Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain aetiology. It occurs mainly in Africa and results in intestinal pseudo-obstruction (IP). Thirtynine patients from the Western Cape region of South Africa have been reported.1 Characteristic clinical features included a chronic, insidious history of repeated attacks of abdominal distension, pain and vomiting. Marked gaseous distension with atony and IP, especially of the colon, was noted on X-ray films. Megacolon was the most common radiological feature, but pseudo-obstruction extended proximally into the small intestine in some patients with advanced disease. In the majority of cases the condition was progressive and eventually affected the entire gastrointestinal (GI) tract.1 We present a single case of a 9-year-old girl with this condition.
South African Journal of Radiology Vol. 10 (2) 2006: pp. 16-17