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JAN/FEB 2012 Vol. 38 No. 1
JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY
Your partner in paediatric
and O&G practice
JUL/AUG 2013 Vol. 39 No. 4
Your partner in paediatric
and O&G practice
CME ARTI CLE
5
S
K
P
Management of
Pregnancies With Previous
Caesarean Section
JOURNAL WATCH
PAEDI ATRI CS
Management of Hearing
Loss in Children
Constipation in Infants
and Children
GYNAECOLOGY
Ovarian Cancer:
Current Management
and Future Directions
OBSTETRI CS
Management of
Early Pregnancy
Complications

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JOURNAL OF PAEDIATRICS, OBSTETRICS & GYNAECOLOGY
Journal Watch
133 Calcium intake and mortality in Swedish women
Outcome of extreme preterm birth in England, 19952006
134 New anti-interleukin therapies for systemic JIA
CPAP vs surfactant and higher vs lower oxygen saturation for
extremely preterm infants: Outcomes at 1822 months
135 Paromomycin for cutaneous leishmaniasis
HMPV infection in young children in the US
136 Risk factors for stillbirth
Fetal macrosomia in developing countries

Board Director, Paediatrics
Professor Pik-To Cheung
Associate Professor
Department of Paediatrics
and Adolescent Medicine
The University of Hong Kong
Board Director, Obstetrics and Gynaecology
Professor Pak-Chung Ho
Head, Department of
Obstetrics and Gynaecology
The University of Hong Kong
Editorial Board
Professor Biran Affandi
University of Indonesia
Dr Karen Kar-Loen Chan
The University of Hong Kong
Professor Oh Moh Chay
KK Womens and Childrens Hospital,
Singapore
Associate Professor Anette Jacobsen
KK Womens and Childrens Hospital, Singapore
Professor Rahman Jamal
Universiti Kebangsaan Malaysia
Dato Dr Ravindran Jegasothy
Hospital Kuala Lumpur, Malaysia
Associate Professor Kenneth Kwek
KK Womens and Childrens Hospital, Singapore
Dr Siu-Keung Lam
Prestige Medical Centre, Hong Kong
Professor Terence Lao
Chinese University of Hong Kong
Dr Kwok-Yin Leung
The University of Hong Kong
Dr Tak-Yeung Leung
Chinese University of Hong Kong
Professor Tzou-Yien Lin
Chang Gung University, Taiwan
Professor Somsak Lolekha
Ramathibodi Hospital, Thailand
Professor Lucy Chai-See Lum
University of Malaya, Malaysia
Professor SC Ng
National University of Singapore
Professor Hextan Yuen-Sheung Ngan
The University of Hong Kong
Professor Carmencita D Padilla
University of the Philippines Manila
Professor Seng-Hock Quak
National University of Singapore
Dr Tatang Kustiman Samsi
University of Tarumanagara, Indonesia
Professor Alex Sia
KK Womens and Childrens Hospital, Singapore
Dr Raman Subramaniam
Fetal Medicine and Gynaecology Centre, Malaysia
Professor Walfrido W Sumpaico
MCU-FDT Medical Foundation, Philippines
Professor Cheng Lim Tan
KK Womens and Childrens Hospital, Singapore
Professor Kok Hian Tan
KK Womens and Childrens Hospital, Singapore
Professor Surasak Taneepanichskul
Chulalongkorn University, Thailand
Professor Eng-Hseon Tay
Thomson Women Cancer Centre, Singapore
Professor PC Wong
National University of Singapore
Adjunct Professor George SH Yeo
KK Womens and Childrens Hospital, Singapore
Professor Hui-Kim Yap
National University of Singapore
Professor Tsu-Fuh Yeh
China Medical University, Taiwan
133
136
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Review Article
Paediatrics
137 The Management of Hearing Loss in Children
Universal neonatal hearing screening aims to detect the 1 in 1,000 babies born in the UK with a permanent
hearing loss detectable at birth. However, children may present later to the paediatrician with hearing
difficulties. This article aims to discuss the clinical assessment of hearing and provides an overview of the
management options available in the treatment of hearing loss.
Marianne D Elloy, Andrew H Marshall
Review Article
Obstetrics
146 Management of Early Pregnancy Complications
Complications of early pregnancy are common, including pregnancy loss, threatened miscarriage,
ectopic pregnancy, molar pregnancy and hyperemesis. This review discusses the different presentations,
diagnoses and management of the common problems complicating early pregnancy.
Harriet Pugsley, Judith Moore
146
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Review Articles
Comprehensive reviews
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Review Article
Gynaecology
155 Ovarian Cancer: Current Management and Future Directions
Ovarian cancer has the highest mortality of all the gynaecological malignancies. Treatment of advanced
epithelial ovarian cancer usually involves debulking surgery and chemotherapy. Treatment may prolong life
and palliate symptoms but it is rarely curative. New treatments are constantly being developed and offer
the hope of improved outcomes.
Sin E Taylor, John M Kirwan
Review Article
Paediatrics
164 Constipation in Infants and Children
Constipation is a common problem in children and is usually functional, related to stool-withholding.
Successful management requires parent education, behavioural strategies, laxative agents (often long
term) and ongoing review.
Taya Dowling, Scott Nightingale
Continuing Medical Education
169 Management of Pregnancies With Previous Caesarean Section
This article reviews the recommendations on vaginal birth after previous caesarean section (VBAC) by
different professional societies, the favourable and unfavourable factors in considering VBAC, the associated
maternal and fetal benefits and risks, as well as the non-medical concerns that play a role in the decision-
making process.
Yung WK, Lau WL, Leung WC
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Early Pregnancy Complications
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169
Indonesia
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Journal Watch
JPOG JUL/AUG 2013 133
PEER REVIEWED
GYNAECOLOGY
Calcium intake and mortality in
Swedish women
Meta-analyses of randomized studies have shown
that taking calcium supplements is associated with
increased risk of coronary disease and stroke. A
Swedish cohort study has confirmed the increased
risk for cardiovascular disease in general but not
for stroke.
The Swedish mammography cohort was set
up in 1987 and included 61,433 women born be-
tween 1914 and 1948. National registries provided
data about all-cause and cardiovascular mortality
over a mean follow-up of 19 years. Food frequency
questionnaires in 1987 and 1997 provided data
about dietary intake and use of calcium supple-
ments for 38,984 women. The relationship between
calcium intake and all-cause mortality took the
form of a J-shaped curve, with higher mortality
at both extremes of intake. An intake of 1,400 mg
a day of calcium was associated with significant
increases of 40% in all-cause mortality, 49% in
cardiovascular mortality, and 114% in coronary
disease mortality, and no significant change in
stroke mortality, compared with a calcium intake of
6001,000 mg a day. After further statistical analy-
sis, low intakes of calcium (< 600 mg/day) were
no longer significantly associated with increased
mortality. Among people taking calcium tablets and
with a dietary calcium intake of > 1,400 mg/day,
all-cause mortality was increased 2.6-fold.
High-calcium intake is associated with in-
creased all-cause and cardiovascular mortality.
Michalson K et al. Long-term calcium intake and rate of all cause and
cardiovascular mortality: community-based prospective longitudinal
cohort study. BMJ 2013; 346: 14 (f228).

Outcome of extreme preterm
birth in England, 19952006
Two successive papers in the BMJ have examined
short-term and long-term outcomes for extremely
preterm births in England in 1995 and 2006.
The prospective national cohort studies pro-
vided short-term data about 666 babies born at 22
25 weeks gestation in England in March to Decem-
ber 1995 and all 3,133 babies born at 2226 weeks
gestation in 2006. In 2006, 56% of infants born at
22 weeks and 98% of those born at 26 weeks were
born alive. Active care at birth was withheld from
73% of infants born at 22 weeks, 16% at 23 weeks,
and < 2% at 24 weeks or later. Survival rates for
live-born infants were 2% at 22 weeks, 19% at 23
weeks, 40% at 24 weeks, 66% at 25 weeks, and
77% at 26 weeks. More than two-thirds (68%) of
survivors had bronchopulmonary dysplasia, 16%
were treated (laser treatment) for retinopathy of
prematurity, and 13% had a serious abnormality on
cerebral ultrasound. In 2006, compared with 1995,
44% more infants born alive at 2225 weeks were
admitted to neonatal intensive care, and survival
of infants born at 23, 24, and 25 weeks increased
by 9.5%, 12%, and 16%, respectively. Overall, the
proportion treated for retinopathy increased from
12% in 1995 to 22% in 2006.
Neurodevelopmental outcomes at ages 23
years were assessed for 1,031 survivors in the 2006
cohort. The prevalence of moderate or severe im-
pairment was 45% among survivors born at 2223
weeks, 30% at 24 weeks, 25% at 25 weeks, and
20% at 26 weeks. Overall, one in seven (14%) had
cerebral palsy, usually mild or moderate. Mean
predicted adjusted mental development index quo-
tients (Bayley scales) were 80 (2223 weeks), 87
(24 weeks), 88 (25 weeks), and 91 (26 weeks). In
the 2006 cohort, a greater absolute number of chil-
dren than in 1995 will need lifelong special care. It
is calculated that of every 100 infants born at 24
weeks, 60 will die despite intensive care and 12
of the 40 survivors will have serious impairments.
Further follow-up of the 2006 cohort is planned.
Between 1995 and 2006 the survival of ex-
tremely preterm infants in England improved, but
PAEDIATRICS
JPOG_JulAug_2013_COMBINE_Final.indd 133 8/5/13 2:18 PM
JPOG JUL/AUG 2013 134
more survived with disability. The writers of an
editorial point out that in the Netherlands, infants
born before 24 completed weeks are not routinely
offered neonatal intensive care.
Costeloe KL et al. Short term outcomes after extreme preterm birth
in England: comparison of two birth cohorts in 1995 and 2006 (the
EPICure studies). BMJ 2012; 345: 14 (e7976); Moore T. Neurological and
developmental outcome in extremely preterm children born in England
in 2006 and 1995: the EPICure studies. Ibid: 15 (e7961), Groenendaal F,
Uiterwaal C. Long-term follow-up of extremely preterm neonates. Ibid:10
(e8252) (editorial).

New anti-interleukin therapies
for systemic JIA
Systemic juvenile idiopathic arthritis (systemic JIA)
frequently leads to joint damage and disability and
is accompanied by systemic features such as fever,
rash, hepatosplenomegaly, and serositis. High-
dose steroid treatment may lead to severe toxicity,
and treatments such as methotrexate and tumour
necrosis factor inhibitors may be ineffective. The
effectiveness of antibodies to the interleukin-6
receptor and to interleukin-1 has been shown in
successive papers in the New England Journal of
Medicine.
The humanized, antihuman interleukin-6-re-
ceptor monoclonal antibody, tocilizumab, was as-
sessed in a placebo-controlled trial at 43 centres
in Europe North America, and South America. The
trial included 112 children aged 217 years with
active, treatment-resistant systemic JIA. Random-
ization (2:1) was to intravenous tocilizumab or
placebo every 2 weeks for 12 weeks. At 12 weeks,
an improvement of at least 30% on the American
College of Rheumatology JIA score (JIA ACR 30)
was achieved by 85% (tocilizumab) vs 24% (pla-
cebo), a highly significant difference. At week 52
a JIA ACR 70 response (at least 70% improvement)
was achieved by 80% in the tocilizumab group and
a JIA ACR 90 response by 59%. Steroid therapy had
been stopped by 52% in this group, and 48% had no
active arthritis. Common adverse events with tocili-
zumab included infections, neutropenia, and raised
aminotransferase levels.
The fully human, anti-interleukin-1 mono-
clonal antibody, canakinumab, was assessed in two
international trials reported together, including 84
and 100 patients. In the first trial, randomization
was to subcutaneous canakinumab or placebo, and
an adapted JIA ACR 30 response was achieved by
84% (canakinumab) vs 10% (placebo). In the sec-
ond trial, 100 patients who had responded to 32
weeks of canakinumab were randomized to con-
tinued canakinumab or to placebo. A disease flare
occurred in 74% (canakinumab) vs 25% (placebo).
The median time to disease flare was incalculable
in the canakinumab group and 236 days in the pla-
cebo group. The disease became inactive in 62%
vs 34%. One in three patients on canakinumab was
able to discontinue steroid therapy. Infections were
frequent with canakinumab and five patients (ver-
sus two in the placebo group) developed the mac-
rophage activation syndrome.
Both tocilizumab and canakinumab were ef-
fective treatment for systemic JIA but more data
are needed about toxicity.
De Benedetti F et al. Randomized trial of tocilizumab in systemic juvenile
idiopathic arthritis. NEJM 2012; 367: 238595; Ruperto N et al. Two
randomized trials of canakinumab in systemic juvenile idiopathic arthritis.
Ibid: 2396406; Sandborg C, Mellins ED. A new era in the treatment of
systemic juvenile idiopathic arthritis. Ibid: 243940 (editorial).

CPAP vs surfactant and higher
vs lower oxygen saturation for
extremely preterm infants:
Outcomes at 1822 months
The Surfactant, Positive Pressure, and Pulse Oxim-
etry randomized trial was a multicentre, random-
ized, controlled trial with a 2 2 multifactorial
design in which 1,316 extremely preterm infants
(born at 24 weeks 0 days to 27 weeks 6 days) were
randomized at 20 US centres to early continuous
positive airway pressure (CPAP), or early surfactant
via an endotracheal tube and to a target oxygen
saturation of 8589% or of 9195%. Early assess-
ment (at 36 weeks postmenstrual age) showed
similar rates of death or bronchopulmonary dyspla-
sia with either CPAP or surfactant, and the lower
target range for oxygen saturation was associ-
ated with less retinopathy of prematurity but more
JPOG_JulAug_2013_COMBINE_Final.indd 134 8/5/13 2:18 PM
Journal Watch
JPOG JUL/AUG 2013 135
PEER REVIEWED PEER REVIEWED
deaths. Now, surviving infants have been assessed
at 1822 months.
Neurodevelopmental status was assessed
at 1822 months in 990 of 1,058 surviving infants
(94%). Death or neurodevelopmental impairment
occurred in 27.9% (CPAP) vs 29.9% (surfactant),
a non-significant difference, and in 30.2% (lower
oxygen saturation target) vs 27.5% (higher oxygen
saturation target), also a non-significant differ-
ence. There was a significant increase in mortality
with the lower oxygen saturation target (22.1% vs
18.2%).
These researchers conclude that outcomes
are similar with early CPAP and with early surfac-
tant, but the lower oxygen saturation target should
not be used in the care of extremely premature
babies.
Vaucher YE et al. Neurodevelopmental outcomes in the early CPAP and
pulse oximetry trial. NEJM 2012; 367: 2495504.

Paromomycin for cutaneous
leishmaniasis
York. It included inpatients and outpatients in No-
vember to May each year between 2003 and 2009
with children presenting with an acute respiratory
illness or fever. The rate of HMPV detection (using
reverse transcriptasepolymerase chain reaction
assay on nose and throat swabs) was 200/3,490
(6%) among children in hospital, 222/3,257 (7%)
among children in outpatient clinics, 224/3,001
(7%) among children in the emergency department,
and 10/770 (1%) among healthy children in well-
child primary care clinics. Rates of hospital admis-
sion with HMPV infection among children aged < 5
years were 1 in 1,000 (3 in 1,000 at age < 6 months,
and 2 in 1,000 at age 611 months). Among chil-
dren admitted to hospital with an acute respiratory
illness or fever, those with HMPV infection were
older, more likely to be diagnosed as pneumonia or
asthma, to need supplemental oxygen, and to stay
longer in intensive care, compared with children
testing negative for HMPV. It was estimated that
among 1,000 children of this age, HMPV would,
each year, cause 55 clinic visits and 13 visits to the
emergency department. Among children admitted
to hospital, coexisting high-risk conditions (pre-
Leishmaniasis is prevalent in Eurasia, Africa, and
the Americas, and although cutaneous leishmani-
asis eventually resolves without treatment it is the
cause of much morbidity. Cutaneous leishmaniasis
due to Leishmania major is prevalent in Tunis, and
a trial there has shown topical paromomycin to be
effective treatment.
A total of 375 patients aged 565 years (half
of them children < 17 years old) were randomized
to three groups: 15% paromomycin, 15% paromo-
mycin plus 0.5% gentamicin, or vehicle alone (pla-
cebo), all applied as topical creams for 20 days to
all ulcerated skin lesions including an index lesion
(15 cm diameter with leishmania demonstrated).
Cure of the index lesion was achieved in 82% (par-
omomycin), 81% (paromomycin/gentamicin), and
58% (placebo). Only seven patients (five in the pla-
cebo group) had any persisting lesions after cure of
the index lesion. Mild to moderate local reactions
occurred with paromomycin.
Topical treatment with paromomycin cream,
with or without gentamicin, was effective treat-
ment for cutaneous leishmaniasis due to L major.
Salah AB et al. Topical paromomycin with or without gentamicin for
cutaneous leishmaniasis. NEJM 2012; 368: 52432.

