Indian Pediatrics 2000;37: 205-207
Pyloric Atresia Associated with Intestinal Atresias
From the Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi 110 002, India.
Reprint requests: Dr. Yogesh Kumar Sarin, Associate Professor and Head, Department of Pediatric Surgery, Maulana Azad Medical College, New Delhi 110 002, India.
Manuscript Received: June 29, 1999;
Initial review completed: August 2, 1999;
Revision Accepted: August 18, 1999
Pyloric atresia is an extremely rare condition representing less than 1% of all atresias of the alimentary tract(1). Rarer still is the association of pyloric atresia with other intestinal atresias. Al-Salem et al. in 1997 could find only eight such cases reported in the literature prior to their case(2). Further, only 5 cases incuding theirs, had combination of pyloric atresia with duodenal atresia. One such unusual case of triple gastro-intestinal atresias encountered recently is being reported.
A 2100 g full term male newborn born to a primigravida mother through normal vaginal delivery was referred with features of abdominal distenson, respiratory distress and excessive salivation. Prenatal ultrasonography had revealed hydramnios and double-bubble picture suggestive of duodenal atresia. Examination revealed a 10´8 cm well-circumscribed mass in the right lower abdomen. The plain radio-graph of the abdomen showed only the gastric bubble, with the rest of the abdomen being opaque. An abdominal ultrasonography could not be done in the late hours of the night. The presence of esophageal atresia was ruled out as a 10 Fr red rubber catheter could be passed into the stomach. A free flow of frank bile was obtained on peritoneal aspiration done in the left lower quadrant. A presumptive diagnosis of peritonitis due to perforation of choledochal cyst or a communicating duplication cyst of intestine was made and the child was taken up for exploratory laparotomy. All the pre-operative biochemical investigations and the hemogram were within normal limits.
The intra-operative findings included triple atresia_pyloric atresia, duodenal atresia at duodeno-jejunal flexure, and atresia of the ascending colon. Although there was about 80 ml of free bile in the peritoneal cavity, no obvious gastro-intestinal perforation could be detected. The bile present in the peritoneum could be the result of persisting leak after the aspiration which may have punctured the dilated duodenum. The pancreas was seen spread over like a sheet on the medial aspect of the massively distended duodenum containing more than 150 ml of bile and pancreatic secretions. The jejunum and ileum were clogged with mucus plugs; however their patency could be ascertained when flushed with saline. The presence of rectal atresia was ruled out by administering saline per rectally.
The pyloric atresia was dealt with Mickulicz procedure. Because of the enormous size of the duodenum, reduction duodenoplasty and duodenojejunostomy were performed for the duodenal atresia. The colonic atresia necessi-tated divided terminal ileostomy. A penrose drain was put after a thorough peritoneal lavage. Post-operatively, the baby was electively ventilated for next four days. The baby continued to be oxygen-dependent. The baby was administered broad-spectrum antibiotics including metronidazole and total parenteral nutrition. No leakage was observed till the 10th post-operative day and the drain was removed. However, the general condition deteriorated with development of septicemia and sclerema and he died on the 13th post-operative day.
Pyloric atresia associated with other intestinal atresias is a rare occurrence. On review of English literature we could find three more references besides that cited by Al-Salem et al.(3-5). There are three distinct groups of pyloric atresias: (i) isolated pyloric atresia; (ii) pyloric atresia in association with other alimentary tract atresias; and (iii) pyloric atresia in association with genetic disorders such as epidermolysis bullosa and aplasia cutis congenita.
Although the prognosis of isolated pyloric atresia has improved remarkably in the last two decades, the outcome in the other two groups has been uniformly poor. None of the neonates born with pyloric atresia associated with other intestinal atresias have survived till date. The fact that two of these did well in respect of intestinal function before succumbing to sepsis, however, provides some reassurance(2,6). It is interesting to note that in five out of the six cases of pyloric atresia associated with duodenal atresia, the duodenal atresia was located distal to the ampulla of Vater(2,3). This combination
resulted, as in our case, in a closed loop obstruction into which bile and pancreatic secretions accumulated, causing massive distention and occasionally perforation and biliary peritonitis. Whereas the incidence of perforation in an isolated duodenal atresia is almost negligible, respiratory problems are common with pyloric atresia, and dyspnea, tachypnea, cyanosis and excessive salivation are often mistaken for instances of esophageal atresia(7,8).
Besides reporting a rarity, the purpose of this manuscript is to make aware the attending physician of this rare association as the underlying cause of an occasional case of neonatal biliary peritonitis or ascitis.
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