Pediatrics in Review - March2011

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91 Common Sleep Problems in Children
Sumit Bhargava
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Diagnosis and Management of Common Sleep Problems in Children
Sumit Bhargava
Pediatr. Rev. 2011;32;91-99
DOI: 10.1542/pir.32-3-91
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Article preventive pediatrics
Diagnosis and Management of

Common Sleep Problems in Children
Sumit Bhargava, MD*
Objectives After completing this article, readers should be able to:
1. Discuss the wide prevalence of sleep problems in children.

Author Disclosure 2. Recognize and manage common sleep problems in children.
Dr Bhargava has 3. Know the American Academy of Pediatrics guidelines for diagnosis and management of
disclosed no financial obstructive sleep apnea in children.
relationships relevant
to this article. This
commentary does not Introduction
contain a discussion Sleep plays a vital and often underestimated role in the growth and development of
of an unapproved/ children. Community surveys have discovered that sleep problems have a high prevalence
throughout childhood and adolescence, with 25% to 50% of preschoolers and up to 40% of
investigative use of a
adolescents experiencing sleep-related problems. (1) Disruption of sleep due to a variety of
commercial
sleep disorders may affect cognitive development and growth, and the child’s sleep
product/device. problems may become a significant family stressor. Studies have shown that sleep issues are
usually not addressed adequately in pediatric practice. (2) This article describes the features
of common sleep disorders that present to the pediatrician and provides strategies for
effective management.
Sleep in Infancy, Childhood, and Adolescence

Sleep architecture, sleep behaviors, and sleep problems change as children progress from
infancy to adolescence. The ability to sleep through the night usually does not develop
until at least 3 to 6 months of age. Sleep duration also varies by age (Table 1). Insufficient
sleep and poor sleep quality may manifest as changes in mood, behavior, memory, and
attention. Parents are usually quick to recognize any changes in their child’s behavior and
mood, and these observations should be ascertained during history taking. Younger
children may develop symptoms of hyperactivity, poor impulse control, and neurocogni-
tive dysfunction that includes attentional problems and impaired vigilance. Adolescents
who are not getting sufficient sleep may have symptoms of excessive daytime sleepiness,
such as falling asleep on the bus on the way to school or during class or not being able to
wake up on time for school. It is important to keep in mind the substantial individual
variation in the need for sleep as well as in the effects of sleep loss on daytime function in
children. Therefore, advice for the optimal duration of sleep may vary from child to child.
Optimal duration of sleep may best be estimated during vacation time, when sleep duration
can be correlated with mood and daytime functioning.
Approach to Pediatric Sleep Disorders

Pediatricians should screen for and recognize the symptoms of childhood sleep disorders in
well children and specific vulnerable populations, such as children who have behavioral and
developmental disorders, genetic syndromes, and chronic medical conditions. A screening
tool (BEARS) (Table 2) has been developed to allow for comprehensive screening for the
major sleep disorders in the pediatric age group. This screening tool has been shown to
increase the likelihood of identifying sleep problems in the primary care setting. (3) It is
divided into five major sleep domains, each with appropriate trigger questions: B: Bedtime
problems, E: Excessive daytime sleepiness, A: Awakenings during the night, R: Regularity
and duration of sleep, S: Snoring.
*Assistant Professor of Pediatrics, Section of Pediatric Respiratory Medicine, Yale School of Medicine, New Haven, CT.
Pediatrics in Review Vol.32 No.3 March 2011 91

preventive pediatrics common sleep problems
Appropriate Duration of
Table 1. Diagnosis and Management of
Common Pediatric Sleep Problems
Sleep by Age Insomnia: Behavioral Insomnia of Childhood
The essential diagnostic feature of this disorder is diffi-
Average Sleep Duration
Age Category (Over a 24-hour Period) culty falling asleep, staying asleep, or both that is related
to an identified behavioral abnormality (Table 3). Be-
Newborns 16 to 20 hours cause children do not usually sleep through the night for
Infants (0 to 1 year) 13 to 15 hours
Toddlers and Preschool 11 to 12 hours total the first 3 to 6 postnatal months, this diagnosis usually is
Children (2 to 5 years) made in toddlers and preschoolers. Behavioral insomnia
School-age Children 10 to 11 hours of childhood can affect parental sleep and may lead to
(6 to 12 years) significant daytime impairment of the parents. Marital
Adolescents (13 to 18 years) 9 hours ideal disputes may arise, and parents may develop negative
Used with permission from Mindell JA, Owens JA. A Clinical Guide to feelings toward a child who repeatedly disrupts their
Pediatric Sleep: Diagnosis and Management of Sleep Problems. Philadel-
phia, PA: Lippincott Williams & Wilkins; 2003. sleep. The sleep difficulties of behavioral insomnia of
childhood are due either to inappropriate sleep associa-
tions or to inadequate limit setting by the caregiver.
SLEEP-ONSET ASSOCIATION TYPE. This disorder pre-

sents typically as frequent night awakenings, with the
child depending on a specific stimulus from the care-
Table 2. BEARS Screening Tool

Toddler/Preschool School Age Adolescent
(2 to 5 years) (6 to 12 years) (13 to 18 years)
Bedtime Problems Does your child have any Does your child have any Do you have any problems
problems going to bed problems at bedtime? falling asleep at bedtime (C)?
or falling asleep? (P)
Do you have any problems
going to bed? (C)
Excessive daytime sleepiness Is your child sleepy Does your child have Do you feel sleepy a lot during
during the day or still difficulty waking, seem the day, in school, or while
taking naps? sleepy during the day, driving? (C)
or take a nap? (P)
Do you feel tired a lot?
(C)
Awakenings during the night Does your child wake up Does your child wake up a Do you wake up a lot at night
a lot at night? lot at night? (P) and have trouble falling back
Do you wake up a lot at asleep? (C)
night and have trouble
falling back asleep? (C)
Regularity and duration of Does your child have a What time does your child What time do you go to bed on
sleep regular bedtime and go to bed and get up school days/weekends?
wake time? on weekdays/weekends? How much sleep do you usually
(P) get? (C)
Do you think your child
gets enough sleep? (P)
Snoring Does your child snore a Does your child have loud Does your child snore loudly or
lot or have difficulty or nightly snoring or nightly? (P)
breathing at night? difficulty breathing at
night? (P)
(C)⫽child, (P)⫽parent
Used with permission from Mindell JA, Owens JA. A Clinical Guide to Pediatric Sleep, Diagnosis and Management of Sleep Problems. Philadelphia, PA:
Lippincott Williams & Wilkins; 2003.
92 Pediatrics in Review Vol.32 No.3 March 2011

and other sleep-disrupting events. In toddlers, separation

Diagnostic Criteria of
Table 3. anxiety may manifest as increased night waking. Prob-
lematic night wakings may also be fostered by the child’s
Behavioral Insomnia of temperament and poor parent-child interactions.
Childhood Management focuses on the establishment of a regu-
lar sleep schedule and bedtime routine. It is crucial for a
successful treatment plan to be tailored specifically to the
A. The child’s symptoms meet the criteria for insomnia
based upon parental report or that of other needs of the child and family. Parents should be advised
caregivers. to have a developmentally appropriate bedtime and a
consistent bedtime routine that allows the child to obtain
B. The child shows a pattern consistent with either the adequate sleep. A sleep-deprived child has more night
sleep-onset association or limit-setting type of wakings. The bedroom should be dark, cool, and quiet
insomnia described below:
because this is the most conducive environment for sleep.
i. Sleep-onset association type includes each of the It should be stressed to parents to put the child to bed
following:
1. Falling asleep is an extended process that
drowsy but awake. The goal is to allow the child to learn
requires special conditions. to “self-soothe” and fall asleep easily after a night waking.
2. Sleep-onset associations are highly Self-soothing at bedtime has been shown to generalize to
problematic or demanding. other night wakings within 2 weeks. The behavioral
3. In the absence of the associated conditions, approaches of extinction and fading can be discussed
sleep onset is significantly delayed, or sleep is
otherwise disrupted.
with parents as methods to teach their child to fall asleep
4. Nighttime awakenings require caregiver independently. These techniques have been proven to be
intervention for the child to return to sleep. effective interventions for behavioral insomnia. (4)
ii. Limit-setting type includes each of the Extinction involves putting the child to bed and sys-
following: tematically ignoring him or her until the next morning.
1. The individual has difficulty initiating or
maintaining sleep.
This approach may not be acceptable to many families
2. The individual stalls or refuses to go to bed because parents may be concerned about the impact of
or refuses to return to bed following a this intervention on their child’s emotional develop-
nighttime awakening. ment. Graduated extinction may be more acceptable. In
3. The caregiver demonstrates insufficient or this method, the child is put to bed drowsy but awake,
inappropriate limit setting to establish
appropriate sleeping behavior in the child.
and the parents check on the child in progressively in-
creasing intervals. On each night, the initial waiting
C. The sleep disturbance is not explained by another period is increased by 5 to 10 minutes. The specific
sleep disorder, medical or neurologic disorder,
mental disorder, or medication use.
waiting period varies for each family and is dependent on
the child’s and the parents’ tolerance for crying. To help
Used with permission from American Academy of Sleep Medicine. The parents try this approach, they can be told that instituting
International Classification of Sleep Disorders. 2nd Ed: Diagnostic and
Coding Manual. Westchester, IL: American Academy of Sleep Medi-
extinction at bedtime soon generalizes to the rest of the
cine; 2005 night. Parents should also be prepared for the “extinc-
tion burst,” which is a worsening of the behavior, usually
occurring on the second night.
giver to fall back to sleep. Such stimuli may include being “Fading” of the adult intervention, which is an option
held or rocked to sleep or needing a specific object or a to the extinction approach, involves establishing a clear
particular environment to fall asleep. Sleep-onset associ- plan for gradually decreasing parental involvement in
ations are widely prevalent in young children, and this the child’s falling asleep. An “exit plan” can be designed,
phenomenon is defined as a disorder if sleep is signifi- with the parents first instituting a regular bedtime and
cantly delayed or repeatedly disrupted in the absence of sleep schedule and then gradually decreasing direct con-
the desired stimulation, with intervention by the care- tact with the child as he or she falls asleep. The goal for
giver required to restore sleep. the plan is for the parent to “exit” the room and allow the
Night wakings are a common phenomenon in young child to fall asleep independently.
children, with a prevalence of 25% to 50% in the first
postnatal year. Night wakings may be associated with LIMIT-SETTING TYPE. Stalling or refusing to go to
cosleeping, breastfeeding, or other medical conditions sleep characterizes limit-setting disorder (Table 3). The

toddler does not respond to parental requests to get

ready for bed. Once in bed, there are frequent demands Diagnostic Criteria:
Table 4.
for parental attention, resulting in delayed sleep onset.
When limits are enforced, sleep onset is not delayed. This
Circadian Rhythm Sleep
type of insomnia is perpetuated when caregivers set few Disorder, Delayed Sleep
or no limits or enforce limits inconsistently and unpre- Phase Type
dictably. The insomnia also may be affected by the child’s
temperament, the sleeping environment (sharing a room A. There is a delay in the phase of the major sleep
with a sibling or other relative), or by an inherent circa- period in relation to the desired sleep time and
dian preference. wake-up time, as evidenced by a chronic or
recurrent complaint of inability to fall asleep at a
Limit-setting disorder typically presents after 2 years
desired conventional clock time together with the
of age, when children are sleeping in a bed or are capable inability to awaken at a desired and socially
of climbing out of the crib. Bedtime resistance is com- acceptable time.
mon, occurring in up to 10% to 30% of toddlers and up to B. When allowed to choose their preferred schedule,
15% of school-age children, and may coexist with night patients will exhibit normal sleep quality and
duration for age and maintain a delayed but stable
wakings.
phase of entrainment to the 24-h sleep/wake
Management of limit-setting disorder is similar to that pattern.
for recurrent night wakings. As with night waking, it is C. Sleep log or actigraphy monitoring (including sleep
important to have a consistent bedtime routine and a diary) for at least 7 days demonstrates a stable
sleep schedule that allows the child to sleep an adequate delay in the timing of the habitual sleep period.
Note: In addition, a delay in the timing of other
number of hours. Parents should establish clear bedtime
circadian rhythms, such as the nadir of the core
rules and put the child to bed drowsy but awake. It is body temperature rhythm or dim light melatonin
crucial for both parents to be persistent and consistent in onset (DLMO), is useful for confirmation of the
behavior modification, and both parents must participate delayed phase.
equally. It is not reasonable to allow children to sleep in D. The sleep disturbance is not explained by another
sleep disorder, medical or neurologic disorder,
the parent’s bed on weekends as a reward for sleeping in
medication use, or substance use disorder.
their own bed during the week. Bedtime “fading” may
Used with permission from American Academy of Sleep Medicine. The
be helpful initially, with bedtime temporarily set at the International Classification of Sleep Disorders. 2nd Ed: Diagnostic and
current sleep-onset time and then gradually advanced to Coding Manual. Westchester, IL: American Academy of Sleep Medi-
cine; 2005.
the desired bedtime. Children who leave their beds
should be taken back to their bedrooms gently but
firmly. Parents should be strongly encouraged to use
positive reinforcement so the child’s compliance with the themselves to be evening types, with optimal functioning
bedtime plan is rewarded. during afternoon, evening, and late evening. This natural
predisposition is exaggerated by the normal phase delay
Circadian Rhythm Disorders: in circadian rhythm of about 2 hours that occurs in
Delayed Sleep Phase Syndrome adolescence. A positive family history is found in up to
Delayed sleep phase syndrome is a common disorder 40% of patients, and polymorphisms in the clock gene
among adolescents and young adults, with a prevalence per3 have been discovered in this disorder.
of 7% to 16%. The condition is characterized by habitual Exact pathophysiologic mechanisms remain un-
sleep/wake patterns that are delayed relative to conven- known. Patients who have this disorder have an abnor-
tional sleep times by 2 hours or more (Table 4). The mal relationship between their endogenous circadian
essential issue with this disorder is the timing rather than rhythm and the homeostatic process that regulates sleep
the quality of sleep. Adolescents who have this disorder and wakefulness.
complain of sleep-onset insomnia and extreme difficulty Sleep logs are useful for demonstrating the habitually
waking in the morning. They make up for the short delayed sleep onset and late awakening. Afternoon naps
nighttime sleep period by afternoon naps or by extending may also be noted, as well as late rise times on weekends.
sleep time on weekends. These behaviors serve to disrupt Polysomnography is not routinely indicated for these
their circadian rhythm even more, and a vicious circle patients unless they also have other symptoms of sleep-
ensues. disordered breathing, such as snoring or apneas. Behav-
Almost all individuals who have this disorder report ioral assessment is important because this syndrome may

be associated with depression, anxiety, school refusal,

and school phobia. Diagnostic Criteria:
Table 5.
The aim of treatment is to realign the sleep schedule
to a more conventional and socially acceptable pattern
Sleep Terrors and Nightmare
and to maintain the realigned sleep schedule. Treatment Disorder
involves improving sleep hygiene by avoiding naps and
caffeinated beverages and decreasing bright light expo- Night Terrors
sure in the evening. Therapy includes restricting tele- A. A sudden episode of terror occurs during sleep,
vision viewing in the late evening. Television sets and usually initiated by a cry or loud scream that is
accompanied by autonomic nervous system and
computers should be removed from the child’s room and behavioral manifestations of intense fear.
cell phones switched off. Other techniques such as chro- B. At least one of the following associated features is
notherapy or bright light therapy are best undertaken in present:
consultation with a pediatric sleep medicine specialist. i. Difficulty in arousing the child.
Successful outcomes are dependent on a highly moti- ii. Mental confusion when awakened from an
episode.
vated patient and family. iii. Amnesia (complete or partial) for the episode.
Maintenance of the sleep schedule is extremely im- iv. Dangerous or potentially dangerous behaviors.
portant, and frequent relapses may occur. Any comorbid C. The disturbance is not explained by another sleep
psychiatric issues must be addressed for the child to disorder, medical or neurologic disorder, mental
continue to maintain the new schedule successfully. disorder, medication use, or substance use disorder.
Continued good sleep hygiene is essential. The patient Nightmare Disorder
may be allowed to sleep a little longer on weekends A. Recurrent episodes of awakening from sleep with
after a few months of strict compliance. However, week- recall of an intensely disturbing dream, usually
end oversleep time should not extend more than 2 hours involving fear or anxiety, but also anger, sadness,
beyond the desired wake time. Close contact with the disgust, and other dysphoric emotions.
B. Full alertness on awakening, with little confusion or
patient and the family during the maintenance phase is disorientation; recall of dream is immediate and
helpful in ensuring compliance with the sleep plan. This clear.
monitoring may be via telephone or regularly scheduled C. At least one of the following features is present:
visits. Patients should be encouraged to continue to i. Delayed return to sleep after the episode.
maintain a sleep log, so deviations from the sleep main- ii. Occurrence of episodes in the latter half of the
habitual sleep period.
tenance plan may be detected early.
Used with permission from American Academy of Sleep Medicine. The
International Classification of Sleep Disorders. 2nd ed: Diagnostic and
Common Parasomnias of Childhood Coding Manual. Westchester, IL: American Academy of Sleep Medi-
Parasomnias are undesirable physical events that occur cine; 2005.
during entry into sleep, within sleep, or during arousal
from sleep (Table 5). These are important clinical dis-
orders of which the pediatrician should be aware because age of 15 years onward. Night terrors are usually seen
they occur commonly during childhood. between 4 and 12 years of age. Genetics may play a role
in night terrors. There is usually a family history of night
NIGHT TERRORS. Night terrors (pavor nocturnus) are terrors in one or both parents. There is no association of
arousals from deep (slow wave) sleep, usually in the first night terrors with psychopathology in young children.
one third of the night and invariably accompanied by Night terrors also may be associated with sleep walking.
behavioral manifestations of intense fear. Tremendous The diagnosis of night terrors is usually based on a
autonomic system discharge occurs, with tachycardia, typical history. History taking should focus on discover-
tachypnea, flushing of the skin, diaphoresis, and in- ing if there is any sleep disorder that could result in
creased muscle tone. The child is found sitting up in bed disrupted sleep, such as obstructive sleep apnea (OSA),
and unresponsive; if awakened, the child is confused and restless legs syndrome/periodic limb movement syn-
disoriented. Vocalization occurs frequently. Amnesia for drome, or seizures. Polysomnography is not routinely
the episodes is usually the case when the child is ques- indicated, although it may be helpful in distinguishing
tioned the next morning. night terrors from nocturnal complex partial seizures or
Prevalence rates vary from 1% to 6.5% in early child- frontal lobe seizures. In addition, polysomnography can
hood, with a relatively stable prevalence of 2.5% from the help to rule out OSA in a snoring child. OSA can lead to

recurrent arousals and shifts in sleep stage and may be Management of nightmares focuses on maintaining
associated with increased night terrors. good sleep hygiene. Exposure to frightening or over-
Management of night terrors is focused primarily on stimulating television shows and movies should be
parental reassurance and education. Parents should be avoided before bedtime. Children may respond well to
informed of the essentially self-limited nature of these parental reassurance or the use of security objects such as
episodes. Most children cease to have them after the blankets. A low-level night light may be helpful. For the
onset of puberty due to the dramatic decrease in slow- child who is excessively disturbed by these events, referral
wave sleep. to a developmental-behavioral pediatrician should be
Scheduled awakening may be considered for the child considered. Affected children respond well to relaxation
who is having nightly episodes. In this approach, the strategies or systemic desensitization.
parents identify the time of the episodes and wake the
child to the point of arousal 15 to 30 minutes before SLEEPWALKING. Sleepwalking consists of a series of
that time. This can be done for 2 to 4 weeks, until the complex behaviors that are related to arousal from slow-
episodes stop occurring, and can be repeated if the wave sleep and culminate in walking around with an
episodes start again. altered state of consciousness and impaired judgment
Short-acting benzodiazepines may be considered in (Table 6). The sleepwalking child may appear confused,
the rare child who has frequent severe episodes that are dazed, or occasionally agitated. The eyes are usually open
excessively violent and place him or her at high risk of during the episode. Children may perform bizarre acts,
injury. Treatment can be considered for 3 to 6 months, such as urinating in inappropriate locations or leaving the
until the episodes cease completely. Benzodiazepines house.
should be slowly tapered because abrupt discontinuation About 15% to 40% of children sleepwalk at least once.
results in slow-wave sleep rebound and a return of the The prevalence of frequent sleepwalking is low (3% to
nocturnal episodes. 5%). Peak occurrence is between 4 and 8 years of age,
and there may be a family history of sleepwalking. Sleep
NIGHTMARES. Nightmares are characterized by dis- deprivation, OSA, and a febrile illness may precipitate
turbing dreams that usually occur in rapid eye movement sleepwalking.
(REM) sleep in the latter half of the night and result in Diagnosis is made by the typical history. Polysomnog-
awakening. There is significant post-awakening anxiety raphy may be helpful if there are associated symptoms of
and difficulty in returning to sleep. This symptomatology
is especially common in younger children, who cannot
distinguish between dreams and reality and may refuse to Diagnostic Criteria:
Table 6.
return to sleep.
Approximately 10% to 50% of children between the Sleepwalking
ages of 3 and 5 years experience nightmares severe
A. Ambulation occurs during sleep.
enough to disturb their own and their parents’ sleep. B. Persistence of sleep, an altered state of
Nightmares can be precipitated by stress or traumatic consciousness, or impaired judgment during
events. They also are associated with sleep deprivation, ambulation is demonstrated by at least one of the
anxiety disorders, and medications, including antide- following:
i. Difficulty in arousing the person.
pressants, antihypertensive agents, and dopamine ago-
ii. Mental confusion when awakened from an
nists. The proportion of children experiencing night- episode.
mares peaks between the ages of 6 and 10 years and iii. Amnesia (complete or partial) about the episode.
subsequently declines. iv. Routine behaviors that occur at inappropriate
Pediatricians should assess both the chronicity and times.
v. Inappropriate or nonsensical behaviors.
severity of nightmares because unusual severity has
vi. Dangerous or potentially dangerous behaviors.
been related to psychopathology. Nightmares can be C. The disturbance is not explained by another sleep
distinguished from night terrors by their occurrence in disorder, medical, or neurologic disorder, mental
the latter half of the night, when REM sleep predomi- disorder, medication use, or substance use disorder.
nates. Also, the child experiences no confusion or disori- Used with permission from American Academy of Sleep Medicine. The
entation with nightmares and can recall the event. Fi- International Classification of Sleep Disorders. 2nd ed. Diagnostic and
Coding Manual. Westchester, IL: American Academy of Sleep Medi-
nally, unlike night terrors, return to sleep is significantly cine; 2005
delayed.

