Download PDF
Eur J Pediatr Surg 2016; 26(02): 207-214
DOI: 10.1055/s-0035-1546755
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Contrast Enema for Hirschsprung Disease Investigation: Diagnostic Accuracy and Validity for Subsequent Diagnostic and Surgical Planning

Giovanni Frongia
1   Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
,
Patrick Günther
1   Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
,
Jens-Peter Schenk
2   Division of Pediatric Radiology, Department of Diagnostic and Interventional Radiology, University Hospital of Heidelberg, Heidelberg, Germany
,
Kai Strube
1   Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
,
Markus Kessler
1   Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
,
Arianeb Mehrabi
3   Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
,
Philipp Romero
1   Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, University Hospital of Heidelberg, Heidelberg, Germany
› Author Affiliations
Further Information

Publication History

30 July 2014

16 December 2014

Publication Date:
24 March 2015 (online)

Also available at
   Permissions and Reprints

Abstract

Introduction A targeted Hirschsprung disease (HD) diagnostic is necessary, as it determines a specific approach primarily based on surgical resection of the affected aganglionic colonic segment. The aim of this study was to evaluate the diagnostic accuracy of a contrast enema (CE) for HD diagnosis and to determine whether it should be performed before or after rectal biopsies (RBs).

Methods A retrospective observational study of children undergoing RB for HD investigation was performed. In the performed CE, the occurrence and the level of a colonic caliber change (CCC) were recorded and its concordance with the histologically assessed level of aganglionosis by RB and the odds ratio were calculated.

Results A total of 107 cases were included. Sensitivity and specificity for a CCC in CE were 74.1% and 94.6%. A CCC present in CE was associated with a 50-fold increased probability for a histologically proven HD. The overall concordance between a CCC and the histologically assessed level of aganglionosis was high (kappa 0.642, p = 0.003), being correct in 94.4% of cases when the CCC was located in the rectosigmoid, but only in 50% of cases when it was located in more proximal segments. By performing a CE only after HD diagnosis confirmation by RB would avoid 67.5% of CE with no loss of diagnostic accuracy.

Conclusion We confirm that CE is a valuable tool for HD diagnosis; however, it should only be performed for subsequent diagnostic and surgical planning following histological confirmation of HD by RB. On the basis of this, an algorithm for an optimized investigation and management of HD is presented.

