Pyloric atresia associated with duodenal and jejunal atresia and duplication

Abstract

A case of congenital pyloric atresia associated with duodenal atresia, jejunal atresia, and a duplication is described. A review of the literature revealed nine previously reported cases of congenital pyloric atresia and multiple intestinal atresias, but this is the first report of such an association and a duplication.

Case of Epidermolysis Bullosa with Pyloric Atresia

“…Pyloric atresia is a rare congenital disease that causes partial or complete obliteration of the gastric outlet known to occur in less than 1% of gastrointestinal atresias6,7. Since the first report in 19688, some cases of EB with pyloric atresia have been reported.…”

Peculiar type 1 congenital pyloric atresia: a case report

“…[3-6] PA may be also part of hereditary multiple intestinal atresias (HMIAs) involving the stomach, duodenum, jejunum, ileum, colon, and rectum. This form has an extremely rare occurrence and a pathogenesis that is still speculative, including the possibility of a combined immunodeficiency syndrome [7-9]. The presence of associated anomalies is a contributing factor for the reported high mortality [10].…”

“…Its incidence is approximately 1 in 100,000 newborns and constitutes about 1% of all intestinal atresias [1,2]. Sometimes it occurs with genetic disorders like epidermolysis bullosa and aplasia cutis congenital [3-6] or in association with other atresias of the gastrointestinal tract [7-9]. The presence of associated anomalies is a contributing factor for the reported high mortality [10].…”

Stomach

“…Chen and Emil (2008) summarized these cases and categorized them into three groups: (1) pyloric atresia as an isolated abnormality; (2) pyloric atresia in association with other intestinal atresias; (3) pyloric atresia in association with other disorders including the esophagus (Müller et al 1990;Centikursun et al 1955;Okoye et al 2000); duodenum (Haller and Cahill 1968;Al-Salem 1999;Chen and Emil 2008); and the jejunum, rectum, and colon (Guttman et al 1973;Puri et al 1985;Al-Salem et al 1997). Chen and Emil (2008) summarized these cases and categorized them into three groups: (1) pyloric atresia as an isolated abnormality; (2) pyloric atresia in association with other intestinal atresias; (3) pyloric atresia in association with other disorders including the esophagus (Müller et al 1990;Centikursun et al 1955;Okoye et al 2000); duodenum (Haller and Cahill 1968;Al-Salem 1999;Chen and Emil 2008); and the jejunum, rectum, and colon (Guttman et al 1973;Puri et al 1985;Al-Salem et al 1997).…”