Pediatric Surgery International

, Volume 19, Issue 4, pp 247–250 | Cite as

Hirschsprung's disease: the Australian Paediatric Surveillance Unit's experience

  • S. J. Singh
  • G. D. H. Croaker
  • P. Manglick
  • C. L. Wong
  • H. Athanasakos
  • E. Elliott
  • D. Cass
Original Article

Abstract.

Hirschsprung's disease (HD) was introduced into the Australian Paediatric Surveillance Unit (APSU) system in 1996 with the objective to collect data on demographics, incidence, family history, associated anomalies, clinical features, investigation, and surgical treatment in Australia. Children under 15 years of age with HD (confirmed by biopsy) were entered into a database maintained at The Children's Hospital at Westmead in Sydney. Nationwide, 127 children with HD were reported from January 1997 to December 2000, and near-complete information was available on 126. Neonatal presentation was seen in 114 cases (90%), whereas 12 (10%) presented post-neonatally. There was a history of delayed passage of meconium (more than 24 h) in 65 of the 114 patients (57%) who presented in the neonatal period. In those presenting post-neonatally there was no history of delayed passage of meconium. Primary pull-through was performed in 42 of the 82 patients (51%) operated upon. The Soave procedure was performed in 53 of the total 82 (65%) operations and 29 of 42(69%) primary pull-through operations. Pre-operative enterocolitis was seen in 15 of 126 (12%) and postoperative enterocolitis in 17 of 82 (21%) children. The majority of children with HD are diagnosed in the neonatal period (90%). Primary pull-through is becoming more popular (51%). The Soave procedure is the most commonly performed operation (65%) in Australia. Pre- and post-operative enterocolitis is still a significant problem.

Keywords.