HMPV infection in young children
in the US
Human metapneumovirus (HMPV) is a paramyxovi-
rus discovered in 2001 as a cause of acute respi-
ratory illness in infants and young children world-
wide. It also affects old people and people with
debilitating illnesses. A study at three US sites
has provided more data about the epidemiology of
HMPV in children under the age of 5 years.
The survey by the Centers for Disease Con-
trol and Prevention (CDC) New Vaccine Surveillance
network took place at three sites, in Cincinnati,
Ohio; Nashville, Tennessee; and Rochester, New
JPOG_JulAug_2013_COMBINE_Final.indd 135 8/5/13 2:18 PM
JPOG JUL/AUG 2013 136
mature birth, immunodeficiency, chronic disease of
lungs, heart, or kidneys, cancer, or sickle-cell dis-
ease) were present in 40% (HMPV-positive) vs 30%
(HMPV-negative).
In the US, HMPV is an important cause of
acute respiratory illness and fever among young
children.
Edwards MK et al. Burden of human metapneumovirus infection in young
children. NEJM 2013; 368: 63343.

Risk factors for stillbirth
There has been little improvement in stillbirth rates
in recent years, and the importance of risk factors
has been uncertain. A study in the West Midlands
region of England has included 91,829 live births
and 389 stillbirths.
Risk factors for stillbirth included parity
(para 0 and 3+), ethnicity (African, Afro-Caribbean,
using country-specific data) for infants born in
hospital in 23 developing countries in Africa and
Latin America (20042005) and Asia (20072008),
including 276,436 singleton live births or fresh still-
births. The 90th percentile for birth weight varied
from 3,250 g in India to 4,050 g in Algeria, and the
prevalence of a birth weight of 4,000 g or greater
was 0.5% in India and 14.9% in Algeria. Factors
found to be significantly associated with macroso-
mia included higher maternal age (2034 years),
higher maternal height, higher parity, higher mater-
nal BMI, maternal diabetes, post-term pregnancy,
and male fetus. Macrosomia was associated with
increased risk of caesarean section and of adverse
maternal outcomes. The risk of adverse perinatal
outcome was increased in Asia.
The increase in obesity and diabetes in
women of reproductive age might have led to an
increase in fetal macrosomia worldwide. Research
into ways of controlling these factors is needed.
Koyanagi A et al. Macrosomia in 23 developing countries: an analysis
of a multicountry, facility-based, cross-sectional survey. Lancet 2013;
381: 47683; Dennedy M, Dunne F. Macrosomia: dening the problem
worldwide. Ibid: 4356 (comment).
Indian, or Pakistani), maternal obesity (BMI 30 or
greater), smoking, pre-existing diabetes, history of
mental health problems, and pregnancy complica-
tions (antepartum haemorrhage, fetal growth re-
striction). The greatest risk factor was fetal growth
restriction which increased the risk of stillbirth by a
factor of 7.8 in non-smoking mothers, 5.7 in smok-
ing mothers, and 10.0 in mothers only exposed to
passive smoking. The population attributable risk
from fetal growth restriction was 6.2% if detected
antenatally and 32.0% when not detected antena-
tally. Antenatal detection of fetal growth restric-
tion was associated with delivery 10 days earlier
on average. The stillbirth rate (per 1,000 births)
was 4.2 overall, 2.4 in pregnancies with no fetal
growth restriction, 9.7 when fetal growth restric-
tion was detected antenatally, and 19.8 when fetal
growth restriction was not detected antenatally.
This study identifies fetal growth restriction
as the main risk factor for stillbirth. Antenatal de-
tection of fetal growth restriction and appropriate
early delivery might prevent 600 stillbirths each
year in the UK.
Gardosi J et al. Maternal and fetal risk factors for stillbirth: population
based study. BMJ 2013; 346: 15 (f108); McCowan LME, Groom KM.
Identifying risk factors for stillbirth. Ibid: 7 (f416) (editorial).

Fetal macrosomia in developing
countries
Fetal macrosomia may lead to perinatal death,
perinatal asphyxia, shoulder dystocia, caesarean
section, maternal haemorrhage, prolonged labour,
and perinatal trauma. In the developed world, the
prevalence of macrosomia has increased along
with maternal obesity and diabetes. Little is
known, however, about fetal macrosomia in devel-
oping countries. Now, the WHO Global Survey on
Maternal and Perinatal Health has provided data
about macrosomia (birth weight > 90th percentile
OBSTETRICS
JPOG_JulAug_2013_COMBINE_Final.indd 136 8/5/13 2:18 PM
Professor Nimish Vakil
talks about management of patients with
refractory GERD.
Successful treatment of refractory GERD
requires thorough investigation of the patient
situation.
Professor David Lieberman
shares his perspective on the present and
future of colorectal cancer screening.
There is a lot of potential to prevent
many cancers if we can improve the rate
of CRC screening.
Dr Markus Cornberg
discusses the management of chronic
hepatitis B.
The aim of therapy should be the cure
or control of HBV infection without the
need for life-long treatment.
In this Series, fnd out what these medical experts have to say about latest
updates in the management of refractory GERD, the management of chronic
hepatitis B and the present & future of colorectal cancer screening.
Current Opinion in
Gastroenterology
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Imaging Paediatric
Brain Tumours
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology
PAEDI ATRI CS I PEER REVI EWED
JPOG JUL/AUG 2013 137
INTRODUCTION
Prompt identification and management of hearing loss in childhood is essential to en-
sure optimal speech and language development in the early years of life and optimal
school performance for older children. The implementation of universal neonatal hear-
ing screening has facilitated earlier identification of congenital losses, with 1 in 1,000
babies being born in the UK with a permanent hearing loss detectable at birth. However,
for more common acquired otological conditions of childhood such as otitis media with
effusion (OME) or hearing loss in the presence of complex medical needs, the paediatri-
cian may be the first port of call for parents or concerned allied health professionals,
and an understanding of the presentation, assessment and management options can
ensure optimal outcomes for this group of children. The aim of this review is to discuss
the clinical assessment of hearing loss and provide an overview of the management
options available.
Types of Hearing Loss
Hearing loss is classified by both type and severity. The types of hearing loss include
conductive, sensorineural, or mixed hearing loss, which can be subdivided into congeni-
tal or acquired. A differential diagnosis is included in Table 1. The severity of hearing
loss in decibels hearing level (dBHL) is based on a pure tone average, which is the
mean hearing threshold at four different sound frequencies (0.5, 1, 2, and 4 kHz). This
allows classification of the patients hearing as normal (< 25 dBHL) or determines the
extent of the loss; mild (2550 dBHL), moderate (5170 dBHL), severe (7190 dBHL),
profound (91110 dBHL), or total (> 110 dBHL). Management of the hearing loss thus
depends on its aetiology and the extent of the loss.
PAEDI ATRI CS I PEER REVI EWED


