Infantile Hypertrophic Pyloric Stenosis

Tresara C. Bell, MD Kings County Medical Center 22 October, 2004

Pyloric Stenosis
Infantile hypertrophic pyloric stenosis (IHPS) is a condition that effects young infants. The pylorus becomes abnormally thickened and manifests as obstruction to gastric emptying. Infants with IHPS are clinically normal at birth, and subsequently develop nonbilious forceful (projectile) vomiting during the first few weeks of postnatal life. Gastric outlet obstruction leads to emaciation and, if left untreated, may result in death

Historical Perspective
Despite its frequency, it was virtually unknown prior to 1627, when a clinical description with survival was described by Fabricious Hildanus. Over the next 2 centuries, only 7 additional cases were described, some without pathologic proof and of doubtful origin. Harald Hirschsprung presented 2 infant girls with pathologically proved IHPS at the German Pediatric Congress in 1887. A profusion of scientific interest was triggered, and by 1910, 598 cases had been recognized. Even as late as 1905, its existence was still occasionally doubted.

Epidemiology
The incidence of IHPS is approximately 2 to 5 per 1,000 births per year in most white populations. Less common in India, and among black and Asian populations, with a frequency that is onethird to one-fifth that in the white population. The male-to-female ratio is approximately 4:1. There is a famlilial link, but a hereditary propensity to the development of IHPS is likely polygenic with no single locus accounting for the fivefold increase in the risk of first-degree relatives.

Epidemiology
Male and female children of affected fathers carry a risk of 5% and 2% respectively, of developing IHPS. Male and female children of affected mothers carry a risk of 20% and 7%, respectively of developing IHPS. Concordance in monozygotic twins is 0.25-0.44, and that in dizygotic twins is 0.05-0.10.

IHPS Anatomy
In IHPS the pyloric ring is no longer a clearly defined separation between the pyloric canal and duodenum. Instead the muscle of the pyloric antrum is hypertrophic (3 or more mm), which separates the normal antrum (1mm thickness) from the duodenum. The lumen is filled with compressed and redundant mucosa, which obstructs the passage of gastric contents.

IHPS Anatomy

Clinical Presentation
Varies with length of symptoms. Recent onset of forceful nonbilious vomiting, typically described as projectile. Frequency of vomiting is initially intermittent, but will progress to follow all feedings. Emesis may become blood tinged with protracted vomiting, likely related to gastritis. Since the child is unable to achieve adequate nutrition, he or she exhibits a voracious appetite.

Clinical Presentation
Starvation can exacerbate diminished hepatic glucoronyl transferase activity, and indirect hyperbilirubinemia may be seen in 1-2% of affected infants. Prolonged vomiting leads to the loss of large quantities of gastric secretions rich in H+ and Cl- . As a result of dehydration, the kidney attempts to conserve Na+ to maintain volume, by exchanging them for K+ and H+ (paradoxical aciduria). The net result is a loss of H+ and K+, which results in hypokalemic, hypochloremic metabolic acidosis.

Etiology
It has been found that, when compared to controls, in IHPS specimens, the muscle layer is deficient in: the quantity of nerve terminals markers for nerve-supporting cells peptide-containing nerve fibers nitric oxide synthase activity mRNA production for nitric oxide synthase Interstitial cells of Cajal
Ohshiro and Puri. Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress. Pediatr Surg Int (1998)13:243-252.

Etiology
IHPS specimens contain increases in:
Insulin-like growth factor platelet-derived growth factor

It is postulated that this abnormal innervation of the muscular layer leads to failure of relaxation of the pyloric muscle, increased synthesis of growth factors, and subsequent hypertrophy, hyperplasia, and obstruction. There is an increased incidence of IHPS in infants receiving erythromycin. The reason is unclear, although a prokinetic effect on gastric muscle contraction is postulated.

Etiology
The hypergastrinemia hypothesis proposes that an inherited increase in the number of parietal cells initiates a cycle of increased acid production, repeated pyloric contraction, and delayed gastric emptying.
Development of IHPS after initiation of feedings, increased postprandial gastrin levels, markedly increased gastric acid secretion in infants with IHPS, and the induction of IHPS in puppies after pentagastrin infusion support this hypothesis.

Diagnosis
Initially suggested by the typical clinical presentation. The mass is firm, mobile, approximately 2 cm, best palpated from the left, located in the midepigasrtrium beneath the liver edge. Palpation of the hard muscle mass or olive is diagnostic in conjunction with a typical history. Diagnosis by palpation of olive only successful 49% of cases in recent years vs. 78% 30 years ago. Palpation requires a calm infant with relaxed abdominal musculature, which is difficult in these hungry babies.
Macadesi and Oates. Clinical diagnosis of pyloric stenosis: a declining art. BMJ 1993;306:553-555.