snoring or restless sleep and typically shows arousal from cor pulmonale, pulmonary hypertension, and systemic
deep sleep in the first half of the night. hypertension as well as poor learning, behavioral problems,
Treatment is focused primarily on protecting the child and attention-deficit/hyperactivity disorder (ADHD).
from harm. The child’s room should be in a safe location, Typical of OSA is a history of loud nightly snoring
not close to stairs. Alarms such as bells may be placed on with observed apnea spells. Parents may note that the
the doorknob to alert parents to the child’s waking. child is a restless sleeper or that he or she sweats substan-
Parents should be reassured that there is no significant tially while sleeping or sleeps in an abnormal position
association between childhood sleepwalking and psycho- with the neck extended. Daytime symptoms can include
pathology. They should be encouraged to maintain good chronic mouth breathing with chronic nasal congestion
sleep hygiene, with a regular sleep/wake schedule. For or morning headaches. Excessive daytime sleepiness is more
the rare child who has nightly events or a history of common among older children. OSA may present with
injury, treatment with benzodiazepines may be consid- subtle neurobehavioral signs, including mood changes,
ered for a period of 3 to 6 months, with slow tapering. ADHD-like symptoms involving inattention and easy dis-
Most sleepwalking episodes resolve by puberty with the tractibility, or academic problems due to difficulty concen-
age-related diminution in slow-wave sleep. trating. Findings on physical examination may include char-
acteristic adenoidal facies as well as signs of atopy or nasal
Sleep-related Breathing Disorders: congestion such as “allergic shiners,” nasal septal deviation,
Pediatric OSA or enlarged turbinates. Oropharyngeal examination may
OSA is characterized by intermittent complete or partial reveal enlarged tonsils or a redundant soft palate with a long
obstruction of the upper airway that leads to obstructive uvula. The rare child may present with cor pulmonale or
apnea or hypopnea, which disrupts normal ventilation systemic hypertension.
during sleep and may be associated with hypoventilation In April 2002, recognizing the significant impact of
and oxyhemoglobin desaturation. OSA must be distin- OSA on children, the American Academy of Pediatrics
guished from primary snoring, which is snoring without issued an evidence-based clinical practice guideline with
associated obstructive events or gas exchange abnormal- recommendations for the diagnosis and management of
ities. OSA in children (Table 7). (5)
The prevalence of this disorder has been reported to
vary from 2% to 4% in healthy children. The disorder can
occur at any age but is most common in the preschool Recommendations for
Table 7.
age group (2 to 6 years) and adolescents. In prepubertal
children, the disease occurs equally in boys and girls. A
the Diagnosis and Treatment
higher prevalence has been reported in African-American of OSA (5)
children. OSA appears to run in families, with a family
1. All children should be screened for snoring.
history of the disorder usually being present. However,
2. Complex, high-risk patients should be referred to a
the relative role of genetic versus environmental factors specialist. These patients include infants and
has not yet been determined. patients who have craniofacial disorders, genetic
The exact pathophysiology of the disorder in children syndromes, neuromuscular disorders, chronic lung
is unknown. It appears to result from a combination of disease, sickle cell disease, and central
hypoventilation syndromes.
upper airway narrowing and upper airway hypotonia.
3. Thorough diagnostic evaluation should be
The primary predisposing factor for OSA in young chil- performed. History and physical examination cannot
dren is adenotonsillar hypertrophy, although size of distinguish between primary snoring and OSA.
the tonsils and adenoids does not predict disease in Polysomnography is the diagnostic test of choice.
individual patients. Obesity appears to be a risk factor in 4. Adenotonsillectomy is the first line of therapy for
most children. Continuous positive airway pressure
older children. In many children, both factors may be
is an option for those who are not surgical
relevant. Other children at high risk of OSA include those candidates or who respond poorly to surgery.
born with craniofacial abnormalities, specifically midface 5. High-risk patients should be monitored as
hypoplasia and micrognathia, and associated hypotonia. inpatients postoperatively.
Among these patients are children who have Down syn- 6. Patients should be reevaluated postoperatively to
determine if additional treatment is required. All
drome, neuromuscular disease, and cerebral palsy.
patients should undergo clinical evaluation. High-
Early diagnosis and treatment of OSA is important risk patients should undergo polysomnography.
because the untreated condition has been associated with

2. Owens JA. The practice of pediatric sleep medicine: results of a

Summary community survey. Pediatrics. 2001;108:e51
3. Owens JA, Dalzell V. Use of the ‘BEARS’ sleep screening tool
in a pediatric residents continuity clinic: a pilot study. Sleep Med.
• Based on community surveys, sleep disorders in
2005;6:63– 69
children are widely prevalent and preventable.
4. Morgenthaler TI, Owens J, Alessi C, et al. Practice parameters
• Based on strong research evidence, behavioral
for the behavioral treatment of bedtime problems and night wak-
treatment of insomnia of childhood is safe and
ings in infants and young children. Sleep. 2006;29:1277–1281
effective.
5. American Academy of Pediatrics. Clinical practice guideline:
• Based on strong research evidence, all children
diagnosis and management of childhood obstructive sleep apnea
should be screened for snoring. Pediatricians are
syndrome. Pediatrics. 2002;109:704 –712
encouraged to follow evidence-based guidelines for
diagnosis and management of OSA, as specified by
the American Academy of Pediatrics in its clinical
practice guideline.
Suggested Reading
American Academy of Sleep Medicine. The International Classification
of Sleep Disorders. 2nd Ed. Diagnostic and Coding Manual.
Westchester, IL: American Academy of Sleep Medicine; 2005
References Schechter MS; Section on Pediatric Pulmonology, Subcommittee
1. Mindell JA, Owens JA. A Clinical Guide to Pediatric Sleep: on Obstructive Sleep Apnea Syndrome. Technical report: diag-
Diagnosis and Management of Sleep Problems. Philadelphia, PA: nosis and management of childhood obstructive sleep apnea
Lippincott Williams & Wilkins; 2003 syndrome. Pediatrics. 2002;109:e69
HealthyChildren.org Parent Resources from AAP

http://www.healthychildren.org/English/ages-stages/baby/sleep/Pages/default.aspx

PIR Quiz
Quiz also available online at http://pedsinreview.aappublications.org.
1. The R in the BEARS screening tool explores:

A. Duration and regularity of sleep.
B. Need for rousing from deep sleep.
C. Rapid eye movement sleep disturbances.
D. Risk for significant sleep apnea.
E. Sleep rituals.
2. An otherwise healthy 21⁄2-year-old-boy refuses to go to bed in the evening. Every night at bedtime, he
cries until he is permitted to sleep in his parent’s bed. Once asleep, he does not snore. The most appropriate
first step toward effective management is:
A. Consistent limit-setting.
B. Disrupting all naps in the afternoon.
C. Scheduling of a sleep study.
D. Trial of a short-acting benzodiazepine.
E. Use of extinction.
3. An otherwise healthy 16-year-old boy complains of difficulty falling asleep at night and extreme difficulty
awakening in the morning. A sleep log documents a normal duration of sleep on weekends. He does well in
school and denies either depression or anxiety. He has eliminated caffeine from his diet. There is no TV in
his bedroom, and he does not use the computer or phone or text at night. He does not snore. The most
appropriate first step toward effective management is:
A. Avoiding naps in the afternoon.
B. Scheduling of a sleep study.
C. Trial of a central alpha agonist.
D. Trial of a selective serotonin reuptake inhibitor.
E. Trial of a short-acting benzodiazepine.
4. An otherwise healthy 12-year-old boy sleepwalks almost every night. His sleep hygiene is appropriate. He
does not snore at night. The home has been made as safe as possible for him. There is a combination lock
on the front door at night and there are no stairs to fall down. However, although no serious injury was
sustained, he did trip and hit his head on a sink last week. The most appropriate next step toward effective
management is:
A. Avoiding naps in the afternoon.
B. Consistent limit-setting.
C. Locking him in his bedroom at night.
D. Scheduling of a sleep study.
E. Trial of a short-acting benzodiazepine.
5. An otherwise healthy 4-year-old boy snores loudly every night. His snoring disrupts the sleep of other
family members, but his mother has not noted any apnea. His body mass index is appropriate for age. His
tonsils protrude slightly from behind the anterior tonsillar pillars. The remainder of his examination
findings are otherwise unremarkable. The most appropriate first step in effective and efficient management
is:
A. Periodic rousing him from sleep.
B. Prescription ear plugs for other members of the family.
C. Reassessment in 1 year.
D. Referral for tonsillectomy.
E. Scheduling of a sleep study.

Diagnosis and Management of Common Sleep Problems in Children
Sumit Bhargava
Pediatr. Rev. 2011;32;91-99
DOI: 10.1542/pir.32-3-91
Updated Information including high-resolution figures, can be found at:

& Services http://pedsinreview.aappublications.org/cgi/content/full/32/3/91
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_pediatrics Respiratory Disorders
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y_disorders Ear, Nose and Throat Disorders
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throat_disorders
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Failure to Thrive: Current Clinical Concepts
Arthur C. Jaffe
Pediatr. Rev. 2011;32;100-108
DOI: 10.1542/pir.32-3-100

Article growth & development

Arthur C. Jaffe, MD*
1. Characterize the limitations of the classic dichotomy of “nonorganic” versus “organic”

Author Disclosure failure to thrive (FTT).
Dr Jaffe has disclosed 2. Recognize that FTT is not a diagnosis but rather a physical sign of inadequate nutrition
no financial to support growth.
relationships relevant 3. Know potential sequelae of FTT.
to this article. This 4. Understand how the diagnostic process must account for the multifactorial nature of
commentary does not FTT.
contain a discussion 5. Describe a systems-based, multidisciplinary approach to treatment of FTT.
of an unapproved/ 6. Discuss potential adverse effects of nutritional repletion of children who fail to thrive.
commercial
product/device.
Introduction
Failure to thrive (FTT) or growth failure has long been a major focus of attention and
critical thought for pediatricians. Over many years, consensus has evolved about its cause,
outcome, diagnosis, and management. By the last half of the 20th century, mainstream
thinking held that FTT developed by one of two alternative mechanisms. On rare
occasions, an underlying medical condition could lead to a failure of growth, which would
present as “organic” FTT. In such cases, correct management of this underlying disorder
would allow the patient to resume his or her normal growth. However, in most FTT cases,
as many as 90% in some series, it was believed that no underlying medical diagnosis could
be made. Affected children were said to have “nonorganic” FTT (NOFTT). This term
and its synonym, “psychosocial” FTT, were often used as euphemisms to imply that the
child was intrinsically normal and healthy and that the observed growth failure was due
to environmental issues, most commonly family factors such as neglect, indifference, or
other failures of parenting. This construct led to children receiving extensive medical
evaluations to rule out diagnosable conditions and to prolonged hospitalizations that were
designed primarily to document parenting difficulties and demonstrate vigorous and
consistent weight gain under the care of hospital personnel. There was concern that, in
addition to being at risk for acute complications of severe malnutrition from neglectful or
disturbed parenting, children would inexorably suffer long-term adverse neurodevelop-
mental outcomes.
This view of FTT is now outmoded. According to a large body of newer thinking, FTT
is not a syndrome. It is, rather, a physical sign that a child is receiving inadequate nutrition
for optimal growth and development. The work of the pediatrician is to determine, in an
ordered and logical process, what may be leading to the inadequate nutrition and, when
possible, to treat the underlying pathophysiologic issues, which often are
multifactorial. Although this newer construct has now become the current
mainstream view of clinicians and researchers working in the field of FTT,
Abbreviations readers should be aware that the older, dichotomous view of “organic” versus
BMI: body mass index “nonorganic” FTT may still color the thinking of some health professionals.
FTT: failure to thrive Specifically, some medical licensure and certification examinations still may
IQ: intelligence quotient ask questions about FTT that reflect this dichotomous approach.
IUGR: intrauterine growth restriction Zenel in 1997 (1) and Gahagan in 2006 (2) wrote thoughtful reviews in
NOFTT: nonorganic failure to thrive this journal that lucidly outline the rationale for reconceptualizing FTT.
Rather than repeating their extensive commentaries, this article seeks to
*Associate Professor, Division of General Pediatrics, Oregon Health & Science University, Doernbecher Children’s Hospital,
Portland, OR.

growth & development failure to thrive
complement their work by asking specific questions that to affect growth. Children born with certain genetic
highlight salient features of the new model and point conditions may appear to have FTT when they are eval-
out areas of concern or uncertainty that call for further uated using normal growth curves, but their perceived
research. growth problems disappear when they are compared
graphically to other children who have the same condi-
How Reliable Are Growth Charts for tion. Growth charts are readily available for such genetic
Diagnosing FTT? conditions as Down, Turner, and Williams syndrome,
Plotting a child’s weight, length, and head circumference among others, and it is essential to plot a child’s growth
on standardized growth charts remains a critically impor- on the appropriate curves to determine if FTT truly is
tant, essential step in providing an objective description present.
of a child’s growth and describing how far off the statis- Others who require special consideration include chil-
tical norm he or she is. Frequently quoted chart-based dren who are chronically immobilized, such as those
definitions of FTT in children younger than 2 years of who have severe neuromotor disabilities. Often, such
age include: a child whose weight is below the 3rd or 5th children have major oromotor dysfunction, are at risk for
percentile for age on more than one consecutive occa- pulmonary aspiration syndrome, or have other issues
sion, a child whose weight drops down two major per- that lead to inability to eat by mouth or maintain ade-
centile lines, a child whose weight is less than 80% of the quate growth. They often receive feeding gastrostomies
ideal weight for age, and a child who is below the 3rd or to optimize nutrition. However, because they are im-
5th percentile on the weight-for-length curve. mobile, they frequently have minimal caloric needs com-
As widely accepted as these definitions are, they are pared with other children and, thus, are at risk for exces-
fraught with confusion. For example, the first two defi- sive weight gain, even obesity. Such children may be
nitions require serial measurements over time. How is difficult to weigh and measure correctly because of their
the clinician to respond when he or she notices a child neurologic status and immobility, making it difficult
who is “below the curves” on the first visit? The last two to assess their status using the standard growth curves.
definitions facilitate diagnosis on one visit, but what Other anthropometric techniques, such as measuring
about a child who does not meet the 80% criterion arm span or the ratio of upper body to lower extremity
precisely or is not quite below the 3rd percentile but length, may be more appropriate.
looks malnourished? These definitions do not provide Another concern with the use of growth charts is that
standards for assessment of the body mass index (BMI) in clinicians intuitively expect that a child is biologically
children older than age 2 years. There is, in fact, some destined to move along his or her predetermined growth
debate about the clinical utility of BMI measurement in channels inexorably and that any deviation from the
very young children. How may the pediatrician most original channel indicates a growth aberration. However,
reliably identify FTT in an older child? Unfortunately, no Mei and associates (3) described shifts in growth curves
standard uniform approach exists to identify reliably each during the first 60 months of age in a cohort of 10,844
child who has FTT solely by use of growth curves. children. Between birth and 6 months of age, 39% of
There are other issues. First, the previously cited healthy children crossed two major percentile lines (up or
definitions do not clearly point out the need to evaluate down) on the weight-for-age curve, as did 6% to 15% of
whether a symmetric fall-off in the growth curves is children between 6 and 24 months of age. Similar shifts
present. They do not explicitly remind the clinician that occurred with the length-for-age curve. Strikingly, on
when the FTT is due to poor food intake, weight tends to the weight-for-height curve, 62% of children between
fall first, sparing the length and head circumference; that birth and 6 months and 20% to 27% of children between
a symmetric fall in weight and height suggests a chronic 6 and 24 months crossed two major percentile lines. The
medical condition; that short stature below the 50th authors concluded that pediatricians should consider
percentile, with relative sparing of weight, suggests an the prevalence of growth rate shifts during infancy and
underlying endocrine disorder; or that an early drop-off early childhood before initiating evaluations of growth
in head circumference suggests a lack of brain growth. concerns.
The pediatrician still must exercise clinical judgment in This caveat is humbling, for it is in precisely this age
interpreting seemingly objective data. range that the question of FTT often is raised. Careful
Also, the widely accepted growth curve-based defini- measurement of weight and length and thoughtful eval-
tions refer to children who are believed to be generally uation of the child’s growth curves remain essential for
healthy and free of underlying conditions that are known diagnosis and management of growth concerns, but the

pediatrician must still use clinical judgment and not be 12-year-old children who had had FTT as infants. Com-
overly reliant on arbitrary mathematical definitions of pared with a control group, they were shorter, lighter,
FTT. and had lower BMIs, and they reported having lesser
appetites. They were satisfied with their body shapes and
What Are the Sequelae of FTT? did not differ from the controls on measures related to
For many years, there has been recognition that the anxiety, depression, or low self-esteem. The authors
undernutrition seen in FTT in first-world environments concluded that FTT in infancy is not associated with
is typically not severe enough to lead to clinical condi- adverse emotional development in childhood. On the
tions such as marasmus or kwashiorkor, although serious other hand, Raikkonen and coworkers (11) measured
nutritional or vitamin deficiency diseases still may occur scores on a standardized assessment of adult hostility in
in very rare cases. However, a rich body of evidence, a large cohort of adults who had a mean age of 63.4 years.
reviewed by Corbett and Drewett (4) in a 2004 meta- The participants’ growth histories were obtained by
analysis, continues to suggest that poor nutrition in review of their birth, child welfare clinic, and school
young children, especially infants, may result in long- records. Men and women who had higher levels of hos-
term problems in cognitive development. On the other tility in adulthood were born lighter and thinner, showed
hand, Rudolf and Logan (5) reached opposite conclu- slower weight gain from birth to 6 months, were lighter
sions in another meta-analysis in 2005. They found that throughout childhood, and were heavier in adulthood
FTT was associated with an intelligence quotient (IQ) than were individuals who had lower levels of hostility.
3 points lower than expected, but the difference was of There is no current consensus about long-term noncog-
questionable clinical significance. They also reported that nitive, emotional sequelae of FTT in early childhood.
early-onset FTT was associated with some persistent Although methodologic difficulties continue to pre-
reduction in weight and height later in childhood. How- sent problems in gathering data and analyzing long-term
ever, they concluded that there was little evidence that outcomes in children who have FTT, the general trend of
FTT was predictive of damaging consequences for the research is to express concern about long-term ad-
growth and intellectual development. In individual stud- verse effects on cognition, learning, and behavior. This
ies, a number of researchers have followed infants or line of evidence strongly supports the common current
toddlers who had FTT for periods as long as 8 years and practice of instituting early and vigorous nutritional ther-
reported measurable deficits in IQ, learning difficulties, apy for children who fail to thrive.
and behavioral difficulties. (6)(7)(8) Some commenta-
tors argue that interpreting these findings is difficult What is the Diagnostic Approach to FTT?
because the study participants tended to have very severe Recognition that FTT is a physical sign of undernutrition
FTT and significantly disordered family environments at allows a framework for classifying its causes. When con-
the time of diagnosis, including problems such as overt sidering the differential diagnosis, it is important to
parental mental illness and substance abuse. remember that FTT often is a multifactorial condition.
Although many children who experience FTT in early Because a given patient may have more than one con-
life eventually seem to have normal function, the overall tributing factor to his or her poor growth, diagnostic
trend is worrisome. A meaningful number of children evaluation should not automatically end as soon as one
have persistent intellectual or behavioral deficits, and it is potential cause is identified. In addition to primary con-
currently impossible to predict which children will do tributing factors, the pediatrician should pay heed to
well and which will have later difficulties. It is unclear possible secondary exacerbating factors. Family stresses
whether energy or protein inadequacy alone is associated associated with the struggle to get an undernourished
with the adverse long-term outcomes or whether defi- child to gain weight or stresses occurring after hearing
ciencies of vitamins, minerals, or other micronutrients that the physician is concerned about FTT may add yet
might be responsible. For example, Lozoff and associates another layer of psychosocial factors that potentiate the
(9) showed that infants who experience iron deficiency condition.
anemia in infancy have measurably poorer developmental Because FTT is a sign of undernutrition, the diagnos-
and behavioral outcomes 10 years later, despite having tic approach must focus on that problem. Undernutri-
received appropriate treatment at the time of diagnosis. tion, broadly speaking, may occur because of one of three
More recently, attention has started to turn to other, mechanisms. The child may have inadequate intake, in-
noncognitive developmental outcomes of early FTT. gesting insufficient nutrients for growth. Even if taking
Drewett and colleagues (10) studied a group of 89 in adequate nutrients, he or she may have malabsorption

that prevents foodstuffs from being available for growth.

