Authors: Ahmed H AlSalem
Publish Date: 2007/03/28
Volume: 23, Issue: 6, Pages: 559-563
Abstract
Congenital pyloric atresia CPA is a very rare condition that was first described by Calder in 1749 Commonly CPA occurs as an isolated lesion which has an excellent prognosis but it can also be seen in association with other malformations which can have a negative impact on the final outcome The medical records of all patients with the diagnosis of CPA treated at our hospital were retrospectively reviewed for age at diagnosis sex presenting symptoms history of polyhydramnios diagnosis associated anomalies operative findings treatment and outcome Eleven cases with the diagnosis of CPA were treated at our hospital There were five males and six females Seven were products of full term normal vaginal delivery and the remaining four were prematures Their birth weights ranged from 12 to 39 kg mean 22 kg Polyhydramnios was seen in seven 636 Associated anomalies were seen in six 545 These included epidermolysis bullosa EB in three hereditary multiple intestinal atresias HMIA in two including a duplication cyst in one of them esophageal atresia in one Down’s syndrome in one congenital heart disease in one cleft palate in one and aplasia cutis congenital ACC in one Intraoperatively five had pyloric diaphragms in two of them there were double diaphragms three had pyloric atresia with a gap between the two ends and two had pyloric atresia with no gap One of them had duodenal perforation as well as ileal perforation The patients with pyloric diaphragms had excision of diaphragms and Heineke–Mickulicz pyloroplasty Four of the other five patients had gastroduodenostomy One of them also had duodeno–jejunostomy due to associated distal duodenal atresia and excision of duplication cyst The patient with duodenal and ileal perforation and because of the marked distension of the duodenum had reduction duodenoplasty gastroduodenostomy and duodeno–jejunostomy The area of ileal perforation was resected and endtoend anastomosis was done after correction of the associated malrotation One patient with associated esophageal atresia had gastrostomy and gastrojejunostomy Postoperatively all did well initially but subsequently six of them died giving an overall survival of 455 Sepsis was the cause of death in all of them CPA is very rare and when it occurs in isolation it has a good prognosis The association of CPA with HMIA is universally fatal CPA in association with EB has a high mortality but there are encouraging results with the use of steroids and phenytoin Sepsis continues to be the main cause of death and an associated combined immunodeficiency should be excluded
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