Hypertrophic Pyloric

Stenosis
 Hypertrophic Pyloric Stenosis
•Hypertrophic pyloric stenosis occurs in 1-3 per 1,000 infants
•It is more common in whites of northern European ancestry, less
common in blacks, and rare in Asians.
•Males are affected 4-6 times as often as females.
•The offspring of a mother and, to a lesser extent, the father who
had pyloric stenosis are at higher risk for pyloric stenosis
•The incidence of pyloric stenosis is increased in infants with B
and O blood groups.
•Pyloric stenosis is occasionally associated with other congenital
defects, including
1. tracheoesophageal fistula
 ETIOLOGY
The cause of pyloric stenosis is unknown, but many
factors have been implicated. Pyloric stenosis is usually
not present at birth and is more concordant in monozygotic
than dizygotic twins. It is unusual in stillbirths and probably
develops after birth
Pyloric stenosis has been associated with
◦ Eosinophilic gastroenteritis
◦ Apert syndrome
◦ Zellweger syndrome
◦ Trisomy 18
◦ Smith-Lemli-Opitz syndrome
 ETIOLOGY
Other Causes
An association has been found with the use of erythromycin in
neonates with highest risk within the 1st 2 wk of life.
Higher incidence of pyloric stenosis among mostly female infants
of mothers treated with macrolide antibiotics during pregnancy
and breastfeeding.
Abnormal muscle innervation,
Elevated serum levels of prostaglandins,
Infant hypergastrinemia
Reduced levels of neuronal nitric oxide synthase have been
 CLINICAL MANIFESTATIONS

Nonbilious vomiting is the initial symptom of pyloric

stenosis.
◦ The vomiting may or may not be projectile initially but is usually
progressive, occurring immediately after a feeding.
◦ Emesis might follow each feeding, or it may be intermittent.
◦ The vomiting usually starts after 3 wk of age, but symptoms
can develop as early as the 1st wk of life and as late as the 5th
mo
◦ After vomiting, the infant is hungry and wants to feed again.
◦ As vomiting continues, a progressive loss of fluid, hydrogen
 CLINICAL MANIFESTATIONS
Hyperbilirubinemia is the most common clinical association of pyloric
stenosis, also known as icteropyloric syndrome.
Unconjugated hyperbilirubinemia is more common than conjugated and
usually resolves with surgical correction. It may be associated with a
decreased level of glucuronyl transferase.
If conjugated hyperbilirubinemia is a part of the presentation, other
etiologies need to be investigated including
• eosinophilic gastroenteritis
• hiatal hernia
• peptic ulcer
• congenital nephrotic syndrome
• congenital heart disease
• congenital hypothyroidism
 Diagnosis
1.Clinical exam..
by palpating the pyloric mass. The mass is firm, movable, approximately 2
cm in length, olive shaped, hard, best palpated from the left side, and
located above and
to the right of the umbilicus in the midepigastrium beneath the
liver’s edge. The olive is easiest palpated after an episode of
vomiting. After feeding, there may be a visible gastric
peristaltic wave that progresses across the abdomen
 Diagnosis
2. Imaging studies
Ultrasound examination
Ultrasonography has a sensitivity of approximately 95%.
confirms the diagnosis in the majority of cases. Criteria for
diagnosis include
◦ pyloric thickness 3-4 mm
◦ pyloric length 15-19 mm
◦ pyloric diameter of 10-14 mm

Contrast studies demonstrate

◦ an elongated Pyloric channel (string sign)
◦ a bulge of the pyloric muscle into the antrum (shoulder sign)
◦ parallel streaks of barium seen in the narrowed channel,
string sign
shoulder sign
double tract sign
 DIFFERENTIAL DIAGNOSIS
1. Gastroesophageal reflux
2. Adrenal insufficiency
3. Inborn errors of metabolism (urea cycle) or acidosis (organic
acidemia)
4. Gastroenteritis
5. A pyloric membrane or pyloric duplication
6. Duodenal stenosis proximal to the ampulla of Vater
 TREATMENT

The preoperative treatment is directed toward correcting

◦ the fluid
◦ acid–base Correction of the alkalosis is essential to prevent
postoperative apnea, which may be associated with anesthesia.
◦ electrolyte losses.
Most infants can be successfully rehydrated within 24 hr. Vomiting
usually stops when the stomach is empty, and only an occasional
infant requires nasogastric suction
 TREATMENT
Surgery
The surgical procedure of choice is pyloromyotomy ( Ramstedt procedure )
Postoperative vomiting occurs in half the infants and is thought to be secondary to
edema of the pylorus at the incision site.
In most infants feedings can be initiated within 12-24 hr after surgery
advanced to maintenance oral feedings within 36-48 hr after surgery.
Persistent vomiting suggests
1. Incomplete pyloromyotomy,
2. Gastritis
3. Gastroesophageal reflux disease
4. Another cause of the obstruction

Endoscopic balloon dilation

has been successful in infants with persistent vomiting secondary to incomplete
 TREATMENT

Conservative management with

Nasoduodenal feedings is advisable In patients who are not good surgical
candidates.
Oral and intravenous atropine sulfate (pyloric muscle relaxant) has also
been described when surgical treatment is not available with 80% success
rate described in some studies.
Inconservative protocols Atropine is administered intravenously at a dose
of 0.01 mg/kg 6 times a day 5 min before feeding.
Oralfeeding is started at a volume of 10 mL formula, 6 times a day. The
volume is increased day by dayu ntil patients tolerate 150 mL/kg/day
unless vomiting occurs more than twice a day.