Skip to main content
Top
Published in: Pediatric Radiology 6/2020

01-05-2020 | Original Article

Accuracy of colonic mucosal patterns at contrast enema for diagnosis of Hirschsprung disease

Authors: Sucari S. C. Vlok, Sam W. Moore, Pawel T. Schubert, Richard D. Pitcher

Published in: Pediatric Radiology | Issue 6/2020

Login to get access

Abstract

Background

Various patterns of colonic mucosal irregularity have been recorded on contrast enema, each with individually very low sensitivity, but high specificity.

Objective

To assess the accuracy of the radiologic features of Hirschsprung disease utilising a unifying stratification of any form of colonic mucosal irregularity on contrast enema.

Materials and methods

We conducted a retrospective study of children with suspected Hirschsprung disease managed at a tertiary South African hospital from January 2009 through April 2015. Three observers independently reviewed abdominal radiographs and contrast enemas. The enema analysis included a unifying category of any form of colonic mucosal irregularity. Radiologic features were compared with rectal biopsy results. We used descriptive statistics and the Fisher exact test to compare the radiologic features of children with and without Hirschsprung disease.

Results

Ninety-two children with median age of 37 days (range 3 days to 11 years) were included; 50 had biopsy-proven Hirschsprung disease. On enema, any mucosal irregularity, a transition zone and recto-sigmoid ratio inversion were associated with Hirschsprung disease (all P<0.01). Mucosal irregularity showed 96% sensitivity (95% confidence interval [CI] 86.3–99.5) and 71.4% specificity (CI 55.4–84.3); a transition zone showed 86% sensitivity (CI 73.3–94.2) and 90.5% specificity (CI 77.4–97.3); and recto-sigmoid ratio inversion showed 78% sensitivity (CI 64.0–88.5) and 83.3% specificity (CI 68.3–93.0).