The Management of
Hearing Loss in Children
Marianne D Elloy, MBBS, MRCS, DOH-NS; Andrew H Marshall, FRCS(ORL-HNS)
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Table 1. Differential diagnosis of paediatric hearing loss
Inheritance/aetiology Sensorineural HL Conductive HL
Congenital Non-
syndromic
Autosomal dominant
Autosomal recessive
X-linked
DFNA chromosome loci: approx
40 gene loci identied. Most
common is GJB2 (connexin 26)
DFNB chromosome loci: approx
35 gene loci identied. Most
common is GJB2 (connexin 26)
DFN chromosome loci: approx 5
gene loci identied
Congenital ossicular
anomalies/xation
Syndromic Autosomal Dominant Branchio-oto-renal syndrome
Craniosynostosis syndromes (Crouzon, Apert,
Muenke, Pfeiffer)
DiGeorge syndrome
Noonan syndrome
Osteogenesis imperfecta
Marshall syndrome
Neurobromatosis type 2
Saethre-Chotzen syndrome
Stickler syndrome
Waardenburg syndrome
Treacher Collins syndrome
Autosomal recessive Albers-Schonberg disease
Pendred syndrome
Enlarged vestibular aqueduct
syndrome
Usher syndrome
X-linked Alport syndrome
Mohr-Tranebjaerg syndrome
Trisomy
Sporadic, autosomal
dominant or autosomal
recessive
Down syndrome
CHARGE syndrome
Goldenhar syndrome
Alport syndrome
Acquired Prenatal Intrauterine infections
Intrauterine exposure to
ototoxic drugs
Postnatal Infectious AOM and complications of
AOM Meningitis
Viral: measles, mumps, CMV
Inammatory Chronic suppurative otitis media
OME
Trauma Tympanic membrane
perforation
Temporal bone fracture
AOM = acute otitis media; CMV = cytomegalovirus; OME = otitis media with effusion.
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CLINICAL ASSESSMENT
Detection
The detection of hearing loss either occurs as a re-
sult of a universal neonatal hearing screening pro-
gramme or as a result of parental or professional
concern.
Universal neonatal hearing screening is now
well established in the United Kingdom, and its aim
is for early identification of hearing loss as early
intervention results in better speech and language
outcomes.
The programme aims for babies to be screened
by the gestational age of 44 weeks but preferably
before discharge from hospital. Premature babies
should not be screened before the gestational age
of 34 weeks. Two screening pathways exist: the
well baby protocol and the neonatal intensive care
unit/special care baby unit (NICU/SCBU) protocol
(for those babies admitted to NICU/SCBU for more
than 48 hours). The exclusions for hearing screen-
ing are ear atresia, microtia, or meningitis, with a
recommendation of direct referral for audiological
assessment in this high-risk group.
A dedicated hearing screening team under-
takes the assessments. The first step of the well
baby protocol comprises otoacoustic emissions
(OAEs) testing. If the baby passes this test and
there is no history of risk factors, then the baby is
discharged from the programme. If the baby fails
the test, it can be repeated on a second occasion;
however, if the test is failed again, an automated
auditory brainstem response (AABR) test is per-
formed. If this is passed and there are no risk fac-
tors, the baby can be discharged; however, if the
AABR is missed or incomplete, or if there are no
clear responses in one or both ears, or risk factors
are identified, further audiological assessment will
be arranged. The NICU/SCBU protocol differs with
all babies undergoing an OAE and AABR test. The
audiological referral criteria are the same as for
the well baby protocol; in addition, if either test
is missed or the tests provide inconsistent results,
then audiological review is recommended. A poten-
tial pitfall using OAEs as a screening tool is the po-
tential failure to identify a condition called auditory
neuropathy. In these children, OAEs are present but
their auditory brainstem response and functional
hearing can be very poor. This condition is more
prevalent in babies that would be identified by the
NICU/SCBU protocol in particular due to prematu-
rity or neonatal infection.
History
The history in neonates should explore the risk fac-
tors identified in the neonatal hearing screening
pathway which includes parental or professional
concern regarding the infants hearing or develop-
ment of auditory or vocal behaviour, high risk of
chronic middle ear problems, for example, Down
syndrome or cleft palate, craniofacial anomalies,
family history of permanent sensorineural hearing
loss from early childhood (in parents or siblings),
intermittent positive pressure ventilation, on NICU
or SCBU for more than 5 days, jaundice or hyperbili-
rubinaemia requiring exchange transfusion, proven
or possible congenital infections, TORCH (Toxoplas-
mosis Other: syphilis Rubella CMV Herpes), neuro-
degenerative or neurodevelopmental disorders, and
exposure to ototoxic drugs with monitored levels
outside the therapeutic range. However, it is im-
portant to be aware that 50% of newborns born
with permanent bilateral congenital hearing loss
do not have any known risk factors. Other risk fac-
tors which have been identified include bacterial
meningitis, whereby 10% of children will develop
a subsequent sensorineural hearing loss, Apgar
scores (04 at 1 minute and 06 at 5 minute), birth
weight less than 1,500 g, and consanguinity.
The history in older children should focus on
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the impact of the hearing loss on the childs speech
and language development, and school perfor-
mance including social interaction with their peers.
The aforementioned risk factors should be explored
and, in addition, enquiry about previous head injury
and temporal bone fracture.
Examination
In neonates with hearing loss, a full clinical exami-
nation should be undertaken as syndromic hearing
loss can occur in up to 30% of children with bilat-
eral permanent hearing loss and other associated
features may be noted.
Otoscopy may be normal. Examination for
craniofacial anomalies, preauricular pits, sinuses,
and branchial pits should be undertaken. Some syn-
dromes with hearing loss can be associated with
ophthalmological anomalies and all children with
a moderate or greater sensorineural hearing loss
should be reviewed by an ophthalmologist.
In older children, the most common cause of
childhood hearing loss is OME, and a number of dif-
ferent characteristic otoscopic appearances have
been identified including a dull tympanic membrane,
loss of the light reflex, flattening of the handle of
the malleus, a golden or blue hue of the tympanic
membrane, and indeed sometimes an air fluid me-
niscus or retrotympanic bubbles. Otosocopy should
also assess for the presence impacted cerumen, a
foreign body, infection, congenital cholesteatoma,
tympanosclerosis (the presence of calcification in
the tympanic membrane which can sometimes also
affect the ossicular chain), perforation (including
the site, extent, and status of the middle ear mu-
cosa), or an attic defect with the possibility of cho-
lesteatoma.
The facial nerve should also be assessed par-
ticularly in the presence of pathology.
Investigations
Audiological Assessments
The objective audiological assessments can be per-
formed for children of any age as no contribution to
the testing process is required by the patient.
Otoacoustic emissions: the principle of
OAEs is that objective sounds are emitted from the
outer hair cells of a normally functioning cochlea.
OAEs can be spontaneous or occur in response to
acoustic stimulus. Transient-evoked OAEs are used
in neonatal hearing screening, whereby broadband
clicks are delivered to the ear by a handheld probe
which also contains a microphone to detect the
emissions. The presence of OAEs indicates a hear-
ing threshold of 2040 dBHL. The test is quick and
easy to administer, is not affected by sleep, and has
a high sensitivity (97%), making it a useful screen-
Syndromic hearing
loss can occur in up
to 30% of children
with bilateral
permanent hearing
loss and other
associated features
may be noted
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ing tool. The limitations include inability to esti-
mate hearing thresholds or assess specific frequen-
cies. Specificity is low and patients can fail if they
have impacted wax or OME. In contrast, a patient
can pass this test but still have hearing problems
due to an auditory neuropathy.
Auditory brainstem response: Auditory
brainstem response can be undertaken as an auto-
mated test or a manually interpreted test to gain in-
formation about frequency-specific hearing thresh-
olds including bone conduction thresholds and
aided thresholds, which is useful in the presence
of ear canal atresia and microtia. The test takes
longer to administer and may require sedation or
general anaesthesia. The test records the activity
of the eighth cranial nerve and auditory pathways in
response to acoustic stimulus (via headphones) by
adhesive scalp electrodes. It has a high specificity
and sensitivity (> 90%).
Tympanometry: this test does not assess
hearing but is used to assess the compliance of
the tympanic membrane and is particularly useful
in the assessment of OME. The shape of the graph
produced gives information about middle ear com-
pliance, and a normal peak (type A) suggests nor-
mal middle ear function, a flattened peak (type B)
is suggestive of OME (or perforation in the presence
of a high ear canal volume), and a peak shifted to a
more negative pressure (type C) suggests Eustachi-
an tube dysfunction.
Behavioural Testing
Visual reinforcement audiometry: this can be
used for children age 6 months to 3 years. It is un-
dertaken in a specially adapted audiology room us-
ing either sound field speakers or ear inserts. The
child must be able to sit on a parents lap and be
able to turn their head to the sound. A distracter en-
tertains the child with toys and sounds are played
via the speakers; when the child turns correctly in
response to the sound, a visual reward is triggered
by the audiologist conducting the test, for example,
an illuminated moving toy. The advantages are that
hearing thresholds can be determined; however,
children can tire and lose interest which reduces
accuracy.
Conditioning/play audiometry: this can be
used for children aged above 23 years who can
obey simple commands. The child is instructed to
perform a task each time they hear a sound pre-
sented by headphones or sound field, for example,
put a wooden man into a wooden boat. This is easy
to perform and can establish hearing thresholds but
is dependent on the compliance of the child.
Pure tone audiometry: this is the same test
as used for testing the hearing in adults, requiring
the patient to press a button each time they hear
a sound. This can be used in children aged 4 years
and above, depending on their compliance. Parents
and siblings should be investigated with pure tone
audiometry.
Imaging: imaging is indicated in patients with
a bilateral severe to profound hearing loss, severe
to profound unilateral hearing loss, or a progres-
sive loss. Local protocols vary and a combination of
computed tomography or magnetic resonance imag-
ing (MRI) scanning can be utilized. The anatomical
features or variations of the cochlear nerves and
structure of the cochlea are well demonstrated by
MRI. However, the presence of ossification within
the cochlea, in particular, following meningitis is
optimally imaged using computed tomography.
Other Investigations
Electrocardiogram (ECG): all children identified
to have a bilateral permanent hearing loss should
undergo an ECG to assess for a prolonged QT inter-
val which is typically found in Jervell and Lange-
Neilsen syndrome, and if anomalies are identified
referral to a cardiologist should be undertaken.
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Blood tests and urinalysis: screening for
congenital infections can be undertaken by test-
ing for antibodies to toxoplasmosis, syphilis, ru-
bella, cytomegalovirus, and herpesvirus hominis. A
blood test for connexin 26, the most common non-
syndromic genetic cause of hearing loss, can be
undertaken. Urea and electrolytes accompanied by
urinalysis for haematuria should be undertaken as
part of a screen for Alport syndrome and branchio-
oto-renal syndrome. Thyroid function tests may be
normal in the early stages of Pendred syndrome,
and the classical investigation described is the per-
chlorate discharge test.
MANAGEMENT
For optimal outcomes, the management of paedi-
atric hearing loss requires a multidisciplinary ap-
proach. This can involve a host of medical speciali-
ties, in addition to ear, nose and throat surgeons,
the general practitioner, paediatricians, ophthal-
mologists, to manage ocular anomalies and the
geneticists to investigate aetiology of hearing loss
and undertake genetic counselling with the childs
family.
The childs family will require a significant
amount of support and input from the allied health
professionals. The audiologists not only assess
the childs hearing thresholds but also are the key
professional group in the provision of adjuncts to
hearing rehabilitation including the management
of hearing aids, bone anchored hearing aids, and
cochlear implant programming. The teachers of
the deaf provide support in the learning environ-
ment before children even start school. They spend
time in the classroom to assess what adjuncts may
be required and train the teachers in mainstream
schools on how to support children with hearing im-
pairment. The speech and language therapists in-
put is essential to optimize language development,
and they often work both in the community and the
school to provide input. At the initial consultation,
simple measures to enhance a childs hearing abili-
ties should be discussed with the parents, including
classroom placement strategies.
Conservative Management
Children who are making satisfactory progress de-
spite a mild to moderate hearing loss may be man-
aged with supportive measures. The majority of
children with OME are managed conservatively as a
significant proportion resolve spontaneously.
In those children requiring intervention, the
use of hearing aids for amplification is the main-
stay of auditory rehabilitation. Most children will
require behind-the-ear digital hearing aids, with
multiple brands and models available. These can be
programmed to the childs hearing loss and a mould
can be made to fit specifically in their ear, which
can be personalized with the logo of their favourite
football team being popular for boys and glitter be-
ing a hit with girls! For children with ear canal atre-
Figure 1. Soft band hearing aid.
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sia, microtia and other conductive deficits, a soft
band hearing aid a bone conductor hearing aid
attached to a soft head band is particularly useful
(Figure 1). Adjunctive devices such as FM systems
for the classroom can also be valuable in hearing
rehabilitation.
Surgical Management
Grommets (ventilation tubes): grommets are
small (plastic or titanium) tubes that are inserted
into the tympanic membrane to aerate the middle
ear. Multiple types of grommets are available with
the design impacting on the duration that the grom-
met is maintained in position. The main indication
for grommet insertion is persistent OME with hear-
ing loss. The National Institute for Clinical Excel-
lence (NICE) guidance (2008) advocates a 3-month
period of active observation as during this period
the effusion will resolve in 60% of children. For
persistent bilateral OME with hearing in the better
ear of 2530 dBHL or worse, grommets can be of-
fered, or if the hearing is less than 2530 dBHL but
is significantly impacting the childs development
or education. Children with Down syndrome often
have persistent problems with OME, and grommet
placement can be technically difficult owing to nar-
row ear canals and children often require multiple
sets of grommets. As such, a separate pathway is
provided for children with Down syndrome, with
more emphasis on utilization of hearing aids than
grommets. A third pathway for children with cleft
palate advocates the use of grommets in the pres-
ence of persistent OME and hearing loss.
The advantages of grommet placement are
instantaneous improvement in hearing. This quick
procedure is carried out under general anaesthetic
in children and often preferred by parents to the
alternative; a hearing aid as compliance is not an
issue. The risks of surgery include bleeding, infec-
tion, and perforation. In the event of infection, a
1-week course of topical antibiotic ear drops is ad-
vocated as the first-line treatment, on rare occa-
sions, for refractory infections grommets have to be
surgically removed.
Bone-anchored hearing aid (BAHA): the
indications for BAHA in children include congeni-
tal aural atresia and microtia, chronic suppurative
otitis media, persistent OME, chronic otitis externa,
unilateral profound hearing loss, failure with con-
ventional aids, and trauma to the external ear ca-
nal. A BAHA is a titanium screw inserted into the
Figure 2. Cochlear implant.
How a cochlear implant works
1. The sound processor (A) captures sound and converts it
into digital code.
2. The sound processor transmits the digitally coded sound
through the coil (B) to the implant (C) just under the skin.
3. The implant converts the digitally coded sound to electrical
signals and sends them along the electrical signals and
sends them along the electrode array, which is positioned
in the cochlea.
4. The implants electrodes stimulate the cochleas hearing
nerve fbres, which relay the sound signals to the brain to
produce hearing sensations.
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calvarial bone behind the ear. Osseointegration
occurs, resulting in bony growth into the titanium
screw. Once this has occurred, typically after 36
months, a bone conduction hearing aid system can
be attached, providing the child with amplification.
Typically, children cannot undergo implantation un-
til the age of 4 years; until a child is old enough to
undergo implantation, a soft band hearing aid can
be used.
The risks of surgery include failure of osse-
ointegration, soft tissue complications, trauma,
fixture displacement, and extradural haematoma.
Outcomes measures include favourable audiologi-
cal outcomes in comparison to conventional bone
conductor aids and good patient usage and satis-
faction after up to 10 years after fitting.
Cochlear implant: A cochlear implant is
a surgically implantable device which bypasses
damaged hair cells in the cochlea and directly
stimulates the cochlear nerve (Figures 2 and 3). It
comprises a microphone (which captures speech
and sound), processor (which converts the sound
into an electronic signal), transmitting coil (which
transmits the electronic signal), internal receiver/
stimulator (which receives the electronic signal and
converts it to electrical impulses), and an electrode
array (which delivers the electrical impulses to the
cochlear nerve). Modern electrodes are multichan-
nel with the common brands having 16 and 22 chan-
nels. Each channel stimulates a specific frequency.
Insertion of an electrode into the cochlear risks
destroying any residual hearing and makes the ear
reliant on the cochlear implant. However, hybrid
cochlear implants have been recently developed
for use in patients with serviceable low-frequency
hearing but a profound high-frequency loss, where-
by a custom made shortened electrode is inserted
with the aim of preserving existing hearing in the
low frequencies.
NICE guidance introduced in 2009 recom-
mended that simultaneous bilateral cochlear im-
plantation was indicated in children with severe
to profound hearing loss who did not receive ad-
equate benefit from conventional hearing aids. The
guidelines defined the severe to profound hearing
loss as only hearing sounds louder than 90 dBHL
at the frequencies of 2 and 4 kHz without hearing
aids and recommended the utilization of speech,
language, and listening skills appropriate for age,
Figure 3. Cochlear implant.
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combined with development stage and cognitive
ability as factors to consider when assessing the
benefit of hearing aids. The main contraindications
to cochlear implantation include lack of a cochlear
nerve and complete ossification of the cochlea.
Patients who are candidates for cochlear im-
plantation will be assessed by a specialist multi-
disciplinary cochlear implant team including au-
diological investigations, imaging, trial of hearing
aids, review by the speech and language therapists,
and teachers of the deaf to assess for suitability.
Confirmation that children are vaccinated against
pneumococcus will be sought preoperatively from
the general practitioner.
The risks of surgery include altered taste, in-
fection, meningitis, device failure, and facial nerve
injury. It is also important to note that children with
cochlear implants cannot undergo MRI.
Outcome measures have been used to assess
the efficacy of cochlear implantation, and there is
a wealth of literature supporting the success of
hearing rehabilitation and these include sound lo-
calization and speech recognition in noise meas-
ures and quality of life. Reports have revealed that
many children who received cochlear implants at an
early age are achieving age-appropriate academic
performance.
FURTHER READING
Browning G. Clinical otology & audiology. London: Arnold, 2001.
Gerber S. The handbook of pediatric audiology. Washington: Gallau-
det, 1996.
Graham J, Scadding G, Bull P. Pediatric ENT. Heidelberg: Springer,
2007.
Johnston J, Durieux-Smith A, OConnor A, Fitzpatrick E. Bilateral
cochlear implants: a critical review. Int J Audiol 2009;48:601617.
Kral A, ODonoghue G. Profound deafness in childhood. N Engl J Med
2010;363:14381450.
Kunst D, Kremer H, Cremers C. Genetics for ENT specialists. London:
Remedica, 2005.
McDermott A-L, Sheehan P. Bone anchored hearing aids in children.
Curr Opin Otolaryngol Head Neck Surg 2009;17:488493.
NHS. Newborn Hearing Screening Programme. Available online at
http://hearing.screening.nhs.uk/; 2011 (accessed 5 Mar 2011).
NICE. Cochlear implants for children and adults with severe to
profound deafness: NICE technology appraisal guidance 166. Avail-
able online at http://guidance.nice.org.uk/TA166; 2009 (accessed 5
Mar 2011).
NICE. Surgical management of otitis media with effusion in children:
NICE clinical guideline 60. Available online at http://guidance.nice.
org.uk/CG60; 2008 (accessed 5 Mar 2011).
Papsin B, Gordon K. Bilateral cochlear implants should be the stan-
dard for children with bilateral sensorineural deafness. Curr Opin
Otolaryngol Head Neck Surg 2008;16:6974.
Phillips J, Yung M, Burton M, Swan I. Use of aminoglycoside contain-
ing ear drops in the presence of a perforation evidence review and
ENT UK consensus statement. Available online at http://www.
entuk.org/news/news/attachments/eardrops; 2007 (accessed 5
Mar 2011).
US Preventative Services Task Force. Universal screening for hearing
loss in newborns: US Preventative Services Task Force recommen-
dation statement. Pediatrics 2008;122:143148.
2011 Elsevier Ltd. Initially published in Paediatrics and Child
Health 2011;22(1):1318.
About the Authors
Marianne D Elloy is Specialist Registrar in Otolaryngology at the
Royal Derby Hospital, Derby, UK. Andrew H Marshall is Consul-
tant in Otolaryngology at the Queens Medical Centre, Notting-
ham, UK.
Practice points
Universal neonatal hearing screening identies 1 in 1,000
babies with a permanent hearing impairment at birth.
Management of hearing loss is a multidisciplinary approach.
Hearing loss can be associated with a syndrome, and associated
features should be identied and investigated.
Early identication and intervention of hearing loss improves
speech and language outcomes.
Conventional digital hearing aids or soft band bone conductor
hearing aids can be used for children.
If hearing aids provide inadequate response to sound, patients
can be referred for assessment for either bone-anchored
hearing aid or cochlear implants, depending on their hearing
thresholds and pathology.
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Imaging Paediatric
Brain Tumours
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology
PAEDI ATRI CS OBSTETRI CS I PEER REVI EWED
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INTRODUCTION
Most women presenting with complications in early pregnancy are assessed, diagnosed
and managed at early pregnancy assessment units (EPAUs). These units aim to provide
thorough assessments, access to specialist investigations (scan, human chorionic gon-
adotrophin [hCG]), a rapid turnaround of results, and co-ordination of further manage-
ment.
The EPAU enables continuity of care, fewer admissions, and planned follow-up. It
is beneficial in the provision of open access for GPs, and ideally patients particularly
following a previous pregnancy loss. By streamlining investigations and treatment, this
system is also more cost-effective.
For women who have had previous pregnancy complication, a familiar setting and
ongoing support in a future pregnancy is a valued service.
A downside of the EPAU system is that it is often only available at limited times,
thus for complications occurring outside of these hours, patients require ward contact
numbers and more frequent inpatient based care.
HYPEREMESIS GRAVIDARUM
Over 50% of women suffer from nausea in pregnancy. Hyperemesis gravidarum is the
inability to maintain hydration, resulting in dehydration and ketonuria as a result of
nausea and vomiting in pregnancy. It affects between 0.1% and 1% of women. Patients
become dehydrated and ketonuric, develop an electrolyte imbalance (hyponatraemia and
hypokalaemia), and in severe untreated cases a nutritional (thiamine) deficiency culmi-
nates in Wernickes encephalopathy.
Management of Early Pregnancy
Complications
Harriet Pugsley, MB ChB, MRCOG; Judith Moore, MRCOG
OBSTETRI CS I PEER REVI EWED
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Symptoms develop around 68 weeks of gesta-
tion and are directly related to levels of hCG, peak-
ing towards the end of the first trimester before
settling in the second trimester. Women present
with nausea, weakness, vomiting, and occasionally
ptyalism (inability to swallow saliva). On examina-
tion, there are signs of dehydration and tachycardia
with or without hypotension. Those who have suf-
fered with hyperemesis in a previous pregnancy are
more likely to develop similar symptoms in subse-
quent pregnancies. Nausea and vomiting present-
ing after 12 weeks of gestation is not hyperemesis
gravidarum.
The proposed pathophysiology behind hy-
peremesis is related to the hCG. The hCG molecule
has common alpha subunit with thyroid-stimulating
hormone and is thought to exert its effect via a
temporary physiological thyrotoxicosis; there may
be evidence of a raised free thyroxine and a low
thyroid-stimulating hormone. This accounts for the
timing of the onset and settling of symptoms in cor-
