Hirschsprung’s Disease with Bowel Duplication A Rarest of Rare Anomalous Association

Submit Manuscript | http://medcraveonline.com infant developed progressive abdominal distention while under observation in the neonatal ICU. On examination, the infant looked generally well, maintaining on room air, vital signs were normal and no dismorphic features. Abdomen was distended all over, there was no visible peristalsis or visible bowel loops with normal overlying skin. It was soft and lax, with no palpable mass, but had exaggerated bowel sounds. Digital rectal examination revealed a normal anal orifice with normal muscle tone. Baby passed little sticky meconium after rectal stimulation, but no gush of gas or stool on withdrawal of finger. A preliminary clinical impression of meconium plug syndrome was made, and conservative treatment started including fleet enema, but unfortunately, the condition of the patient did not improve in spite of repeated enemas. Blood counts, Hemoglobin, serum chemistry, renal functions were within normal values. Liver function showed unconjugated hyperbilirubinemia, but other parameters were normal. Plain abdominal x ray revealed dilated bowel loops all over the abdomen, no air fluid levels, no free intraperitoneal gas, no intramural gas, and no evidence of any mass lesion. However it revealed diffuse gaseous distention of colon, more prominent on the left side. Gastrograffin contrast enema study was undertaken, for diagnostic as well as therapeutic purposes, due to suspicion of meconium plug syndrome. It revealed classic cone shaped narrowing in recto sigmoid area, with proximal dilatation of bowel and distal narrowing suggesting the presence of Hirschsprung’s disease (Figure 1).