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Multicenter Study
. 2017 Aug;102(8):722-727.
doi: 10.1136/archdischild-2016-311872. Epub 2017 Mar 9.

Hirschsprung's disease in the UK and Ireland: incidence and anomalies

T J Bradnock  1 , M Knight  2 , S Kenny  3 , M Nair  2 , G M Walker  1 ; British Association of Paediatric Surgeons Congenital Anomalies Surveillance System
Collaborators, Affiliations
Multicenter Study

Hirschsprung's disease in the UK and Ireland: incidence and anomalies

T J Bradnock et al. Arch Dis Child. 2017 Aug.

Abstract

Objectives: To describe clinical characteristics and preoperative management of a national cohort of infants with Hirschsprung's disease (HD).

Design: Population-based cohort study of all live-born infants with HD born in the UK and Ireland from October 2010 to September 2012.

Setting: All 28 paediatric surgical centres in the UK and Ireland.

Participants: 305 infants presenting before 6 months of age with histologically proven HD.

Main outcome measures: Incidence, clinical characteristics including gestational age, birth weight, gender, associated anomalies; age and clinical features at presentation; and use of rectal washouts or stoma.

Results: The incidence of HD in the UK and Ireland was 1.8 per 10 000 live births (95% CI 1.5 to 1.9). Male to female ratio was 3.3:1. An associated anomaly was identified in 23% (69), with 15% (47) having a recognisable syndrome. The proportion of infants who presented and were diagnosed in the neonatal period was 91.5% (279) and 83.9% (256), respectively. 23.9% (73) and 44.2% (135) passed meconium within 24 and 48 hours of birth. 81% (246) first presented to a hospital without tertiary paediatric surgical services, necessitating interhospital transfer. Initial colonic decompression was by rectal washouts in 86.2% (263) and by defunctioning stoma in 12.8% (39). Subsequently, 27.4% (72) of infants failed management with rectal washouts and required a delayed stoma, resulting in 36.4% (111) of infants having a stoma.

Conclusions: In this population-based cohort, presentation outside the neonatal period was rare. Nearly half of the infants with HD passed meconium within 48 hours of birth and over one third were managed with a stoma.

Keywords: Associated anomalies; Hirschsprung's disease; Incidence; Management.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Case ascertainment and data collection.
Figure 2
Figure 2
Maintenance of colonic decompression and the proportion of infants undergoing definitive surgery at 1 year after diagnosis. *Three infants not included—one died and two had a primary pull-through without preceding stoma or rectal washouts.
See this image and copyright information in PMC

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References

    1. Singh SJ, Croaker GD, Manglick P, et al. . Hirschsprung's disease: The Australian Paediatric Surveillance Unit's experience. Pediatr Surg Int 2003;19:247–50. 10.1007/s00383-002-0842-z - DOI - PubMed
    1. Swenson O, Bill AH. Resection of rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolons: an experimental study. Surgery 1948;24:212–20. - PubMed
    1. Duhamel B. A new operation for the treatment of Hirschsprung's disease. Arch Dis Child 1960;35:38–9. 10.1136/adc.35.179.38 - DOI - PMC - PubMed
    1. Soave F. A new surgical technique for treatment of Hirschsprung's disease. Surgery 1964;56:1007–14. - PubMed
    1. Boley SJ. New modification of the surgical treatment of Hirschsprung's disease. Surgery 1964;56:1015–17. - PubMed

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