relation with levels of hCG. There may also be psy-
chological and cultural factors.
In all presentations, a multiple or molar preg-
nancy should be excluded as these conditions also
result in an increased hCG level.
Many women with mild symptoms are managed
in the community; once women are ketotic and un-
able to maintain hydration, admission is necessary.
For the majority of women, intravenous rehydration
with antiemetic medication, on a day case basis
without admission, is sufficient to break the cycle
and allow the patient to re-establish oral intake.
Many units have managed to expand the role of the
EPAU to incorporate this service and release capac-
ity on the admitting wards. However, more severe
cases do require an inpatient admission, further
rehydration, antiemetics, thiamine supplements,
daily electrolyte analysis, and a gradual resumption
of oral intake and monitoring of ketonuria. Intrave-
nous rehydration with normal saline or Hartmanns
with potassium supplementation and monitoring of
electrolytes and ketonuria should be employed. In-
fusions containing dextrose should be avoided as
they may precipitate Wernickes encephalopathy.
Women requiring admission are intravascularly de-
hydrated posing an increased thrombotic risk, thus
thromboprophylaxis should be considered. Rarely,
intractable cases may require treatment with ster-
oids to relieve symptoms.
Hyperemesis is not uncommon; the majority of
cases can be successfully treated as a day admis-
sion to an EPAU. The outcome for the both preg-
nancy and patient are excellent.
MISCARRIAGE
Fifteen percent to 20% of pregnancies end in mis-
carriage.
Miscarriage was traditionally classified as
threatened, inevitable, complete, incomplete, or
missed. Most women present with pain and bleed-
ing in early pregnancy. Alternatively, miscarriage
may be diagnosed at the early dating scan with
no prior warning symptoms, this is classified as a
missed miscarriage.
Practice points I
Hyperemesis
50% of women suffer from nausea in pregnancy
0.11% of women suffer from hyperemesis gravidarum
Symptoms peak at the end of the first trimester
In severe cases, there is a risk of Wernickes encephalopathy re-
sulting from a nutritional imbalance of reduced thiamine
Treatment includes intravenous rehydration, electrolyte monitor-
ing and restitution, antiemetics, thiamine supplementation and,
in non-resolving cases, steroid therapy
Molar and multiple pregnancies should be excluded
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OBSTETRI CS I PEER REVI EWED
An episode of pain or bleeding in an early
pregnancy subsequently demonstrated to be a vi-
able pregnancy is termed a threatened miscarriage.
Traditionally, classifications of complete, in-
complete and inevitable miscarriage have been
used. Diagnosis and management are based on
clinical findings: (1) examination of vaginal loss
for products of conception, and (2) examination
of the cervical, to determine whether it is open or
closed. More recently, there has been a tendency
to be increasingly conservative in management of
miscarriage with routine use of ultrasound to aid
management. Once an intrauterine pregnancy is
demonstrated by ultrasound, with uncertain viabil-
ity, a follow-up scan will be arranged 1014 days
later to determine whether the pregnancy is ongo-
ing or not. If initial symptoms are of heavy bleeding,
or if they worsen between appointments, then med-
ical intervention in terms of a surgical evacuation
may be necessary at an earlier stage. Patients with
a pregnancy of unknown viability, who are waiting
for follow-up, must be informed of the risk of pain
and bleeding between appointments and be given
appropriate contact numbers and advised to return
if their bleeding becomes heavy.
If there are symptoms (or with minimal symp-
toms), treatment of miscarriage is based on three
alternatives: expectant, medical, or surgical. Ex-
pectant management employs awaiting the natural
course of events, for the products to pass sponta-
neously. Medical management involves combina-
tions of oral or vaginal prostaglandins to induce the
completion of miscarriage. Surgical management
involves an operation, usually vacuum aspiration,
to remove any remaining products of pregnancy.
There are risks and benefits associated with
all approaches:
1. Expectant management requires a longer fol-
low-up, often multiple visits, and is associated
with more prolonged and heavier bleeding.
Owing to the unpredictable of length of follow-
up and number of visits associated with expect-
ant management, many women veer away from
this option. There is a higher risk of treatment
failure and an increased need for later surgery.
This method of treatment may be more effec-
tive for those who are symptomatic of pregnan-
cy loss (ie, those presenting with an incomplete
miscarriage) because the process of spontane-
ous loss will have already begun. The timescale
for completion of expectant management can
extend over 26 weeks with the emphasis on
planned follow-up at specified intervals for
assessments usually with ultrasound scans
to ensure all products have passed. Although
figures differ depending on the length and type
of follow-up that patients have (clinical versus
ultrasound), around 80% complete without
intervention. When counselling women, the
possibility of a period of prolonged follow-up
and of the risk of incomplete loss with the
subsequent need for surgery should be stressed.
2. Medical management also aims to avoid
Practice points II
Miscarriage
Affects up to 1 in 5 pregnancies
Treatment options are expectant, medical, and surgical
Expectant management aims to avoid surgery, may result in
prolonged follow-up with a risk of heavier bleeding and failed
treatment
Medical management aims to avoid surgery, may be uncomfort-
able with heavier bleeding and risk of later surgery
Surgical management allows early completion of treatment with
the risk of surgical and anaesthetic complications
There is no difference in post-treatment conception rates regard-
less of method of management
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OBSTETRI CS I PEER REVI EWED
surgery and is an accelerated method of
conservative management with a more predict-
able timescale of completion. It can be carried
out as an inpatient or outpatient procedure,
provided support is available for women at
home if needed. If managed in the community,
follow-up is necessary once again, to ensure
completion and that ongoing complications are
recognized and treated.
3. The benefit of surgical management is to
limit the need for prolonged follow-up,
reduce the number of symptomatic days, and
for early closure of treatment. Complications,
although infrequent, include uterine perforation,
cervical trauma, incomplete evacuation, the
risk of the anaesthetic, and a slightly higher
infection rate when compared with expectant
management.
The miscarriage treatment trial and subse-
quent Cochrane reviews (2006, 2010) have con-
cluded that there is no superior method of man-
agement and have recommended that the womans
preferences are taken into account when planning
care; treatment should therefore be patient-guided,
based on an informed decisions.
Importantly, the long-term follow-up of the
miscarriage treatment trial concluded that there
is no difference in later conception rates follow-
ing the different approaches to management. It has
also been suggested that empowering patients by
choice in their management reduces subsequent
anxiety and depression rates. Women who have
been involved in the decision-making process have
subsequently scored more favourably on quality-of-
life outcome questionnaires.
Recurrent Miscarriage
Recurrent miscarriage is defined as three or more
consecutive miscarriages and affects 1% of couples
trying to conceive.
It is recommended that investigations into
why women have miscarried should be implement-
ed following the third consecutive miscarriage.
For the majority of women, the results are normal.
Prognosis for the future worsens with increasing
numbers of pregnancy losses, advancing maternal
age, normal histopathology, and a normal parental
karyotype. Recurrent miscarriage is particularly
devastating for couples as they may be able to
conceive relatively easily but not carry an ongoing
pregnancy; they may thus need increased support
and counselling. EPAUs arrange specialist follow-
up and offer a patient educational role with the
provision of information combined with access to
support groups and counselling.
Following recurrent miscarriage, investiga-
tions may include screening for antiphospholipid
syndrome, karyotyping the products of conception,
diagnostic imaging for structural abnormalities, and
parental karyotyping looking for balanced translo-
cations. The incidence of controlled diabetes and
thyroid disease are no different in this population
when compared with that of the general public.
As stated, for the majority of patients, inves-
tigations will be normal and no cause is identified.
In a minority, there may be a treatable haemato-
logical factor such as antiphospholipid syndrome.
In the case of recurrent miscarriage, positive serum
Practice points III
Recurrent miscarriage
Defined as three or more consecutive miscarriages
Affects 1% of couples
For the majority of patients, all investigations are normal
A minority of patients will have antiphospholipid syndrome,
which can be treated with heparin and aspirin to improve the
chance of an ongoing pregnancy
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OBSTETRI CS I PEER REVI EWED
blood results on two separate occasions 12 weeks
apart with a history of three consecutive pregnancy
losses before 10 weeks are necessary to confirm
antiphospholipid syndrome. (Positive bloods in as-
sociation with a single pregnancy loss above 10
weeks or a poor obstetric outcome before 34 weeks
secondary to placental disease also fulfil the crite-
ria for antiphospholipid syndrome.) For these wom-
en, treatment with aspirin and heparin will improve
the chance of an ongoing pregnancy. Without treat-
ment, the likelihood of a term pregnancy may be as
low as 10%; with treatment, the miscarriage rate
may be reduced by up to 54%.
MOLAR PREGNANCY
Molar pregnancies are rare, affecting roughly 1 in
700 conceptions. They are the result of an abnor-
mal conception and can be either complete moles
or partial moles. They are more common at the ex-
tremes of reproductive ability and among people
living in Asia. The risk of recurrence is low: 12%.
Complete moles are diploid, contain only pa-
ternal chromosomes, and are either the result fer-
tilization of an empty ovum with duplication of a
single sperm or dispermic fertilization. They contain
no fetal tissue.
Partial moles are triploid in nature, may con-
tain fetal parts, and are the result of dispermic fer-
tilization.
The concern with molar conceptions is the
risk of progression to neoplasia if left untreated.
Molar pregnancies can present as a miscarriage
or, more rarely, after a normal pregnancy. They are
associated with characteristic appearances on an
ultrasound scan (snow storm/placental vacuoles).
Molar pregnancies are usually detected in the
first trimester because women either present with
bleeding, resulting in an early ultrasound scan, or
are suspected on the dating ultrasound scan. They
are also associated with a raised hCG level, and the
condition should be excluded in women presenting
with hyperemesis.
Diagnosis is sometimes retrospective follow-
ing histological examination of products of concep-
tion removed following a surgical evacuation. In
cases of miscarriage, products should be screened
for gestational trophoblastic disease.
Once suspected, the majority are easily treat-
ed by surgical evacuation alone, with histological
confirmation. However, owing to the low but poten-
tially life-threatening risk of ongoing trophoblastic
tissue and neoplasia, registration and referral to
specialist tertiary care is crucial.
Due to the nature of hydatidiform gestational
trophoblastic disease/neoplasia (GTN) with the po-
tential for progressive disease, long-term follow-up
is necessary. This involves prolonged measurement
of urine hCG levels to ensure disease-free status,
avoidance of future pregnancy until hCG levels have
been normal for 6 months, and further monitoring
after all future pregnancies.
Treatment with surgical evacuation and pro-
longed monitoring of hCG levels are usually all that
is required, with repeat monitoring of levels in sub-
sequent pregnancies. All UK cases of gestational
Practice points IV
Molar pregnancy
Affects 1 in 700 conceptions
The concern is the risk of progression to neoplasia if unrecog-
nized or untreated
All cases must be registered at a tertiary referral centre and re-
quire long-term follow-up
The risk of recurrence is low (12%)
Cases which have progressed to neoplasia are responsive to
treatment; 56% require chemotherapy with a 98% cure rate
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OBSTETRI CS I PEER REVI EWED
trophoblastic disease are monitored via a national
registration programme consisting of three centres
based in Sheffield, Dundee, and Charring Cross.
hCG is a marker of ongoing trophoblastic dis-
ease. If levels are persistently elevated, further
treatment is required; this is usually medical with
methotrexate and, in rare cases, chemotherapy; a
repeat evacuation is not usually helpful. GTN is
extremely responsive to treatment. Chemotherapy
is necessary in only 56% of cases with a greater
than 98% cure rate and is more commonly required
following complete rather than partial molar preg-
nancies.
Following diagnosis and treatment, women
are advised to delay further conception until 6
months following normalization of the hCG levels
and a year after completion of chemotherapy. Bar-
rier contraception is advised; hormonal contracep-
tion is an option once hCG levels have normalized.
If contraception has been commenced before diag-
nosis, there may be a slight increase in the risk of
gestational trophoblastic disease progressing to
neoplasia with combined preparations, but there
is no evidence of detriment from single agent pro-
gestogens.
Thankfully, the recurrence risk is low, so wom-
en who continue to have a future pregnancy can be
reassured that 98 out of 100 conceptions will not be
a further molar pregnancy. In the women in whom a
second molar pregnancy does occur, it is usually of
the same histological type as the first.
ECTOPIC PREGNANCY
Ectopic pregnancy complicates approximately 1 in
50 pregnancies. Risk factors include previous ec-
topic pregnancy, a history of pelvic infection, or
past pelvic surgery. If not recognized or managed
inappropriately, it can become a life-threatening
condition as a result of invasion and rupture of a
blood vessel, resulting in massive pelvic haemor-
rhage; this can happen with or without rupture of
the Fallopian tube.
Presentation, as with most early pregnancy
complications, is with pain with or without vaginal
bleeding, or as collapse with associated concern-
ing symptoms of dizziness and fainting. Diagno-
sis is based on history, clinical examination, and
investigations, including transvaginal ultrasound
scan, serial hCG measurements, and laparoscopy.
In a normal pregnancy, the serum hCG level should
rise by at least 60% in 48 hours, although anything
above 30% can be consistent with normal early
pregnancy. The hCG levels in an ectopic pregnancy
may initially rise at a normal rate, but by the time
of presentation the rise is usually suboptimal. Once
suspected, ectopic pregnancies might be suggested
by ultrasound evidence of gestational tissue out-
side of the body of the uterus with an empty uter-
ine cavity. However, laparoscopy remains the gold
standard for confirmation of diagnosis and allows
concurrent treatment.
Although diagnosis can be suggested by pel-
vic ultrasound and hCG, confirmation can often
take several days because of presenting features
in common with other early pregnancy complica-
tions, uncertainty on ultrasound scan, and the need
Practice points V
Ectopic pregnancy
Affects 1 in 50 pregnancies
The main concern is the risk of life-threatening bleeding
Management options depend largely on presentation and include
mainly surgery or methotrexate (rarely, expectant management
may be employed in the case of resolving trophoblast)
Salpingotomy is only recommended during surgical treatment if
there is a concern that the contralateral tube is non-functional
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OBSTETRI CS I PEER REVI EWED
for serial blood levels. In the stable patient in this
situation, the 48-hour hCG trend and symptoms are
crucial factors in determining timing and options
for treatment. In cases of haemodynamic insta-
bility secondary to a suspected bleeding ectopic
pregnancy, emergency surgery is necessary either
by laparotomy or laparoscopy, in order to stem the
bleeding and remove the ectopic as rapidly and
safely as possible.
Once diagnosed, there are several options for
the management of an ectopic pregnancy, depend-
ing mainly on presentation and individual circum-
stances. For example, in the acute emergency due
to a bleeding ectopic, urgent surgery is required
as a lifesaving procedure. However, the majority
of ectopic pregnancies are treated by scheduled
laparoscopic surgery or by medical management
with methotrexate. Conservative management is
also an option with resolving trophoblast (the trend
is for the hCG level to fall). Laparoscopic surgery
may be initially diagnostic to confirm the ectopic
pregnancy as the cause of pain and then therapeu-
tic by salpingectomy or salpingotomy as definitive
treatment.
Any surgery performed will have implications
for future fertility potential. At the time of surgery,
both Fallopian tubes should be assessed as the
health of the unaffected Fallopian tube is also rele-
vant. The surgical recommendation is for salpingec-
tomy to be performed if the contralateral tube is
deemed healthy and that salpingotomy should only
be performed if there is doubt about fertility with
the remaining tube. This is because preservation of
the Fallopian tube following an ectopic pregnancy
retains the risk of a subsequent ectopic pregnancy
in the same tube, whereas a salpingectomy removes
this risk. The fertility rate from a single functional
tube should be sufficient to allow conception.
If there is a diseased contralateral Fallopian
tube (such as a hydrosalpinx) and a salpingectomy
is performed, future options for conception are like-
ly to depend on in vitro fertilization. The limitation
of visual assessment of the tube should be remem-
bered as it will give an indication of gross disease
but not assess tubal patency for which a dye test is
more accurate.
Owing to the risk of life-threatening bleeding
from an ectopic pregnancy and the need to be ab-
solutely certain that a viable intrauterine pregnancy
has been excluded, there are rigid criteria to be met
before medical management becomes an option.
It is recommended that ultrasound findings
should show a suspected ectopic mass less than 3
cm in size with minimal free fluid and no visible
fetal heart. There should be minimal pain on ex-
amination and a suboptimal rise in hCG which is
initially less than 3,000 IU/L. (The Royal College of
Obstetricians and Gynaecologists disseminated na-
tional recommendation, from which individual units
For the majority of patients with recurrent miscarriage,
investigations are normal and no cause is identied.
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JPOG JUL/AUG 2013 153
OBSTETRI CS I PEER REVI EWED
set their own treatment pathways). These criteria
are employed to enable patient selection so that
medical management is an option for appropriate
patients who are unlikely to have spontaneous
bleeding whilst undergoing treatment. Success of
treatment is assessed by subsequent hCG estima-
tions.
The main benefit of methotrexate is the avoid-
ance of surgery. The downside includes the low risk
of failure of treatment in cases in which ongoing
trophoblast results in bleeding from the ectopic
site and the need for surgical intervention.
Methotrexate has antifolate properties and is
teratogenic, so patients must be suitably advised
to delay conception following treatment, and care
must be taken to encourage folic acid prior to and
during subsequent conceptions.
Expectant management can be employed for
cases of resolving trophoblast, which may either be
a resolving ectopic pregnancy or an early pregnan-
cy miscarriage. For safety, levels of hCG must be
decreasing and women must be able to seek appro-
priate help and attend hospital easily should they
need to. Follow-up needs to be rigorous to ensure
that the hCG titres have returned to non-pregnant
levels and complications are not missed.
For women who have experienced an ectopic
pregnancy, fears for the future include the risk of
a recurrent ectopic pregnancy, concerns regarding
the ability to conceive again, and fear relating to
the health risks of a recurrent ectopic pregnancy.
It may be particularly worrying for women to plan a
further pregnancy if they have already experienced
life-threatening emergency surgery from a ruptured
ectopic or a previous pregnancy complication. If one
Fallopian tube is diseased, it is likely that the other
tube is also affected, and spontaneous conception
may be delayed or impossible. Therefore fertility
may well depend on in vitro fertilization which may
not be an option for many women (limited National
Health Service funding, personal finances, and
emotional reasons).
CONCLUSION
For the majority of women, conception, pregnancy,
and birth are straightforward, resulting in an uncom-
plicated confinement, delivery, and healthy baby.
For women who do experience complications,
explanation, advice, follow-up, and necessary in-
vestigations will help when planning for the future
and deciding whether to try for a further pregnancy.
Women who are informed of the long-term effects of
treatment, for example, that surgical/medical man-
agement following a miscarriage does not convey
differences in conception rates, and who are given
an informed choice regarding their management,
may find it easier to come to terms with their loss
and have less anxiety and depression long-term. It
must be remembered that each case is individual,
all circumstances are different and women need
Women who are informed
of the long-term effects
of treatment... and who are
given an informed choice
regarding their management,
may find it easier to come
to terms with their loss
and have less anxiety and
depression long-term.
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OBSTETRI CS I PEER REVI EWED
FURTHER READING
Royal College of Obstetricians and Gynaecologists. The manage-
ment of early pregnancy loss. Green-top Guideline No. 25.
London: RCOG, 2006.
Trinder J, Brocklehurst P, Porter R, Read M, Vyas S, Smith L.
Management of miscarriage: expectant, medical or surgical?
Results of randomized controlled trial (miscarriage treatment
(MIST) trial). BMJ 2006;332:1235.
Smith LFP, Ewings PD, Quinain C. Incidence of pregnancy after
expectant, medical or surgical management of spontaneous
first trimester miscarriage. Long term follow-up of miscar-
riage treatment (MIST) randomized controlled trial. BMJ
2009;339:b3827.
Nanda K, Peloggia A, Grimes DA, Lopez LM, Nanda G. Expect-
ant care versus surgical treatment for miscarriage. Cochrane
Database Syst Rev 2006. Issue 2. Art. No.: CD003518. pub. 2.
Neilson JP, Gyte GML, Hickey M, Vaquez JL, Don L. Medical
treatments for incomplete miscarriage (less than 24 weeks).
Cochrane Database Syst Rev 2010. Issue 1. Art. No.: CD007223.
pub. 2.
Neilson JP, Hiskey M, Vaquez JL. Medical treatment for early
fetal death (less than 24 weeks). Cochrane Database Syst Rev
2006. Issue 3. Art. No.: CD002253. pub. 3.
Rai RS, Clifford K, Cohen H, Regan L. High prospective fetal
loss rate in untreated pregnancies of women with recurrent
miscarriage and antiphospholipid antibodies. Hum Reprod
1995;10:33013304.
Royal College of Obstetricians and Gynaecologists. The manage-
ment of recurrrent miscarriage. Green-top Guideline No. 17.
London: RCOG, 2011.
Royal College of Obstetricians and Gynaecologists. The manage-
ment of gestational trophoblastic neoplasia. Green-top Guide-
line No. 38. London: RCOG, 2010.
Royal College of Obstetricians and Gynaecologists. The manage-
ment of tubal pregnancy. Green-top Guideline No. 21. London:
RCOG, 2004.
2012 Elsevier Ltd. Initially published in Obstetrics, Gynaecol-
ogy and Reproductive Medicine 2012;22(3):7680.
About the Authors
Harriet Pugsley is Specialist Registrar in Obstetrics and Gynae-
cology. Judith Moore is Consultant Obstetrician and Gynaecolo-
gist at Nottingham United Hospitals NHS Trust, Nottingham City
Hospital, Nottingham, UK.
to be informed of the risks and benefits of all the
available treatment options.
The role of the EPAU, with open access and
support in future pregnancies in terms of regular
scanning and as a point of contact for the individual,
has been shown to improve the successful outcome
of future pregnancies following miscarriage. Open
access and choice in care options have also been
advocated as helping women to feel more in con-
trol of their treatment and reducing post-treatment
rates of anxiety and depression.
Besides the feeling of loss, it is normal for
women to feel guilt, blame, and anxiety. Women
will have concern for their own health, particularly
if they have required emergency treatment. Part-
ners may also harbour feelings of guilt, responsi-
bility, and helplessness for the situation, and may
have conflicting emotions with concerns regarding
the risk to their loved ones health in a future preg-
nancy, balanced with their desire to increase their
family.
It is important for patients who have had an
ectopic pregnancy to understand that they require
an early ultrasound in subsequent pregnancies to
exclude a recurrence and to seek a medical assess-
ment early in pregnancy.
Previously, in future conceptions, early open
access and regular specialist follow-up have been
available, enabling reassurance, investigations,
early detection of further complications, along with
support throughout subsequent pregnancies. Unfor-
tunately as healthcare services are put under ever
increasing pressure to reduce costs, open access
may no longer be deemed a financially viable ser-
vice.
It is important that appropriate investigations
are arranged and results collated and explained. It
is also necessary that patients are given appropri-
ate counselling, information, and links to support
groups should they require additional care.
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Autism Spectrum Disorders
Patricia Howlin, BA MSc PhD FBSP, Professor of Clinical Child Psychology
GYNAECOLOGY I PEER REVI EWED
JPOG JUL/AUG 2013 155
GYNAECOLOGY I PEER REVI EWED
INTRODUCTION
The lifetime prevalence of ovarian cancer in the developed world is 12%. It is often
