Diagnostic Marathon (Hirschsprun0g´s Disease)

obst_22a_n.jpg: Operative findings in a 1-year-old boy with constipation on laparotomy in the left lower abdomen, with a view from the right side of the patient: On the left side the rectum is dippig into the pelvis which continues after a funnel-shaped transitional zone into a grotesquely dilated sigmoid segment (on the right side and at the top). During surgery the diagnosis of a classic aganglionosis is more than obvious by the described findings and the intraoperatively performed biopsies. Compare the grotesquely dilated sigmoid segment with the following work-up examinations. obst_22b_n.jpg: Second of several preoperative contrast enemas dating further back. The transverse colon is filled with air. Contrast within the rectal ampulla and sigmoid. In spite of a $$obst_2??££pathological rectal biopsy§§ and absent relaxations of the internal sphincter muscle as in figure obst_21a_n.jpg and obst_21b_n.jpg the diagnosis of Hirschsprung's disease has been discarded because both contrast enema were interpreted as normal. Because a second, this time surgical biopsy in general anesthesia yielded findings compatible with Hirschsprung's disease, a pediatric surgeon was finally consulted. obst_22c_n.jpg and obst_22d_n.jpg: Additional pictures of the former contrast enema. obst_22c_n.jpg: Plain X-ray prior to a further contrast enema with the colon filled with air; on the right side of the spinal column an overdistended intestinal loop without haustration is visible. obst_22d_n.jpg: Also with contrast filling the enormously dilated intestinal loop is visible in the same place. obst_22c_n.jpg and obst_22d_n.jpg: Although a transitional zone is not clearly recognizable, the diagnosis of Hirschsprung's disease is obvious from the constant presentation of a megacolon (in this case of a megasigmoid). Compare with $$obst_15??££the enema in idiopathic constipation.§§ The operation as well as the work-up examinations belong in the hands of an experienced pediatric surgeon who is used to compare the radiological with the intraoperative findings, and who knows the value of the other additonal examinations.