Duodenal atresia with familial apple peel syndrome: case study with review of literature

BMJ Case Rep. 2019 Aug 26;12(8):e230160. doi: 10.1136/bcr-2019-230160.

Abstract

This is a case report of a neonate who was antenatally diagnosed with jejunal atresia which turned out to be duodenal atresia with apple peel syndrome. A previous sibling, who also had apple peel but with jejunal atresia, succumbed to sepsis after surgery. The first sibling had jejunal stenosis and had died of sepsis following surgery. Combination of duodenal atresia with apple peel is extremely rare. This coupled with a familial condition is rarer still. This case was challenging due to the short length of the gut and prolonged need for total parenteral nutrition and sepsis in postoperative period.

Keywords: congenital disorders; neonatal and paediatric intensive care; paediatric surgery.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Diagnosis, Differential
  • Digestive System Surgical Procedures / methods*
  • Duodenal Obstruction* / diagnosis
  • Duodenal Obstruction* / etiology
  • Duodenal Obstruction* / physiopathology
  • Duodenal Obstruction* / surgery
  • Duodenum / abnormalities
  • Duodenum / diagnostic imaging
  • Duodenum / surgery
  • Female
  • Humans
  • Infant, Newborn
  • Intestinal Atresia* / diagnosis
  • Intestinal Atresia* / genetics
  • Intestinal Atresia* / physiopathology
  • Intestinal Atresia* / surgery
  • Jejunostomy / methods*
  • Jejunum / abnormalities
  • Jejunum / diagnostic imaging
  • Jejunum / surgery
  • Medical History Taking
  • Neonatal Sepsis* / diagnosis
  • Neonatal Sepsis* / etiology
  • Neonatal Sepsis* / therapy
  • Parenteral Nutrition, Total / methods
  • Pregnancy
  • Prenatal Diagnosis / methods
  • Rare Diseases / diagnosis
  • Siblings
  • Treatment Outcome