Does Pre-Biopsy Contrast Enema Delay the Diagnosis of Long Segment Hirschsprung's Disease?

Abstract

Introduction: The diagnosis of long segment Hirschsprung's disease (LSHD) is frequently delayed. Our purpose was to: 1) summarize contrast enema (CE) findings in patients with LSHD, and 2) evaluate the utility of CE by comparing LSHD patients managed with/without pre-biopsy CE.

Methods: All LSHD cases (transition zone [TZ] proximal to the splenic flexure) treated between 1984 and 2009 were stratified according to whether a pre-biopsy CE was done (Group 1) or not (Group 2). CE were reviewed by a single pediatric radiologist, and the original reports were categorized as “helpful”, “inconclusive” or “misleading”. Group comparisons included elapsed days from admission to diagnostic rectal biopsy/first operation and initial hospitalization length of stay (LOS).

Results: 29 patients (16 in Group 1; 13 in Group 2) were identified. CE review revealed TZ in 7/16 (44%); and of these, 6 (86%) underestimated the actual aganglionic segment length. 6/16 (38%) original CE reports were “misleading”. Overall, Group 1 patients experienced a significant delay in time to biopsy (p=0.047), first operation (p=0.005), and showed a trend towards prolonged LOS.

Conclusions: Pre-biopsy CE offers little to the diagnosis of LSHD and may contribute to diagnosis/treatment delays. Even if a TZ is recognized in biopsy proven HD, the predicted aganglionic segment length should not guide the operative planning.

Key words

long segment Hirschsprung's disease - contrast enema - diagnosis - outcome

References

• 1 Haricharan RN, Georgeson KE. Hirschsprung disease.  Semin Pediatr Surg. 2008;  17 266-275
• 2 Martucciello G. Hirschsprung's disease, one of the most difficult diagnoses in pediatric surgery: a review of the problems from clinical practice to the bench.  Eur J Pediatr Surg. 2008;  18 140-149
• 3 O’Donovan AN, Habra G, Somers S. et al . Diagnosis of Hirschsprung's disease.  AJR Am J Roentgenol. 1996;  167 517-520
• 4 Diamond IR, Casadiego G, Traubici J. et al . The contrast enema for Hirschsprung disease: predictors of a false-positive result.  J Pediatr Surg. 2007;  42 792-795
• 5 Jamieson DH, Dundas SE, Belushi SA. et al . Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease?.  Pediatr Radiol. 2004;  34 811-815
• 6 de Lorijn F, Kremer LC, Reitsma JB. et al . Diagnostic tests in Hirschsprung disease: a systematic review.  J Pediatr Gastroenterol Nutr. 2006;  42 496-505
• 7 Proctor ML, Traubici J, Langer JC. et al . Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach.  J Pediatr Surg. 2003;  38 775-778
• 8 Langer JC, Durrant AC, de la Torre L. et al . One-stage transanal Soave pullthrough for Hirschsprung disease: a multicenter experience with 141 children.  Ann Surg. 2003;  238 569-583 discussion 583–585
• 9 Somme S, Langer JC. Primary versus staged pull-through for the treatment of Hirschsprung disease.  Semin Pediatr Surg. 2004;  13 249-255
• 10 Dasgupta R, Langer JC. Hirschsprung disease.  Curr Probl Surg. 2004;  41 942-988
• 11 Moore SW. Total colonic aganglionosis and Hirschsprung's disease: shades of the same or different?.  Pediatr Surg Int. 2009;  25 659-666
• 12 De Campo JF, Mayne V, Boldt DW, De Campo M. Radiological findings in total aganglionosis coli.  Pediatr Radiol. 1984;  14 205-209
• 13 Stranzinger E, DiPietro MA, Teitelbaum DH, Strouse PJ. Imaging of total colonic Hirschsprung disease.  Pediatr Radiol. 2008;  38 1162-1170

Correspondence