Conclusion

Colonic mucosal irregularity on contrast enema has high sensitivity and moderate specificity for Hirschsprung disease.
Appendix
Available only for authorised users
Literature
1.
go back to reference McDonald RG, Evans WA (1954) Hirschsprung’s disease; roentgen diagnosis in infants. AMA Am J Dis Child 87:575–585PubMed McDonald RG, Evans WA (1954) Hirschsprung’s disease; roentgen diagnosis in infants. AMA Am J Dis Child 87:575–585PubMed
2.
go back to reference Kosloske AM, Goldthorn JF (1984) Early diagnosis and treatment of Hirschsprung’s disease in New Mexico. Surg Gynecol Obstet 158:233–237PubMed Kosloske AM, Goldthorn JF (1984) Early diagnosis and treatment of Hirschsprung’s disease in New Mexico. Surg Gynecol Obstet 158:233–237PubMed
3.
go back to reference Stensrud KJ, Emblem R, Bjørnland K (2012) Late diagnosis of Hirschsprung disease — patient characteristics and results. J Pediatr Surg 47:1874–1879CrossRef Stensrud KJ, Emblem R, Bjørnland K (2012) Late diagnosis of Hirschsprung disease — patient characteristics and results. J Pediatr Surg 47:1874–1879CrossRef
4.
go back to reference Berman CZ (1956) Roentgenographic manifestation of congenital megacolon (Hirschprung’s disease) in early infancy. Pediatrics 18:227–228PubMed Berman CZ (1956) Roentgenographic manifestation of congenital megacolon (Hirschprung’s disease) in early infancy. Pediatrics 18:227–228PubMed
5.
go back to reference Hayakawa K, Hamanaka Y, Suzuki M et al (2003) Radiological findings in total colon aganglionosis and allied disorders. Radiat Med 21:128–134PubMed Hayakawa K, Hamanaka Y, Suzuki M et al (2003) Radiological findings in total colon aganglionosis and allied disorders. Radiat Med 21:128–134PubMed
6.
go back to reference Sheng TW, Wang CJ, Lo WC et al (2012) Total colonic aganglionosis: reappraisal of contrast enema study. J Radiol Sci 37:11–19 Sheng TW, Wang CJ, Lo WC et al (2012) Total colonic aganglionosis: reappraisal of contrast enema study. J Radiol Sci 37:11–19
7.
go back to reference Basnet A, Zheng S (2006) Total colonic aganglionosis: diagnosis and treatment. World J Pediatr 2:97–101 Basnet A, Zheng S (2006) Total colonic aganglionosis: diagnosis and treatment. World J Pediatr 2:97–101
8.
go back to reference Taxman TL, Yulish BS, Rothstein FC (1986) How useful is the barium enema in the diagnosis of infantile Hirschsprung’s disease? Am J Dis Child 140:881–884PubMed Taxman TL, Yulish BS, Rothstein FC (1986) How useful is the barium enema in the diagnosis of infantile Hirschsprung’s disease? Am J Dis Child 140:881–884PubMed
9.
go back to reference Ouladsaiad M (2016) How to manage a late diagnosed Hirschsprung's disease. Afr J Paediatr Surg 13:82–87CrossRef Ouladsaiad M (2016) How to manage a late diagnosed Hirschsprung's disease. Afr J Paediatr Surg 13:82–87CrossRef
10.
go back to reference Lewis NA, Levitt MA, Zallen GS et al (2003) Diagnosing Hirschsprung’s disease: increasing the odds of a positive rectal biopsy result. J Pediatr Surg 38:412–416CrossRef Lewis NA, Levitt MA, Zallen GS et al (2003) Diagnosing Hirschsprung’s disease: increasing the odds of a positive rectal biopsy result. J Pediatr Surg 38:412–416CrossRef
11.
go back to reference Alehossein M, Roohi A, Pourgholami M et al (2015) Diagnostic accuracy of radiologic scoring system for evaluation of suspicious Hirschsprung disease in children. Iran J Radiol 12:e12451CrossRef Alehossein M, Roohi A, Pourgholami M et al (2015) Diagnostic accuracy of radiologic scoring system for evaluation of suspicious Hirschsprung disease in children. Iran J Radiol 12:e12451CrossRef
12.
go back to reference Peyvasteh M, Askarpour S, Ostadian N et al (2016) Diagnostic accuracy of barium enema findings in Hirschprung’s disease. Arq Bras Cir Dig 29:155–158CrossRef Peyvasteh M, Askarpour S, Ostadian N et al (2016) Diagnostic accuracy of barium enema findings in Hirschprung’s disease. Arq Bras Cir Dig 29:155–158CrossRef
13.
go back to reference O’Donovan AN, Habra G, Somers S et al (1996) Diagnosis of Hirschsprung’s disease. AJR Am J Roentgenol 167:517–520CrossRef O’Donovan AN, Habra G, Somers S et al (1996) Diagnosis of Hirschsprung’s disease. AJR Am J Roentgenol 167:517–520CrossRef
14.
go back to reference Meier-Ruge W, Lutterbeck PM, Herzog B et al (1972) Acetylcholinesterase activity in section biopsies of the rectum in the diagnosis of Hirschsprung’s disease. J Pediatr Surg 7:11–17CrossRef Meier-Ruge W, Lutterbeck PM, Herzog B et al (1972) Acetylcholinesterase activity in section biopsies of the rectum in the diagnosis of Hirschsprung’s disease. J Pediatr Surg 7:11–17CrossRef
15.
go back to reference Swenson O, Neuhauser EB, Picklett LK (1949) New concepts of the etiology, diagnosis and treatment of congenital megacolon (Hirschprung's disease). Pediatrics 4:201–209PubMed Swenson O, Neuhauser EB, Picklett LK (1949) New concepts of the etiology, diagnosis and treatment of congenital megacolon (Hirschprung's disease). Pediatrics 4:201–209PubMed
16.
go back to reference Hope JW, Borns PF, Berg PK (1965) Roentgenologic manifestations of Hirschprung's disease in infancy. Am J Roengenol Radium Ther Nucl Med 95:217–229CrossRef Hope JW, Borns PF, Berg PK (1965) Roentgenologic manifestations of Hirschprung's disease in infancy. Am J Roengenol Radium Ther Nucl Med 95:217–229CrossRef
