Naima Zamir, Jamshed Akhtar.
Hirschsprung`s Disease: Pattern of Clinical Presentation.
J Surg Pak Jan ;19(1):40-4.

Objective: To report clinical symptoms and signs and age at initial presentation of patients with Hirschsprung`s disease. Study design: Descriptive case series. Place & Duration of study: Department of Paediatric Surgery, National Institute of Child Health Karachi, From September 2008 to December 2013. Methodology: Patients who were proven cases of Hirschsprung’s disease on rectal biopsy report were included. The data was collected prospectively. Variables recorded included clinical presentation including symptoms, signs, age, gender, mode of presentation either elective or acute etc. The data was analyzed using descriptive statistics like frequency, percentage and proportions in relation to quantitative and qualitative variables. Results: A total of 84 patients with Hirschsprung’s disease were managed during the study period. There were 63 male and 21 female patients with male to female ratio of 3:1 . Fifty-five (65.5%) patients belonged to Karachi. Fifty percent (n=42) patients presented by day 6 of life. The commonest presentation was delayed passage of menonium but it was reported in 39 (46.4%) patients only. All these patients were operated and extent of the disease was up to rectosigmoid level in 78 (93%) patients. Conclusion: Clinical presentation in present study had similarities to reported literature but important differences were noted with regards to passage of meconium and extent of aganglionosis.

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