Duodenal atresia and stenosis

Abstract

Congenital duodenal atresia and stenosis is a common cause of intestinal obstruction in the neonate, with an incidence of 1 in 5000 to 10,000 live births and an increased prevalence in males. More than 50% of affected patients have associated congenital anomalies, including annular pancreas, intestinal malrotation, esophageal atresia, Meckel's diverticulum, imperforate anus, renal anomalies, lesions of the central nervous system, and biliary tract malformations. The most common anomalies associated with duodenal atresia include Trisomy 21, diagnosed in one third of patients, and isolated cardiac defects, which occur in approximately 30%. The presence of trisomy carries an increased risk for congenital heart defects requiring operative repair. Approximately 45% of patients are born prematurely, with one-third exhibiting failure to thrive and persistent growth retardation. Presently, laparoscopic or open duodenoduodenostomy has become the standard of care, with survival rates of greater than 95% and mortality primarily attributed to associated anomalies of other organ systems.