Hirschsprung's disease experience at King Hussein Medical Center

الملخص EN

Objective : To describe the clinical presentation and complications of Hirschsprung's disease at King Hussein Medical Center.

Methods : This is a retrospective review of 94patients treated during the period between 1980-1996 for Hirschsprung's disease.

Data collected included: age at presentation, sex, level of aganglionosis and presence of associated congenital anomalies.

Peri-operative complications and outcome were analyzed.

Results: The majority of patients were males (81%).

Thirty-five patients (3 7 %) were diagnosed within the first 72 hours after birth while the rest were diagnosed between one month and 9 years.

Familial association was found in 14.9 %.

Short segment Hirschsprung's disease was found in 71.3 % of cases.

Peri-operative complications occurred in the majority of cases (60 patients, 63.8 %).

The most common complications were: Hirschsprung's disease enterocolitis in 32 patients (34 %) and sigmoid loop colostomy prolapse in 17patients (18 %).

Fourteen patients (14.9 %) died; eleven died post colostomy but before the definitive pull-through and the other three died following the pull-through.

Deaths related to HEC were seven, six of them pre-pullthrough and only one post-pullthrough.

Enterocolitis, sepsis, and associated congenital anomalies were the main contributing factors for mortality.

Conclusion: Hirschsprung's disease is a potentially life- threatening disease.

All efforts should be made to recognize and treat the complications early.