described as a silent killer; however, the majority of women frequently experience symp-
toms in the months leading to diagnosis. The majority of ovarian cancers are diagnosed
at an advanced stage. In England and Wales, ovarian cancer kills more women than all
of the other gynaecological malignancies combined.
Pregnancy, breastfeeding, and use of the oral contraceptive pill all appear to pro-
tect against the development of epithelial ovarian cancer; the lower incidence seen in
less developed countries may be related to a higher birth rate.
Patients with ovarian cancer are best managed by multidisciplinary teams. These
usually include nurse specialists, medical oncologists, histopathologists, radiologists,
palliative care specialists, and gynaecological oncologists, in collaboration with the
patients and their families.
TYPES OF OVARIAN CANCER
Primary ovarian tumour types include epithelial, sex cordstromal and germ cell tu-
mours. Tumours not specific to the ovaries also occur, such as sarcomas and lymphomas.
Metastatic tumours from breast, stomach, and endometrial primaries are not uncommon.
Epithelial Tumours
More than 80% of ovarian cancer is epithelial in origin. The most common subtype
is serous, accounting for about 50%, followed by endometrioid, mucinous, clear cell,
transitional (Brenner), mixed, and undifferentiated tumours. These have previously been
Ovarian Cancer:
Current Management
and Future Directions
Sin E Taylor, BSc, MB ChB, MRCOG, MD; John M Kirwan, MB ChB, MRCOG
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JPOG JUL/AUG 2013 156
GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
treated as essentially the same disease with dif-
fering histology; however, it is becoming increas-
ingly evident that they behave as distinct entities.
For example, endometrioid and clear cell cancers
are strongly linked with endometriosis, whilst many
mucinous cancers originate in the appendix and evi-
dence increasingly points to serous tumours arising
from dysplastic endometrium in the distal fallopian
tube. Response to chemotherapy varies; serous tu-
mours tend to be highly chemosensitive, but clear
cell and mucinous tumours are more resistant to
conventional chemotherapy.
Primary peritoneal cancer is histologically
indistinguishable from metastatic serous ovarian
cancer. It is diagnosed in the absence of any clear
ovarian primary. Treatment is the same as for ovar-
ian cancer, although, as there is often no mass to
debulk, chemotherapy is more often used as the
primary treatment.
Borderline ovarian tumours are not truly can-
cers but are termed borderline because they show
histological features that are intermediate between
benign and malignant tumours. They are staged in
exactly the same way and sometimes spread be-
yond the ovary to produce non-invasive implants in
the omentum and the peritoneum. They can recur
after long periods; cases have been documented
with disease returning over 30 years after the initial
presentation. They are typically found in a younger
population compared with epithelial cancers, one-
third occur in women under the age of 40. The main
treatment is surgical excision, and opinions differ
in the extent of surgery required. It is probable that
they represent premalignant disease for low-grade
ovarian carcinomas.
Sex CordStromal Tumours
Sex cordstromal tumours account for approximate-
ly 7% of all malignant ovarian tumours. They arise
from a combination of the hormone-producing cells
of the ovary and stromal fibroblasts. Seventy per-
cent of all malignant sex cordstromal tumours are
granulosa cell tumours. The majority occur in wom-
en in their sixth decade, although a small proportion
arises in young women and prepubertal girls. Gran-
ulosa cell tumours may secrete sex hormones; most
secrete oestrogen (although androgen-secreting va-
rieties do occur) potentially leading to endometrial
hyperplasia and carcinoma. Presenting symptoms
include abdominal distension, acute abdominal
pain, and abnormal vaginal bleeding. As most pre-
sent at an early stage, the prognosis is good. Treat-
The histological subtypes of epithelial ovarian cancer are
distinct entities, requiring different treatment strategies.
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
ment is principally surgical with platinum-based
chemotherapy for advanced disease. Surgical treat-
ment is as for epithelial ovarian cancer, although in
young women with early disease, fertility preserva-
tion is an option. Other stromal tumours are rare
and include thecomas, fibromas, Sertoli-Leydig cell
tumours, and gynandroblastomas.
Malignant Germ Cell Tumours
Malignant germ cell tumours occur chiefly in girls
and young women. The most common variety is the
dysgerminoma, the counterpart to the seminoma in
the male. Other types include the yolk sack tumour,
embryonal carcinoma, polyembryoma, non-gesta-
tional choriocarcinoma, and teratoma. They usually
present with abdominal pain, which is sometimes
acute, and a palpable pelvic mass. Treatment for
early-stage disease is surgical. As over 60% are
confined to one ovary at diagnosis, fertility-sparing
surgery is usual with unilateral salpingo-oophorec-
tomy or even ovarian cystectomy in selected cases
with otherwise normal ovaries. Dysgerminomas are
highly radiosensitive, but platinum-based chemo-
therapy is currently the preferred option as radio-
therapy usually results in premature ovarian failure.
Non-dysgerminoma tumours are treated with the
chemotherapy combination of bleomycin, etopo-
side, and cisplatin. Response rates are excellent
with cure rates approaching 100% in early-stage
disease and up to 75% in advanced disease.
FAMILIAL OVARIAN CANCER
Approximately 510% of all ovarian cancer is as-
sociated with a genetic predisposition. Individuals
carrying these gene defects have a significantly
higher risk of developing ovarian cancer than the
general population (Table 1).
A strong family history of breast and/or ovar-
ian cancer, especially at a relatively young age,
may indicate the presence of one of several pos-
sible BRCA1 or BRCA2 gene mutations. The gene
products are involved in DNA repair. These gene
mutations are particularly common amongst the
Ashkenazi Jewish population. Men carrying these
genes are at increased risk of pancreatic cancer
as well as male breast cancer. Endometrial, colon,
ovarian, and other cancers cluster in families with
the Lynch II or hereditary non-polyposis colorectal
cancer (HNPCC) syndrome. This is caused by a vari-
ety of defects in the DNA mismatch repair system.
BRCA1/2 and HNPCC are inherited in an autosomal
dominant fashion; further mutation or epigenetic
silencing of the second allele results in malignancy.
Prophylactic risk-reducing surgery is recom-
mended for BRCA1 and BRCA2 carriers when they
reach the age of 35 or have completed their fami-
lies. This usually consists of a laparoscopic bilateral
salpingo-oophorectomy. The risk of ovarian cancer
under 35 years in BRCA patients is low, and remov-
al of the ovaries has the dual advantage of reducing
the risks of both ovarian and breast cancer. HNPCC
carriers are offered hysterectomy and bilateral sal-
pingo-oophorectomy when their family is complete.
Until patients are ready to undergo surgery, annual
screening is commonly offered with cancer antigen
125 (CA 125) and transvaginal ultrasound; however,
Table 1. Approximate lifetime percentage risk of developing
ovarian cancer
General population
One rst-degree relative affected under 55 years
One rst-degree relative affected over 55 years
Two rst-degree relatives affected
BRCA1 carrier
BRCA2 carrier
HNPCC carrier
1.6%
5.2%
3.4%
7%
2844%
27%
12%
HNPCC = hereditary non-polyposis colorectal cancer.
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
there is no evidence that this is effective. Even if
the ovaries are removed, there is a small continuing
risk of developing primary peritoneal cancer.
The lack of one of these specific conditions
does not exclude an increased cancer risk, as not all
inherited ovarian cancer syndromes have had their
genetic origin fully characterized.
BIOMARKERS AND SCREENING
The most commonly used marker for ovarian cancer
is CA 125. This is a glycoprotein that is released
into the bloodstream by any condition that disturbs
the peritoneum, including any peritoneal cancer,
cirrhosis, congestive cardiac failure, endometrio-
sis, and pelvic inflammatory disease. Pregnancy
also causes a variable increase in serum levels. It
is therefore notoriously non-specific at low levels.
However, serous ovarian cancer can cause dramatic
increases in CA 125. This is only likely to occur
when the disease has already spread beyond the
ovary. Other tumour subtypes, especially mucinous,
often produce a more modest elevation.
Other markers are also used. Germ cell ovarian
cancers often secrete highly specific tumour mark-
ers which are useful in diagnosis and monitoring,
eg, -fetoprotein, -hCG, and lactate dehydroge-
nase. These should be tested, in addition to CA
125, in women under the age of 40 years with a
suspicious pelvic mass. In addition, inhibin may be
of use as a marker for mucinous and granulosa cell
tumours.
Novel markers are currently under development
both as panels and individually. Human epididymis
protein 4 has been suggested as a biomarker with
equal or greater sensitivity and specificity than CA
125, however clinical trials are awaited.
The PLCO (prostate, lung, colon, ovary) trial,
a huge US-based randomized controlled trial of
cancer screening, has recently reported. It con-
Screening the anxious low-risk patient for ovarian cancer may not be benecial because of the risk of false-
positive result.
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cluded that screening the general population with
annual CA 125 and transvaginal ultrasound does
not reduce ovarian cancer mortality. Indeed, a sig-
nificant number of women had major complications
from surgery performed because of a false-positive
screening test.
There are also two large UK-based multicentre
trials investigating ovarian cancer screening. The
first is in postmenopausal women without a signifi-
cant family history of ovarian cancer and also uses a
combination of transvaginal ultrasound and CA 125
(UK Collaborative Trial of Ovarian Cancer Screen-
ing [UKCTOCS]). The second is in women with a
significant family history and is testing a panel of
biomarkers in addition to CA 125 and transvaginal
ultrasound (UK Familial Ovarian Cancer Screening
Study). Both have now finished recruitment and are
due to report in the next few years.
Screening with a combination of CA 125, ul-
trasound, and pelvic examination is commonly per-
formed for anxious patients who desire screening
for ovarian cancer. If the findings of the PLCO trial
are duplicated in the UKCTOCS trial, this practice is
likely to become hard to defend in low-risk women.
INVESTIGATION
The first symptoms of ovarian cancer usually
emerge some time before diagnosis. These com-
monly include early satiety, changes in bowel
habit, bloating, urinary frequency, and pelvic and
abdominal pain. Patients with advanced cancer of-
ten complain of abdominal swelling and discomfort
due to ascites with or without a large abdomin-
opelvic mass. Eating is often difficult, and patients
may notice weight loss, apart from the distended
abdomen. It is not uncommon for patients to pre-
sent with a swollen leg secondary to a deep vein
thrombosis. However, most small ovarian cancers
are asymptomatic when confined to the ovaries and
therefore difficult to detect.
Recent National Institute for Clinical Excel-
lence (NICE) guideline recommend that women,
especially those over 50 years old, who experience
symptoms persistent or frequent symptoms as de-
scribed above, or new-onset symptoms suggestive
of irritable bowel syndrome, should have CA 125
Table 2. FIGO staging of ovarian cancer
Stage Description
I Conned to ovaries
Ia One ovary, no ascites present containing malignant
cells, no tumour on external surface, capsule intact
Ib Both ovaries, no ascites present containing malignant
cells, no tumour on external surfaces, capsule intact
Ic Tumour limited to one or both ovaries with any of
the following: tumour on the surface on one or both
ovaries, capsule ruptured, ascites present with malig-
nant cells or positive peritoneal washings
II Growth involving one or both ovaries with pelvic exten-
sion
IIa Extension and/or metastases to uterus and/or fallopian
tubes
IIb Extension to other pelvic tissues
IIc Tumour stage IIa or IIb but with tumour on surface of
one or both ovaries, capsule ruptured, ascites pres-
ent containing malignant cells or positive peritoneal
washings
III Tumour involving one or both ovaries with microscopi-
cally conrmed peritoneal implants outside the pelvis
and/or regional lymph node metastasis
IIIa Microscopic peritoneal metastasis beyond the pelvis
IIIb Macroscopic peritoneal metastasis beyond the pelvis, 2
cm or less in greatest dimension
IIIc Abdominal implants greater than 2 cm in diameter and/
or regional lymph nodes metastasis
IV Distant metastasis beyond the peritoneal cavity.
Includes liver parenchymal metastasis and/or pleural
effusion with positive cytology
FIGO = International Federation of Gynecology and Obstetrics.
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
measured. If the level is > 35, an ultrasound of the
abdomen and pelvis should be performed.
The ultrasound and CA 125 together are used
to calculate the risk of malignancy index (RMI): RMI
= U M CA 125, where U = ultrasound score (1
point for each of multilocular cysts, solid areas,
ascites, bilateral lesions, metastases. U = 0 for 0
points, U = 1 for 1 point, U = 3 for 25 points); M =
menopausal status (premenopausal = 1, postmeno-
pausal = 3); CA 125 = serum CA 125 level.
A score of 250 has been chosen by NICE to
guide triage to either surgery in a cancer centre un-
der the care of a specialist multidisciplinary team,
including subspecialist gynaecological oncologists
( 250), or to care under a general gynaecologist
with an interest in gynaecological oncology in a
cancer unit (< 250).
If ovarian cancer is suspected, a computed to-
mography of the abdomen and pelvis (and thorax if
clinically indicated) should be performed, prior to
surgery, to assess the extent of the disease.
STAGING
Staging is performed to guide treatment and to
provide information on prognosis. Traditionally, this
has been achieved by performing a staging lapa-
rotomy. Information can also be gleaned from ra-
diological investigations, guided biopsy, and cytol-
ogy of ascitic or pleural fluid. The current staging
system was devised by International Federation of
Gynecology and Obstetrics (FIGO) (see Table 2).
TREATMENT
Treatment in ovarian cancer depends upon the stage
at presentation and the histological subtype. Non-
epithelial cancers are discussed earlier. In general,
epithelial ovarian cancers are treated with a combi-
nation of surgery and chemotherapy. Except in very
early disease, treatment is rarely curative but it can
provide symptom relief and prolong life.
Traditionally, ovarian cancer is treated with a
staging and debulking laparotomy followed by six
cycles of chemotherapy. Second-look laparotomy,
where a second surgical debulking procedure is
performed after completion of chemotherapy, is not
beneficial. Data from the EORTC 55971 trial, which
compared interval debulking surgery performed
midway between six cycles of chemotherapy with
the traditional laparotomy and six cycles of post-
operative chemotherapy, suggested that neoadju-
vant chemotherapy in bulky stage IIIc/IV disease
did not adversely affect prognosis and that interval
debulking is associated with a lower post-operative
morbidity and mortality. The CHORUS (CHemother-
apy OR Upfront Surgery) trial also addresses this
question and its publication is awaited.
In light of this, interval debulking surgery, per-
Advanced epithelial ovarian cancer is difcult to cure.
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
formed midway through chemotherapy, is gaining
popularity.
SURGERY
Surgical treatment, whether primary or as an inter-
val debulking procedure, involves a midline laparot-
omy, sampling of ascitic fluid or peritoneal wash-
ings for cytology, full assessment by palpation of
all peritoneal surfaces and biopsy of any suspicious
areas, removal intact of any encapsulated masses
or debulking of tumour, sampling of suspicious
pelvic and para-aortic lymph nodes, omentectomy,
total abdominal hysterectomy, and bilateral salpin-
go-oophorectomy. The aim is to completely remove
all visible disease. This is thought to promote an
optimum response to chemotherapy. Surgery also
provides ample material for diagnostic histological
assessment. The evidence for debulking improving
chemosensitivity is not absolute. Many argue that
the ability to perform optimal cytoreduction is a
more a reflection of favourable tumour biology with
an intrinsically better prognosis than the surgery
itself influencing outcome. However, it has been
demonstrated that survival in stage III disease is
improved by the primary surgery being performed by
a specialist gynaecological oncologist, rather than
an obstetrician/gynaecologist or a general surgeon.
Surgical aggressiveness varies considerably
between continents, countries, and individual units.
Some studies have shown improvements in survival
with radical surgery, including liver resection, bowel
resection, and splenectomy. Published case series
show small or no increases in mortality and morbid-
ity. However, these studies usually involve highly se-
lected cases from single institutions and may not be
directly applicable to wider practice. Some disease
remains unresectable to even the most adventurous
surgeon; this includes small bowel mesenteric dis-
ease and disease of the portal triad.
Recent NICE guidance recommends that lym-
phadenectomy is restricted to removal of clinically
involved nodes. Full pelvic and para-aortic node dis-
section in suspected stage I disease is not recom-
mended.
Less aggressive surgery is preferred in early
epithelial ovarian cancer in young women who wish
to preserve their fertility. About 8% of stage I epi-
thelial ovarian cancers occur in women under the
age of 35. A proportion of these will not have com-
pleted childbearing and may wish to consider fertil-
ity-sparing surgery. Suitable patients include those
with stage IA, grade 1 or possibly grade 2 disease.
Such conservative surgery would typically consist
of peritoneal fluid cytology, unilateral salpingo-
oophorectomy, omental biopsy, and careful inspec-
tion of the contralateral ovary and nodal chain. One
case series describes 282 women treated conserva-
tively for epithelial ovarian cancer. Just over 30%
The decision to adopt palliation in patients with ovarian cancer
can be challenging.
The decision to adopt palliation in patients with ovarian cancer can
be challenging.
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
subsequently went on to have term deliveries. Four
percent died of conditions related to their disease.
Surgery also plays a role in palliative care of
patients with ovarian cancer. Bowel obstruction is
common in the end stages of the disease; post-mor-
tem studies of women dying with ovarian cancer
revealed bowel obstruction in almost 50%. Before
contemplating such surgery, consideration must be
given as to whether it is appropriate. Surgery should
only be performed if it has a reasonable chance of
success, and risks need to be carefully balanced
against potential symptom relief. Contraindications
to surgery include patient refusal, rapidly accumu-
lating acsites, high obstruction, multiple levels of
obstruction, and poor nutritional status. Decisions
involving palliative surgery should involve the mul-
tidisciplinary team and careful discussion with the
patient and her relatives.
CHEMOTHERAPY
Patients should have histological confirmation of
their ovarian cancer prior to starting chemotherapy.
This may be obtained through image-guided percu-
taneous biopsy, or where this is not possible or the
results are inadequate, by laparoscopic biopsy.
The current standard first-line chemotherapy
regimen for ovarian cancer involves intravenous ad-
ministration of a platinum-based drug with a taxa-
ne, usually paclitaxel, given 3 weekly for six cycles.
Most units prefer carboplatin to cisplatin as it has
a less toxic side effect profile. Evidence for the use
of paclitaxel is drawn from its efficacy in relapsed
ovarian cancer. However, paclitaxel significantly in-
creases the risk of neuropathy when compared with
carboplatin alone, and some patients have anaphy-
lactic reactions to taxanes. For these reasons, it
is not universally used. Chemotherapy can also be
given via the intraperitoneal route; however, cur-
rently in the UK its use is not recommended outside
of clinical trials.
Epithelial ovarian cancer is one of the more
chemosensitive solid tumours, and complete clini-
cal and radiological response occurs in up to 50%
with the above regimen. Conversely, 2030% will
show no evidence of response. Unfortunately, the
majority of patients with advanced ovarian cancer
will relapse. Chemotherapy for recurrent disease is
determined in part by the length of time before re-
lapse occurs. If it is more than 6 months afterwards,
it is potentially platinum-sensitive and, unless con-
traindicated, a regimen containing platinum will be
used again. A taxane is likely to be included, espe-
cially if not used initially. Paclitaxel is sometimes
used at a lower dose at more frequent intervals;
this appears to reduce the adverse effects experi-
enced. Response rates are in the order of 30%. If
relapse occurs within 6 months, second-line drugs,
such as liposomal doxorubicin and topotecan, may
be considered. As response rates are low, approxi-
mately 1020%, the choice of drug is made bearing
in mind side effect profiles and ease of administra-
tion.
Novel chemotherapeutic agents are constantly
being developed, some of the most promising effect
the functioning of vascular endothelial growth fac-
Practice points
Ovarian cancer is best managed within a cancer centre by a
multidisciplinary team
Women with symptoms suggestive of ovarian cancer should
have a CA 125 performed as an initial screen, followed by
ultrasound if abnormal
Ovarian cancer screening with ultrasound and CA 125 does not
appear to be helpful in the low-risk population
Treatment involves a combination of surgery and platinum-
based chemotherapy
Fertility-sparing surgery is possible in women with early-stage
disease
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GYNAECOLOGY GYNAECOLOGY I PEER REVI EWED
tor. Interim analysis of the ICON 7 trial of standard
therapy with or without bevacizumab (a monoclo-
nal antibody to vascular endothelial growth factor)
shows a sustained improvement in progression-free
survival in a subgroup of women with advanced dis-
ease and suboptimal surgical debulking.
Hormonal therapies are occasionally used;
these probably act by reducing oestrogen activity
and include tamoxifen, aromatase inhibitors, and
gonadotropin-releasing hormone analogues. Re-
sponse rates of 1015% have been achieved in re-
lapsed disease. Their main advantage is their mini-
mal side effects when compared with conventional
chemotherapy.
PALLIATIVE CARE
Palliation is an integral part of the care of pa-
tients with ovarian cancer. Maintaining a balance
between optimism and pragmatism, together with
knowing when the emphasis of care should tilt to-
wards palliation, are some of the most challenging
aspects of caring for this group of patients.
Almost all patients with advanced ovarian
cancer, whether in the terminal phase or not, will
have distressing symptoms that require treatment.
These symptoms may be due to the disease itself
or secondary to their treatment. Common symptoms
include nausea, pain, loss of appetite, constipation,
and abdominal distension. This is too small a space
to describe all the possible treatment strategies
involved, but it is worth emphasizing a few princi-
ples. It is important to take a history, examine the
patient, and review the drug chart and case notes.
Then consider what investigations may be helpful
and what drug or intervention will best treat the
most likely cause of her symptoms. These patients
are usually complex with multiple problems; how-
ever, a logical and systematic approach will help to
identify the best treatment. Subcutaneous infusions
delivered by syringe driver are useful as they permit
a steady concentration of drug and oral medications
may be poorly absorbed. Several hospices publish
their guidelines for managing symptoms in pallia-
tive care on the internet, which can be very helpful.
Finally, the physical needs of a seriously ill pa-
tient are only one facet of their care. Social, spiritu-
al, and emotional needs also need to be addressed,
both for the patient and their relatives. Specialist
oncology nurses and palliative care input is essen-
tial, as are discussions on resuscitation status and
preferred place of death. These aspects of care are
easily overlooked but can make the difference be-
tween a peaceful and a difficult death.
FURTHER READING
Agarwal R, Linch M, Kaye SB. Novel therapeutic agents in ovarian
cancer. EJSO 2006;32:875886.
Aletti GD, Gallenberg MM, Cliby WA, Jatoi AJ, Hartmann LC.
Current management strategies for ovarian cancer. Mayo Clin Proc
2007;82:751770.
Blagden S, Gabra H. Future directions in the management of epithelial
ovarian cancer. Future Oncol 2008;4:403411.
Cannistra SA. Cancer of the ovary. N Engl J Med 2004;351:25192529.
Colombo N, Parma G, Zanagnolo V, Insinga A. Management of ovarian
stromal cell tumours. J Clin Oncol 2007;25:29442951.
Gershenson DM. Management of ovarian germ cell tumours. J Clin
Oncol 2007;25:29382943.
Goff BA, Mandel L, Muntz HG, Melancon CH. Ovarian carcinoma
diagnosis, results of a national ovarian cancer survey. Cancer
2000;89:20682075.
Markman M. New, expanded, and modied use of approved antineo-
plastic agents in ovarian cancer. The Oncologist 2007;12:186190.
NICE clinical guideline 122, Ovarian Cancer.
Nickles Fader A, Rose PG. Role of surgery in ovarian carcinoma. J Clin
Oncol 2007;25:28732883.
Rao G, Crispens M, Rothenberg ML. Intraperitoneal chemotherapy
for ovarian cancer: overview and perspective. J Clin Oncol
2007;25:28672872.
2011 Elsevier Ltd. Initially published in Obstetrics, Gynaecology
and Reproductive Medicine 2011;22(2):3437.
About the Authors
Sin E Taylor is Subspecialty Trainee in the Department of Gynae-
cologial Oncology at Liverpool Womens Hospital, Liverpool, UK.
John M Kirwan is Consultant Gynaecological Oncologist at Liverpool
Womens Hospital, Liverpool, UK.
JPOG_JulAug_2013_COMBINE_Final.indd 163 8/5/13 2:18 PM
Imaging Paediatric
Brain Tumours
Tang Phua Hwee, MBBS, FRCR, MMed Diagnostic Radiology
PAEDI ATRI CS I PEER REVI EWED
JPOG JUL/AUG 2013 164
Constipation in
Infants and Children
Taya Dowling, BMedSci, MB BS; Scott Nightingale, BMed(Hons), MClinEpid, FRACP

