SPR unknown #34 -- Final
Publication Date: 2010-07-09
total colonic Hirschsprung's
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 . 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 . It is sssociated with Down syndrome (8%), genitourinary abnormalities (6%), gastrointestinal anomalies (4%), cardiac defects (8%) . It can be treated by ERPT, Swenson’s operation, or Duhamel operation .
At plain radiography, TCH is characterized by multiple dilated loops of bowel and retention of contrast material in the colon . 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 . Significant clinical features include family history, distended abdomen, and bilious emesis. Diagnosis is confirmed by biopsy .
- Stranzinger E, DiPietro MA, Teitelbaum DH, Strouse PJ (2008) Imaging of total colonic Hirschsprung disease. Pediatr Radiol 38: 1162-1170.
- Narla LD, Hingsbergen EA (2000) Case 22: Total colonic aganglionosis—long-segment Hirschsprung disease. Radiology 215: 391-394.
- Wildhaber BE, Teitelbaum DH, Coran AG (2005) Total colonic Hirschsprung’s disease: a 28-year experience. J Pediatr Surg 40: 203-207.
- 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.