Abstract
BackgroundCongenital pyloric atresia (CPA) is a rare gastrointestinal anomaly frequently associated with epidermolysis bullosa (EB). Although the complications of familial isolated CPA are minor, delays in diagnosis can increase the chances of morbidity.Case presentationThree female infants born to a Japanese mother presented with CPA at birth. There was no consanguinity between the parents, and the spacing between pregnancies was 2 years in each case. All 3 siblings had a prenatal diagnosis of CPA owing to polyhydramnios and a dilated stomach, without dilatation of the rest of the gastrointestinal tract. All patients underwent reconstructive surgeries for establishing bowel continuity (Case 1, pyloromyotomy; Case 2, gastroduodenostomy in a diamond fashion; and Case 3, gastroduodenostomy in a side-to-side fashion) soon after birth. Their postoperative courses were uneventful, and they grew up healthily, without any complications.ConclusionFetal ultrasonography is useful for diagnosing CPA prenatally. Successful prenatal diagnosis can lead to timely intervention after birth.
Highlights
Congenital pyloric atresia (CPA) is a rare gastrointestinal anomaly frequently associated with epidermolysis bullosa (EB)
CPA can be classified into 3 subtypes: type A, pyloric membrane or web; type B, pylorus replaced by solid tissue; and type C, complete
CPA can be classified into 3 anatomical types: type A, which shows a pyloric membrane or web, which can be multiple (57%); type B, in which the pyloric channel is replaced by a solid cord (34%); and type C, which shows complete interruption between the stomach and the duodenum (9%) [3]
Summary
Congenital pyloric atresia (CPA) is a rare gastrointestinal anomaly that accounts for less than 1% of all intestinal atresias [1]. A nasojejunal tube was inserted for postoperative enteral feeding, and intestinal patency was confirmed with saline injection Her postoperative course was uneventful, except neonatal apnea, which resolved spontaneously by 2 weeks duodenotomy) without excision of the atretic segment. A nasojejunal tube was inserted, and patency of the intestine was confirmed with saline injection Her postoperative course was uneventful, and she achieved full oral intake 9 days postoperatively. Following decompression of the stomach, plain abdominal radiography and an upper gastrointestinal contrast study confirmed the diagnosis of CPA. Prenatal ultrasonography showed polyhydramnios (AFI: 27 cm) and a dilated fetal stomach (45 × 16 mm) at 30 weeks of gestation. Plain abdominal radiography and an upper gastrointestinal contrast study revealed complete obstruction of the pylorus, leading to the diagnosis of CPA. She is a healthy 6-year-old with normal growth and development
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