REMEMBER
Constipation is a common paediatric presentation.
Constipation is defined based on the frequency of stooling (which varies widely
depending on the age of the child), and more importantly the consistency, size and
difficulty with which stools are passed (see the box on page 165).
1
Constipation arising beyond the neonatal period is most often functional (ie, does not
result from any identifiable organic pathology), and may be perpetuated by voluntary
withholding of stool to avoid painful defaecation.
There are several red flags that should prompt further investigation for a contrib-
uting medical or surgical condition, but these are uncommon (see the box on page
165).
2,3
Faecal incontinence is thought to be related to over distension of the rectum with
stool, shortening of the anal canal and resulting impairment of normal continence
mechanisms. Incontinence is not deliberate or caused by laziness on the part of the
child.
Untreated constipation and faecal incontinence (encopresis) can have a significant
psychosocial impact on a child.
Management of constipation is often a long- term process that requires the com-
plementary approaches of careful education of the child and parents, behavioural
techniques, laxative agents and review.
4

ASSESSMENT
The focus should be on identifying the rare child with an organic cause for consti-
PAEDI ATRI CS I PEER REVI EWED
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PAEDI ATRI CS PAEDI ATRI CS I PEER REVI EWED
pation, and determining whether the child has
faecal impaction.
The history should include a detailed descrip-
tion of the childs stool, stool frequency, incon-
tinence, withholding behaviour and any symp-
toms associated with defaecation, such as pain,
bleeding and straining.
Important aspects of the history include age at
onset, growth trends, diet history and the pres-
ence of red flags (see the box on this page).
A thorough physical examination should be per-
formed, particularly focusing on growth param-
eters, palpable abdominal faecal masses, in-
spection of the perianal and lumbosacral regions
and lower limb neurological examination. Poor
growth may occur with Hirsch sprung disease,
hypothyroidism and coeliac disease.
Digital rectal examination should be avoided in
primary health care as it rarely contributes to the
clinical assessment and can be particularly dis-
tressing for the child.
Impaction is suggested by faecal incontinence or
a palpable faecal mass (preferably determined
via abdominal palpation).
If the likely diagnosis is functional constipation
then no further invest igation is needed. Abdomi-
nal X- rays are not needed to diagnose constipa-
tion or to determine response to therapy.
5
If a pathological cause for constipa tion is sus-
pected then appropriate investigations should be
performed in consultation with a paediatrician or
paediatric surgeon.
MANAGEMENT
A combination of management approaches that
complement each other are almost always re-
quired in the management of childhood consti-
pation. Individual elements in isolation (eg, di-
simpaction without maintenance laxatives) are
unlikely to be unsuccessful.
Education of parents and caregivers about the
relationship between behavioural aspects (eg,
fear of pain and withholding) and functional
Red ag feature
2,3
History
Constipation from the neonatal period
Failure to pass meconium by the age of 48 hours
Ribbon stools suggesting anorectal stricture or stenosis
Abdominal distension and vomiting
Poor weight gain or weight loss
Leg weakness or delayed gross motor development
Examination
Gross abdominal distension
Abnormal appearance, position or patency of anus fistulae, bruis-
ing, multiple fissures or fissures away from the midline, tight or
patulous anus, anteriorly placed anus, absent anal wink
Lumbosacral abnormalities evidence of sacral agenesis, discol-
oured skin, naevi, sinus, hairy patch, lipoma, central pit, scoliosis
Gluteal asymmetry or wasting
Absent cremasteric reflex
Abnormal results on lower limb neurological examination de-
formity such as talipes, abnormal reflexes
Rome III diagnostic criteria for functional constipation in
children
1
At least two of the following features have been present for at least 2
months in a child aged 4 years or older (developmental age):
two or fewer defaecations in the toilet per week
at least one episode of faecal incontinence per week
history of retentive posturing or excessive volitional stool retention
history of painful or hard bowel movements
presence of a large faecal mass in the rectum
history of large diameter stools that obstruct the toilet
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PAEDI ATRI CS PAEDI ATRI CS I PEER REVI EWED
constipation is vital. They should be informed
that this is usually a chronic problem, requiring
long-term management. The rationale behind the
various aspects of management should be made
clear, and education should be reviewed on sub-
sequent visits.
Faecal disimpaction, if necessary, should be
achieved using laxatives (see the Table). Except
in infants, oral therapy should generally be tried
first. In children, rectal therapy should usually
be reserved for those with more severe or un-
responsive constipation, leading to persistent
rectal discomfort or unproductive straining. Oral
macrogol 3350 (polyethylene glycol) was found
to be equally effective to enema therapy for dis-
impaction in a randomized study of 90 children.
6
If multiple enemas or nasogastric lavage are re-
quired for disimpaction then a paediatrician or
paediatric surgeon should be involved.
Maintenance laxative therapy should be started
after disimpaction and often needs to continue
for many months after normalization of stool-
ing. A plan should be made to restart laxatives
promptly on signs of relapse.
The objectives of maintenance therapy are that:
the child passes regular soft stools (eg, 1 to
2 per day) without discomfort or excessive ef-
fort
the rectum remains empty to prevent re-
impaction.
There are many options available for mainte-
nance laxative therapy (see the Table), with
choice influenced by the age of the child, pre-
vious experience, ease of administration and
palatability to the child:
Liquid paraffin should be avoided in infants
and those at risk of aspiration because of the
risk of lipoid pneumonia, and should not be
given within 2 hours of sleep.
In children, the osmotic laxative macrogol
Parents and caregivers are to be educated to encourage the child with faecal incontinence develop a healthy
stooling habit.
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PAEDI ATRI CS PAEDI ATRI CS I PEER REVI EWED
was found to result in more stools per week
and less need for additional therapy when
compared with lactulose in a meta -analysis of
four studies.
7
Macrogol is not currently recom-
mended for children younger than 2 years in
Australia because of lack of safety data; how-
ever, these data are accumulating.
Stimulant laxatives such as bisacodyl, sen-
nosides and sodium picosulfate are effective
adjuncts to osmotic laxatives when necessary.
There are few data to support widely held con-
cerns regarding long- term use of laxatives, in
particular stimulant laxatives. Clinical studies
show that long- term use of osmotic laxatives is
safe and well tolerated.
7
Caregivers should be
educated about this safety and the need for long-
term maintenance therapy.
Behavioural measures combined with laxative
therapy are superior to either therapy alone in
children with faecal incontinence.
8
It is impor-
tant that behavioural measures are applied con-
sistently with the aim of developing a healthy
stooling habit. The child should be encouraged to
sit on the toilet for 5 minutes, two or three times
a day, within 30 minutes after meals to take ad-
vantage of the gastrocolic reflex. Positive rein-
forcement for sitting (eg, using reward charts)
should be encouraged. Children should never be
punished for being constipated or incontinent.
A healthy diet should be encouraged rather than
a high- fibre diet, as there is little evidence that
increasing dietary fibre is an effective treat-
ment for childhood constipation.
2
There is lim-
ited evidence that avoiding cow milk may result
in improvement in some children with chronic
constipation, particularly those with atopic ten-
dencies.
9,10
Any trial of such an elimination diet
should be limited to 2 to 4 weeks, and the child
should be re- challenged to confirm any effect.
Prolonged elimination diets require supervision
by a qualified dietitian to prevent any nutritional
deficiencies and there should be repeated at-
tempts to normalize the diet.
Aside from situations of clinical dehydration,
there is no evidence that increasing water intake
is beneficial in constipation. However, ensuring
adequate fluid intake is important when using
osmotic laxatives to avoid dehydration.
Regular review is required to monitor response
to therapy, adjust laxative dose, reinforce educa-
Table. Initial laxative options for infants and children
Infants (< 12 months) Children
Disimpaction Glycerol suppository 700 mg Macrogol 3350 1 to 1.5 g/kg/day (max 52 g for
children aged 2 to 5 years and 78 g for children
aged 6 years and over) until disimpacted
4
*
Maintenance

