Hirschsprung’s disease (HD) is characterised by an absence of ganglion cells in the distal bowel and extending proximally for varying distances. The pathophysiology of Hirschsprung’s disease is not fully understood. There is no clear explanation for the occurrence of spastic or tonically contracted aganglionic segment of bowel. The aganglionosis is confi ned to rectosigmoid in 75% of patients, sigmoid, splenic fl exure or transverse colon in 17% and total colon along with a short segment of terminal ileum in 8%. The incidence of HD is estimated to be 1 in 5,000 live births. The disease is more common in boys with a male-to-female ration of 4:1. The male preponderance is less evident in long-segment HD, where the male-to-female ratio is 1.5–2:1.
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Keywords
- Internal Sphincter
- Total Colonic Aganglionosis
- Intestinal Neuronal Dysplasia
- Aganglionic Segment
- Suction Rectal Biopsy
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Further Reading
Dasgupta R, Langer JC (2005) Transanal pullthrough for hirschsprung's disease. Semi Pediatr Surg 14:64–71
Puri P (2003) Newborn Surgery. Arnold, London
Puri P (2006) Hirschsprung's Disease. In P Puri, ME Höllwarth (eds) Pediatric Surgery, Springer-Verlag Berlin Heidelberg, New York, pp 275–288
Puri P, Shinkai T (2004) Pathogenesis of hirschsprung's disease and its variants: Recent progress. Semi Pediatr Surg 13:18–24
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Puri, P. (2009). Hirschsprung's Disease and Variants. In: Puri, P., Höllwarth, M. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-69560-8_46
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