Finally, although a child may have both appropriate Diagnostic Classification
Table.
intake of food and normal absorption across the gut wall,
he or she may have increased metabolic demand due to
of Causes and Selected
chronic disease or genetic or metabolic disorders. As seen Examples of Failure to Thrive
in the Table, this classification scheme allows the clinician
to approach the diagnosis of a child methodically and Inadequate Nutritional Intake
rationally. • Not enough food offered
As always in medicine, careful and thoughtful consid- –Food insecurity
–Poor knowledge of child’s needs
eration must be given to the clinical history. The assess- Poor transition to table food
ment of whether adequate nutrition is being presented to Avoidance of high-calorie foods
the child requires in-depth questioning to determine –Formula dilution
what kinds of milk, formula, foods, and vitamins are –Excessive juice
being offered. Does the family know what an appropriate –Breastfeeding difficulties
–Neglect
diet is for their child’s age and developmental level? Are • Child not taking enough food
there adequate financial resources for the family to obtain –Oromotor dysfunction
these foods? Is breastfeeding proceeding well, both from –Developmental delay
the mother’s and the child’s perspective? Is formula –Behavioral feeding problem
being prepared correctly or is it too dilute? Is the child Altered oromotor sensitivity
Pain and conditioned aversion
getting an excessive amount of juice, which may lead to • Emesis
satiety without supplying adequate calories? Is there ev- –Gastroesophageal reflux
idence of neglect? Does the child have intrinsic condi- –Malrotation with intermittent volvulus
tions that interfere with food intake, such as oromotor –Increased intracranial pressure
dysfunction, developmental delay, or feeding aversions Malabsorption
due to behavior problems or in learned response to pain? • Cystic fibrosis
Is there recurrent emesis, suggestive of conditions such • Celiac disease
as gastroesophageal reflux disease, malrotation with in- • Food protein insensitivity or intolerance
termittent volvulus, or increased intracranial pressure? Increased Metabolic Demand
Often, obtaining an accurate history of nutritional intake
• Insulin resistance (eg, intrauterine growth
requires the use of a 3-day food diary, with consultation
restriction)
by a nutritionist to calculate actual intake of energy, • Congenital infections (eg, human immunodeficiency
protein, vitamins, and minerals. virus, TORCH)
The history also should address the possibility of • Syndromes (eg, Russell-Silver, Turner, Down)
malabsorption syndromes, with detailed questions about • Chronic disease (eg, cardiac, renal, endocrine)
stool frequency and consistency. Many states now re-
quire newborn screening for cystic fibrosis, and those
results should be obtained, although such screening does be undertaken to search for subtle suggestions of issues
not have 100% sensitivity or specificity. A family history such as congestive heart failure, chronic renal disease, or
of respiratory and gastrointestinal disorders should be endocrine disorders that may be associated with FTT.
elicited. Most children older than 6 months of age have Importantly, the physician should ask about the perinatal
been exposed to baby foods that contain gluten, so celiac history, with attention to possible intrauterine growth
disease is a frequent diagnostic consideration. Question- restriction (IUGR). Evidence now strongly suggests that
ing should probe for a family history suggestive of celiac IUGR from any cause may be associated with insulin
disease or endocrine conditions that may be associated resistance, which has long-term implications for growth
with that diagnosis. In addition, if the child is having potential and adverse metabolic sequelae if excessive
abnormal stools, the physician should ask about a family weight gain is induced.
history suggestive of milk protein intolerance or sensi- The complete physical examination should focus on
tivity. any clinical questions that have been raised during the
If concerns about a specific chronic disease that could history. It is appropriate to look carefully for subtle signs
increase metabolic demand are not raised during the of dysmorphology, such as minimal discrepancies in limb
initial clinical history, a detailed review of systems should length that may be found in Russell-Silver syndrome. If

initial growth measurements have not included the head their child’s clinical status. Developmental, oromotor, so-
circumference and weight-length ratio or BMI, they cial work, or mental health consultations are part of the
should be obtained. If readily available, caliper measure- routine diagnostic armamentarium and are not deferred
ment of skinfold thickness and mid-arm muscle circum- until an occult organic disease has been proven not to exist.
ference can provide valuable information about a child’s When the physician feels that laboratory studies are
nutritional status. warranted, even when there are no specific clinical indi-
cations, a reasonable selection might include a complete
What is Involved in the blood count with red cell indices (to evaluate for anemia
Laboratory Evaluation of FTT? and iron deficiency), a complete chemistry panel (includ-
According to the classic view of FTT, in which it was ing tests for renal and hepatic function), celiac screening,
essential to identify any “organic” causes, extensive stool examination for fats and reducing substances, and a
screening was undertaken to eliminate any and all possi- sweat chloride test for cystic fibrosis. Screening for hypo-
bilities before making the diagnosis, by exclusion, of thyroidism or growth hormone deficiency should be
“nonorganic” or “psychosocial” FTT, with its implica- considered only if the child’s length has decelerated and
tion of faulty parenting. However, as far back as 1978, is below the 50th percentile on the length-for-age chart.
Sills (12) clearly showed that laboratory studies had very Of note, a length above the 50th percentile is strong
little, if any, clinical utility in the evaluation of FTT. In his evidence that no endocrine disorder is present.
retrospective review of 185 children hospitalized for
FTT, he found that 34 (18%) had proven organic causes. How Much of the Evaluation May Occur in
In all 34 patients, the specific diagnosis was strongly the Pediatrician’s Office and When is
suggested by the history and physical examination find- Hospitalization Indicated?
ings. A total of 2,607 laboratory tests were undertaken The chronicity and the multifactorial nature of FTT
for the entire group of 185 patients. Only 36 tests (1.4%) mean that a major investment of time and energy is
were of positive diagnostic assistance, and all of them necessary for optimal evaluation and management. The
were in the 34 patients whose diagnosis was strongly professional expertise of the pediatrician caring for a
suggested by history and examination. No unsuspected child who has FTT is best demonstrated by use of a
diagnoses were uncovered by the results of any of the systems-based approach. In addition to the pediatrician’s
2,607 laboratory tests. skills in medical diagnosis and management, there is a
Today, there still is no evidence to support the exten- need for evaluation of the child’s temperament and de-
sive, systematic use of screening laboratory evaluations in velopment, oromotor functioning, nutritional needs and
diagnosing FTT. Consideration of diagnostic possibili- deficits, and family and social support systems. The pedi-
ties according to the modern construct portrayed in the atrician is served best by working closely with other
Table should lead to a rational, cost-efficient, case-based professionals, such as nurses, occupational or physical
approach to evaluation. However, this more narrow ap- therapists, speech and language pathologists, nutrition-
proach has potential difficulties. Often, when the physi- ists, social workers, psychiatrists, and psychologists. If the
cian identifies FTT and tells the family that their child is physician can access and use a team comprising some or
not growing normally, the family fails to see a problem. all of these disciplines in his or her medical home, it may
They may look at growth curves and even understand be reasonable to manage the child in that setting. How-
them, but they see a child who appears to be healthy, ever, if it is difficult or impractical to provide this neces-
active, and meeting developmental milestones. sary range of services, the child, family, and physician are
Before the physician can investigate possible psycho- all better served by referring the patient to an interdisci-
social barriers to adequate nutritional intake, he or she plinary team of experts who have that capability.
must make a therapeutic alliance with the family. Achiev- The role of hospitalization in FTT has evolved. In the
ing this goal may require convincing the family that due dichotomous “organic” versus “nonorganic” FTT con-
attention is being given to the possibility of occult or- struct, there were several indications for admission. One
ganic disease, necessitating the ordering of some labora- was to expedite the efficient and complete performance
tory tests to foster the relationship with the family. of a vast panoply of laboratory studies, including blood
Obtaining laboratory studies also allows the pediatrician tests, stool and urine assays, and radiologic examinations,
to explain to the family that early investigation of envi- and to have the child seen by a range of consultants to
ronmental, psychosocial, or behavioral causes of FTT is a ensure that occult organic disease was not being over-
coequal part of the overall comprehensive evaluation of looked. Another reason for admission was to present an

appropriate type and amount of food to the child, doc- cessful medical or surgical interventions may not, by
ument that the child was ingesting that food, and observe themselves, be sufficient to restore the child’s nutritional
subsequent consistent weight gain. This approach was status to normal. Anorexia, nausea, emesis, pain, or mal-
believed to serve the dual purposes of confirming the aise due to the condition or its treatment may potentiate
diagnosis of NOFTT, with its assumption that faulty a child’s disinterest in eating or inadvertently positively
parenting was the mechanism of disease, and returning reinforce undesirable or aversive feeding behaviors. Fam-
the child to health because exogenously caused poor ily members may respond to illness-associated anxieties
nutrition was the sole problem. It was assumed that this and fears by understandably but inappropriately commu-
process could take 2 weeks or more. nicating their stress to the child during mealtimes or
For a variety of reasons, this approach is no longer feeding situations.
advisable or feasible. Cost constraints plus the recogni- In such cases, as well as in the more common situation
tion that children and their families are best served by in which the FTT is due, in large measure, to poor intake
staying out of the hospital, if at all possible, have com- of nutrients, the first step in successful treatment is the
bined to limit the use of hospital admissions in evaluating careful, structured observation of the child’s eating or
children who have FTT. Most diagnostic and consulta- feeding behavior and of how he or she interacts with
tive evaluations can be performed on an ambulatory basis. family members during meals. Does the child exhibit
The argument can be made that, in appropriate circum- hunger? Can the child attend to eating at mealtimes for a
stances, there is value in having repetitive, objective assess- developmentally appropriate length of time and tolerate
ments of a child’s feeding behaviors while he or she interacts sitting in a highchair or at a table? Does the child have
with all usual caretakers. Such serial evaluations may be difficulty chewing or swallowing food of certain consis-
undertaken in a hospital setting if experts are readily avail- tencies or temperatures? Does the child have aversive
able and can attend each feeding session to evaluate the eating or feeding behaviors, such as screaming, crying,
behaviors and coach the family members to improve the gagging, hitting, or biting himself or herself or others
child’s food intake. Routine 2-week admissions for FTT are during the meal? Does the child turn his or her head away
not practical today. Children occasionally may be admitted from food, induce emesis, or pocket food in the mouth
for several days to document weight gain with measured for long periods of time? Based on the results of these
food intake, but even this practice is problematic. It is very observations, it may be important to have a detailed
difficult to evaluate weight changes over 2 or 3 days. Acute evaluation performed by an expert in oromotor function
gains or losses may be explained by edema or diuresis or by in young children, such as an occupational therapist or
whether food or liquid intake, urination, or defecation speech and language pathologist.
occur before or after the child is weighed. Confirmation of Depending on the results of such evaluation, initial
real weight gain requires tracking growth measurements treatment may include procedures such as decreasing
over several weeks, which can and should be performed in oromotor hypersensitivity; altering food textures, colors,
an ambulatory environment. or temperatures in a stepwise fashion; spacing meal times
The current approach to FTT, emphasizing undernu- optimally to drive hunger; and working with the family
trition, leads naturally to efforts to maximize nutritional to develop child-specific behavioral interventions, such as
repletion by supplemental means. Such efforts may in- positive reinforcement for desired eating behaviors and
clude nasogastric tube feeding or gastrostomy feeding if extinction procedures to eliminate undesired behaviors.
a lengthy period of repletion is predicted. There may be Punishment of undesired behaviors should be explicitly
a role for a brief admission (ie, several days) to teach the avoided. Only in the most unusual circumstances, in
family how to place and manage a nasogastric tube. controlled settings with rigorous safeguards, have some
Typically, the general pediatrician or team of experts in a behavioral experts reported success in using carefully de-
specialized care setting institutes nasogastric tube feed- signed punishment paradigms to improve eating behaviors.
ing in the home environment because of limitations on Behavioral interventions, even with successful dietary
hospitalization coverage. manipulations to maximize nutritional intake, might take
weeks or months to demonstrate appropriate catch-up
What is the Treatment of FTT? weight gain and, in fact, may fail to produce the desired
Because FTT is a sign of undernutrition, nutritional improvement. Because of the concerns about potential
repletion is the sine qua non of successful therapy. Even long-term intellectual, emotional, or behavioral sequelae
in cases in which a malabsorption syndrome is present or of FTT, it may be desirable to maximize nutritional
in which a chronic medical condition is diagnosed, suc- intake by supplemental enteral feeding.

In recent years, many pediatricians caring for patients into frank metabolic syndrome. Although an individual’s
who have FTT have turned to early and vigorous use of weight or BMI might be statistically within the normal
nasogastric tube feeding to accelerate weight gain and a range, that person might be functionally obese. Recent
return to statistical normalcy on the growth curves. This endocrinologic research has suggested other evidence to
maneuver often is successful but may still take a number support this possibility, including perturbations of hor-
of months to achieve the desired results. During that mones such as leptin, ghrelin, and adiponectin. There is
time, there will almost always be loss of whatever appetite no straightforward method of identifying such a child
the child does have, an increase in aversive behaviors, because body habitus, truncal obesity, or skinfold mea-
possible problems maintaining proper placement of the surements may not meet current standards for obesity.
tube, and nasal irritation and pulmonary aspiration of No evidence refutes the essential hypothesis that some
formula. These issues raise concerns for families and for individuals may be programmed to be smaller and
the professionals caring for the child. Thus, a number of slighter than typically is considered normal and that
children receive a long-term feeding gastrostomy with or well-intended nutritional interventions may produce un-
without an initial trial of nasogastric tube feeding. expected, deleterious outcomes.
Clearly, decisions about supplemental feeding are com- This line of thinking creates a major dilemma for the
plex, and advising families about their benefits and risks is pediatrician caring for a child who has FTT. On the one
best left, whenever possible, to teams that have expertise hand, there is substantial evidence, although not definite
in this arena. proof, that some children who have FTT may have
adverse neurodevelopmental outcomes. That evidence
Are There Long-term Risks to argues for nutritional intervention. However, some chil-
Vigorous Nutritional Repletion? dren who have “FTT,” according to the usual defini-
Reports on the long-term growth of young children who
tions, are at risk of developing metabolic syndrome later
have FTT are widely discrepant. Some individuals appear
in life if clinicians overcorrect their growth parameters.
to continue to be small and thin throughout life, others
For these children, nutritional therapy should be assidu-
appear to be normal, and some even appear to become
ously avoided. There is no definite guidance for the
overweight. Consideration is being given to the possibil-
physician at this point; each child must be evaluated
ity that certain individuals may be biologically pro-
individually.
grammed to be at weights or heights that are below the
standards that are generally accepted as appropriate,
based on population-based growth curves. In other
words, what is statistically normal may not be biologically
normal. According to this view, aggressively increasing
Summary
nutritional intake to produce weight gain may not be • Based on consensus, FTT is best considered a physical
sign of undernutrition and not a clinical syndrome
beneficial to all patients.
caused by “organic” or “nonorganic” factors. (1)(2)
Insulin resistance has been suggested as one cause for • Based on strong research evidence, infants and
concern. In 1999, Cianfarani and associates (13) pro- young children may cross major percentile lines on
posed that children who experienced IUGR might even- growth curves during a normal course of growth.
tually develop insulin resistance as a defense mechanism Therefore, documentation of weights or lengths
falling off of growth channels is not, by itself, proof
to protect against postnatal hypoglycemia. In 2000,
of FTT. (3)
Jaquet and colleagues (14) showed that young adults • Based on conflicting research evidence, it is unclear
who had been born with IUGR did, in fact, demonstrate how many children have adverse neurodevelopmental
such insulin resistance. Steward (15) summarized grow- outcomes from FTT. (4)(5)(6)(7)(8)
ing evidence that a subpopulation of term infants whose • Based on some research evidence, extensive
laboratory screening is of little utility in the
birthweights were above the traditional cut-off value of
evaluation of FTT. (12)
2,500 g may actually have unrecognized IUGR. Infants • Based on some research evidence, certain children
who have clear-cut IUGR are known to be at risk for who appear to have FTT may be biologically
subsequent FTT, and this additional group of unrecog- programmed to be smaller and thinner than most
nized infants may bear a similar risk. According to this children. Insulin resistance may be a mechanism, and
aggressive nutritional intervention may put these
line of thought, vigorous nutritional intervention in such
children at risk of developing metabolic syndrome.
infants or young children who have FTT might exacer- (13)(14)(15)
bate their insulin resistance and eventually drive them

References ing in infancy and IQ levels at 8 years in the Avon Longitudinal

1. Zenel JA. Failure to thrive: a general pediatrician’s perspective. Study of Parents and Children. Pediatrics. 2007;120:e1051– e1058
Pediatr Rev. 1997;18:371–378 9. Lozoff B, Jimenez E, Hagen J, Mollen E, Wolf A. Poorer
2. Gahagan S. Failure to thrive: a consequence of undernutrition. behavioral and developmental outcome more than 10 years after
Pediatr Rev. 2006;27:e1– e11 treatment for iron deficiency in infancy. Pediatrics. 2000;105:e51
3. Mei Z, Grummer-Strawn LM, Thompson D, Dietz WH. Shifts 10. Drewett RF, Corbett SS, Wright CM. Physical and emotional
in percentiles of growth during early childhood: analysis of longi- development, appetite and body image in adolescents who failed to
tudinal data from the California Child Health and Development thrive as infants. J Child Psychol Psychiatry. 2006;47:524 –531
Study. Pediatrics. 2004;113:e617– e627 11. Raikkonen K, Pesonen A-K, Heinonen K, Lahti J, et al. Infant
4. Corbett SS, Drewett RF. To what extent is failure to thrive in growth and hostility in adult life. Psychosomatic Med. 2008;70:
infancy associated with poorer cognitive development? A review 306 –313
and meta-analysis. J Child Psychol Psychiatry. 2004;45:641– 654 12. Sills RH. Failure to thrive: the role of clinical and laboratory
5. Rudolf MCJ, Logan S. What is the long term outcome for evaluation. Am J Dis Child. 1978;132:967–969
children who fail to thrive? A systematic review. Arch Dis Child. 13. Cianfarani S, Germani D, Branca F. Low birthweight and adult
2005;90:925–931 insulin resistance: the “catch-up growth” hypothesis. Arch Dis
6. Drotar D, Sturm L. Prediction of intellectual development in Child Fetal Neonatal Ed. 1999;81:F71–F73
young children with early histories of nonorganic failure to thrive. 14. Jaquet D, Gaboriau A, Czernichow P, Levy-Marchal C. Insulin
J Pediatr Psychiatr. 1988;13:281–296 resistance early in adulthood in subjects born with intrauterine
7. Black MM, Dubowitz H, Krishnakumar A, Starr RH. Early growth retardation. J Clin Endocrinol Metab. 2000;85:1401–1406
intervention and recovery among children with failure to thrive: 15. Steward DK. Biological vulnerability in infants with failure to
follow-up at age 8. Pediatrics. 2007;120:59 – 69 thrive: the association with birthweight. Child Care Health Dev.
8. Emond AM, Blair PS, Emmett PM, Drewett RF. Weight falter- 2001;27:555–567

http://www.healthychildren.org/English/health-issues/conditions/chronic/Pages/
Failure-to-Thrive.aspx

