How to manage low gut obstruction in neonates with immature ganglion cells in the colonic wall?

BACKGROUND

Neonates with immature ganglion cells in the colonic wall may have the clinical picture similar to Hirschsprung's disease, especially total colonic aganglionosis. Management of this entity depends on the judgment of each clinician.

OBJECTIVE

The aim of this study was to review management of clinical low gut obstruction in neonates with presence of immature ganglion cells in the colon.

METHODS

A retrospective study of neonates with clinical low gut obstruction due to presence of immature ganglion cells treated between 2007 and 2012 was reviewed.

RESULTS

Six patients, one term and 5 pre-term neonates, were proven to have immature ganglion cells in the colonic wall. They presented with delay or failure to pass meconium, progressive abdominal distension and bilious vomiting after birth. Abdominal films showed generalized small bowel dilatation and barium enemas revealed a microcolon in 4 of them. They underwent laparotomy between 4 and 11 days of life. A microcolon with a transitional zone (Tz) was seen at the terminal ileum, 30-75 cm proximal to the ileocecal valve. Colonic biopsy and the appendix revealed presence of immature ganglion cells and ileal biopsy at the Tz showed presence of normal ganglion cells. An ileostomy was performed at the Tz in all of the 5 premature neonates, while an enterostomy was not done in term infant. Closure of the enterostomy in the 5 premature cases was performed after they had been proven to have mature ganglion cells in the colonic wall by a rectal biopsy after the age of 3 months. All of the 6 cases were doing well on the last follow-up between 1 and 3 years.

CONCLUSIONS

Functional low gut obstruction in neonates caused by immaturity of the colonic ganglion cells should be managed by laparotomy including biopsies of the colon, appendix and terminal ileum with enterostomy at the Tz. Closure of the enterostomy is done after presence of mature ganglion cells proven by a rectal biopsy after 3 months of age. Full-term neonates with immature ganglion cells in the colonic wall may be successfully managed conservatively without enterostomy.