A 3.3 kg, 36-week baby girl was born prematurely after labor caused by ruptured membranes. The prenatal ultrasound revealed a polyhydramnios at 26 weeks. Fetal echocardiogram was normal and amniocentesis was without genetic aberrance. On examination there was a normal anus, an nasogastric tube (NGT) drained bile stained fluid. The baby passed some mucus from below but no typical dark meconium. A chest and abdominal x-ray showed a "double bubble sign". What is the most likely diagnosis?
The most common form of duodenal atresia is where the obstruction occurs below the ampulla of Vater. Hence bilious NGT output is reported in the scenario. Also the most common variant is characterized by a membranous intraluminal atresia (type I). Nearly 35% of babies with congenital duodenal obstruction have syndrome and of those a majority have associated cardiac defects. If the duodenal obstruction is incomplete, we call this a duodenal stenosis. Clinically this manifests much later in life and is characterized by failure to thrive, chronic vomiting, electrolyte abnormalities and is called a duodenal windsock variant. Here a membranous web in the most often second and third part of the duodenum is causing clinical symptoms of high, incomplete bowel obstruction. The treatment is resection of the web and a side-to-side duodenoduodenostomy.
Acute pancreatitis
Neonatal Hirschsprung's disease
Duodenal atresia
Malrotation of midgut
Plastic & Reconstructive Surgery