PIR Quiz
Quiz also available online at http://pedsinreview.aappublications.org
6. You are evaluating a 2-year-old child who has been referred to you for poor weight gain. Before seeing the
patient, you examine his growth chart, which reveals weight below the 5th percentile (50th percentile for
12 months), height at the 25th percentile, and head circumference at the 50th percentile. Solely considering
the growth chart parameters, which of the following is the most likely reason for his poor weight gain?
A. Cystic fibrosis.
B. Growth hormone deficiency.
C. Inadequate caloric intake.
D. Metabolic disorder.
E. Underlying genetic disorder.
7. You are seeing an 18-month-old boy who has Down syndrome for a health supervision visit. His mother is
concerned that he is not gaining weight well. She reports that he is a picky eater and often spits up after
feedings. He has no history of cardiac or intestinal conditions but has been diagnosed with otitis media
three times. Of the following, which is the most important to do first?
A. Initiate ranitidine therapy for reflux.
B. Obtain his neonatal records.
C. Plot his weight, height, and head circumference on a Down syndrome growth chart.
D. Refer him to a dietitian for nutritional counseling.
E. Refer him to an otolaryngologist for insertion of tympanostomy tubes.
8. You diagnose FTT in a 15-month-old boy at his first visit to the clinic. His development is normal and he
has no history of vomiting, diarrhea, or chronic infections. He has had no primary care physician before
now. His growth parameters are: weight at the 10th percentile, height less than the 5th percentile
(50th percentile for 10 months), and head circumference at the 25th percentile. There are no abnormal
findings on physical examination. Which of the following is most likely to reveal a diagnosis in this child?
A. Complete blood count with differential count.
B. Complete metabolic panel.
C. Growth hormone testing.
D. Immunologic testing.
E. Sweat test.
9. A 2-year-old girl in your clinic has FTT, with her weight and height less than the 5th percentile for age.
Her physical examination findings are normal. Which of the following additional historical or examination
findings would most likely lead you to recommend hospitalization at this time?
A. Failure to gain weight on several visits despite dietary intervention.
B. Head circumference also below the 5th percentile.
C. History of gastroesophageal reflux.
D. Moderate speech delay.
E. Recent divorce of parents.
10. You are giving a lecture to a group of medical students about the management of FTT, and one of them
asks whether hospitalization is always indicated. Of the following, the most likely benefit of hospitalizing
children who have FTT is that:
A. Laboratory evaluation can be performed more efficiently, leading to a diagnosis more quickly.
B. Nurses can educate parents on the proper use of a nasogastric feeding tube.
C. Parents finally understand that the child’s FTT is a serious problem.
D. Therapists and consultants can efficiently and repeatedly evaluate the child’s eating behaviors.
E. Weight gain in the hospital proves that there is nonorganic FTT.

Arthur C. Jaffe
Pediatr. Rev. 2011;32;100-108
DOI: 10.1542/pir.32-3-100

Nutrition and Nutritional Disorders
http://pedsinreview.aappublications.org/cgi/collection/nutritional
_disorders Preventive Pediatrics
_pediatrics Growth and Development
http://pedsinreview.aappublications.org/cgi/collection/growth_d
evelopment

Sacral Dimples
Holly A. Zywicke and Curtis J. Rozzelle
Pediatr. Rev. 2011;32;109-114
DOI: 10.1542/pir.32-3-109

Article nervous system
Sacral Dimples
Holly A. Zywicke, MD,*
Curtis J. Rozzelle, MD†
1. Explain the difference between open and closed neural tube defects.
2. Describe the characteristics of spinal skin dimples that warrant further evaluation.
Author Disclosure 3. Describe the characteristics of spinal skin dimples that do not warrant further
Drs Zywicke and evaluation.
Rozzelle have 4. Discuss the evaluation of spinal skin dimples and name the findings that suggest
disclosed no financial occult spinal dysraphism.
relationships relevant 5. Discuss the neurosurgical treatment of occult spinal dysraphism.
to this article. This 6. Explain the natural history and clinical manifestations of occult spinal dysraphism.
commentary does not
contain a discussion
of an unapproved/ Definitions
Neural tube defects are among the most common forms of birth defect, affecting 1 in every
1,000 pregnancies. (1)(2) These defects, which result from abnormal fusion of the neural
commercial
tube during embryonic development, are placed into two broad categories: open and
product/device. closed. Open neural tube defects are lesions in which brain, spinal cord, or spinal nerves are
exposed through obvious defects of the meninges and skull or vertebral column. Examples
are anencephaly, myelomeningocele, and meningocele. Closed neural tube defects are
skin-covered lesions under which the nervous system structures have not formed normally.
These include split cord malformation, dermal sinus tract, tethered spinal cord, and
intraspinal lipoma (Table).
Spina bifida is an imprecise term often used to describe a variety of congenital spinal
anomalies that range in consequence from insignificant to severe. Spina bifida occulta
(SBO) is a radiographic finding that describes incomplete osseous fusion of the posterior
elements. It may occur in conjunction with a cutaneous abnormality but is clinically benign
and is considered a normal variant. (3) Occult spinal dysraphisms (OSDs) are much less
common than SBO and encompass a variety of skin-covered neural tube defects. Because
the neural structures are affected, however, neurologic impairment is common. Most
forms of OSD have an associated overlying cutaneous abnormality.
Most open neural tube defects are diagnosed prenatally with ultrasonography and
serum marker concentrations. Those defects not identified before delivery are apparent at
birth. An OSD, on the other hand, is less obvious and may not be diagnosed until later in
life, despite its presence at birth. The occult nature can be problematic because the clinical
impairments associated with closed neural tube defects, which include paresis, spasticity,
sensory disturbance, orthopedic deformity or contracture, and bowel and bladder dysfunc-
tion, often progress insidiously over time.
Diagnosis
More than 50% of OSDs are diagnosed when a dimple (Fig. 1) is noted in the lower
spine/sacral region. Although the natural history of OSD is not fully understood, early
diagnosis and intervention are believed to improve outcome in almost all cases. (4) Hence,
the recognition of a suspicious skin dimple and identification of underlying anomalies with
prompt radiographic evaluation and neurosurgical referral is crucial. However, not all
dimples are associated with an OSD. Distinguishing between cutaneous stigmata associ-
*Division of Pediatric Neurosurgery, Department of Surgery, University of Alabama at Birmingham and Children’s Hospital of
Alabama, Birmingham, AL.
†
Assistant Professor, Department of Surgery, Division of Pediatric Neurosurgery, University of Alabama at Birmingham and
Children’s Hospital of Alabama, Birmingham, AL.

nervous system sacral dimples
Table. Definitions
Open Neural Tube Defects
Anencephaly An exposed rudimentary brainstem due to failed closure of the
cephalic portion of the neural tube.
Myelomeningocele Herniation of the spinal cord through an unfused portion of
the spinal column.
Meningocele Protrusion of the meninges through a spinal column opening.
Closed Neural Tube Defects
Split Cord Malformation Diastematomyelia Division of the spinal cord into two parts that are usually
separated by bone or cartilage.
Dermal Sinus TractⴞInclusion Tumor Incomplete dysjunction (separation of the cutaneous ectoderm
and neuroectoderm), resulting in an epithelial-lined tract
that terminates in neural structures.
Tethered Spinal Cord Abnormal attachment of the lower end of the spinal cord to
surrounding structures.
Lipomyelomeningocele or Spinal Cord Lipoma Premature dysjunction allows mesodermal infiltration between
cutaneous ectoderm and neuroectoderm, resulting in a
tethered spinal cord attached to a benign fatty tumor in the
back.
ated with OSD and innocent skin dimples can be difficult association with milder forms of OSD and warrant fur-
and may lead to costly and unnecessary tests or referrals. ther evaluation. Therefore, a deviated or duplicated
(5) Therefore, the focus of this review is to provide (“split”) gluteal cleft (Fig. 3) should raise concern for
information on how to identify skin dimples that require OSD, whether or not a dimple is present. The manage-
further evaluation, what method of evaluation should be ment of a “dimple” alone, however, demands greater
used, and when to refer to a specialist. diagnostic acumen because some dimples over the spine
Most cutaneous stigmata associated with OSD are represent dermal sinus tracts, although most do not.
found in the midline overlying the spinal lesion. A find- Dermal sinus tracts are not classified as open neural tube
ing of hypertrichosis, capillary hemangioma, atretic me- defects because characteristically they do not feature
ningocele, subcutaneous mass (eg, lipoma), or a caudal overt meningeal, osseous, and cutaneous defects (despite
appendage (Fig. 2) requires further investigation. Glu- potential communication between the skin and nervous
teal cleft anomalies other than dimples also have a weak elements). These skin-covered lesions are marked simi-
larly to other OSDs with overlying dimples or other
cutaneous stigmata. (6)
Clinical findings do not predict with certainty which
dimples are associated with OSD. However, the follow-
ing criteria have been found to differentiate best between
dimples that require further evaluation and those that
require only routine follow-up evaluation: multiple dim-
ples, dimple diameter larger than 5 mm, location greater
than 2.5 cm above the anal verge, and association of the
dimple with other cutaneous markers. Review of the
literature shows that 2% to 4% of all children have a
dimple identified in the sacrococcygeal region, but only
seven cases have ever been found to be associated with an
OSD. (7) For those patients in whom a coccygeal dimple
was found in conjunction with an OSD, most had more
Figure 1. Solitary dimple whose location greater than 2.5 mm than one dimple. Notably, the second dimple often was
above the anus indicated the need for further evaluation, found more rostrally along the spine (ie, cervical, tho-
which revealed an occult spinal dysraphism requiring neuro- racic, or lumbar). Hence, clinical examination should
surgical intervention. seek to identify dimple location and the number present.

Figure 3. A duplicated gluteal cleft associated with occult

spinal dysraphism.
pediatrician must perform a careful physical examination,

with particular attention to the neurologic and ortho-
pedic aspects. Associated orthopedic findings can include
clubfeet, arthrogryposis (contracture of multiple joints
leading to fixation of the joints in extension or flexion) of
the lower extremities, and hip dislocation. Abnormal cur-
vature of the spine, including kyphosis or scoliosis, also
may be present. Abnormal neurologic or orthopedic
examination findings indicate the need for further eval-
uation.
Management
When detailed history and physical examination raise the
Figure 2. Cutaneous lesions. Skin dimples are often the cu-
clinical suspicion for OSD, radiographic imaging should
taneous marking found with occult spinal dysraphism. How-
ever, multiple other markings can signify underlying spinal be obtained (Fig. 7). Either ultrasonography or magnetic
element malformation, including caudal appendage (A) and resonance imaging (MRI) can be employed to evaluate
hypertrichosis (B).
Solitary sacrococcygeal pits located entirely within the

gluteal cleft (Fig. 4) have no clinical significance and
should be considered anatomic variations of normal.
Typically, the coccyx is palpable beneath the dimple and
intact skin can be seen at the base (Fig. 5). If there is
difficulty discerning whether the lesion is covered com-
pletely by skin, otoscopic examination of the dimple
often can determine if there is a bottom to the pit.
Although most lesions occur in the midline, eccentric
lesions (Fig. 6) are not in themselves abnormal unless
occurring in conjunction with other lesions or outside
the sacral spine. No radiographic evaluation or neuro-
surgical consultation is required; parental reassurance is Figure 4. A prototypical benign sacral dimple that is located
the only intervention necessary. within the gluteal cleft (less than 2.5 cm above the anus) and
In addition to a thorough inspection of the skin, the solitary.

Figure 5. Solitary sacrococcygeal dimple that demonstrates

complete covering with skin over the entire dimpled area when
the skin is stretched laterally and, therefore, is not an occult
spinal dysraphism-associated lesion.
OSD. Ultrasonography of the lumbosacral spine gener-

ally is useful only in children younger than 3 months of
age because ossification of the vertebral arches has not
yet occurred. (8) However, the decision to use ultra-
sonography versus MRI (for children of any age) as first-
line imaging appears somewhat institution-dependent. In Figure 6. A right eccentric dimple that occurs outside of the
one study of a pediatric population who had sacrococcy- midline but does not carry a high degree of suspicion for an
geal cutaneous lesions, a discordance rate of 17% be- occult spinal dysraphism because it is an isolated sacrococcy-
tween ultrasonography and MRI studies was found in geal lesion.
which ultrasonography suggested an OSD while MRI
yielded normal results. (9) Pediatricians, therefore, hallmark of dermal sinus tracts that predispose the pa-
should be aware of the possible discrepancy in findings tient to bacterial meningitis or intraspinal abscess. (10)
with these imaging modalities and know which study is Surgical intervention is aimed at untethering the spinal
most appropriate at their respective institutions. cord and removing abnormal tissue, when present.
Spinal ultrasonography can assess the level of the
conus medullaris, the diameter and echogenicity of the Prognosis
filum terminale, and the position and movement pattern Almost all neurosurgical referrals for suspected OSD in
of the spinal cord and nerve roots. Abnormal findings can children younger than 1 year of age are for evaluation
include a low-lying conus, in which the tip is below the of a dimple. Although the natural history of OSD is
level of the second lumbar vertebral body; a filum termi- somewhat unpredictable, the overall risk of neurologic
nale diameter greater than 2 mm; and a posteriorly compromise increases with time. Neurologic deficits can
positioned or nonmobile cord, which can indicate teth- be difficult to identify in young children because the
ering. If ultrasonographic findings are abnormal, MRI of onset of dysfunction is generally insidious and occurs
the spine is indicated. Findings on MRI vary, based on about the same time as expected neurologic function
the type of OSD present. In general, MRI is more reliable development (eg, crawling, walking, standing). Accord-
and exact in diagnosing OSD. ingly, OSD deficits may be mistaken for delayed accrue-
Neurosurgical referral is appropriate if radiographic ment of normal function, and irreversible damage may
evaluation reveals any spinal abnormality. Consideration occur before symptomatic manifestation. The reasons for
for early referral (before imaging) is appropriate for dim- neurosurgical referral for children older than 1 year of
ples superior to the gluteal cleft, especially if any dis- age suspected of having OSD include chronic urinary
charge is observed or reported. Such dimples are the tract infections, lower limb deformity (eg, foot drop,

aging and neurosurgical referral. (11) Optimal outcome

is most likely with early diagnosis and surgical interven-
tion.
Summary
• Spinal skin dimples and other cutaneous markings
located outside of the sacrococcygeal region are
associated most often with closed neural tube
defects or OSD.
• The presence of more than one skin dimple
anywhere along the neural axis is an indicator of the
likely presence of OSD.
• The neurologic deficits associated with OSD are
progressive and frequently not detected until
permanent dysfunction has been sustained when
diagnosed later in life.
• Early neurosurgical intervention is believed to
prevent or halt progression of neurologic deficits
due to spinal cord tethering.
References
1. Wiswell TE, Tuttle DJ, Northam RS, Simonds GR. Major
Figure 7. An algorithm for evaluation of dimples overlying congenital neurologic malformations. A 17-year survey. Am J Dis
the neural axis. MRIⴝmagnetic resonance imaging Child. 1990;144:61– 67
2. Williams LJ, Rasmussen SA, Flores A, Kirby RS, Edmonds LD.
Decline in the prevalence of spina bifida and anencephaly by race/
weakness or atrophy in a lower extremity, talipes equino-
ethnicity: 1995–2002. Pediatrics. 2005;116:580 –586
varus, or dragging one foot), bowel/bladder dysfunc- 3. Boone D, Parsons D, Lachmann SM, Sherwood T. Spina bifida
tion, pain, and lower extremity spasticity or paresis. occulta: lesion or anomaly? Clin Radiol. 1985;36:159 –161
However, careful inspection of this population often 4. Soonawala N, Overweg-Plandsoen WCG, Brouwer OF. Early
reveals subtle cutaneous stigmata. Therefore, it is impor- clinical signs and symptoms in occult spinal dysraphism: a retrospec-
tive case study of 47 patients. Clin Neurol Neurosurg. 1999;101:
tant for the pediatrician to be vigilant in searching for
11–14
midline skin anomalies. Even as common a condition as 5. Medina LS, Crone K, Kuntz KM. Newborns with suspected
primary nocturnal enuresis warrants careful examination occult spinal dysraphism: a cost-effectiveness analysis of diagnostic
for midline skin anomalies. strategies. Pediatrics. 2001;108:e101– e108
6. Ackerman LL, Menezes AH. Spinal congenital dermal sinuses: a
30-year experience. Pediatrics. 2003;112:641– 647
Conclusion 7. Weprin BE, Oakes WJ. Coccygeal pits. Pediatrics. 2000;105:
Early diagnosis of OSD often comes from identification e69 – e73
of spinal skin dimples. Recognition of suspicious lesions 8. Szyszko TA, Watson M. The value of ultrasonographic exami-
is important to reduce the risk of neurologic, urologic, nation of the lumbar spine in infants with specific reference to
and orthopedic dysfunction. During examination, the cutaneous markers of occult spinal dysraphism. Clin Radiol. 2005;
60:935
pediatrician should not only look for dimples along the
9. Sasani M, Asghari B, Asghari Y, Afsharian R, Ozer AF. Correla-
spine but also for other markings such as abnormal tion of cutaneous lesions with clinical radiological and urodynamic
hair growth, asymmetric gluteal creases, dermal sinuses/ findings in the prognosis of underlying spinal dysraphism disorders.
dimples/pits, hyper- or hypopigmentation, capillary Pediatr Neurosrg. 2008;44:360 –370
hemangiomas, skin tags, and subcutaneous fatty masses 10. Bhatia S, Gullu MS, Date NB, Muzumdar D, Muranjan MN,
Lahiri KR. Anterior sacral pyocele with meningitis: a rare presenta-
that are associated with OSDs. Any lesion along the spine
tion of occult spinal dysraphism with congenital dermal sinus.
outside of the sacrococcygeal region or identification of J Child Neurol. 2010;25:1393–1397
more than one skin marking anywhere along the spine 11. Hall DE, Udvarhelyi GB, Altman J. Lumbosacral skin lesions
warrants further evaluation, including radiographic im- as markers of occult spinal dysraphism. JAMA. 1981;246:2606

PIR Quiz
11. Which of the following is the best example of an open neural tube defect?
A. Anencephaly.
B. Dermal sinus tract.
C. Diastematomyelia.
D. Spinal cord lipoma.
E. Tethered spinal cord.
12. Among the following, the child most likely to benefit from early referral to a neurosurgeon is:
A. 1-month-old who has an eccentric sacral dimple.
B. 1-week-old who has a solitary sacrococcygeal pit.
C. 2-month-old who has a sacrococcygeal dimple.
D. 3-month-old who has a dimple superior to the gluteal cleft with discharge.
E. 3-week-old who has a palpable coccyx beneath the dimple.
13. An 8-month-old girl presents to your clinic with multiple dimples superior to the gluteal cleft, and you
suspect OSD. Among the following, the most appropriate next step in her evaluation is:
A. Computed tomography scan of the spine.
B. Lumbar puncture.
C. Magnetic resonance imaging of the spine.
D. Ultrasonography of the spine.
E. Radiographs of the spinal column.
14. A father brings his 6-year-old son to you for evaluation of nocturnal enuresis and occasional daytime
wetting. On physical examination, you note a sacral dimple. Among the following, the feature most
concerning for OSD is:
A. Eccentric sacral location of the dimple.
B. History of one urinary tract infection.
C. Hypertrophy of one foot.
D. Spasticity of the lower extremities.
E. Truncal hypotonia.
15. Of the following, the feature that best distinguishes a dimple associated with OSD is:
A. Cutaneous marker associated with the dimple.
B. Greater than 3 mm maximal dimension.
C. Location greater than 1 cm above the anus.
D. Sacrococcygeal location.
E. Single dimple.