Sorbitol-containing fruit juices, such as prune,


pear, apple (50 to 100 mL/day) or
Lactulose 5 mL daily
Macrogol 3350 0.4 to 0.8 g/kg daily up to 17 g* or
Paraffin oil 1 to 3 mL/kg daily (start 10 to 20 mL
daily)

or
Lactulose 10 to 15 mL daily
* Macrogol 3350 (polyethylene glycol) is available in Australia in a variety of formulations, in scoop packs or sachets.

Doses are a guide only and should be titrated to effect every 2 to 3 days as required, with consideration of the maximum recommended dose.

Not recommended in infants, or in children with gastro-oesophageal reux or risk of aspiration.


JPOG_JulAug_2013_COMBINE_Final.indd 167 8/5/13 2:18 PM
JPOG JUL/AUG 2013 168
PAEDI ATRI CS PAEDI ATRI CS I PEER REVI EWED
REFERENCES
tion and support the family through what is often
a long and frustrating period.
Lack of response to management should prompt
review of all aspects of the management plan.
Persisting failure to respond may prompt recon-
sideration of pathological causes and investiga-
tion for these.
CONCLUSION
Constipation is a common paediatric presenta-
tion and is usually functional.
Further investigation or referral is guided by the
presence of red flags.
Untreated constipation and faecal incontinence
(encopresis) can have a significant psychosocial
impact on a child.
Management of constipation is often a long-
term process that requires the complementary
approaches of careful education of the child and
parents, behavioural techniques, laxative agents
and review.
2013 Medicine Today Pty Ltd. Initially published in Medicine Today
July 2013;14(7):7173. Reprinted with permission.
About the Authors
Dr Dowling is a Paediatric Registrar, Hunter New England Local Health
District, Newcastle. Dr Nightingale is a Paediatric Gastroenterolo-
gist, John Hunter Childrens Hospital; and Conjoint Lecturer, School
of Medicine and Public Health, University of Newcastle, Newcastle,
NSW, Australia.
Series Editor: Associate Professor Simone Strasser, MD, FRACP, Clini-
cal Associate Professor, Central Clinical School (Medicine), University
of Sydney; and Senior Staff Specialist, AW Morrow Gastroenterol-
ogy and Liver Centre, Royal Prince Alfred Hospital, Sydney, NSW,
Australia.
1. Drossman DA. Rome III: the functional gastro-
intestinal disorders. 3rd ed. McLean, VA: Degnon
Associates; 2006.
2. National Institute for Health and Care Excellence
(NICE). Constipation in children and young people:
diagnosis and management of idiopathic childhood
constipation in primary and secondary care: quick
reference guide. London: NICE; 2010.
3. Constipation Guideline Committee of the North
American Society for Pediatric Gastroenterology,
Hepatology and Nutrition. Evaluation and treatment
of constipation in infants and children: recommen-
dations of the North American Society for Pediatric
Gastroenterology, Hepatology and Nutrition. J Pedi-
atr Gastroenterol Nutr 2006;43:e1-e13.
4. North American Society for Pediatric Gastroen-
terology, Hepatology and Nutrition. Evaluation and
treatment of constipation in children: summary of
updated recommendations of the North American
Society for Pediatric Gastroenterology, Hepatol-
ogy and Nutrition. J Pediatr Gastroenterol Nutr
2006;43:405-407.
5. Berger MY, Tabbers MM, Kurver MJ, Boluyt N,
Benninga MA. Value of abdominal radiography, co-
lonic transit time, and rectal ultrasound scanning in
the diagnosis of idiopathic constipation in children:
a systematic review. J Pediatr 2012;161:44-50,
e41-42.
6. Bekkali NL, van den Berg MM , Dijkgraaf MG, et
al. Rectal fecal impaction treatment in childhood
constipation: enemas versus high doses oral PEG.
Pediatrics 2009;124:e1108-E1115.
7. Gordon M, Naidoo K, Akobeng AK, Thomas AG.
Osmotic and stimulant laxatives for the manage-
ment of childhood constipation. Cochrane Database
Syst Rev 2012;7:CD009118.
8. Brazzelli M, Grifths PV, Cody JD, Tappin D. Be-
havioural and cognitive interventions with or with-
out other treatments for the management of faecal
incontinence in children. Cochrane Database Syst
Rev 2011;12:CD002240.
9. Iacono G, Cavataio F, Montalto G, et al. Intoler-
ance of cows milk and chronic constipation in chil-
dren. N Engl J Med 1998;339:1100-1104.
10. Irastorza I, Ibanez B, Delgado-Sanzonetti L,
Maruri N, Vitoria JC. Cows milkfree diet as a
therapeutic option in childhood chronic constipa-
tion. J Pediatr Gastroenterol Nutr 2010;51:171-176.
JPOG_JulAug_2013_COMBINE_Final.indd 168 8/5/13 2:18 PM
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Essential
Reference
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JPOG JUL/AUG 2013 169
Management of Pregnancies With Previous
Caesarean Section
Yung WK, MBBS, MRCOG, FHKAM (O&G); Lau WL, MBBS, FRCOG, FHKAM (O&G); Leung WC, MBBS, MD, FRCOG, FHKAM (O&G), Cert RCOG (Maternal and Fetal Med)
INTRODUCTION
Over the past few decades, there has been
widespread concern about the increasing
proportion of births born by caesarean de-
livery. The rising rate of primary caesarean
section has led to the increased number of
obstetric population with a history of prior
caesarean delivery. Although this group
of women may be offered planned vagi-
nal birth after previous caesarean section
(VBAC) or elective repeat caesarean sec-
tion (ERCS), the VBAC rate is generally low
particularly in well-developed countries.
In the United States, the VBAC rate has
decreased to 8.5% by 2006, while the total
caesarean rate has increased to 31.1%.
1

In this article, we review the recom-
mendations on VBAC by different profes-
sional societies, the favourable and unfa-
vourable factors in considering VBAC, the
associated maternal and fetal benefits
and risks, as well as the non-medical
concerns that play a role in the decision-
making process.
WHO ARE SUITABLE TO
ATTEMPT VBACWHAT
DOES THE EVIDENCE SAY?
There have been numerous studies evalu-
ating the risks and benefits of VBAC and
One of the obstetricians roles is to identify women who are most likely to achieve
successful vaginal birth after previous caesarean section.
ERCS. International authorities have
published their guidelines to provide
evidence-based obstetric care. Examples
are guidelines from the American College
of Obstetrics and Gynecology (ACOG) in
2010, the Royal College of Obstetrics and
Gynecology (RCOG) in 2007, and the Society
of Obstetricians and Gynaecologists of
Canada (SOGC) in 2005. All three organi-
zations emphasized that their data were
limited by several factors: (1) there is no
randomized controlled trial on VBAC ver-
sus ERCS; (2) adverse maternal and peri-
natal outcomes rarely occur; (3) there was
selection bias in the published reports, as
a womans decision to attempt VBAC re-
lies on the counselling by the health-care
provider and local resources.
2

The three societies share similar
opinion on patient selection undergoing
5
S
K
P
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 169 8/5/13 2:09 PM
JPOG JUL/AUG 2013 170
planned VBAC. Previous uterine rupture
and high vertical classical caesarean sec-
tion are the contraindications, as they are
associated with a substantial risk of scar
rupture. For the other scar variants such
as prior inverted T or J incision, lower ver-
tical incision, previous myomectomy and
prior complex uterine surgery, the avail-
able data are, however, insufficient and
conflicting owing to the limited number
of studies. Planned VBAC in such circum-
stances should be assessed by experi-
enced obstetricians on an individual basis.
The risk of uterine rupture for women
with one previous caesarean section of a
low transverse incision is low. The risk fig-
ures by ACOG, RCOG and SOGC are < 1%,
0.20.7%, and 0.21.5%, respectively.
In some patients, the type of uterine
incision of prior caesarean delivery can-
not be confirmed. Although some have
questioned the safety of offering VBAC
under these circumstances, two case se-
ries reported a similar rate of successful
VBAC and uterine rupture to known low
transverse scar.
3,4
It is based on the fact
that nowadays most caesarean incisions
are low transverse. Therefore, the ACOG
and SOGC guidelines suggest that VBAC
is not contraindicated in women with one
previous caesarean delivery with unknown
type of scar unless there is a high clinical
suspicion of a previous classical uterine
incision. The RCOG states that the opera-
tive notes of previous operation should be
reviewed where possible.
Although the risk of uterine rupture
increases with the number of previous
caesarean sections, the chance of achiev-
ing VBAC appears to be similar.
1
Both the
RCOG and ACOG suggest that planned
VBAC is contraindicated in women with
more than two previous caesarean sec-
tions.
1,5
In clinical practice, most women
undergoing planned VBAC have one prior
caesarean delivery. The quoted success
rates of planned VBAC by the ACOG, RCOG
and SOGC are 6080%, 7276%, and 50
85%, respectively.
One of the obstetricians roles is to
identify women who are most likely to
achieve successful VBAC. Prior vaginal
birth, spontaneous onset of labour, prior
caesarean section for a non-recurring
condition and preterm labour are the fa-
vourable factors for successful VBAC.
1,5
On the contrary, recurrent indication for
prior caesarean section (eg, labour dys-
tocia), increased maternal age, maternal
obesity, gestation greater than 40 weeks,
increased neonatal birth weight, and short
pregnancy interval are the negative pre-
dictor factors.
1
Macrosomia has been recognized
as a significant factor leading to failed
VBAC, with a higher risk of uterine rupture
in women with no previous vaginal deliv-
ery.
2,6,7
Peaceman et al
8
evaluated the ef-
fect of fetal size on VBAC. If prior caesar-
ean delivery was performed for dystocia
defined as failed induction, cephalopelvic
disproportion or failure to progressor
failed instrumental delivery, the VBAC
success rate was reduced to 54% com-
pared with 67% for other indications. If
fetal weight of the current pregnancy ex-
ceeded the prior one by 500 g, the success
rate was only 38%.
8
Other retrospective
analysis showed that infants with birth
weight of more than 4 kg were less likely
to be delivered vaginally. However, obste-
tricians should be aware that so far there
is a lack of reliable methods in estimat-
ing fetal weight with accuracy. Therefore,
clinical suspicion of fetal macrosomia
alone should not be a contraindication to
offering planned VBAC.
Womens age might be considered
during the counselling on planned VBAC.
Although there were studies showing that
the success rate decreased with advanced
age,
9
maternal age alone should not be
used to discourage women from attempt-
ing a vaginal delivery.
Maternal obesity adversely influenc-
es the success rate.
10
The available evi-
dence consistently demonstrates a gradu-
al reduction of vaginal delivery rate with
increasing body mass index (BMI). Juhasz
et al
11
evaluated 1,213 women in their
study. The VBAC rate fell from 83.1% for
BMI < 19.8, to 79.9% for BMI 19.826, fur-
ther to 69.3% for BMI 26.129, and down
to 68.2% for BMI > 29 (P < 0.001). Of inter-
est, retrospective studies by Durnwald et
al
12
found that women who gained weight
between pregnancies had a lower VBAC
Maternal age alone
should not be used to
discourage women
from attempting a
vaginal delivery.
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 170 8/5/13 2:09 PM
Continuing Medical Education
JPOG JUL/AUG 2013 171
Table. Experience of Kwong Wah Hospital, Hong Kong, in the management of women with one prior caesarean
delivery
Year
2007 2008 2009 2010
Total no. of maternities 5,395 5,446 5,682 5,945
Women with prior caesarean
deliveries (%)
9.1 9.9 9.7 9.4
Successful planned VBAC rate (%) 94.3 88.5 86.3 87.2
Total VBAC rate (%) 21.1 21.9 22.9 26.9
Uterine rupture (%) 0 0.8 0 0
VBAC = vaginal birth after previous caesarean section.
rate. Women with normal BMI who turned
overweight (BMI 2529.9) before their
second pregnancy had a 56.6% success
rate; which was in contrast to the 74.2%
for women whose BMI remained normal (P
= 0.006).
12
Some authorities consider short
inter-delivery interval as a risk factor for
failed VBAC and uterine rupture. As uter-
ine rupture is an uncommon complication,
evidence from RCOG based on three small
observational studies suggested a two-
to threefold increased scar rupture risk
with inter-delivery interval below 1224
months.
5
THE DELIVERY
INTRAPARTUM
MANAGEMENT
Slight variations in opinion exist among
authorities on the standard of an institu-
tion offering VBAC. Nevertheless, they
are all in the direction that planned VBAC
should be conducted in a suitably staffed
and equipped delivery suite, with avail-
able resources for immediate laparotomy
and neonatal resuscitation.
Early diagnosis of uterine scar rup-
ture followed by expeditious laparotomy
and resuscitation is essential to reduce
adverse maternal and fetal outcome.
Although there is no single pathognomon-
ic clinical feature of the rupture, an abnor-
mal tracing on cardiotocography is present
in 5587% of the cases.
5
The RCOG and
SOGC recommend continuous electronic
fetal monitoring for all women attempting
VBAC.
Other associated features of uterine
rupture include acute onset of scar ten-
derness, severe abdominal pain persisting
between contractions, chest pain or shoul-
der tip pain, sudden onset of shortness of
breath, abnormal vaginal bleeding or hae-
maturia, cessation of previously efficient
uterine activity, maternal tachycardia, hy-
potension or shock, and loss of station of
the fetal presenting part.
5
For women attempting VBAC, mater-
nal and fetal condition should be regularly
assessed once labour begins. The likeli-
hood of vaginal delivery may be modified
by intrapartum conditions. Studies report-
ed that women admitted with a more fa-
vourable cervical status (eg, cervical dila-
tation > 4 cm, > 75% effacement) in spon-
taneous labour have a twofold increase in
success rate.
1315
Induction of labour for maternal or
fetal condition is increasingly common in
obstetric practice. Labour induction and
augmentation in women with previous
caesarean delivery is associated with an
increased risk of uterine rupture. The risk
is highest with misoprostol (6%), followed
by prostaglandin E
2
(2%) and lowest with
oxytocin (1.1%).
16
All the three organiza-
tions discouraged the use of misoprostol
and prostaglandins in most women with
previous caesarean delivery.
Studies on mechanical cervical ripen-
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 171 8/5/13 2:09 PM
JPOG JUL/AUG 2013 172
ing and labour induction with transcervical
catheter were limited and retrospective. The
risk on uterine rupture was inconclusive.
1
Induced labour is also associated
with lower success rate of VBAC com-
pared with spontaneous labour. If oxytocin
was used alone for induction or augmen-
tation, the vaginal delivery rate was 62%
(95% confidence interval, CI, 5370%) and
68% (95% CI, 6472%), respectively.
17

