In our experience with 150 patients having Hirschsprung's disease, 2 patients have been encountered in whom the entire colon was deficient in ganglion cells. Diagnosis and treatment of this condition required variations from that followed in the usual cases of Hirschsprung's disease where the aganglionic segment is limited to the rectum and rectosigmoid.
The two patients with deficient Auerbach's plexus ganglion cells throughout the colon presented so bizarre a clinical picture in comparison with that typically encountered in congenital megacolon, that they posed a considerable diagnostic problem. Both of these infants were admitted to the hospital because of abdominal distention and vomiting. Roentgenograms with barium revealed the colon to be essentially normal in size, the abdominal distention being due to small bowel dilatation. There was some attempt to empty the colon; however, a portion of the barium was retained in the colon and was still there in one patient several
SWENSON O, FISHER JH. Treatment of Hirschsprung's Disease with Entire Colon Involved in Aganglionic Defect. AMA Arch Surg. 1955;70(4):535-538. doi:10.1001/archsurg.1955.01270100061010