Keywords

Hirschsprung disease - contrast enema - rectal biopsy - diagnostic accuracy
 

  • References

  • 1 Haricharan RN, Seo JM, Kelly DR , et al. Older age at diagnosis of Hirschsprung disease decreases risk of postoperative enterocolitis, but resection of additional ganglionated bowel does not. J Pediatr Surg 2008; 43 (6) 1115-1123
    CrossrefPubMedGoogle Scholar
  • 2 White FV, Langer JC. Circumferential distribution of ganglion cells in the transition zone of children with Hirschsprung disease. Pediatr Dev Pathol 2000; 3 (3) 216-222
    CrossrefPubMedGoogle Scholar
  • 3 Lewis NA, Levitt MA, Zallen GS , et al. Diagnosing Hirschsprung's disease: increasing the odds of a positive rectal biopsy result. J Pediatr Surg 2003; 38 (3) 412-416 , discussion 412–416
    CrossrefPubMedGoogle Scholar
  • 4 Martucciello G, Pini Prato A, Puri P , et al. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies. J Pediatr Surg 2005; 40 (10) 1527-1531
    CrossrefPubMedGoogle Scholar
  • 5 Moore SW, Johnson G. Acetylcholinesterase in Hirschsprung's disease. Pediatr Surg Int 2005; 21 (4) 255-263
    CrossrefPubMedGoogle Scholar
  • 6 Das K, Kini U, Babu MK, Mohanty S, D'Cruz AJ. The distal level of normally innervated bowel in long segment colonic Hirschsprung's disease. Pediatr Surg Int 2010; 26 (6) 593-599
    CrossrefPubMedGoogle Scholar
  • 7 de Lorijn F, Kremer LC, Reitsma JB, Benninga MA. Diagnostic tests in Hirschsprung disease: a systematic review. J Pediatr Gastroenterol Nutr 2006; 42 (5) 496-505
    CrossrefPubMedGoogle Scholar
  • 8 Diamond IR, Casadiego G, Traubici J, Langer JC, Wales PW. The contrast enema for Hirschsprung disease: predictors of a false-positive result. J Pediatr Surg 2007; 42 (5) 792-795
    CrossrefPubMedGoogle Scholar
  • 9 Noviello C, Cobellis G, Romano M, Amici G, Martino A. Diagnosis of Hirschsprung's Disease: an age-related approach in children below or above one year. Colorectal Dis 2010; 12 (10) 1044-1048
    CrossrefPubMedGoogle Scholar
  • 10 Garcia R, Arcement C, Hormaza L , et al. Use of the recto-sigmoid index to diagnose Hirschsprung's disease. Clin Pediatr (Phila) 2007; 46 (1) 59-63
    CrossrefPubMedGoogle Scholar
  • 11 Pochaczevsky R, Leonidas JC. The “recto-sigmoid index”. A measurement for the early diagnosis of Hirschsprung's disease. Am J Roentgenol Radium Ther Nucl Med 1975; 123 (4) 770-777
    CrossrefPubMedGoogle Scholar
  • 12 Seigel DG, Podgor MJ, Remaley NA. Acceptable values of kappa for comparison of two groups. Am J Epidemiol 1992; 135 (5) 571-578
    PubMedGoogle Scholar
  • 13 De la Torre-Mondragón L, Ortega-Salgado JA. Transanal endorectal pull-through for Hirschsprung's disease. J Pediatr Surg 1998; 33 (8) 1283-1286
    CrossrefPubMedGoogle Scholar
  • 14 De La Torre L, Langer JC. Transanal endorectal pull-through for Hirschsprung disease: technique, controversies, pearls, pitfalls, and an organized approach to the management of postoperative obstructive symptoms. Semin Pediatr Surg 2010; 19 (2) 96-106
    CrossrefPubMedGoogle Scholar
  • 15 Maerzheuser S, Bassir C, Rothe K. Hirschsprung disease in the older child: diagnostic strategies. Clin Pediatr (Phila) 2012; 51 (11) 1087-1090
    CrossrefPubMedGoogle Scholar
  • 16 Reid JR, Buonomo C, Moreira C, Kozakevich H, Nurko SJ. The barium enema in constipation: comparison with rectal manometry and biopsy to exclude Hirschsprung's disease after the neonatal period. Pediatr Radiol 2000; 30 (10) 681-684
    CrossrefPubMedGoogle Scholar
  • 17 Stranzinger E, DiPietro MA, Teitelbaum DH, Strouse PJ. Imaging of total colonic Hirschsprung disease. Pediatr Radiol 2008; 38 (11) 1162-1170
    CrossrefPubMedGoogle Scholar
  • 18 AWMF online. Available at: http://www.awmf.org/leitlinien/detail/ll/006-001.html . Accessed March 16, 2015
    PubMedGoogle Scholar