Although induction of labour is not
contraindicated in women with prior cae-
sarean delivery, the fact of lower success
rate together with higher rupture risk
should be considered in the counselling.
Nonetheless, individual circumstanc-
es must be considered in all cases. For ex-
ample, if a patient, who may not otherwise
be a candidate for planned VBAC, presents
in advanced labour, obstetricians may judge
it best to proceed with vaginal delivery.
MATERNAL BENEFIT AND
RISK
Both planned VBAC and ERCS carry mater-
nal and neonatal risks. The risks of either
approach include maternal haemorrhage,
infection, operative injury, thromboembo-
lism, hysterectomy, and death. The risk
of uterine rupture is essentially limited
to the VBAC group. As successful VBAC
is associated with the least complications
while a failed one with more complica-
tions, careful selection of women who
would most likely deliver vaginally is the
key to management of pregnancy after
prior caesarean section.
Short term
Successful VBAC offers several distinct,
consistently reproducible short-term ad-
vantages compared with ERCS, such as
fewer hysterectomies, fewer thromboem-
bolic events, lower blood transfusion rate,
and shorter hospital stay. However, when
VBAC fails, emergency caesarean section
is associated with increased uterine rup-
ture, hysterectomy, operative injury, blood
transfusion, endometritis, and longer hos-
pital stay.
18
At present, clinical prediction
of the optimal candidate for planned VBAC
remains imprecise.
Long term
It is well-known that caesarean delivery
increases the risk of long-term problems.
Chronic pain is a common condition expe-
rienced by some women after caesarean
delivery. The risk appears to be higher
with increasing number of operations
performed. A group in Denmark followed
up 244 women for chronic pain after cae-
sarean section. Of those women, 18.6%
had pain after 3 months and 12.3% still
suffered from pain at 10 months after de-
livery. Furthermore, 5.9% of the women
characterized their pain as being present
daily or almost daily.
19
One of the causes
of moderate to severe pelvic pain was
neuropathic pain caused by entrapment of
iliohypogastric or ilioinguinal nerves.
20
As with chronic pain, pelvic adhe-
sions increase with the number of cae-
sarean sections. Dense adhesions make
subsequent operation more difficult, and
increase the operating time, blood loss
and the risk of injury to the surrounding or-
gans.
20
Adhesions were assessed in a co-
hort study involving 3,190 women in Saudi
Arabia.
21
Adhesions were described as se-
vere if they were dense or causing fusion
of uterus to the abdominal wall or blad-
der. In that study, severe adhesions were
present in 0.2%, 11.5%, 26.0%, 44.8%,
54.5% and 50.6% of women having their
first, second, third, fourth, fifth, and sixth
or more caesarean sections, respectively.
A strong association exists between
previous caesarean delivery and abnormal
placentation. A meta-analysis performed
by Ananth et al
22
showed that a woman
with at least one previous caesarean
was at 2.6 times greater risk of placenta
previa in her subsequent pregnancy. The
risk increased with the number of prior
Both planned vaginal birth after
previous caesarean section and elective
repeat caesarean section carry maternal
and neonatal risks.
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 172 8/5/13 2:09 PM
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JPOG JUL/AUG 2013 173
Continuing Medical Education
caesarean sections.
The most significant long-term risk of
caesarean delivery is placenta accreta oc-
curring in subsequent pregnancies. As the
placenta does not properly separate from
the uterus after delivery, it may result in
massive bleeding, leading to disseminated
intravascular coagulopathy, multi-organ
failure and maternal mortality. Even if cae-
sarean hysterectomy is to be performed as
the life-saving procedure, the operation
could be difficult with its own set of com-
plications and results in permanent loss of
fertility.
20
As with previa, it is certain that
the risk of placental accreta increases with
the number of prior caesarean sections.
The combination of placenta previa and
previous caesarean delivery dramatically
increases the risk further. In a cohort study
of 723 women with placenta previa, ac-
creta occurred in 3%, 11%, 40%, 61% and
67% of those having their first, second,
third, fourth, and fifth or more caesarean
sections, respectively.
23
FETAL BENEFIT AND RISK
Perinatal morbidity and mortality are other
concerns when considering the option of
delivery. The largest population-based
evaluation of perinatal mortality was per-
formed by Smith et al.
24
The rate of deliv-
ery-related perinatal death was signifi-
cantly greater in the VBAC group than in the
ERCS group (12.9 vs 1.1 per 10,000 births).
The marked excess of perinatal deaths was
mainly due to uterine rupture in the VBAC
group. What further complicated the rela-
tive risk is that elective caesarean delivery
at 39 weeks gestation would prevent two
fetal deaths per 1,000 live births compared
with expectant management.
As with perinatal death, asphyxia-
related injury in VBAC usually occurs af-
ter uterine rupture. In a study by Landon
et al,
25
the incidence of hypoxic ischemic
encephalopathy was 0.08% in the VBAC
group compared with 0% in the ERCS
group, at a background uterine rupture rate
of 0.7%.
Sepsis is a frequent indication for
admission to the neonatal intensive care
unit. Both maternal fever and prolonged
rupture of membrane greater than 18 hours
are more common in the VBAC group.
Neonates who were born after failed VBAC
requiring emergency caesarean delivery
had a significantly greater rate of suspect-
ed sepsis.
26
Although the evidence suggests an
increased perinatal risk for women un-
dergoing VBAC, the absolute numbers of
serious morbidity and mortality remain
low. One should also note that a large
proportion of women undergoing VBAC
would deliver successfully. In fact, there
are numerous beneficial effects of labour
and vaginal delivery to the newborn. Even
at term gestation, babies born vaginally
are at lower risk of respiratory morbidity
(respiratory distress syndrome or transient
tachypnoea of newborn) compared with
those born by prelabour caesarean sec-
tion.
27,28
This finding was demonstrated by
a large cohort study
29
in Denmark involving
34,458 babies over 9 years. The risk of res-
piratory morbidity for infants delivered by
elective caesarean section was increased
compared with that by the vaginal route at
37 weeks gestation (odds ratio, OR, 3.9;
95% CI, 2.46.5), 38 weeks gestation (OR,
3.0; 95% CI, 2.14.3) and 39 weeks gesta-
tion (OR, 1.9; 95% CI, 1.23.0). A same
pattern was observed for risk of serious
respiratory morbidity at an even higher
odds ratio at 37 weeks gestation (OR 5.0;
95% CI, 1.616.0). This is the reason why
most authorities recommend that ERCS be
performed after 39 weeks gestation. Other
potential benefits of vaginal delivery are
the lower risk of hypothermia and hypoten-
sion at birth, higher likelihood of success-
ful breastfeeding, and lower incidence of
asthma in childhood.
28
NON-MEDICAL FACTORS
Despite the numerous research-based evi-
dence on the safety of trial of labour after
caesarean section, the VBAC rate remains
low in some developed countries such as
the United States (8.5% in 2006). It is ap-
parent that some non-clinical factors play
a role in the decision-making.
The factors which might affect VBAC
rate in an institution include administrative
policies, medicolegal pressures, profes-
sional society guidelines, and patient and
obstetrician preferences.
According to a survey by Wells
30
in the
United States, reasons for obstetricians
not offering VBAC included (1) them be-
ing unwilling to accept the risk of adverse
outcome, (2) them not believing that VBAC
is safe, (3) medico-legal liability concerns,
and (4) lack of immediate availability of fa-
cilities for laparotomy.
The decision may also be affected
by non-clinical patient priorities, such as
patients desire for a vaginal delivery, her
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 173 8/5/13 2:09 PM
JPOG JUL/AUG 2013 174
wish to experience (or avoid) labour, her
need for scheduling the delivery, and her
positive or negative feeling about the pre-
vious delivery.
31
It is important to discuss
with the women their risk perception and
priorities when considering the mode of
delivery.
COUNSELLING AND
DECISION-MAKING
Depending on the clinical situation, either
planned VBAC or ERCS is usually appropri-
ate for most women with prior caesarean
delivery. Understanding the womens be-
liefs and values of the process and out-
come is essential in providing evidence-
based, patient-centred care.
32
Discussing
the options in early pregnancy allows the
women and their family to evaluate the
risks and benefits based on their own
perception. While patient pamphlets play
an important role in general information-
giving, the obstetricians advice should
be more specific to the individuals condi-
tion. The decision made in early pregnancy
should never be considered final, as medi-
cal and social circumstances continue to
evolve. For instance, women who intend to
deliver by ERCS might present with spon-
taneous labour before the scheduled date.
If labour progresses well and the chance
of a successful VBAC is high, she might
change her preference to vaginal delivery.
According to the international guidelines,
ERCS should be scheduled after 39 weeks
gestation in an otherwise uncomplicated
pregnancy.
1
If a woman does not show any strong
preference regarding the mode of delivery
in early pregnancy, planned VBAC could be
encouraged with the flexibility of a later
change of plan. A local study has been
conducted to assess the psychological im-
pact of women being assigned to the mode
of delivery.
33
It involved 298 women, who
showed no preference for VBAC or ERCS,
being randomly assigned to either option
with flexibility. Women in the planned
VBAC group achieved high success rate of
vaginal delivery (73%) without an increase
in the psychological stress and morbidity.
EXPERIENCE FROM A PUBLIC
OBSTETRIC UNIT
Kwong Wah Hospital is one of the eight
public hospitals in the Hong Kong terri-
tory providing obstetric service. There are
almost 6,000 deliveries annually. Women
receive obstetric service at minimal per-
sonal cost, as the service is largely funded
by the government. Obstetricians and mid-
wives are salaried, and the advices given
are therefore not influenced by personal
financial gain. Women with history of
prior caesarean deliveries are assessed by
obstetricians at the booking visit. The pre-
vious operation records are traced via the
electronic system of the public hospitals
or by an enquiry letter to the correspond-
ing obstetricians/hospitals. Planned VBAC
is offered to women with one previous
uncomplicated transverse lower segment
caesarean delivery. Information pamphlets
on planned VBAC and ERCS are given out
during antenatal visits. The pregnancy
conditions are continuously reviewed. The
mode of delivery is discussed before 37
weeks gestation and documented in the
record. Women who have no strong pref-
erence for either option are encouraged to
consider planned VBAC when the pregnan-
cy conditions are favourable. Women who
intend to undergo ERCS are forewarned of
a possible re-evaluation of the mode of
delivery if they present themselves in an
advanced stage of labour. All women at-
tempting VBAC receive electronic continu-
ous fetal heart monitoring during labour.
Labour progress is assessed regularly by
the obstetricians. Facilities and expertise
for emergency operation are available in
the delivery suite. The authors experience
over a 4-year period is shown in the Table.
CONCLUSION
At present, there is no reliable formula
to calculate the best mode of delivery
in women with prior caesarean section.
This should be a shared decision made by
the obstetricians and the women, in-
corporating medical factors, social
Successful VBAC carries
the lowest risk,
followed by ERCS,
and the risk is highest
with failed VBAC,
requiring caesarean
delivery.
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 174 8/5/13 2:09 PM
Continuing Medical Education
JPOG JUL/AUG 2013 175
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circumstances and patients preferences.
Successful VBAC carries the lowest risk,
followed by ERCS, and the risk is high-
est with failed VBAC, requiring caesarean
delivery.
Although uterine rupture increases
the risk of adverse outcome, it is still a rare
complication in high-resource settings.
The incidence has remained the same
(< 1%) in UK, where the rate of primary
caesarean section has increased over the
past decade. Moreover, the resulting seri-
ous adverse outcome after uterine rupture
is very low in absolute number.
Given the high success rate of VBAC
in carefully selected pregnancies, obste-
tricians should not overstate the risk and
consequence of uterine rupture so as to
turn women away from attempting VBAC
without balancing the short- and long-
term complications of ERCS.
About the Authors
Dr Yung is Associate Consultant, Dr Lau is Consultant, and
Dr Leung is Chief of Service at the Department of Obstet-
rics and Gynaecology, Kwong Wah Hospital, Hong Kong.
JPOG_JulAug_2013_CME_ID_Final_MagneticOfPregWiPrevCaeSec.indd 175 8/5/13 2:09 PM
Please indicate on your answer sheet whether the following statements are True or False.
1. Previous classical caesarean section is a contraindication to planned vaginal birth after previous caesarean section
(VBAC).
2. Patients with unknown type of previous caesarean scar should not be offered planned VBAC.
3. Obese patients (body mass index, BMI, > 30) should not be offered planned VBAC, as high BMI is associated with a low
success rate.
4. Spontaneous labour increases the likelihood of successful VBAC.
5. Continuous electronic fetal monitoring should be offered to all women attempting VBAC.
6. Labour induction and augmentation by oxytocin are associated with increased risk of uterine rupture in patients with prior
caesarean delivery.
7. The risk of placenta previa/placenta accreta is directly related to the number of previous caesarean deliveries.
8. Perinatal mortality and asphyxia-related fetal injury are more signicant in the VBAC group compared with the elective
repeat caesarean section (ERCS) group.
9. Babies who are born vaginally are at lower risk of respiratory morbidity in both the short and long term.
10. Women who decline planned VBAC should be offered ERCS at 37 weeks gestation in order to avoid spontaneous labour
before the scheduled operation.
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