SPR unknown #34 -- Final

Emma Boylan

Children's Memorial Hospital

Mary Wyers, MD

Children's Memorial Hospital


Hirschsprung's Disease, Total Colonic Hirschsprung's, aganglionosis, SPR unknown 34

Publication Date: 2010-07-09


Abdominal distention. History of prior upper GI several months previously.


Unknown 1 & 2: Initial abdominal radiographs with multiple distended loops of bowel, and subtle chronic retention of contrast material in the colon.

Unknown 3&4: Barium enema shows fairly uniform caliber of the colon with poor demarcation of the flexures (a “question mark” configuration)


total colonic Hirschsprung's


meconium plug syndrome, meconium ileus, small left colon syndrome, imperforate anus, megacystis-microcolon-intestinal hypoperistalsis syndrome


Hirschsprung’s disease, also called colonic aganglionosis, is a functional obstruction of the bowel found in neonates. It is caused by a congenital lack of intrinsic enteric ganglion cells [1]. Although Hirschsprung’s is a common cause of bowel obstruction in neonates, accounting for one-third of all cases, total colonic Hirschsprung’s is much rarer and makes up just 3-12% of Hirschsprung’s diagnoses [2, 3]. Total colonic Hirschsprung’s carries higher morbidity and mortality than short-segment type. However, long-term favorable outcomes have been reported with 82% of patients having favorable-range bowel movement frequency [3]. It is sssociated with Down syndrome (8%), genitourinary abnormalities (6%), gastrointestinal anomalies (4%), cardiac defects (8%) [2]. It can be treated by ERPT, Swenson’s operation, or Duhamel operation [3].

At plain radiography, TCH is characterized by multiple dilated loops of bowel and retention of contrast material in the colon [2]. Contrast radiography may show a rectosigmoid caliber ratio of <1, a transition zone, denervation hyperspasticity of the distal segment, and sawtooth configuration due to contractions; however, some studies have found that rectosigmoid index and transition zone “are not reliable signs to diagnose TCHD” [2, 1]. A microcolon, question mark-shaped colon, or normal colon may be seen, as may intraluminal calcifications in the right-lower quadrant [1, 4]. A contrast enema and clinical information may help to differentiate from meconium plug syndrome, meconium ileus, small left colon syndrome, imperforate anus, megacystis-microcolon-intestinal hypoperistalsis syndrome, or colonic, ileal, or distal jejunal atresia or stenoses [2]. Significant clinical features include family history, distended abdomen, and bilious emesis. Diagnosis is confirmed by biopsy [1].


  1. Stranzinger E, DiPietro MA, Teitelbaum DH, Strouse PJ (2008) Imaging of total colonic Hirschsprung disease. Pediatr Radiol 38: 1162-1170.
  2. Narla LD, Hingsbergen EA (2000) Case 22: Total colonic aganglionosis—long-segment Hirschsprung disease. Radiology 215: 391-394.
  3. Wildhaber BE, Teitelbaum DH, Coran AG (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40: 203-207.
  4. Cowles RA, Berdon WE, Holt PD, Buonomo C, Stolar CJ (2006) Noenatal intestinal obstruction simulating meconium ileus in infants with long-segment intestinal aganglionosis: Radiographic findings that prompt the need for rectal biopsy. Pediatr Radiol 36: 133-137.

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