Pseudo Hirschsprung's disease.

THE DEVELOPMENT of a successful surgical treatment for Hirschsprung's disease 11 and the establishment of a sound pathologic and etiologic basis 3 for the disease have been among the most gratifying events in Pediatric Surgery of the last decade. The result has been to render accurate diagnosis possible with precision. At the same time the knowledge that an effective surgical treatment is available has perhaps led to rather more frequent early surgical consultation in children with obstinate constipation. In true Hirschsprung's disease, as Hirschsprung 5 reported (in his original two cases, difficulty with defecation is noted from birth), there may be delay in the first passage of meconium. If a reliable history in the obstetric nursery is available, distention, constipation, and the necessity for enemas will be noted. regularly. The condition persists or progresses. The children develop large protuberant abdomens. The costal margins flare. Fecal masses are regularly palpable in the abdomen. The phildren rarely have spontaneous stools and then only hard seybali. Flatus is passed annoyingly frequently, and in large amounts. A successful enema yields enormous quantities of stool followed for perhaps a day or two by inadequate evacuations and then obdurate constipation. Pain is not common. Distention may be so extreme, as in infants, to cause death from respiratory embarrassment, with diaphragm so high that the diagnosis of eventration has been made. Repeated hospital admissions are required for crises of constipation. With assiduous home care and regular enemas such crises may be made infrequent but are rarely altogether eliminated. Incontinence does not occur. At times ulceration of the distended bowel-described and correctly interpreted by Hirschsprung as secondary to stasis and erosion by fecal masses-results in bloody diarrhea in the face of continued,distention and palpable abdominal fecal masses. Ordinarily sphincter tone is normal, the rectal ampulla is empty, and not remarkable. The radiographic picture, clearly described by Neuhauser and Swenson,10 invariably demonstrates a normal sized or narrow rectal segment, and a dilated proximal sigmoid or descending colon. Hirschsprung, once more, plainly described at autopsy the normal sized or narrowed rectum, and the sharp transition to a huge sigmoid. If doubt remains as to the diagnosis, biopsy of the rectal wall will show an absence of the ganglion cells in the myenteric plexus. We have encountered four types of patients referred for treatment of megacolon in whom the difficulty lay elsewhere than in the congenital absence of ganglion cells of the myenteric plexuses of a segment of the rectum or of the colon and rectum. 1) Pseudo Hirschsprung's disease on a psychogedc basis in otherwise organically normal children. 2) Megacolon and obstinate constipation in mentally defective children. 3) Megacolon and obstinate constipation associated with organic anal obstruction.