Is this neonatal small left colon syndrome (NSLCS) and what's the next step?

This kiddo read the book before
he was born!

Mom with maternal diabetes. 24 hour old baby born term, had bowel movement only with stimulation, and then started having bilious emesis. Exam and AXR were consistent with a distal bowel obstruction. UGI to r/o malrotation showed a normally located ligament of Trietz, while the contrast enema showed a small left colon with a transition point at the splenic flexure (above).

Dilemma!... Given the kid's history and the imaging findings, this is very likely NSLCS. But do we not need to be sure we're not missing Hirschsprung's disease?

Time for some PubMed action and an article titled: "Neonatal small left colon syndrome in the offsprings of diabetic mothers-an analysis of 105 children". Perfect!

In this article by Ellis et al, the authors looked at 105 patients born to mothers with diabetes in pregnancy (pregestational or gestational). Of these neonates, 6 had intestinal obstruction. 5 had NSLCS and one had Hirschsprung's disease (recto-sigmoid transition zone). The obstructive symptoms in the neonates with NSLCS resolved after a contrast enema.

The authors emphasized that 50% of infants born with NSLCS are born to mothers with diabetes during pregnancy. Additionally, when such infants are born with distal intestinal obstruction, the chance of having NSLCS vs. Hirschsprung's disease is >10:1. Given that NSLCS is a self limited disease that resolves with a diagnostic/theraputic contrast enema, it would seem reasonable to treat this baby expectantly, and perform a rectal biopsy if he does not resume a normal pattern of bowel function.

Reference:
Ellis H, Kumar R, Kostyrka B. Neonatal small left colon syndrome in the offsprings of diabetic mothers-an analysis of 105 children. Journal of Pediatric Surgery (2009)44,2343-2346