Sacral Dimples
Holly A. Zywicke and Curtis J. Rozzelle
Pediatr. Rev. 2011;32;109-114
DOI: 10.1542/pir.32-3-109

Supplementary Material Supplementary material can be found at:

/DC1
Neurologic Disorders
http://pedsinreview.aappublications.org/cgi/collection/neurologi
c_disorders Fetus and Newborn Infant
http://pedsinreview.aappublications.org/cgi/collection/fetus_new
born_infant
Errata An erratum has been published regarding this article. Please see
next page or:

hematology thrombocytopenia
5. A 7-year-old girl presents with a 3-day history of bruising and an episode of epistaxis lasting 30 minutes.
On physical examination, the only abnormalities are scleral icterus, widespread bruising, and cutaneous as
well as mucosal petechiae. Laboratory results include a platelet count of 3ⴛ103/␮L (3ⴛ109/L), hemoglobin
of 7.8 g/dL (78 g/L), white blood cell count of 12.9ⴛ103/␮L (12.9ⴛ109/L), absolute neutrophil count of
8.8ⴛ103/␮L (8.8ⴛ109/L), and mean corpuscular volume of 86 fL. Urinalysis is negative for red blood cells.
The most appropriate next study is:
A. Antiplatelet antibodies.
B. Bone marrow aspirate.
C. Direct antiglobulin (Coombs) test.
D. Flow cytometry on peripheral blood.
E. Serum blood urea nitrogen and creatinine assessment.
Corrections
The caption for Figure 2 in the article entitled “Focus on Diagnosis: Urine Electrolytes” in
the February issue of the journal (Pediatr Rev. 2011;32:65– 68) is incorrect. The correct
caption should read, “A graphic illustration of a positive urine anion gap, with the number
of unmeasured anions exceeding the number of unmeasured cations. When this situation
occurs in the context of metabolic acidosis, it is consistent with renal tubular acidosis,
indicating an impaired ability to excrete protons in the urine as ammonium.” We regret the
error.
The caption for Figure 1 in the article entitled “Sacral Dimples” in the March issue of
the journal (Pediatr Rev. 2011;32:109 –114) is incorrect. The correct caption should read,
“Solitary dimple whose location is greater than 2.5 cm above the anus indicated the need
for further evaluation. . . .” We regret the error.
Pediatrics in Review Vol.32 No.4 April 2011 151

Venereal Warts in Children
Kelly A. Sinclair, Charles R. Woods and Sara H. Sinal
Pediatr. Rev. 2011;32;115-121
DOI: 10.1542/pir.32-3-115

Article infectious diseases

Kelly A. Sinclair, MD,*
Charles R. Woods, MD,
MS,† Sara H. Sinal, MD§ 1. Describe the natural history and epidemiology of anogenital human papillomavirus
infection.
2. Discuss diagnosis and treatment options for children who have anogenital warts.
Author Disclosure 3. Recognize when anogenital warts are suggestive of child sexual abuse and what steps
Drs Sinclair and Sinal are needed to manage this clinical problem.
have disclosed no
financial relationships
relevant to this Introduction
More than 24 million cases of human papillomavirus (HPV) infection occur in adults in the
article. Dr Woods has
United States, with an estimated 1 million new cases developing each year. The number of
disclosed that he outpatient visits for adults who have venereal warts (condyloma acuminata) increased
received a research fivefold from 1966 to 1981. (1) HPV infections in children may present as common skin
grant from Pfizer warts, anogenital warts (AGW), oral and laryngeal papillomas, and subclinical infections.
Laboratories. This The increased incidence of AGW in children has paralleled that of adults.
commentary does AGW in children present a unique diagnostic challenge: Is the HPV infection a result of
child sexual abuse (CSA), which requires reporting to Child Protective Services (CPS), or
contain a discussion
acquired through an otherwise innocuous mechanism? Practitioners must balance “miss-
of an unapproved/ ing” a case of CSA if they do not report to CPS against reporting to CPS and having
investigative use of a parents or other caregivers potentially suffer false accusation and its potential ramifications,
commercial product. which may include losing custody of children.
In the past, simply identifying AGW in a young child was considered indicative of CSA
by some experts. However, there is no defined national standard beyond the limited
guidance provided in the 2005 American Academy of Pediatrics (AAP) Policy Statement,
which states that AGW are suspicious for CSA if not perinatally acquired and the rare
vertical, nonsexual means of infection have been excluded. (2) Guidance in determining
perinatal acquisition or nonsexual transmission is not provided. This review examines the
pathophysiology of HPV causing AGW in children and adolescents, diagnostic challenges,
treatment options, and a clinical pathway for the evaluation of young children who have
AGW when CSA is of concern.
Epidemiology and Transmission

HPV are double-stranded DNA viruses that belong to the family of
Papillomaviridae. More than 200 HPV serotypes have been identified, but
only 85 have been more thoroughly characterized. Skin warts caused by
Abbreviations types 1, 2, 3, 4, 7, and 10 may occur in as many as 10% to 20% of all
AAP: American Academy of Pediatrics children, with the peak incidence between ages 12 and 16 years. Types
AGW: anogenital warts 6 and 11 are responsible for up to 75% to 90% of genital infections and are
CPS: Child Protective Services the most common types found in oral lesions. HPV 16 and 18 also are
CSA: child sexual abuse common causes of genital tract infection and cause about two thirds of
FDA: Food and Drug Administration cervical cancers and many vulvar, penile, and anal cancers as well as oral
HPV: human papillomavirus cancers of epithelial origin.
PPV: positive predictive value HPV infections in humans fall into two general categories: cutaneous
STI: sexually transmitted infection and mucosal. There is no host preference based on age, sex, or race. HPV
may be present in normal-appearing cells and remain latent for months to
*Emergency Medical Services, The Children’s Mercy Hospital and Clinics, Kansas City, MO.
†
Department of Pediatrics, University of Louisville School of Medicine, Louisville, KY.
§
Department of Pediatrics, Wake Forest University Health Sciences, Winston-Salem, NC.

infectious diseases venereal warts
years before generating visible lesions. Multiple factors definitively distinguish among the possible mechanisms
have been shown to change susceptibility and progres- of acquisition of infection. Such testing is expensive and
sion of disease after infection: nutrition, endogenous and generally not recommended. Serotyping the child’s le-
exogenous hormones, tobacco use, parity, and immune sion is not considered helpful because AGW in children
status. Immunocompetent children and adolescents usu- are often caused by cutaneous types (up to 40% in some
ally can clear HPV infection within a 2-year period by a studies). HPV serotypes 6 and 11, most commonly con-
cell-mediated immune response. Persistence of infection, sidered the sexually transmitted serotypes of HPV, occa-
including latency, appears to increase from adolescence sionally cause cutaneous warts in children.
to adulthood. Three recent large case series have shown that most
In adolescents and adults, transmission of HPV types young children who have AGW have no physical or
causing AGW is primarily sexual. HPV is likely the most forensic evidence of CSA, with rates of CSA ranging
common sexually transmitted infection (STI), affecting from 3% to 10%. (5)(6)(7) CSA as the means of HPV
up to 80% of adults. Intercourse with an infected partner infection in children does increase with age. In a 2005
results in transmission of disease in two thirds of expo- study of 74 children who had AGW in North Carolina,
sures. Condoms are not as effective in preventing sexual among those 4 to 8 years old and 8 years and older, AGW
transmission of HPV as they are in preventing trans- was 2.9 times and 12.1 times, respectively, more likely
mission of other STIs. Most adolescents who become to have been due to CSA than in children younger than
infected with HPV do so within 2 years of first sexual 4 years. (5) For children younger than 4 years, when a
intercourse. Infection in adolescents is more commonly forensic history and thorough physical examination
subclinical or transient than in adults, but HPV infection raised no concerns, the positive predictive value (PPV) of
in sexually active adolescents can lead to cervical cancer. HPV infection alone was 21% for girls and 16% for boys
In female adolescents, perianal infection may result from and girls combined. For boys and girls 4 to 8 years of age
autoinoculation from secretions caused by primary cer- and those older than 8 years, the PPVs were 36% and
vicovaginal infection or from anal intercourse. In male 70%, respectively. In a 2006 Canadian study of 72 chil-
adolescents, anal intercourse is the primary cause of dren who had AGW, CSA was suspected or confirmed in
perianal lesions. 26% of those 2 to 6 years old and in 85% of those more
The prevalence of anogenital HPV infection in chil- than 6 years old. (6) In a 2007 study of 131 6-month-old
dren and adolescents is unknown but parallels that of to 9-year-old children in Kentucky referred to a pediatric
adults. The average age of presentation of AGW in chil- gynecologist for AGW, (7) 50% had a maternal history of
dren is 2.8 to 5.6 years. (3)(4)(5)(6) AGW in young girls AGW or cervical dysplasia. Among 81 patients who had
are seen in the vulvar, perianal, hymenal, vaginal, and siblings, 49% of siblings also had AGW. After full legal
urethral areas, regardless of method of transmission. system investigations, only three of 131 cases (2%) were
Young boys have perianal lesions; penile lesions are rare. “ruled to be suspicious for abuse.” Perinatal transmission
In adolescent boys, penile lesions are more common than was considered the most likely mode of transmission for
perianal lesions. AGW, like other STIs such as trichomo- most of these children.
niasis, often are asymptomatic in males.
HPV serotypes that cause AGW in young children Diagnosis
may be transmitted by perinatal exposure, heteroinocu- AGW is diagnosed primarily by clinical appearance
lation, autoinoculation, and indirect fomite transmis- (Figs. 1 and 2). The warts start as a small, flesh-colored
sion, in addition to sexual abuse. Children may auto- papule in the perianal area in males or females and
inoculate themselves, usually from a wart on the hand to subsequently grow on the hymen, in the vestibule or
another location on the body, including the genital area. vulvar areas, or around the urethra in females or less
Nonsexual transmission via sharing underwear, bath commonly on the penile shaft in males. Over a period of
towels, and swimsuits as well as bidet use has been re- months or sometimes very rapidly, these lesions develop
ported. Transmission to young children from family into clusters of skin-colored flat warts or somewhat pe-
members or other caregivers during diapering or routine dunculated larger cauliflower or berrylike keratinized
care has also been described. masses, similar to skin warts. Warts on the skin of the
Lesions from an affected child and those found on penis, scrotum, labia, and perianal areas tend to be flatter,
caregivers or others having contact with the child may be shiny, and less verrucous. In most cases, internal (specu-
assessed for concordance of serotype or genotype. How- lum) examination is not performed, but when surgical
ever, documented concordance or discordance does not treatment is undertaken for extensive warts, warts are

presents as shiny papules with central umbilication, and

most lesions measure 1 to 2 mm. Giant molluscum
lesions have been reported in the anogenital area in
children and are more easily confused with HPV. Con-
dyloma lata lesions, which are seen in secondary syphilis,
tend to be flatter and smoother. Syphilis serologies
should be positive in these cases, and a brushing for
dark-field testing should show spirochetes. In adoles-
cents, vulvar papillomatosis can be confused with AGW,
and these lesions are not associated with HPV infection.
Hymenal remnants may also be confused with condy-
loma acuminatum. Other conditions in the differential
diagnosis are epidermal verrucous nevus, Bowenoid
papulosis, pseudoverrucous papules and nodules, neuro-
fibromas, Langerhans histiocytosis, and malignant growths.
Figure 1. Findings in an 18-month-old girl evaluated for
anogenital warts, likely from perinatal transmission.
Treatment
Seventy-five percent of AGW resolve spontaneously
often found in the vagina and rectum as well. In adoles- within months to a few years in children who have
cents, smooth, flat warts are seen more commonly on the healthy immune systems. (8) Those persisting for more
cervix when there is clinically identifiable infection. Out- than 2 years are less likely to resolve spontaneously or
side of the cervix, the vulva is the most commonly with treatment. (9) Many clinicians now prefer watchful
affected site in adolescent females, and nearly 25% of waiting over immediate treatment. Treatment may be
affected women also have perianal lesions. desired in long-lasting cases or when warts are causing
If the clinician is unsure if the lesion is a wart, which is symptoms such as pain with defecation, itching, or bleed-
more likely to be a concern in single lesions, a biopsy is ing. There are no United States Food and Drug Admin-
indicated. Brushing the lesion with a Papanicolaou brush istration (FDA)-approved treatments for AGW in chil-
and sending the specimen for HPV testing has been dren 12 years of age and younger. Treatments can be
recommended when the diagnosis is in doubt. We have divided into nonsurgical and surgical. No approach has
not found this technique to be very helpful because the been shown to be universally successful, and recurrence is
test result often is negative. A negative test result could common after any treatment. Some children may require
be true (the lesion is not due to HPV) or false due to a combination of therapies. Because recurrence is com-
inadequate sampling or limitations of the assay. In our mon, once a child has had AGW, the appearance of new
experience, molluscum contagiosum is the condition lesions after spontaneous resolution or treatment does
most easily confused with AGW. Molluscum typically not necessarily indicate a new exposure. This fact is
particularly important when the source of infection is
believed to be CSA.
Nonsurgical approaches include those that cause non-
specific tissue destruction and immunomodulators. (8)
Podophyllin is available as a resin (10% to 25%) that is
applied to the warts by the clinician every 1 to 2 weeks
until the warts resolve. The resin should be washed off
after 4 hours to minimize burning. Many parents and
children find this treatment unpleasant. A similar but less
potent drug is podofilox, available as a 0.5% gel or
solution, which is applied to the wart once or twice daily,
several days per week, by the caretaker at home, as
tolerated. Podofilox is not approved for use in children
younger than 12 years of age, although one study of 17
Figure 2. Anogenital warts are visible in the vestibule and on children suggests that podofilox is likely to be safe and
the hymen of a 9-year-old girl who was a victim of incest. effective in children and can be applied by the caretaker

at home with clearance rates of 88%. (10) Imiquimod, younger than 4 years of age as suspicious for CSA. How
an immunomodulator, has been used for treatment of then to proceed in the midst of this unfortunate clinical
AGW. It is not approved for treatment in children uncertainty?
younger than 12 years of age, but case series show
clearance rates approaching 75% in children. The 5% Areas of Consensus
cream is applied to the AGW and kept on overnight three There is general agreement that the evaluation of a child
times per week, with improvement expected in weeks to who has AGW for possible CSA should include the
months. (10) Local irritation and pruritus can occur. following:
Surgical approaches include cryotherapy with liquid 1. Primary caregivers (usually parents) should be in-
nitrogen, electrodesiccation, and pulsed dye laser. Sur- terviewed for history regarding 1) cutaneous or AGW for
gical excision is also an option for treating a limited themselves and other family members and 2) abnormal
number of AGW. Clearance rates with these methods Papanicolaou smears or surgeries for cervical cancer in
in adults range from 27% to 100%, but recurrence rates the biologic mother (although oncogenic HPV serotypes
are approximately 25%. In our experience, surgical ap- usually do not cause AGW in children, they may coexist
proaches are reserved for children who have extensive, with nononcogenic types). In addition, caregivers should
symptomatic AGW. Because most affected children are be asked whether they suspect sexual abuse or if a sexual
younger than 4 years, general anesthesia is usually re- offender has had access to the child.
quired. Postoperative pain and scarring can occur. 2. An interview with the child regarding CSA should
be performed if the child is old enough to be interviewed,
Prevention usually 3 to 4 years of age. The interview must be
Two vaccines for HPV are currently available in the performed by a person trained in interviewing children
United States for administration to females between ages and who is familiar with acceptable interview techniques
9 and 26 years. They have been shown to be highly for determining the likelihood of CSA. In a recent study
effective in prevention of precancerous cervical lesions of 987 children ages 2 to 17 years who had been sexually
for at least 5 years after vaccination. Vaccination does not abused, 73% fully disclosed the abuse, 12% partially dis-
affect existing infection. Therefore, vaccination is most closed, 10% did not disclose, and 5% denied abuse. (12)
effective when administered to females before first sexual Fifty percent of children ages 2 to 6 years of age dis-
intercourse. The quadrivalent vaccine protects against closed. Factors associated with disclosure were age (older
HPV 16, 18, 11, and 6. The bivalent vaccine protects children more likely), sex (girls more likely), disclosure
against HPV 16 and 18. The Advisory Committee on before the interview (results in much higher disclosure rate
Immunization Practices recommends vaccination of during the interview itself), a positive relationship between
11- to 12-year-old girls with a three-dose schedule of 0, caregiver and child, and age of onset of CSA (the earlier the
2, and 6 months. (11) Vaccination also is recommended onset of abuse, the more likely the disclosure).
for females 13 to 26 years of age, and the series may be 3. An inventory should be taken of frequently seen
started as young as 9 years of age. Quadrivalent HPV signs, symptoms, and behaviors that occur in children
vaccine is now approved by the FDA for males and may who have been sexually abused. Among the common
be administered to 9- to 26-year-old males to reduce behaviors are nightmares, advanced sexual knowledge
their likelihood of acquiring genital warts. (See cdc.gov/ for age, and acting out sexually with peers. Twenty-eight
hpv for additional information.) percent of sexually abused children exhibit sexual behav-
ior problems, but in children who demonstrate intrusive
The Issue of CSA or aggressive sexual behavior, only 48% have been sexu-
Specific age cutoffs, typically 24 months or older at the ally abused. (13) Normal sexual behaviors must be dif-
time of diagnosis of AGW, have been suggested in the ferentiated from repetitive, intrusive, or abusive sexual
past as a primary criterion for reporting the child to CPS behaviors.
for potential CSA. The variability of the incubation pe- 4. A thorough physical examination should be per-
riod from HPV infection to the development of visible formed, looking for any evidence of physical or sexual
AGW, the potential for small lesions from perinatally abuse. This evaluation should include a careful and mag-
acquired infection to go unnoticed for weeks to months nified examination of the genital and anal areas, evaluat-
(or years), and the possibility of “innocent” postnatal ing for signs of acute trauma such as petechiae or bruising
acquisition of AGW make it difficult to justify a specific to the hymen and anal tears as well as signs of chronic
age cutoff as a sole criterion for reporting children trauma such as absent hymen tissue at the posterior

