Skip to main content

Advertisement

SpringerLink
Log in

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

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. McDonald RG, Evans WA (1954) Hirschsprung’s disease; roentgen diagnosis in infants. AMA Am J Dis Child 87:575–585

    CAS  PubMed  Google Scholar 

  2. Kosloske AM, Goldthorn JF (1984) Early diagnosis and treatment of Hirschsprung’s disease in New Mexico. Surg Gynecol Obstet 158:233–237

    CAS  PubMed  Google Scholar 

  3. Stensrud KJ, Emblem R, Bjørnland K (2012) Late diagnosis of Hirschsprung disease — patient characteristics and results. J Pediatr Surg 47:1874–1879

    Article  Google Scholar 

  4. Berman CZ (1956) Roentgenographic manifestation of congenital megacolon (Hirschprung’s disease) in early infancy. Pediatrics 18:227–228

    CAS  PubMed  Google Scholar 

  5. Hayakawa K, Hamanaka Y, Suzuki M et al (2003) Radiological findings in total colon aganglionosis and allied disorders. Radiat Med 21:128–134

    PubMed  Google Scholar 

  6. Sheng TW, Wang CJ, Lo WC et al (2012) Total colonic aganglionosis: reappraisal of contrast enema study. J Radiol Sci 37:11–19

    Google Scholar 

  7. Basnet A, Zheng S (2006) Total colonic aganglionosis: diagnosis and treatment. World J Pediatr 2:97–101

    Google Scholar 

  8. 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 140:881–884

    CAS  PubMed  Google Scholar 

  9. Ouladsaiad M (2016) How to manage a late diagnosed Hirschsprung's disease. Afr J Paediatr Surg 13:82–87

    Article  Google Scholar 

  10. 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 38:412–416

    Article  Google Scholar 

  11. 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:e12451

    Article  Google Scholar 

  12. Peyvasteh M, Askarpour S, Ostadian N et al (2016) Diagnostic accuracy of barium enema findings in Hirschprung’s disease. Arq Bras Cir Dig 29:155–158

    Article  Google Scholar 

  13. O’Donovan AN, Habra G, Somers S et al (1996) Diagnosis of Hirschsprung’s disease. AJR Am J Roentgenol 167:517–520

    Article  Google Scholar 

  14. 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 7:11–17

    Article  CAS  Google Scholar 

  15. Swenson O, Neuhauser EB, Picklett LK (1949) New concepts of the etiology, diagnosis and treatment of congenital megacolon (Hirschprung's disease). Pediatrics 4:201–209

    CAS  PubMed  Google Scholar 

  16. Hope JW, Borns PF, Berg PK (1965) Roentgenologic manifestations of Hirschprung's disease in infancy. Am J Roengenol Radium Ther Nucl Med 95:217–229

    Article  CAS  Google Scholar 

  17. Rosenfield NS, Ablow RC, Markowitz RI et al (1984) Hirschsprung’s disease: accuracy of the barium enema examination. Radiology 150:393–400

    Article  CAS  Google Scholar 

  18. Smith GHH, Cass D (1991) Infantile Hirschsprung’s disease — is barium enema useful? Pediatric Surg Int 6:318–321

    Google Scholar 

  19. 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 63:80–85

    Article  Google Scholar 

  20. Lockhart-Mummery HE, Morson BC (1964) Crohn’s disease of the large intestine. Gut 5:493–509

    Article  CAS  Google Scholar 

  21. Snyder MJ (2004) Imaging of colonic diverticular disease. Clin Col Rect Surg 17:155–162

    Article  Google Scholar 

  22. Lee CC, Lien R, Chian MC et al (2012) Clinical impacts of delayed diagnosis of Hirschsprung’s disease in newborn infants. J Pediatr Neonatol 53:133–137

    Article  Google Scholar 

  23. 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 32:723–727

    Article  Google Scholar 

  24. 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 58:265–281

    Article  Google Scholar 

  25. 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 38:775–778

    Article  CAS  Google Scholar 

  26. Jamieson DH, Dundas SE, Belushi SA et al (2004) Does the transition zone reliably delineate aganglionic bowel in Hirschsprung's disease? Pediatr Radiol 34:811–815

    Article  Google Scholar 

  27. 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 28:597–601

    Article  Google Scholar 

  28. 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 33:581–586

    Article  Google Scholar 

  29. 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 54:2028–2031

    Article  Google Scholar 

  30. 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 60:606–612

    Article  CAS  Google Scholar 

Download references

Acknowledgements

Special thanks to Prof. Martin Kidd from the Centre of Statistical Consultation, Department of Statistics and Actuarial Sciences, Stellenbosch University.

Author information

Authors and Affiliations

  1. Division of Radiodiagnosis, Department of Medical Imaging and Clinical Oncology, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

    Sucari S. C. Vlok & Richard D. Pitcher

  2. Division of Paediatric Surgery, Department of Surgical Sciences, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

    Sam W. Moore

  3. Division of Anatomical Pathology, Department of Pathology, Faculty of Medicine and Health Sciences, Stellenbosch University and Tygerberg Hospital, Cape Town, South Africa

    Pawel T. Schubert

Authors
  1. Sucari S. C. Vlok
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sam W. Moore
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Pawel T. Schubert
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Richard D. Pitcher
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sucari S. C. Vlok.

Ethics declarations

Conflicts of interest

None

Additional information

Publisher’s note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Online Resource 1

(JPG 39 kb)

Online Resource 2

(JPG 43 kb)

Online Resource 3

(XLSX 50 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Vlok, S.S.C., Moore, S.W., Schubert, P.T. et al. Accuracy of colonic mucosal patterns at contrast enema for diagnosis of Hirschsprung disease. Pediatr Radiol 50, 810–816 (2020). https://doi.org/10.1007/s00247-020-04631-2

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-020-04631-2

Keywords

Search

Navigation