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Case of the Month

by Dr Noreen Norfaraheen Lee Abdullah MD (UKM), MMed Radiology (UKM), AM(Mal)

Answer to Case Of the Month 
Hirschsprung’s Disease

Discussion of Findings
The plain abdomen radiograph demonstrates gross dilatation of the entire large bowel loops.

The barium enema examination was performed using a Foley’s catheter. The balloon was not inflated. There was free flow of barium into the rectum. A persistent short segment narrowing of the rectum was seen followed by an abrupt dilatation of the rectosigmoid colon. The transitional zone was situated approximately 1cm from the anal orifice and measured 1 cm in length.
The diagnosis was Hirschsprung’s disease. 

Hirschsprung’s Disease

Hirschsprung’s disease is a developmental disorder of the colon and commonly known as colonic aganglionosis. Hirschsprung’s disease is due to the absence of the myenteric plexus. This occurs because there is failure of the migration of the neural crest cells throughout the total length of the gastrointestinal tract.

The involvement can be either long or short segment. Its occurrence is 1 in 5,000 live births and usually presents in the neonatal period. The clinical picture is of constipation, obstruction or both. Males are frequently affected in the short segment aganglionosis while equal sex incidence observed in the long segment disease. Down’s syndrome is associated with this clinical entity.

In performing this procedure, it is important that the patient does not undergo any bowel preparation procedure. The reason being - the dilated bowel facilitates easier recognition of the transition zone. Another point of note is that the catheter is inserted no more than one centimeter into the anal canal and the balloon must not be inflated. The examination is terminated once the aganglionic segment is identified.
 

About the Author 
Dr Noreen, a Consultant Radiologist is currently with the Universiti Sains Malaysia, Kubang Kerian, Kelantan, Malaysia.

 

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Last Updated:
Tuesday, 04 January 2005