hymenal rim or scarring in the anogenital area. The

majority of examinations in abused children yield no Findings in a Child Who
Table 1.
results of note.
5. Screening for other STIs should be performed.
Has Anogenital Warts That
The 2005 AAP recommendations include testing for Require a Report to Child
gonorrhea, Chlamydia infection, Trichomonas infection, Protective Services
human immunodeficiency virus infection, hepatitis B and
C, and syphilis, depending on the circumstance of the • Parents suspect abuse
CSA, age of the child, and time since sexual contact. (2) • Child discloses abuse
6. Referral to a child abuse specialist is appropriate if • Child’s behaviors suggest abuse
• Physical examination suggests abuse
the primary care practitioner or specialists involved do
• Finding of a sexually transmitted infection in
not feel comfortable or adequately trained to perform addition to HPV
any parts of the previously noted evaluation. • Any child older than 48 months of age
Exceptions include adolescents who report consensual sexual activity
Lack of Consensus with an appropriate-age peer, and severely immunosuppressed children
In 2007, we surveyed members of The Ray Helfer Soci- who have multiple warts at other sites with no other findings of abuse.
HPV⫽human papillomavirus
ety (an honorary society of physicians with a mission to
provide leadership in prevention, diagnosis, and treat-
ment of child abuse), most of whom are child abuse
specialists, regarding their views on management of interviewer or is unable for some other reason to obtain a
young children who develop AGW. Of 195 members comprehensive evaluation, a decision to report may be
who could be reached by email, 65 (33%) responded. the default option.
There was significant disagreement as to whether chil- Table 1 outlines instances when reporting to CPS is
dren who have AGW should be reported to CPS regard- necessary. For a child who is younger than 4 years of age
less of whether further evaluation (interview of parent when AGW are first seen and the entire evaluation (his-
and child, behavioral inventory, genital/anal examina- tory, behavioral inventory, physical examination, and
tion for trauma, and cultures for other STIs) suggested STI screening studies) yields negative results, including
CSA. Given the scenario of a 4-year-old child who has no concerns regarding household or other locations of
AGW and whose comprehensive evaluation suggested no child care, a report to CPS is not mandatory because
other evidence of CSA, 36% agreed and 44% disagreed perinatal or “innocent” postnatal acquisition is highly
with reporting to CPS. In response to an item regarding likely in this context.
whether CSA was unlikely to be the source of AGW in a Because of increased risk of CSA with age, when AGW
3-year-old child, 45% agreed, 18% disagreed, and 37% are first seen in a child 4 years and older, we routinely
were unsure. This variation, even among a small subset of make a CPS report because the physical examination can
child abuse specialists responding to the survey, is indic- be negative for any other evidence of CSA and children
ative of the lack of certainty regarding whether CSA is a do not always disclose their abuse, even to skilled forensic
common cause of AGW in young children. In addition to interviewers. Some clinicians may choose routine report-
confusing clinicians, this ambiguity is a problematic real- ing of children too young to be reliably interviewed to
ity for CPS workers who have the responsibility to inves- CPS to elicit a home visit and bring to light any CPS
tigate the cases when reported as suspected abuse. CPS record of previous concerns potentially unknown to the
may refuse to accept such cases for investigation because clinician. In these cases, the clinician accepts as unavoid-
a physician cannot say whether AGW represents CSA, able the stress a CPS investigation will bring to a poten-
and CPS workers may already have significant case loads tially innocent family.
involving situations that have more definitive evidence. For a patient who is pubertal, the possibility of con-
sensual sexual activity must be explored. If consensual
Suggested Clinical Approach for activity occurred with a peer of appropriate age, no re-
Reporting to CPS port is needed. If the partner is of inappropriate age
First, it is important for the practitioner to be aware of (especially if meeting the definition of statutory rape by
the reporting laws in his or her state and be sure that a state law) or if the patient says sexual activity was not
decision not to report is acceptable under the law. In consensual, a report should be made. If no report is
addition, if a practitioner does not have access to a skilled made, careful follow-up by the primary care physician

a CPS report must be made. Parents also should be told

Follow-up Screening for
Table 2. that CPS will be made aware of the scientific limitations
in the area of AGW.
Sexual Abuse in Children Who
Have Anogenital Warts Not
Reported as Suspected Child
Sexual Abuse Summary
• HPV is widely prevalent and acquired from a variety
History of sources and a variety of routes.
• New caretaker concerns about abuse • The average age for presentation of AGW in
• New disclosure by the child prepubertal children is 3.75 to 4 years. (3)(4)(5)(6)
• Symptoms of anal/genital trauma or infection • AGW in children younger than 4 years of age are
(vaginal discharge, vaginal/anal bleeding, or pain) typically acquired from nonsexual transmission.
• Behaviors (nonspecific but often seen in CSA cases) (5)(6)(7)
–Unusual fears • CSA must be considered in any child who has AGW;
–Sleep disturbances the older the child, the more likely the AGW has
–Change in school performance resulted from sexual abuse. However, thorough
–Anger and acting out evaluation is necessary before determining if
• Behaviors (more specific for CSA) reporting to CPS is necessary.
–Sexual knowledge unusual for age of child • Adolescents generally acquire AGW through sexual
–Acting out sexual acts with peers activity that is usually but not always consensual.
–Inappropriate exposure or excessive touching of Adolescents are now encouraged to be immunized
genitalia of self or others against specific types of HPV to prevent cervical
cancer.
Examination Findings • AGW can be treated, but it is unclear whether
• Trauma to genital/anal area lesions resolve more rapidly with treatment. (8)(9)
• Trauma to skin in area of breasts, such as bite marks Most AGW in young children and adolescents do not
• Trauma to the mouth or pharynx, such as bruising of need to be treated because they resolve
tongue or palate spontaneously.
• AGW recurrence is common after treatment. In a child
Laboratory Testing who has been a victim of CSA, recurrence does not
• Confirmed sexually transmitted infection necessarily mean the child has been reabused. (9)
CSA⫽child sexual abuse
References
is indicated to observe for any future signs of CSA 1. Condyloma acuminatum - United States, 1966 –1981. MMWR
(Table 2). Morb Mortal Wkly Rep. 1983;32:306 –308
2. Kellogg N. The evaluation of sexual abuse in children. Pediat-
When the report is made, the CPS workers should be rics. 2005;116:506 –512
told that HPV infection causing AGW may be a result of 3. Obalek S, Jablonska S, Favre M, Walczak L, Orth G. Condylo-
CSA, but other sources of infection cannot be totally mata acuminata in children: frequent association with human pap-
eliminated. However, the case being reported is consid- illomaviruses responsible for cutaneous warts. J Am Acad Dermatol.
1990;23:205–213
ered at high risk for CSA because of the age of the child 4. Obalek S, Jablonska S, Orth G. Anogenital warts in children.
or the presence of other positive factors (eg, disclosure by Clin Dermatol. 1997;15:369 –376
the child, suspicion on the part of the parent, sexualized 5. Sinclair KA, Woods CR, Kirse DJ, Sinal SH. Anogenital and
behaviors, abnormal physical findings, or another STI). respiratory tract human papillomavirus infections among children:
age, gender, and potential transmission through sexual abuse.
If CSA is suspected by a nonfamily member or caretaker, Pediatrics. 2005;116:815– 825
a report to law enforcement would take the place of 6. Marcoux D, Nadeau K, McCuaig C, Powell J, Oligny LL.
reporting to CPS. In the case of young children, this is an Pediatric anogenital warts: a 7-year review of children referred to a
uncommon scenario. tertiary-care hospital in Montreal, Canada. Pediatr Dermatol. 2006;
23:199 –207
Parents need to be informed that state law requires 7. Jones V, Smith SJ, Omar HA. Nonsexual transmission of ano-
reporting of suspected abuse and although other causes genital warts in children: a retrospective analysis. Sci World J.
of HPV infection are possible, based on high risk factors, 2007;7:1896 –1899

8. Culton DA, Morrell DS, Burkhart CN. The management of 11. ACIP Provisional Recommendations for HPV Vaccine. 2009.
condyloma acuminata in the pediatric population. Pediatr Ann. Accessed January 2011 at: http://www.cdc.gov/mmwr/preview/
2009;38:368 –372 mmwrhtml/mm5920a4.htm?s_cid⫽mm5920a4_e
9. Allen AL, Siegfried EC. The natural history of condyloma in 12. Lippert T, Cross TP, Jones L, Walsh W. Telling interviewers
children. J Am Acad Dermatol. 1998;39:951–955 about sexual abuse: predictors of child disclosure at forensic inter-
10. Moresi JM, Herbert CR, Cohen BA. Treatment of anogenital views. Child Maltreat. 2009;14:100 –113
warts in children with topical 0.05% podofilox gel and 5% imi- 13. Kellogg ND. Clinical report–the evaluation of sexual behaviors
quimod cream. Pediatr Dermatol. 2001;18:448 – 450 in children. Pediatrics. 2009;124:992–998
PIR Quiz
16. You find perivulvar warts in a 2-year-old girl. She has a thin vaginal discharge, but the remainder of her
examination findings are normal. History reveals she has difficulty sleeping. Of the following, the
strongest argument that this finding should be reported to CPS is:
A. Age of the child.
B. Frequent nightmares.
C. Location of the warts.
D. Sex of the child.
E. Trichomonas vaginalis in the wet prep.
17. You find perivulvar warts in a 7-year-old girl. Aside from a thin vaginal discharge, the remainder of her
examination findings are normal. Wet prep and culture of the discharge yield no evidence for STI. History
reveals occasional sleepwalking. Of the following, the strongest argument that this finding should be
reported to CPS is:
A. Age of the child.
B. Her denial of abuse.
C. Her sleep disturbance.
D. Presence of discharge.
E. Sex of the child.
18. A 17-year-old girl has had asymptomatic vulvar warts for 3 years for which she never has received
treatment. These warts:
A. Are likely to resolve without treatment.
B. Are very unlikely to recur after treatment.
C. May be transmitted despite condom use.
D. Most likely reflect nonsexual contact.
E. Require treatment.
19. When administered as recommended, currently available HPV vaccines:

A. Are not useful for preventing HPV infections in males.
B. Conceal CSA.
C. Mitigate pre-existing HPV infections.
D. Prevent anogenital infection by all HPV serotypes.
E. Prevent the development of precancerous cervical lesions.

Kelly A. Sinclair, Charles R. Woods and Sara H. Sinal
Pediatr. Rev. 2011;32;115-121
DOI: 10.1542/pir.32-3-115

Genital System Disorders
http://pedsinreview.aappublications.org/cgi/collection/genital_sy
stem_disorders Infectious Diseases
http://pedsinreview.aappublications.org/cgi/collection/infectious
_diseases

Index of Suspicion • Case 1: Fatigue, Shortness of Breath, and Chest Pain in an
Adolescent Boy • Case 2: Poor Feeding and Weight Gain, Tachypnea, and
Vomiting in a Neonate • Case 3: Afebrile Seizures After a Bout of Diarrhea in a
2-year-old Girl
Claudeen K. F. Scott, Monvadi B. Srichai, Dina M. Kulik, Farid H. Mahmud,
Christina Olson and Julia Paz
Pediatr. Rev. 2011;32;123-128
DOI: 10.1542/pir.32-3-123

index of suspicion
Case 1: Fatigue, Shortness of Breath, and Chest

Pain in an Adolescent Boy
Case 2: Poor Feeding and Weight Gain, Tachypnea,
and Vomiting in a Neonate
Case 3: Afebrile Seizures After a Bout of Diarrhea
in a 2-year-old Girl
Case 1 Presentation cardiac silhouette and bilateral pleural
A 15-year-old boy presents with a effusions. Electrocardiography docu-
The reader is encouraged to write ments sinus tachycardia, poor R-wave
6-day history of progressive general-
possible diagnoses for each case before
turning to the discussion. We invite ized weakness, shortness of breath, progression, and 4-mm Q waves in the
readers to contribute case presentations and chest pain that he describes as inferior leads. Additional evaluation
and discussions. Please inquire first by squeezing in nature whenever he lies leads to the diagnosis.
contacting Dr. Deepak Kamat at flat. He has had difficulty sleeping
dkamat@med.wayne.edu.
and says that he feels that his “heart is
getting bigger.” Since yesterday, his Case 2 Presentation
symptoms have worsened, and he has A 16-day-old female infant presents
Author Disclosure
developed pain in his left arm and with a 1-day history of nonbilious
abdomen. He is more comfortable and nonbloody vomiting, poor feed-
Drs Scott, Srichai, Kulik, Mahmud,
sitting up. He denies any change in ing, and diaphoresis in the absence of
Olson, and Paz have disclosed no weight, night sweats, visual distur- fever. She was born at term via spon-
financial relationships relevant to bances, neck stiffness, or rashes. His taneous, uncomplicated vaginal de-
these cases. This commentary does past medical and family history con- livery to a healthy 27-year-old primi-
not contain a discussion of an tain no findings of note. He is not gravida. Her birthweight was 3.2 kg,
unapproved/investigative use of a taking any medication and denies use and she is breastfed, with occasional
commercial product/device.
of stimulants or illicit drugs. formula supplementation. The par-
On physical examination, his ents are nonconsanguineous. She is
weight is 81 kg, height is 179 cm, referred to the ED for poor weight
and body mass index is 25, all above gain, tachypnea, and poor feeding.
99th percentile. His resting heart On arrival, physical examination
Frequently Used Abbreviations rate is 121 beats/min, blood pres- reveals a 2.86-kg, mottled newborn
ALT: alanine aminotransferase sure is 91/55 mm Hg (which drops who is making no respiratory effort
AST: aspartate aminotransferase to 75/55 mm Hg with inspiration), and has a heart rate of less than
BUN: blood urea nitrogen and respiratory rate is 22 breaths/ 60 beats/min. The infant is intu-
CBC: complete blood count min. His oxygen saturation is 100% bated and a fluid bolus of 40 mL/kg
CNS: central nervous system in room air. He has intermittent sub- of normal saline is given. Intravenous
CSF: cerebrospinal fluid costal retractions, and air entry is de- (IV) antibiotics are initiated after
CT: computed tomography creased at both lung bases. The apex blood culture and lumbar puncture
ECG: electrocardiography beat is not displaced. Jugular venous samples are obtained.
ED: emergency department distension to 6 cm is present. Heart CBC shows Hgb of 18.6 g/dL
EEG: electroencephalography sounds are diminished, but there are (186 g/L), WBC count of 15.5⫻
ESR: erythrocyte sedimentation rate no murmurs, gallops, or rubs. There 103/mcL (15.5⫻109/L), and plate-
GI: gastrointestinal is no hepatomegaly or peripheral let count of 537⫻103/mcL (537⫻
GU: genitourinary edema. 109/L). Serum electrolytes are: so-
Hct: hematocrit CBC and values of serum electro- dium, 123 mEq/L (123 mmol/L);
Hgb: hemoglobin lytes, amylase, lipase, cardiac enzymes potassium, 11 mEq/L (11 mmol/L);
MRI: magnetic resonance imaging (including troponin), and liver en- chloride, 102 mEq/L (102 mmol/
WBC: white blood cell zyme are within age-appropriate limits. L); and bicarbonate, 17 mEq/L
Chest radiograph shows an enlarged (17 mmol/L), BUN is 23.8 mg/dL

index of suspicion
(8.5 mmol/L) and glucose is 65 mg/ are obtained, and she is admitted for 800 mL of bloody fluid. Pericardial
dL (3.6 mmol/L). The CSF has 4 observation. fluid cytology demonstrated clusters
WBCs and normal concentrations Early in the hospitalization, she has of reactive mesothelial cells.
of glucose and protein. Urinalysis two more afebrile seizures, one par- A subsequent cardiac MRI con-
reveals a specific gravity of less than tial and one generalized. Phenytoin firmed the large intracardiac mass,
1,005, 3⫹ red blood cells/high- administration results in complete with the epicenter in the inferior sep-
power field, and ⫹1 protein. Chest resolution of the seizures, despite tal wall involving large portions of
and abdominal radiographs are un- the patient having a subtherapeutic both ventricles (Fig. 2). Gadolinium
remarkable. Arterial blood gas shows drug concentration. Brain MRI and contrast administration resulted in
a pH of 7.41, PCO2 of 28 mm Hg, and EEG are read as normal. The hospital heterogeneous enhancement of the
PO2 of 151 mm Hg. Serum lactate course is complicated by an ileus that mass, suggesting cardiac neoplasm
measures 36.0 mg/dL (4 mmol/L) develops on the third day of hospi- with areas of necrosis. On biopsy of
(normal, ⬍ 21.6 mg/dL [2.4 mmol/ talization, requiring several days of the mass, histopathology was consis-
L]). The infant is treated for hyper- bowel rest and nasogastric tube de- tent with a desmoplastic small round
kalemia in the ED and transferred compression. A laboratory result is cell tumor (DSRCT). Bone scan and
to the critical care unit. Additional received a few days after discharge CT scan of the neck, chest, abdo-
laboratory evaluation leads to the that confirms the diagnosis. men, and pelvis did not show any
diagnosis. metastatic disease.
Due to the extensive involvement
Case 1 Discussion of both ventricular chambers with the
Transthoracic echocardiography neoplasm, resection was not deemed
Case 3 Presentation showed a large circumferential peri- possible. A chemotherapy regimen
A 2-year-old girl is brought to the cardial effusion measuring up to was initiated consisting of etoposide
ED because of a prolonged afebrile 6 cm at its maximum dimension and ifosfamide, followed by vincris-
seizure characterized by eye devia- and a large homogeneous, echogenic tine, cyclophosphamide, doxorubi-
tion and lip smacking. Two doses of 4⫻6⫻9-cm intracardiac mass (Fig. cin, and mesna. Despite several cycles
diazepam (0.1 mg/kg IV) are re- 1) that was confined to the mid and of chemotherapy, the tumor size re-
quired to stop the seizure. She has apical interventricular septum, oblit- mained unchanged, and serial echo-
no history of previous seizures or erating the right ventricular apex. cardiography demonstrated decreas-
other medical problems, and the only Emergency pericardiocentesis yielded ing cardiac function. Evaluation for
medication she has received is acet-
aminophen. She is developmentally
normal. Family history is noncon-
tributory.
She has had fever and vomiting for
the past 3 days, but by today, the
fever had resolved, the vomiting was
improving, and she was tolerating
oral fluids. Her parents also report
she has had loose stools in the past
12 hours and recent fussiness.
ED evaluation, including CBC,
comprehensive metabolic panel, se-
rum magnesium assessment, uri-
nalysis, and noncontrast head CT
scan, yields unremarkable results. A
lumbar puncture is performed, and
CSF results are within normal limits
(0 WBCs, 0 red blood cells, protein
of 18 mg/dL, and glucose of 51 mg/ Figure 1. Transthoracic echocardiography shows a large circumferential pericardial
dL). Blood, urine, and stool cultures effusion and a large homogeneous, echogenic intracardiac mass.

index of suspicion
such as hydrazine, procainamide, and

isoniazid.
Cardiac tumors should be consid-
ered in patients who present with a
sudden onset of heart failure and
tamponade. The second most com-
mon presentation of cardiac tumors
involves symptoms related to sys-
temic embolization. Other manifes-
tations include systemic and consti-
tutional symptoms, local invasion
causing arrhythmia, and obstruction
of intracardiac blood flow.
Management
Initial urgent management must be
relief of the life-threatening cardiac
tamponade. The pericardial space
normally contains 25 mL of serous
Figure 2. A cardiac MRI shows a large intra-cardiac mass involving large portions of
fluid, which allows the heart to con-
both ventricles. tract without creating friction. An in-
crease in pericardial fluid in this space
by as little as 100 mL can interfere
potential heart transplantation was atic until the tumors have advanced with filling of the heart. This effect
initiated, but the patient died sud- significantly. results in decreased stroke volume,
denly at home, approximately 1 year This patient presented at a late leading to a decrease in systolic blood
after his initial presentation. stage of his disease. To our knowl- pressure during inspiration (pulsus
edge, there has only been one re- paradoxus), cardiovascular shock, and
ported case of DSRCT occurring death.
The Condition
in the heart. This tumor is extremely Patients who have benign tumors
Cardiac tumors are extremely rare.
rare, is malignant, and usually is found can be cured by resection of the
They are classified into primary be-
in the abdominal cavity of males dur- mass. However, patients who de-
nign or malignant neoplasms or sec-
ing adolescence and early adulthood. velop malignant cardiac tumors usu-
ondary metastatic tumors. In some
ally present late, and resection gener-
autopsy series in children, the preva-
ally is not possible. The prognosis for
lence of primary tumors ranged from Differential Diagnosis
patients who have malignant tumors
0.017% to 0.28%. Only 10% of all Cardiac tamponade often is associ-
is poor. Therefore, treatment usually
primary cardiac tumors that occur in ated with pericarditis. Causes of peri-
is palliative, with radiation therapy
children and young people are ma- carditis include cardiomyopathy;
with or without chemotherapy.
lignant, most commonly arising from collagen vascular disorders such as
the right side of the heart or from systemic lupus erythematosus; hy-
the pericardium. Most primary car- pothyroidism; systemic viral, fungal, Lessons for the Clinician
diac malignant tumors are sarcomas, or bacterial illnesses, including infec-
commonly angiosarcomas and rhab- tions with tuberculosis and human ● Clinicians should consider the di-
domyosarcomas. These tumors can immunodeficiency virus; radiation agnosis of cardiac tumor in a pa-
be found in the endocardium, myo- therapy; and primary and secondary tient who presents with subtle
cardium, or pericardium. malignancies, especially from the signs and symptoms suggestive of
Pericardial effusions caused by breast and lung. Other causes of car- congestive heart failure and possi-
malignant tumors can progress to diac tamponade can include dissect- ble tamponade.
tamponade. Patients who have ma- ing aortic aneurysm; trauma (pene- ● Most intracardiac tumors in chil-
lignant tumors rarely are symptom- trating and rarely blunt); and drugs dren are benign.