17.
go back to reference Rosenfield NS, Ablow RC, Markowitz RI et al (1984) Hirschsprung’s disease: accuracy of the barium enema examination. Radiology 150:393–400CrossRef Rosenfield NS, Ablow RC, Markowitz RI et al (1984) Hirschsprung’s disease: accuracy of the barium enema examination. Radiology 150:393–400CrossRef
18.
go back to reference Smith GHH, Cass D (1991) Infantile Hirschsprung’s disease — is barium enema useful? Pediatric Surg Int 6:318–321 Smith GHH, Cass D (1991) Infantile Hirschsprung’s disease — is barium enema useful? Pediatric Surg Int 6:318–321
19.
go back to reference Marei MM, Abdelsattar AH, Yassin TM et al (2015) Reducing the frequency of unnecessary rectal biopsies by combined interpretation of clinical and radiological findings in Egyptian children with suspected Hirschsprung’s disease. Gaz Egypt Paediatr Assoc 63:80–85CrossRef Marei MM, Abdelsattar AH, Yassin TM et al (2015) Reducing the frequency of unnecessary rectal biopsies by combined interpretation of clinical and radiological findings in Egyptian children with suspected Hirschsprung’s disease. Gaz Egypt Paediatr Assoc 63:80–85CrossRef
20.
go back to reference Lockhart-Mummery HE, Morson BC (1964) Crohn’s disease of the large intestine. Gut 5:493–509CrossRef Lockhart-Mummery HE, Morson BC (1964) Crohn’s disease of the large intestine. Gut 5:493–509CrossRef
21.
go back to reference Snyder MJ (2004) Imaging of colonic diverticular disease. Clin Col Rect Surg 17:155–162CrossRef Snyder MJ (2004) Imaging of colonic diverticular disease. Clin Col Rect Surg 17:155–162CrossRef
22.
go back to reference Lee CC, Lien R, Chian MC et al (2012) Clinical impacts of delayed diagnosis of Hirschsprung’s disease in newborn infants. J Pediatr Neonatol 53:133–137CrossRef Lee CC, Lien R, Chian MC et al (2012) Clinical impacts of delayed diagnosis of Hirschsprung’s disease in newborn infants. J Pediatr Neonatol 53:133–137CrossRef
23.
go back to reference De Haro JI, Palazón Bellver P, Julia Masip V et al (2016) Effectiveness of calretinin and role of age in the diagnosis of Hirschsprung disease. Pediatr Surg Int 32:723–727CrossRef De Haro JI, Palazón Bellver P, Julia Masip V et al (2016) Effectiveness of calretinin and role of age in the diagnosis of Hirschsprung disease. Pediatr Surg Int 32:723–727CrossRef
24.
go back to reference Tabbers MM, DiLorenzo C, Berger MY et al (2014) Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 58:265–281CrossRef Tabbers MM, DiLorenzo C, Berger MY et al (2014) Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr 58:265–281CrossRef
25.
go back to reference Proctor ML, Traubici J, Langer JC et al (2003) Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: implications for surgical approach. J Pediatr Surg 38:775–778CrossRef Proctor ML, Traubici J, Langer JC et al (2003) Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: implications for surgical approach. J Pediatr Surg 38:775–778CrossRef
26.
go back to reference Jamieson DH, Dundas SE, Belushi SA et al (2004) Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease? Pediatr Radiol 34:811–815CrossRef Jamieson DH, Dundas SE, Belushi SA et al (2004) Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease? Pediatr Radiol 34:811–815CrossRef
27.
go back to reference Muller CO, Mignot C, Belarbi N et al (2012) Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung's disease? Pediatr Surg Int 28:597–601CrossRef Muller CO, Mignot C, Belarbi N et al (2012) Does the radiographic transition zone correlate with the level of aganglionosis on the specimen in Hirschsprung's disease? Pediatr Surg Int 28:597–601CrossRef
28.
go back to reference Chen X, Xiaojuan W, Zhang H et al (2017) Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung's disease. Pediatr Surg Int 33:581–586CrossRef Chen X, Xiaojuan W, Zhang H et al (2017) Diagnostic value of the preoperatively detected radiological transition zone in Hirschsprung's disease. Pediatr Surg Int 33:581–586CrossRef
29.
go back to reference Moore SW, Maluleke T, El Hosny AA (2019) Is Hirschsprung disease a purely neurological condition? A study of the actin G2 smooth muscle gene in Hirschsprung disease. J Pediatr Surg 54:2028–2031CrossRef Moore SW, Maluleke T, El Hosny AA (2019) Is Hirschsprung disease a purely neurological condition? A study of the actin G2 smooth muscle gene in Hirschsprung disease. J Pediatr Surg 54:2028–2031CrossRef
30.
go back to reference Budianto IR, Obata S, Kinoshita Y et al (2015) Reevaluation of acetylcholinesterase staining for the diagnosis of Hirschsprung disease and allied disorders. J Pediatr Gastroenterol Nutr 60:606–612CrossRef Budianto IR, Obata S, Kinoshita Y et al (2015) Reevaluation of acetylcholinesterase staining for the diagnosis of Hirschsprung disease and allied disorders. J Pediatr Gastroenterol Nutr 60:606–612CrossRef
Metadata
Title
Accuracy of colonic mucosal patterns at contrast enema for diagnosis of Hirschsprung disease
Authors
Sucari S. C. Vlok
Sam W. Moore
Pawel T. Schubert
Richard D. Pitcher
Publication date
01-05-2020
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 6/2020
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-020-04631-2

Other articles of this Issue 6/2020

Pediatric Radiology 6/2020 Go to the issue