  • 19 Esayias W, Hawaz Y, Dejene B , et al. Barium enema with reference to rectal biopsy for the diagnosis and exclusion of Hirschsprung disease. East Cent Afr J Surg 2013; 18: 141-145
    PubMedGoogle Scholar
  • 20 Smith GH, Cass D. Infantile Hirschsprung's disease, is a barium enema useful?. Pediatr Surg Int 1991; 6: 318-321
    PubMedGoogle Scholar
  • 21 Swenson O, Sherman JO, Fisher JH. Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 1973; 8 (5) 587-594
    CrossrefPubMedGoogle Scholar
  • 22 Taxman TL, Yulish BS, Rothstein FC. How useful is the barium enema in the diagnosis of infantile Hirschsprung's disease?. Am J Dis Child 1986; 140 (9) 881-884
    PubMedGoogle Scholar
  • 23 Pini-Prato A, Avanzini S, Gentilino V , et al. Rectal suction biopsy in the workup of childhood chronic constipation: indications and diagnostic value. Pediatr Surg Int 2007; 23 (2) 117-122
    CrossrefPubMedGoogle Scholar
  • 24 Alizai NK, Batcup G, Dixon MF, Stringer MD. Rectal biopsy for Hirschsprung's disease: what is the optimum method?. Pediatr Surg Int 1998; 13 (2-3) 121-124
    CrossrefPubMedGoogle Scholar
  • 25 Ghosh A, Griffiths DM. Rectal biopsy in the investigation of constipation. Arch Dis Child 1998; 79 (3) 266-268
    CrossrefPubMedGoogle Scholar
  • 26 Rahman N, Chouhan J, Gould S , et al. Rectal biopsy for Hirschsprung's disease—are we performing too many?. Eur J Pediatr Surg 2010; 20 (2) 95-97
    Article in Thieme ConnectPubMedGoogle Scholar
  • 27 Langer JC. Laparoscopic and transanal pull-through for Hirschsprung disease. Semin Pediatr Surg 2012; 21 (4) 283-290
    CrossrefPubMedGoogle Scholar
  • 28 Knowles CH, De Giorgio R, Kapur RP , et al. Gastrointestinal neuromuscular pathology: guidelines for histological techniques and reporting on behalf of the Gastro 2009 International Working Group. Acta Neuropathol 2009; 118 (2) 271-301
    CrossrefPubMedGoogle Scholar
  • 29 Aldridge RT, Campbell PE. Ganglion cell distribution in the normal rectum and anal canal. A basis for the diagnosis of Hirschsprung's disease by anorectal biopsy. J Pediatr Surg 1968; 3 (4) 475-490
    Google Scholar
  • 30 Tafazzoli K, Soost K, Wessel L, Wedel T. Topographic peculiarities of the submucous plexus in the human anorectum—consequences for histopathologic evaluation of rectal biopsies. Eur J Pediatr Surg 2005; 15 (3) 159-163
    Google Scholar
  • 31 Athow AC, Filipe MI, Drake DP. Problems and advantages of acetylcholinesterase histochemistry of rectal suction biopsies in the diagnosis of Hirschsprung's disease. J Pediatr Surg 1990; 25 (5) 520-526
    CrossrefPubMedGoogle Scholar
  • 32 Dobbins III WO, Bill Jr AH. Diagnosis of Hirschsprung's disease excluded by rectal suction biopsy. N Engl J Med 1965; 272: 990-993
    CrossrefPubMedGoogle Scholar
  • 33 Jamieson DH, Dundas SE, Belushi SA, Cooper M, Blair GK. Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease?. Pediatr Radiol 2004; 34 (10) 811-815
    PubMedGoogle Scholar
  • 34 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 (5) 775-778
    CrossrefPubMedGoogle Scholar
  • 35 De Campo JF, Mayne V, Boldt DW, De Campo M. Radiological findings in total aganglionosis coli. Pediatr Radiol 1984; 14 (4) 205-209
    CrossrefPubMedGoogle Scholar
  • 36 Marquez TT, Acton RD, Hess DJ, Duval S, Saltzman DA. Comprehensive review of procedures for total colonic aganglionosis. J Pediatr Surg 2009; 44 (1) 257-265 , discussion 265
    CrossrefPubMedGoogle Scholar
  • 37 Miyano G, Ochi T, Lane GJ, Okazaki T, Yamataka A. Factors affected by surgical technique when treating total colonic aganglionosis: laparoscopy-assisted versus open surgery. Pediatr Surg Int 2013; 29 (4) 349-352
    CrossrefPubMedGoogle Scholar
  • 38 Muensterer OJ, Chong A, Hansen EN, Georgeson KE. Single-incision laparoscopic endorectal pull-through (SILEP) for Hirschsprung disease. J Gastrointest Surg 2010; 14 (12) 1950-1954
    CrossrefPubMedGoogle Scholar