PYLORIC STENOSIS

AFUGBUOM CHIBUEZE IKENNA

IM-640
 INTRODUCTION

Pyloric stenosis, also known as infantile hypertrophic

pyloric stenosis (IHPS), is an uncommon condition in
infants characterized by abnormal thickening of the pylorus
muscles in the stomach leading to gastric outlet obstruction.

Clinically infants are well at birth. Then, at 3 to 6 weeks of

age, the infants present with "projectile" vomiting,
potentially leading to dehydration and weight loss
 ETIOLOGY
The exact etiology of infantile hypertrophic pyloric stenosis is unknown.
Some studies have shown that young infants treated with macrolide
antibiotics had an increased incidence of infantile hypertrophic pyloric
stenosis.

Postnatal exposure to erythromycin has also been associated with an

increased risk for the development of pyloric stenosis. Other risk factors
include bottle feeding, preterm birth, cesarean section delivery, and first-
born infants (30% to 40% of cases). If the mother was a heavy
smoker during pregnancy, the risk of hypertrophic pyloric stenosis could
increase by 1.5. to 2.0 fold.
 PATHOPHYSIOLOGY

The hallmark of pyloric stenosis is marked hypertrophy and

hyperplasia of both the circular and longitudinal muscular layers of
the pylorus. This thickening leads to the narrowing of the lumen of
the gastric antrum.

The pyloric canal becomes lengthened. The muscles of the pylorus

become thickened. The mucosa becomes edematous and
thickened. When severe, the stomach becomes dilated secondary to
gastric outlet obstruction. This obstruction induces immediate
postprandial, nonbilious, projectile emesis.
HISTORY AND PHYSICAL EXAMINATION

Infants with pyloric stenosis classically present with projectile, non-bilious

vomiting. Vomiting may be intermittent or occur after each feeding. Emesis
should not be bilious.
In about 60% to 80% of the infants with pyloric stenosis, a firm, non-
tender, hard pylorus measuring 1 to 2 cm in diameter, classically described
as an "olive," may be present in the right upper quadrant. The clinician may
also observe reverse peristaltic waves.
Infants may show dehydration. Signs of dehydration in infants are:
depressed fontanelles, dry mucous membranes, decreased tearing, poor skin
turgor, and lethargy
 HISTORY AND PHYSICAL
EXAMINATION
The classic electrolyte imbalance of pyloric stenosis is
hyperchloremic, hypokalemic metabolic alkalosis. The diagnosis of
pyloric stenosis is being made earlier because of earlier use of
sonography so those laboratory abnormalities are now present in less
than 50% of cases.
Dehydration may cause either hypernatremia or hyponatremia; both
can lead to prerenal renal failure. Elevated unconjugated bilirubin
levels may be present.
 EVALUATION
Ultrasonography has become the standard imaging technique for diagnosing hypertrophic pyloric
stenosis. It is reliable, highly sensitive, highly specific, and easily performed. An experienced
ultrasonographer is necessary to diagnose pyloric stenosis

A pyloric wall thickness of 3 mm or greater and a pyloric channel length of 15 mm or greater

are considered abnormal and indicate pyloric stenosis. Ultrasound findings also include target signs and
lack of gastric emptying.

Barium upper can help to diagnose pyloric stenosis when ultrasonography is not diagnostic but is rarely
necessary. It should demonstrate an elongated pylorus and can show marked delayed gastric emptying.
Findings may include a string sign, double-track sign, or beak sign.

Sometimes, upper endoscopy, performed by a pediatric gastroenterologist, can be used as an additional

diagnostic tool in rare, select cases when the other imaging tests are nondiagnostic or when the infant
presents with atypical clinical features.

Abdominal X-ray lacks sensitivity and specificity significantly. Findings may include large stomach gas
with the absence of gas in the colon secondary to gastric obstruction. Caterpillar sign may be present,
which is a radiological finding that shows a dilated stomach appearance secondary to hypertrophied
pylorus gastric contractions.
 DIFFERENTIAL DIAGNOSIS
Midgut volvulus: This is a twisting of the malrotated bowel, which can present in the first
month of life with bilious vomiting. An upper GI series will show a "corkscrew" appearance
of the bowel. An abdominal CT scan will show an inverted relationship between the superior
mesenteric artery and the superior mesenteric vein.

Gastroenteritis: It also involves episodes of vomiting that are typically non-bilious, but it is
often associated with anorexia, fever, lethargy and diarrhoea. Plain abdominal films may show
dilated small and large bowel loops

Urinary tract infection: UTI may present with fever, foul-smelling urine, lethargy, frequent
and painful urination, anorexia and vomiting. Urinalysis and culture establish the diagnosis.
Plain film in UTI may reveal an ileus (lack of movement) pattern; ultrasound or CT may show
evidence of urologic abnormality or pyelonephritis in advanced cases.

Other diseases that could be differentiated include acute renal failure, sepsis, hernia, colic,
constipation, necrotizing enterocolitis, trauma, toxic megacolon, Hirschsprung disease,
testicular torsion, and appendicitis.
 TREATMENT

First, medical treatment is necessary and usually consists of rehydration and correction of
electrolyte imbalances. If no or mild signs of dehydration are evident, 5% dextrose with
0.25% NaCl and 2 meq KCl per 100 mL is given.

If moderate or severe, recommend higher IVF NaCl concentrations. Bicarbonate levels
should be corrected and monitored, given the impact on potential hypoventilation. NG tube
should be considered.

Once the infant is rehydrated, surgery is the next step.

Treatment is surgical and is called pyloromyotomy. In this surgery, the pyloric muscle is
divided down to the submucosa. The surgery can be performed open or laparoscopically,
depending on the surgeon. The operation is curative and has very low morbidity.