index of suspicion
● MRI allows detailed evaluation of a clude aldosterone deficiency due to a activity are increased because resis-
cardiac mass. disruption in the final steps in aldoste- tance to these hormones is high.
● Let a symptomatic patient sit up if rone synthesis and resistance to aldo- Patients born with generalized au-
he or she prefers until you know sterone resulting in salt wasting despite tosomal recessive PHA1 (arPHA1)
why. elevated serum aldosterone concentra- present in the neonatal period and
tions. The latter cause of salt wasting is suffer from life-threatening salt
(Claudeen K. F. Scott, MBBS, West
termed pseudohypoaldosteronism. wasting and hyperkalemia. They ex-
Side Pediatrics (CHS), Fremont, OH;
hibit salt loss from the kidney, colon,
Monvadi B. Srichai, MD, Depart-
The Condition sweat glands, and salivary glands and
ment of Radiology, NYU School of
The infant had normal female geni- require large doses of potassium
Medicine, New York, NY)
talia, and abdominal ultrasonogra- binders and sodium replacements to
phy confirmed normal female repro- sustain life. Inadequate absorption of
ductive organs, kidneys, and adrenal sodium and water leads to excessive
Case 2 Discussion glands. Urine culture was negative, fluid in the lungs and recurrent lung
Due to her significant hyperkalemia, which ruled out pseudohypoaldoste- infections. Homozygous mutations
the infant received IV calcium glu- ronism caused by a urinary tract in- in the subunits of ENaC (␣, ␤, ␦)
conate 10% (10 mg/kg), nebulized fection. Karyotype was 46 XX (nor- have been found to cause arPHA1.
albuterol (0.1 mL/kg in 3 mL normal female). Newborn screening was Autosomal dominant PHA1
mal saline), IV insulin (0.15 units/ negative. Furthermore, confirmatory (adPHA1) has a milder course. A
kg), IV 10% dextrose solution, and testing for 17-OH progesterone, tes- loss-of-function mutation in the min-
IV sodium bicarbonate (2 mmol/ tosterone, dihydrotestosterone, and eralocorticoid receptor causes af-
kg). The repeat potassium measure- androstenedione showed normal con- fected patients to have episodes of
ment 4 hours later dropped to centrations, thus ruling out CAH as salt wasting, hyperkalemia, and met-
6.9 mEq/L (6.9 mmol/L). The in- the cause of her salt wasting. Her abolic acidosis during neonatal life,
fant received a stress dose of hydro- aldosterone concentration was mark- but symptoms often subside in early
cortisone (25 mg IV) and additional edly elevated at 1,054.8 ng/dL childhood, and no treatment is re-
doses of insulin, calcium gluconate, (57,000 pmol/L) (normal, 5.0 to quired after this time. Unlike patients
and salbutamol for the elevated se- 89.8 ng/dL [140 to 2,490 pmol/L]), who have arPHA1, those who have
rum potassium concentrations. CSF as was the renin value at 65 ng/L adPHA1 do not have salt wasting
bacterial and viral studies as well as per second (normal ⬍13.9 ng/L per from sweat or salivary glands.
cultures were negative. second).
Physiologically, aldosterone binds Treatment
Differential Diagnosis the mineralocorticoid receptor to Salt wasting episodes recur fre-
This infant presented in shock and regulate electrolyte excretion and in- quently, and patients require ther-
severe salt wasting, as indicated by travascular volume. Through its ef- apy with IV or dietary salt of up to
the low sodium and high potassium fects on the distal renal tubule and 45 g/day. Patients often require
values. The most common cause of collecting ducts, aldosterone acts to long-term potassium binder therapy
salt wasting in children is congenital increase sodium resorption via the as well as bicarbonate treatment for
adrenal hyperplasia (CAH) due to epithelial sodium channel (ENaC) metabolic acidosis. Lung infections
21-hydroxylase deficiency (90% of to restore intravascular volume. The are treated with antibiotics.
cases), although this complication is electrical gradient created by this Treatment usually is lifelong for
seen less commonly since the initia- process drives protons and potassium patients who have arPHA1, although
tion of newborn screening programs. into the urine, leading to electrolyte there have been case reports of chil-
Two thirds of children born with and pH balance in the serum. dren born with the condition who
CAH have insufficient aldosterone Pseudohypoaldosteronism type 1 subsequently experienced clinical
and present commonly in the first 4 (PHA1) is characterized by renal re- improvement in childhood that re-
postnatal weeks with salt wasting. Fe- sistance to aldosterone and elevated quired ongoing sodium replacement
male infants who have CAH usually serum aldosterone concentrations, but not potassium binder therapy.
have masculinized genitalia caused leading to salt wasting, hyperkale- Treatment for adPHA1 usually ceases
by excess secretion of adrenal andro- mia, and metabolic acidosis. Plasma in childhood once hyponatremia, hy-
gens. Other causes of salt wasting in- aldosterone concentrations and renin perkalemia, and metabolic acidosis

index of suspicion
no longer occur. Because the degree formed to look for the most common ing on CSF and serum antigen assess-
of amelioration depends on the ge- causes of afebrile seizure and in- ment have been used in the research
notype, counseling about prognosis cluded a CT scan of the head to rule setting but are not available routinely
is necessary. out acute bleeding or trauma, a lum- for clinical application, and findings
To manage this patient’s meta- bar puncture to rule out meningitis, a have been negative or inconclusive in
bolic acidosis, hyperkalemia, and hy- chemistry panel to rule out electro- some cases.
ponatremia, dicitrate (3 mmol three lyte or glucose abnormalities, and an Proposed mechanisms include di-
times a day), sodium polystyrene sul- EEG to evaluate for encephalitis or rect viral infection of the CSF and
fonate potassium binder (1 to 1.5 g evidence of an underlying seizure dis- secondary irritation of the CNS from
twice a day), and 3% sodium chloride order. MRI, which is not always in- neurotoxins or an immune response.
(15 mmol/kg per day) were admin- dicated in the evaluation of a first-time Elevated nitric oxide metabolite con-
istered with good effect. The girl seizure, can be helpful for diagnos- centrations have been reported in
had a homozygous mutation in the ing congenital malformations, space- the CSF of patients who have sei-
alpha subunit of the sodium channel occupying lesions, or encephalitis. zures and rotavirus gastroenteritis
ENaC. Mutations in this region re- In this patient, results of all of when compared with patients who
sult in arPHA1, and, thus, she will these studies were normal. A clinical experience typical febrile seizures or
require ongoing treatment with so- suspicion for rotavirus-associated sei- seizures associated with meningitis
dium chloride, potassium binders, zures was raised. Typically, severe or encephalitis. In addition, elevated
and bicarbonate. Parental genetic dehydration and electrolyte distur- CSF carnitine values have been re-
testing is indicated for future preg- bances are responsible for afebrile ported in patients who have rotavirus-
nancies. seizures in children who have gastro- associated seizures.
enteritis. However, this patient had Seizures are associated more
Lessons for the Clinician adequate urine output at the time of clearly with rotavirus infection than
her admission and normal serum with other causes of infectious gas-
● Any child who fails to reach birth-
electrolyte values. Her rotavirus anti- troenteritis. The incidence rate in the
weight at 2 weeks of age and has
gen test did come back positive, United States is difficult to quantify
evidence of poor feeding or vomit-
which confirmed the suspected diag- because most of the literature is
ing should have electrolytes assessed
nosis. However, due to the remote based on case reports from Asia,
to rule out metabolic abnormality.
location of the medical facility, the and clinicians often do not look for
● Any infant found to be wasting salt
result was not available until 2 weeks an association between seizures and
who does not appear to have sepsis
after admission. In hospitals that have mild diarrheal illnesses.
or CAH might have an aldosterone
readily available rotavirus testing, evi- Patients usually are diagnosed
synthesis or resistance abnormality.
dence suggests that extensive evalua- clinically based on a history of gastro-
● PHA1 leads to frequent episodes of
tion may not be necessary for self- enteritis, lack of evidence for another
salt wasting, hyperkalemia, and
limited afebrile seizures in the setting cause of seizures, and a stool test that
metabolic acidosis
of rotavirus-positive gastroenteritis. is positive for rotavirus. In general,
● Treatment may be lifelong and in-
children are afebrile at the time of the
volves high-dose salt therapy, po-
Pathogenesis and seizures and have relatively normal
tassium binders, and bicarbonate.
Clinical Features electrolyte values, although there is
(Dina M. Kulik, MD, Paediatric Although rotavirus-associated sei- significant variation in presentation.
Emergency Medicine, Farid H. Mah- zures in children have been described Seizures can be either focal or gener-
mud, MD, Paediatric Endocrinology, in several case series (primarily from alized and can recur early in the ill-
The Hospital for Sick Children, To- Asian hospitals), the pathogenesis is ness. Most patients have a benign,
ronto, Ontario, Canada) not well understood. Routine CSF self-limited course, although a few
studies, EEG, and brain imaging typ- case reports have described signifi-
ically yield normal findings, although cant encephalopathy.
Case 3 Discussion a few case reports have described ab-
Afebrile seizures in children are more normalities consistent with encepha- Management
suggestive of underlying pathology litis, including enhancement on MRI There is no specific treatment for ro-
than typical febrile seizures. There- and transient EEG changes. Rota- tavirus infection beyond supportive
fore, an extensive evaluation was per- virus polymerase chain reaction test- care. Some patients are started on

index of suspicion
anticonvulsant therapy when seizures ation several months later, she was cines may reduce the incidence of
recur. However, anticonvulsants are still seizure-free and no longer taking afebrile seizures, which can be men-
associated with significant adverse ef- anticonvulsants. tioned when counseling parents
fects. Most patients reported in the about the benefits of that immuniza-
literature have been managed suc- Lessons for the Clinician tion.
cessfully with observation alone and ● Rotavirus testing should be consid-
have no long-term complications. ered for children who experience (Christina Olson, MD, Julia Paz,
This patient was given IV diaze- afebrile seizures and have had re- DO, Pediatrics Department, US Na-
pam in the ED because her seizure cent gastroenteritis-like symptoms. val Hospital Guam, Agana Heights,
had been continuous for more than If rotavirus test results are positive, GU)
30 minutes when she arrived. She an extensive evaluation and anti- The views in this article are those of
also was started on phenytoin after convulsant therapy for seizures that the authors and do not necessarily re-
the third seizure, but if the rotavirus recur in the first few days may be flect the official policy or position of the
test result had been available at that avoidable if the child is doing well Navy, the Department of Defense, or
time, there would have been a higher otherwise. the United States government.
threshold for beginning anticonvul- ● Parents can be reassured that To view Suggested Reading lists
sant therapy. She was discharged on rotavirus-associated seizures are for the cases, visit pedsinreview.
phenytoin with a plan to wean her off likely to be self-limited. aappublications.org and click on
as an outpatient. At follow-up evalu- ● Widespread use of rotavirus vac- Index of Suspicion.
Clarifications
Question #14 in the June 2010 issue follows the article on periorbital and orbital cellulitis,
and the correct answer is said to be A, which is a typographical error. The correct answer
is C: Periorbital cellulitis and ophthalmology consultation and IV antibiotics. We regret
the error.
In the article on urinary tract infections and vesicoureteral reflux in the November 2010
issue (Pediatrics in Review. 2010;31:451– 463), the sentence on page 452 in the first
column that starts, “The negative predictive value . . .” should read as follows: “The
negative predictive value is the proportion of patients who have negative test results and do
not have the disease.” We regret the error.

Index of Suspicion • Case 1: Fatigue, Shortness of Breath, and Chest Pain in an
Adolescent Boy • Case 2: Poor Feeding and Weight Gain, Tachypnea, and
Vomiting in a Neonate • Case 3: Afebrile Seizures After a Bout of Diarrhea in a
2-year-old Girl
Claudeen K. F. Scott, Monvadi B. Srichai, Dina M. Kulik, Farid H. Mahmud,
Christina Olson and Julia Paz
Pediatr. Rev. 2011;32;123-128
DOI: 10.1542/pir.32-3-123


/DC1
Cardiovascular Disorders
http://pedsinreview.aappublications.org/cgi/collection/cardiovas
cular_disorders Infectious Diseases
http://pedsinreview.aappublications.org/cgi/collection/infectious
_diseases Neurologic Disorders
http://pedsinreview.aappublications.org/cgi/collection/neurologi
c_disorders Metabolic Disorders
http://pedsinreview.aappublications.org/cgi/collection/metabolic
_disorders Fluid and Electrolyte Metabolism
http://pedsinreview.aappublications.org/cgi/collection/fluid_elec
trolyte_metabolism Disorders of Blood/Neoplasms
http://pedsinreview.aappublications.org/cgi/collection/disorders_
of_blood_neoplasms Emergency Care
http://pedsinreview.aappublications.org/cgi/collection/emergenc
y_care Critical Care
http://pedsinreview.aappublications.org/cgi/collection/critical_ca
re Endocrine Disorders
http://pedsinreview.aappublications.org/cgi/collection/endocrine
_disorders

Clarifications
Pediatr. Rev. 2011;32;128
DOI: 10.1542/pir.32-3-128

index of suspicion
anticonvulsant therapy when seizures ation several months later, she was cines may reduce the incidence of
recur. However, anticonvulsants are still seizure-free and no longer taking afebrile seizures, which can be men-
associated with significant adverse ef- anticonvulsants. tioned when counseling parents
fects. Most patients reported in the about the benefits of that immuniza-
literature have been managed suc- Lessons for the Clinician tion.
cessfully with observation alone and ● Rotavirus testing should be consid-
have no long-term complications. ered for children who experience (Christina Olson, MD, Julia Paz,
This patient was given IV diaze- afebrile seizures and have had re- DO, Pediatrics Department, US Na-
pam in the ED because her seizure cent gastroenteritis-like symptoms. val Hospital Guam, Agana Heights,
had been continuous for more than If rotavirus test results are positive, GU)
30 minutes when she arrived. She an extensive evaluation and anti- The views in this article are those of
also was started on phenytoin after convulsant therapy for seizures that the authors and do not necessarily re-
the third seizure, but if the rotavirus recur in the first few days may be flect the official policy or position of the
test result had been available at that avoidable if the child is doing well Navy, the Department of Defense, or
time, there would have been a higher otherwise. the United States government.
threshold for beginning anticonvul- ● Parents can be reassured that To view Suggested Reading lists
sant therapy. She was discharged on rotavirus-associated seizures are for the cases, visit pedsinreview.
phenytoin with a plan to wean her off likely to be self-limited. aappublications.org and click on
as an outpatient. At follow-up evalu- ● Widespread use of rotavirus vac- Index of Suspicion.
Clarifications
Question #14 in the June 2010 issue follows the article on periorbital and orbital cellulitis,
and the correct answer is said to be A, which is a typographical error. The correct answer
is C: Periorbital cellulitis and ophthalmology consultation and IV antibiotics. We regret
the error.
In the article on urinary tract infections and vesicoureteral reflux in the November 2010
issue (Pediatrics in Review. 2010;31:451– 463), the sentence on page 452 in the first
column that starts, “The negative predictive value . . .” should read as follows: “The
negative predictive value is the proportion of patients who have negative test results and do
not have the disease.” We regret the error.

Clarifications
DOI: 10.1542/pir.32-3-128

Related Articles A related article has been published:

1

Facilitating Parent-Child Communication About Sexuality
Pediatr. Rev. 2011;32;129-130
DOI: 10.1542/pir.32-3-129

in brief
In Brief
Sara Buchdahl Levine, MD, MPH mote condom use. However, national lute answers to these questions and
Greenwich Adolescent Medicine, LLC surveys of adolescents also have shown many others. Rather, pediatricians can
Greenwich, CT that only about 1 in 10 adolescents help parents realize that many of these
discussed sex with their parents before questions about sexuality have differ-
having sex, and fewer than half have ent answers based on the parents’ own
Author Disclosure ever discussed how to know if they are preferences, values, and beliefs.
Drs Levine and Adam have disclosed ready to have sex. Pediatricians can For young children, parent-child
no financial relationships relevant to promote parent-child communication communication about sexuality is not
this In Brief. This commentary does about sexuality from an early age and particularly “sexual.” Rather, discus-
support parents and teens as these sions may be about how girls and boys
not contain a discussion of an
conversations evolve. have bodies that are much the same,
unapproved/investigative use of a
Although the literature on promot- but also different; about appropriate
commercial product/device. ing parent-child communication about and inappropriate touching; and about
sexuality grows, methods of fostering love and relationships. Pediatricians
healthy sexuality among children and
also can help parents identify “teach-
Sexuality Education for Children and teens remains controversial. The debate
able moments” for age-appropriate dis-
Adolescents. American Academy of goes on about abstinence-only versus
Pediatrics, Committee on Psychoso- cussions of sexuality and reproduction,
comprehensive reproductive education.
cial Aspects of Health and Commit- such as the birth of a sibling or pet.
Children, teens, parents, and pediatri-
tee on Adolescence. Pediatrics. 2001; As children reach school age, con-
cians receive complicated messages
108:498 –502 versations about sexuality begin to
from the media; peers; and profes-
Timing of Parent and Child Communi- change. Both parents and children may
cation About Sexuality Relative to
sional, religious, and lay leaders. Pedi-
find such discussions embarrassing and
Children’s Sexual Behaviors. Beckett atricians can help parents clarify their
more difficult. Pediatricians again need
MK, Elliott MN, Martino S, et al. own values and beliefs, provide medi-
to provide information about normal
Pediatrics. 2010;125:34 – 42 cally accurate information, and identify
community resources to support posi- physical and sexual development and
From Diapers to Dating: A Parent’s
Guide to Raising Sexually Healthy tive communication about sexuality. encourage parents to listen as much
Children—From Infancy to Middle Parents often do not know how to as they talk. Children receive much of
School. Haffner DW. 2nd ed. New start conversations about sexuality, their sexuality “education” from peers,
York, NY: New Market Press; 2004 and pediatricians can help facilitate often with great inaccuracies. Parents
Beyond the “Big Talk”: The Roles of this process. Discussions of sexuality can correct misinformation and rein-
Breadth and Repetition in Parent- force their own family’s values and
should begin long before puberty and
Adolescent Communication about expectations for healthy and responsi-
Sexual Topics. Martino SC, Elliott
adolescence, but parents often wait
until their children are preteens or ble sexual behavior.
MN, Corona R, et al. Pediatrics. 2008;
teens to have the “big talk.” Beginning With adolescents, conversations
121:e612-e618
Advocates for Youth. Parents’ Sex Ed when their patients are young, pedia- about sexuality become more explicit
Center. Multiple resources available tricians should address parents’ con- and detailed. Pediatricians should take
at www.advocatesforyouth.org cerns and provide anticipatory guid- a confidential sexual history from all
ance about normal physical and sexual adolescents while also encouraging
A growing body of research indicates development. Often, pediatricians are teens to talk to their parents. Data have
that early, frequent, and repetitive asked questions such as, “Can my chil- shown that adolescents want informa-
communication about sexuality be- dren [of opposite sex] still bathe to- tion about sex from their parents but
tween parents and children helps to gether?” or “At what age should we often do not get it. Physicians can
prevent early sexual activity, protect [the adults] stop allowing our children provide facts, but parents remain a key
against risky sexual behaviors, and pro- to see us naked?” There are few abso- resource for helping teens make safe

in brief
and responsible decisions that are con- figure out what a child’s question is about sexuality and having those con-
sistent with their values. before answering it. Conversely, provid- versations in the office can promote
One area of particular difficulty for ing too little information or responding healthy development and help to pre-
some families is addressing concerns “We’ll discuss that when you’re older” vent distress and disease.
of gay, lesbian, bisexual, transgendered, can make a child fearful, ashamed, or
or questioning (GLTBQ) youth. Studies simply unlikely to ask questions in the
have shown consistently that this pop- future. Comment: Teenagers in the United
ulation is at increased risk for mental Most parents would agree that States are more likely than their peers
health problems and risky sexual be- eventually they want their children to in other industrialized countries to have
haviors. Parents may find themselves have healthy sexual relationships—just had sexual intercourse by the time they
struggling to accept their child’s sexual not too soon. Parents can communicate are 15 years old, with one third having
identity and provide appropriate guid- with their children about healthy rela- intercourse by the time they have
ance and support. In these cases, it can tionships, personal safety, and their reached ninth grade. They are more
be life-saving for pediatricians to refer own values and expectations before likely to have shorter relationships and
to community services serving GLBTQ the onset of sexual activity. Communi- more partners, putting them at in-
youth and their families. Furthermore, a cating the importance of respect, inti- creased risk for sexually transmitted
nonjudgmental office environment is macy, connection, and love can provide infections and pregnancy. Our country
critical to facilitating care. the foundation for later conversations “boasts” the highest rates in the devel-
At all stages of a child’s develop- about abstinence and the prevention of oped world of adolescent childbearing
ment, pediatricians can help parents infection and unwanted pregnancy. and abortion. What more, really, is
with age-appropriate discussions of Finally, pediatricians should become there to say about the need for com-
sexuality. Parents often are themselves comfortable with discussing sexuality munication at home and for education
so uncomfortable that they may pro- and providing resources for children at in our schools? Pediatricians are in a
vide too much information in response various ages. Many books have been position to advocate for and facilitate
to a child’s question. Sometimes the published aimed at children as young as both.
question, “Where did I come from?” toddlers and through adolescence. Mul-
really is just, “Was I born in a tiple web sites are aimed at parents and
hospital/in New York/in America?” Pe- teens. Empowering and assisting par- Henry M. Adam, MD
diatricians should encourage parents to ents to talk openly with their children Editor, In Brief

http://www.healthychildren.org/English/ages-stages/teen/dating-sex/Pages/How-
to-Talk-About-Sex-With-Your-Teen.aspx

Pediatr. Rev. 2011;32;129-130
DOI: 10.1542/pir.32-3-129

Adolescent Medicine/Gynecology
http://pedsinreview.aappublications.org/cgi/collection/adolescent
_medicine_gynecology Psychosocial Issues and Problems
http://pedsinreview.aappublications.org/cgi/collection/psychosoc
ial_issues_problems Preventive Pediatrics
_pediatrics

Cover Art Contest
DOI: 10.1542/pir.32-3-131

ATTENTION ARTISTS AGES 5 TO 16 YEARS
ART CONTEST FOR COVERS

OF 2012 and 2013
PEDIATRICS IN REVIEW
INSTRUCTIONS TO ARTISTS:
Your drawing should be no smaller than 8.5⬙ ⫻ 11⬙ and no larger than 18⬙ ⫻ 24⬙.
Remember, your drawing will be reduced to 5.5⬙ ⫻ 6⬙ on the cover of the journal, so try to
make your drawing fit the space (vertical, not horizontal). Be sure to make your drawing
colorful. Draw about any of the following:
● Your favorite thing to do

● Your dream of what you would like to happen someday
● What you would like to be doing when you grow up
● Etc, etc!
ENTRY INFORMATION (Please fill in ALL information):
Name:
Address:
City/State/Zip:
Telephone Number:
My Parents’/Legal Guardians’ Email Address (for notification of selected artwork):
My Pediatrician is:
SUBMIT ARTWORK AND THIS COMPLETED FORM TO:
Kathleen Bernard
Editorial Assistant, Pediatrics in Review
American Academy of Pediatrics
Division of Scholarly Journals and Professional Periodicals
141 Northwest Point Blvd.
Elk Grove Village, IL 60007
All entries must be received by December 30, 2012.