Hischsprung's disease Pull-through procedure Surveillance 

References

  1. 1.
    Kleinhaus S, Boley SJ, Sheran M, Sieber WK (1979) Hirschsprung's disease – a survey of the members of the Surgical Section of the American Academy of Pediatrics. J Pediatr Surg 14: 588–597PubMedCrossRefGoogle Scholar
  2. 2.
    Ikeda K, Goto S (1984) Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients. Ann Surg 199: 400–405PubMedCentralPubMedCrossRefGoogle Scholar
  3. 3.
    Spouge D, Baird PA (1985) Hirschsprung disease in a large birth cohort. Teratology 32: 171–177PubMedCrossRefGoogle Scholar
  4. 4.
    Rajab A, Freeman NV, Patton MA (1997) Hirschsprung's disease in Oman. J Pediatr Surg 32: 724–727PubMedCrossRefGoogle Scholar
  5. 5.
    Carneiro PMR, Brereto RJ, Drake DP (1992) Enterocolitis in Hirschsprung's disease. Pediatr Surg Int 7: 356–360CrossRefGoogle Scholar
  6. 6.
    Goldberg E (1991) An epidemiological study of Hirschsprung's disease. Int J Epidemiol 13: 479–485CrossRefGoogle Scholar
  7. 7.
    Passage E (1967) The genetics of Hirschprung's disease. N Eng J Med 276: 138–143CrossRefGoogle Scholar
  8. 8.
    Russell MB, Russell CA, Niebuhr E (1994) An epidemiological study of Hirschsprung's disease and additional anomalies. Acta Paediatr 83: 68–71PubMedCrossRefGoogle Scholar
  9. 9.
    Spouge D, Baird PA (1985) Hirschsprung disease in a large birth cohort. Teratology 32: 171–177PubMedCrossRefGoogle Scholar
  10. 10.
    Births (1999) Australian Bureau of Statistics, Sydney, 3301.0, p 35Google Scholar
  11. 11.
    Bill AH, Chapman ND (1962) The enterocolitis of Hirschsprung's disease: Its natural history and treatment. Am J Surg 103: 70–74CrossRefGoogle Scholar
  12. 12.
    Elhalaby EA, Coran AG, Blane CE, Hirschl RB, Teitelbaum DH (1995) Enterocolitis associated with Hirschsprung's disease: a clinical-radiological characterisation based on 168 patients. J Pediatr Surg 30: 76–83PubMedCrossRefGoogle Scholar
  13. 13.
    Fujimoto T, Puri P (1988) Persistence of enterocolitis following diversion of fecal stream in Hirschsprung's disease. A study of mucosal defence mechanisms. Pediatr Surg Int 3: 141–146Google Scholar
  14. 14.
    Shanbhogue LLR, Bianchi A (1990) Experience with primary Swenson resection and pull-through for neonatal Hirschsprung's disease. Pediatr Surg Int 5: 446–448Google Scholar
  15. 15.
    Ikeda K, Goto S (1984) Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients. Ann Surg 199: 400–405PubMedCentralPubMedCrossRefGoogle Scholar
  16. 16.
    Jung PM (1995) Hirschsprung's disease: one surgeon's experience in one institution. J Pediatr Surg 30: 646–651PubMedCrossRefGoogle Scholar
  17. 17.
    Marty TL, Seo T, Matlak ME, Sullivan JJ, Black RE, Johnson DG (1995) Gastrointestinal function after surgical correction of Hirschsprung's disease: long-term follow-up in 135 patients. J Pediatr Surg 30: 655–658PubMedCrossRefGoogle Scholar
  18. 18.
    Teitelbaum DH, Caniano DA, Qualman SJ (1989) The pathophysiology of Hirschsprung's-associated enterocolitis: importance of histologic correlates. J Pediatr Surg 24: 1271–1277PubMedCrossRefGoogle Scholar
  19. 19.
    Carcassonne M, Guys JM, Morrison-Lacombe G, Kreitmann B (1989) Management of Hirschsprung's disease: curative surgery before 3 months of age. J Pediatr Surg 24: 1032–1034PubMedCrossRefGoogle Scholar
  20. 20.
    Teitelbaum DH, Qualman SJ, Caniano DA (1988) Hirschsprung's disease. Identification of risk factors for enterocolitis. Ann Surg 207: 240–244PubMedCentralPubMedCrossRefGoogle Scholar
  21. 21.
    Swenson O, Sherman JO, Fisher JH (1973) Diagnosis of congenital megacolon: an analysis of 501 patients. J Pediatr Surg 8: 587–594PubMedCrossRefGoogle Scholar
  22. 22.
    Lister J (1977) Hirschprung's: the man and the disease. J Roy Coll Surg Edinb 22: 378PubMedGoogle Scholar
  23. 23.
    Klein MD, Coran AG, Wesley JR, Drongowski RA (1984) Hirschsprung's disease in the newborn. J Pediatr Surg 19: 370–374PubMedCrossRefGoogle Scholar
  24. 24.
    Klein MD, Philippart AI (1993) Hirschsprung's disease: three decades' experience at a single institution. J Pediatr Surg 28: 1291–1293PubMedCrossRefGoogle Scholar
  25. 25.
    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: 881–884PubMedGoogle Scholar
  26. 26.
    Soave F (1966) Hirschsprung's disease. Technique and results of Soave's operation. Br J Surg 53: 1023–1027PubMedCrossRefGoogle Scholar
  27. 27.
    Soave F (1966) A new surgical method for the treatment of Hirschsprung's disease [Russian]. Khirurgiia 42: 60–65PubMedGoogle Scholar
  28. 28.
    Soave F (1969) Surgery of rectal anomalies with presentation of the relationship between the colonic muscular sleeve and the puborectalis muscle. J Pediatr Surg 4: 705–712PubMedCrossRefGoogle Scholar
  29. 29.
    Soave F (1970) Hirschsprung's disease: Soave's operation [French]. Ann Chir Inf 11: 84–88Google Scholar
  30. 30.
    Boley SJ (1964) New modification of the surgical treatment of Hirschsprung's disease. Surgery 56: 1005–1010Google Scholar
  31. 31.
    Jasonni V, Martucciello G (2000) Soave's extramucosal endorectal pullthrough. In: Holschneider AM, Puri P (eds) Hirschsprung's disease and allied disorders. Harwood, Amsterdam, pp 336–351Google Scholar
  32. 32.
    Hendren WH, Fishman SJ (2000) Experience with Soave endorectal pull-through procedure. In: Holschneider AM, Puri P (eds) Hirschsprung's disease and allied disorders. Harwood, Amsterderm, pp 352–361Google Scholar
  33. 33.
    Cass D (1990) Aganglionosis: associated anomalies. J Paediatr Child Health 26: 351–354PubMedCrossRefGoogle Scholar
  34. 34.
    Ryan ET, Ecker JL, Christakis NA, Folkman J (1992) Hirschsprung's disease: associated abnormalities and demography. J Pediatr Surg 27: 76–81PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • S. J. Singh
    • 1
  • G. D. H. Croaker
    • 2
  • P. Manglick
    • 2
  • C. L. Wong
    • 2
  • H. Athanasakos
    • 2
  • E. Elliott
    • 2
  • D. Cass
    • 2
  1. 1.Consultant Paediatric Surgeon, Department of Paediatric Surgery, University Hospital, Queen's Medical Centre, Nottingham NG7 2UH, UKUK
  2. 2.Department of Academic Surgery, The Children's Hospital at Westmead, Westmead, Sydney NSW 2124, AustraliaAustralia

Personalised recommendations