Diagnosis
If the olive is not palpable in an infant who has a clinical picture suggestive of IHPS, further studies are warranted. Ultrasonography is used to measure the thickness of the pyloric wall and the length of the pyloric canal. normal wall thickness <2mm, IHPS >4 mm normal length of the pyloric canal <10 mm, IHPS >14 mm Sensitivity and specificity as high as 100%
Heller, et al. Application of new imaging modalities to the evaluation of common pediatric conditions. J Pediatr 1999; 135(5): 632-639.

Diagnosis
False-negative result can occur if antrum is measured. False-positive can occur is pyloric spasm is present. If ultrasound is not diagnostic and IHPS remains a concern, the next test of choice is an upper GI series.

Diagnosis
The canal is outlined by a string of contrast material coursing through spaces between redundant mucosa (string sign). Alternatively, there may be several linear tracts of contrast material separated by intervening mucosa (double-track sign). Mass impression on gastric antrum (shoulder sign) may be present.

Diagnosis
Upper endoscopy is used in rare occasions when other imaging modalities are inconclusive. Nasogastric aspirates after 3-4 hours of fasting. IHPS was present in 92% of patients with nasogastric aspirate of 10ml or more. GER was diagnosed in 86% of patients with nasogastric aspirates of less than 10ml.

Differential Diagnosis
Gastroesophageal reflux, with or without hiatal hernia. Differentiated by radiologic studies. Also amount of vomitus is smaller, and the infant does not usually lose weight. Adrenal insufficiency. Differentiated by absence of metabolic acidosis, hyperkalemia, and elevated urinary sodium. Viral gastroenteritis. Unusual in infants less than 6 weeks of age. Associated with significant diarrhea and sick contacts.

Treatment
The preoperative treatment is directed toward correcting the fluid, acid-base, and electrolyte losses. Intravenous fluid therapy is begun with 0.450.9% saline, in 510% dextrose, with the addition of potassium chloride in concentrations of 30 50 mEq/L. Fluid therapy should be continued until the infant is rehydrated and the serum bicarbonate concentration is less than 30 mEq/dL, which implies that the alkalosis has been corrected.

Treatment
Correction of the alkalosis is essential to prevent postoperative apnea, which may be associated with anesthesia Most infants can be rehydrated within 24 hours. Vomiting will usually stop once the stomach is empty. Occasionally an infant will require nasogastric suction. Once resuscitated the infant can undergo the Fredet-Ramstedt pylormyotomy, which is the procedure of choice.

Treatment
If the mucosa is entered (usually on the duodenal side), it can be primarily repaited and reinforced with an omental patch. Large perforations are managed by closing the pyloromyotomy, rotating the pylorus 90, and repeating the myotomy. Mortality and morbidity of less that 0.5%

Post-operative complications:
Wound infection Imcomplete myotomy, treated by repeat myotomy or endoscopic balloon dilation.

Treatment
Diet can be resumed within 6 to 12 hours postoperatively. Post-operative vomiting may occur in up to 50% of infants. Thought to be secondary to edema of the pylorus at the incision site. Most infants will tolerate full diet within 24 to 48 hours.

Treatment
Laparoscopic pyloromyotomy is performed primarily for improved cosmesis and shorter operative time, with comparable length of stay and morbidity. Decreased rate of duodenal perforation. Increased rate of incomplete myotomies and incisional hernias, requiring re-operation.
Yagmurlu, et al. Laparoscopic pyloromyotomy: a concurrent single institution series. J Pedatr Surg 2004;(39)3:292-296.

Treatment
Endoscopic balloon dilation has been used to treat IHPS. The seromuscular ring was not reliably disrupted to relieve the obstruction. Most patients failed balloon dilation and were treated with pyloromyotomy.
Ogawa, et al. Successful endoscopic balloon dilatation for hypertrophic pyloric stenosis. J Pediatr Surg 1996; (31)12:1712-1714. Hayashi, et a. Balloon catheter dilation for hypertrophic pyloric stenosis. J Pediatr Surg 1990; (25)11:1119-1121.

Treatment
Atropine sulfate has been used to treat IHPS with some success, however infants may not tolerate diet for at least 5 days. Surgery was necessary in 30% of the patients.
Yamatata, et al. Pyloromyotomy vs. atropine sulfate for infantile hypertrophic pyloric stenosis. J Pediatr Surg 2000; 35(2):338-41.