Note: All submitted artwork becomes the property of the American Academy of Pediatrics, and
all selected artists will be notified.
CLINICIANS: Please post this notice in your office for your patients!

Cover Art Contest
DOI: 10.1542/pir.32-3-131


Ethics for the Pediatrician: Providing Culturally Effective Health Care
Alissa Hurwitz Swota and D. Micah Hester
Pediatr. Rev. 2011;32;e39-e43
DOI: 10.1542/pir.32-3-e39
http://pedsinreview.aappublications.org/cgi/content/full/32/3/e39

ethics for the pediatrician
Providing Culturally Effective

Health Care
Alissa Hurwitz Swota, PhD,* D. Micah Hester, PhD†
Introduction into the values and interests that af-

There is no strong consensus on fect decision-making for a specific
what is meant by the concept of patient. In light of this perspective,
“culture.” Anthropologists and be- “cultural competency” and “sensi-
havioral scientists note that it is “an tivity” have become common buzz-
abstraction . . . , a generalization ex- words in society in general and in
trapolated from the specific material medicine specifically. (4) Despite the
products, interactions, or ideations. content and implications of some of
. . . We do not, in fact, observe cul- the existing training material in this
ture; we observe phenomena that area, “competence” and “sensitivity”
Author Disclosure we choose to group together and to cultural aspects of individual lives
Drs Hurwitz Swota and Hester have label culture.” (1) As applied to the do not require a comprehensive un-
disclosed no financial relationships health-care setting, “Culture defines derstanding of selected “cultural”
relevant to this article. This how health care information is re- practices and values. It is not simply
ceived, how rights and protections because a practitioner has memo-
commentary does not contain a
are exercised, what is considered to rized a large quantity of information
discussion of an unapproved/
be a health problem, how symptoms about the cultural practices of various
investigative use of a commercial and concerns about the problem are groups that he or she becomes cul-
product/device. expressed, who should provide treat- turally sensitive. Rather, what should
ment for the problem, and what type be sought is a careful recognition of
of treatment should be given.” (2) and willingness to attend to specific
(3) In short, culture is the lens culturally influenced attitudes and
through which one views the world, interests as they are held and expressed
affecting everything that an individ- by individual patients and families.
ual perceives and does, often at an That is, culturally effective health care
unconscious level. Although com- “take[s] into account the beliefs,
mon use often treats the term “cul- values, actions, customs, and unique
ture” as equivalent to “race,” “eth- health care needs of distinct popula-
nicity,” or “nationality,” in the more tion groups” but does so to “assess
rigorous sense (and the sense in how [if at all] the belief and behaviors
which it is used in this article), this of . . . cultural group[s] affect the
common use is overly constricted. patient and family.” (4)
Although looking at issues in “His- Central to providing culturally
panic” culture or “American” culture effective health care is developing
may be instructive, it is equally rea- and maintaining good communica-
sonable to discuss “gay and lesbian” tion skills. Communication between
culture or “Protestant” culture. practitioners and patients and fami-
In patient/family-centered care, lies, however, is not without diffi-
it is important to understand how culties. The power differential be-
family, community, and culture play tween the physician and patient/
family creates an additional obstacle
*Department of Philosophy, University of North in communication, especially for dis-
Florida; Wolfson Children’s Hospital, Jacksonville, FL. empowered persons dealing with
†
Division of Medical Humanities/Pediatrics,
University of Arkansas for Medical Sciences; “experts.” Explaining complex med-
Arkansas Children’s Hospital, Little Rock, AR. ical issues to people who often are
Pediatrics in Review Vol.32 No.3 March 2011 e39

not well-versed in medical terminol- often relies on the boy to interpret, patient and family and how the
ogy can be laborious. Further, “cul- rarely speaks directly to the parents, family sees or encounters the world
tural variations in verbal and nonver- and gives most spoken information ● Developing and providing options
bal communication can be a major and direction to the boy. He also relies in light of those values (not neces-
barrier to effective pediatric care.” on visual aids, hand gestures, and his sarily “in agreement with”)
(5) When different languages are interpretation of the parents’ body ● Dealing professionally with dis-
spoken, these barriers become more language. agreements or conflicts (eg, by re-
pronounced. The latest visit was precipitated questing an ethics consultation or
One population consistently re- by continued erratic blood glucose by bringing in a cultural broker)
porting lower subjective health-care readings that were confusing and con-
status is deaf and hard-of-hearing cerning. The physician also is confused Participation: More Than
persons (for the case presented, we because he has spent several visits re- Just Consent
focus on deaf parents who use Amer- viewing how to care for an insulin- Since the 1970s, concern about re-
ican Sign Language [ASL] as a first dependent child and what to do with specting patient “autonomy” has been
language). (5)(6) The following case various specific blood glucose values, paramount, and the practical expres-
highlights the triadic relationship in- going so far as to send home carefully sion of this respect is the need to
herent in pediatric health care among written and comprehensive directions. obtain “informed consent.” (7) As
patient, clinician, and parents; the important and useful as these ethical
difficulties that arise when barriers to Discussion obligations are, they have several
communication are present; and the This case raises many practical and shortcomings. For example, gaining
importance of considering the lan- ethical concerns, but our focus is on a patient’s or family’s consent does
guage of the patient and family to how cultural issues have a direct im- not guarantee that an ethically ap-
provide the best health care. Specifi- pact on patient care. We have purpose- propriate process has occurred. Even
cally, this case draws out some of fully chosen to address culture through more importantly in pediatrics, the
the many complexities that arise in the case of a child who has deaf par- concept of “patient autonomy” is
caring for patients when their paren- ents (acting as primary decision- difficult to apply because many (if
tal decision-makers are deaf. makers) to highlight both specific con- not most) minors are developmen-
siderations when dealing with deaf tally incapable of acting autono-
Case decision-makers and general concerns mously. Thus, morally and legally,
FB is a 10-year-old boy who has type 1 about what it means to demonstrate parents are granted the authority to
diabetes. His primary care physician appropriate cultural sensitivity to pro- make decisions for their children.
has been seeing the boy for the past year vide optimal patient care. The focus on Such authority, in fact, is not an ex-
to help regulate his blood glucose. Be- and importance of cultural sensitivity is ercise of “autonomy” because par-
cause FB is dependent on insulin, his driven by some of the central precepts ents are making decisions not for
parents are closely involved in his care, of current medical practice, particularly themselves but for their children,
but regulating his diabetes has proven patient/family-centered care. Patient/ who themselves are presumed not to
difficult. His parents regularly bring family-centered care is now a well- have autonomy. (8)(9)
him in when his numbers are askew, established tenet of medical ethics. Thus, reliance on “autonomy”
but his physician has never been com- Such “centering” demands that fami- alone to ground the ethical basis of
fortable communicating with the par- lies (patients) be allowed to participate patient- and family-centered decision-
ents, who are both deaf. The boy is nei- fully in, and even take the lead on, making seems doomed to failure. In-
ther deaf nor hard-of-hearing. decision-making (at least as far as ap- stead, a full sense of the patient’s life
The parents were both born deaf propriate). To allow for full partici- story, which includes some under-
and met in a high school for deaf chil- pation, physicians must demonstrate standing of the familial, communal,
dren. They can read lips but are more general sensitivity and more specifically and cultural influences on that story,
comfortable with and prefer ASL. The cultural sensitivity by: is necessary to promote adequate
physician has struggled in communi- participation. (10) In the case of
cating with them. His is a fairly new ● Creating opportunities for the pa- pediatrics, parents necessarily shape
private practice, and for expediency tient and family to be heard and much of that story. (11) Therefore,
and to save costs, he has only used an understood on their terms helping parents (and patients, to the
ASL interpreter on a few occasions. He ● Learning about the values of the extent possible) to become invested
e40 Pediatrics in Review Vol.32 No.3 March 2011

in the decision-making process not bers of the deaf population, often is realized that, among other tools such
only demonstrates respect but helps unknown or overlooked by health- as written handouts and good Inter-
to garner robust informed consent. care practitioners. (15) Deaf patients net sites, engaging a trained inter-
In the case presented, it clearly is who use ASL (some deaf or hard-of- preter would be the best approach to
not enough to rely on the boy to hearing patients may read lips or use facilitating good communication.
translate for his parents. Such reli- sign-to-English or some other mode
ance compromises his medical care of communication in lieu of ASL) “are Use of Interpreters
by making a 10-year-old boy the key the nonEnglish speaking minority at Although cost-effective and expedi-
communicator in his own care for a greatest risk for physician-patient mis- ent, the use of children as interpreters
complex medical condition. Even if communication.” (6) between physician and parents is a
unwittingly, poor communication or For example, “ASL communica- dubious practice at best. (3)(16) Chil-
use of resources can deny parents tion starts with the main point and dren often lack not only the vocabu-
the opportunity to participate in then winds down,” contrary to En- lary but the experience necessary to
their child’s medical care. One nega- glish communication, which “works convey both the basic information
tive effect of such actions could be its way up to the main point and then and substantive meaning of that in-
a less active role for parents in the concludes.” (6) One result of this formation within the context of the
care and treatment of their child. difference may be that “physicians medical encounter. Further, a child
This effect is especially problematic in may believe that communications is caught between two sets of author-
cases of children who have chronic are finished when deaf patients are ity figures: parental and medical.
conditions in which the care plans can still ‘winding down’ the conversa- These relationships may cause the
be complicated and necessarily regi- tion. When physicians give treatment child to shape the discussion in ways
mented and necessitate family involve- recommendations to deaf patients, that he or she considers most appro-
ment and support (eg, in administer- the patient may ask the same ques- priate to each authority. All of these
ing medications, dosing, influencing tions multiple times.” (6) If the dif- factors can lead to significant mis-
diet and nutrition, encouraging exer- ferences in communication style are communication that, in turn, can
cise, and monitoring of the condi- not identified and explored, deaf pa- have negative effects on the child’s
tion). (12)(13) Further, poor commu- tients may hold a paucity of infor- health care. Further, according to a
nication can lead to a lack of comfort mation, leading to a decision that is recommendation from the United
on both sides, with physicians not en- not fully informed. At the same time, States Department of Health and
gaging with parents and parents de- practitioners would be under the Human Services report entitled “Na-
laying or even avoiding bringing their mistaken impression that they have tional Standards for Culturally and
child to the physician because of answered all the questions of the pa- Linguistically Appropriate Services
frustration and increased anxiety over tients and families. Deaf patients may in Health Care,” “Minor children
the miscommunications affecting their feel as if they are being dismissed or should never be used as interpreters,
child’s health. This physician’s reti- slighted in some way if physicians nor be allowed to interpret for their
cence to engage the parents in discus- try to end the conversation, believing parents when they are the patients/
sion, coupled with the limited context that patients have finished asking consumers.” (16)
that a 10-year-old boy can provide, questions, when, in fact, questions Trained interpreters are skilled
results in the boy not only not receiv- still remain unanswered and unasked. not only at conveying the meanings
ing culturally effective care but actually Presumptions may run deep about of the words, but doing so at levels
receiving substandard health care. (14) how communication should occur, that patients and families can grasp.
both in form and content, and such Further, they help provide some con-
Communicating With Deaf presumptions may interfere with text, such as the conviction and force
Patients and Surrogates necessary sensitivity. The physician in behind the messages being sent. (17)
For those who rely on ASL, English the case presentation may be entirely Interpreters play a significant and
should not necessarily be considered unaware that ASL is not the same as useful role in connecting different
their “first” language. In fact, both the spoken English he attempts to cultures through the medium of lan-
the grammar and syntax of ASL is use through speaking, gestures, body guage. As a result, they have a signif-
different from that of written and language, and even the boy’s inter- icant impact on creating a participa-
spoken English. This important fact, preting. Had he been aware of the tory process for patients and families
central to communicating with mem- language differences, he might have within the health-care setting.

Importance of Inclusion that enables family members to par- the clinician an opportunity to gain
Essential in creating an environment ticipate fully in caring for their loved access to such personal and impor-
in which patients and families can one. tant information.
participate in the decision-making Although we encourage all physi- Another, more simplified, varia-
process is establishing a relationship cians to learn what they can about tion of this principle is what has been
based on trust. In attempting to fos- various characteristics and traditions called the LEARN model offered by
ter trust, practitioners need to help of the many cultures that they may Berlin and Fowkes: (21)
patients and families feel comfortable encounter (eg, learning how ASL dif-
enough to engage in a dialogue and
● Listen with sympathy and empathy
fers from written and spoken English
share information. One approach is to the patient’s perception of the
or how some religious tenets affect
being careful not to prejudge as fool- problem
end-of-life decisions), such an educa-
ish or ill-conceived specific cultural
● Explain your perception of the
tional endeavor will not allow the cli-
practices and beliefs about health problem
nician to connect well with all patients
care. Specifically, practitioners must
● Acknowledge and discuss differ-
and families. Instead (or at least in tan- ences and similarities
solicit information and listen care- dem with other tools), we recommend
fully, with an open mind, to the re-
● Recommend treatment
using some variation of Arthur Klein- ● Negotiate agreement
sponses. Whether the information man’s questions for eliciting “explana-
gleaned from these encounters is tory models”: (18)(19)(20) Of course, none of this effort will
about traditional foods or beverages succeed if the practitioner cannot
that are being given to the patient, ● What do you call your problem? find an effective method of commu-
herbal remedies or salves, or end-of- nication, either through a commonly
What name does it have?
life rituals, such information, and the shared language or through inter-
● What do you think has caused your
fact that patients and families are will- preters trained to connect health-
problem? (cause)
ing to share it, can prove to be invalu- care professionals with patients and
● Why do you think it started when it
able. For example, a perinatal nurse families and overcome language bar-
did? (time of onset)
case manager describes a practice riers. To decrease the potential for
● What does your sickness do to
common in Hispanic culture in conflict, it is imperative to identify a
you? How does it work? (patho-
which a baby who has a sunken fon- successful means of communication
physiology)
tanelle is held upside down by the at the outset of practitioner/patient/
● How severe is it? Will it have a short
feet to “fill” the hole. (3) Instead of family relationships.
or long course?
deeming such a practice foolish and
scoffing at it, clinicians should let the
● What do you fear most about your
families know that a more effective sickness?
● What are the chief problems your References
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● What are the most important re- Linguistically Competent Health Promotion
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Care Planning. Lanham, MD: Lexington
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e42 Pediatrics in Review Vol.32 No.3 March 2011

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Ethics for the Pediatrician: Providing Culturally Effective Health Care
Alissa Hurwitz Swota and D. Micah Hester
Pediatr. Rev. 2011;32;e39-e43
DOI: 10.1542/pir.32-3-e39

& Services http://pedsinreview.aappublications.org/cgi/content/full/32/3/e39

http://pedsinreview.aappublications.org/cgi/content/full/32/3/e39
/DC1
Psychosocial Issues and Problems
http://pedsinreview.aappublications.org/cgi/collection/psychosoc
ial_issues_problems Ethics for the Primary Care Pediatrician
http://pedsinreview.aappublications.org/cgi/collection/ethics_pe
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EgyptianPediatrics

Cover: The artwork on the cover of this CME Statements:

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Diagnosis and Management of

1. Discuss the wide prevalence of sleep problems in children.

Sleep in Infancy, Childhood, and Adolescence

Approach to Pediatric Sleep Disorders

Pediatrics in Review Vol.32 No.3 March 2011 91

SLEEP-ONSET ASSOCIATION TYPE. This disorder pre-

Table 2. BEARS Screening Tool

92 Pediatrics in Review Vol.32 No.3 March 2011

and other sleep-disrupting events. In toddlers, separation

Pediatrics in Review Vol.32 No.3 March 2011 93

toddler does not respond to parental requests to get

94 Pediatrics in Review Vol.32 No.3 March 2011

be associated with depression, anxiety, school refusal,

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Pediatrics in Review Vol.32 No.3 March 2011 97

2. Owens JA. The practice of pediatric sleep medicine: results of a

HealthyChildren.org Parent Resources from AAP

98 Pediatrics in Review Vol.32 No.3 March 2011

1. The R in the BEARS screening tool explores:

Pediatrics in Review Vol.32 No.3 March 2011 99

Updated Information including high-resolution figures, can be found at:

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Failure to Thrive: Current Clinical Concepts

1. Characterize the limitations of the classic dichotomy of “nonorganic” versus “organic”

100 Pediatrics in Review Vol.32 No.3 March 2011

Pediatrics in Review Vol.32 No.3 March 2011 101

102 Pediatrics in Review Vol.32 No.3 March 2011

that prevents foodstuffs from being available for growth.

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106 Pediatrics in Review Vol.32 No.3 March 2011

References ing in infancy and IQ levels at 8 years in the Avon Longitudinal

HealthyChildren.org Parent Resources from AAP

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108 Pediatrics in Review Vol.32 No.3 March 2011

Updated Information including high-resolution figures, can be found at:

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Pediatrics in Review Vol.32 No.3 March 2011 109

110 Pediatrics in Review Vol.32 No.3 March 2011

Figure 3. A duplicated gluteal cleft associated with occult

pediatrician must perform a careful physical examination,

Solitary sacrococcygeal pits located entirely within the

Pediatrics in Review Vol.32 No.3 March 2011 111

Figure 5. Solitary sacrococcygeal dimple that demonstrates

OSD. Ultrasonography of the lumbosacral spine gener-

112 Pediatrics in Review Vol.32 No.3 March 2011

aging and neurosurgical referral. (11) Optimal outcome

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Updated Information including high-resolution figures, can be found at:

Supplementary Material Supplementary material can be found at:

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Pediatrics in Review Vol.32 No.4 April 2011 151

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Venereal Warts in Children