DEGENERATIVE LEIOMYOPATHY IN AFRICAN CHILDREN - A REVIEW OF CURRENT PERSPECTIVES

The syndrome of degenerative leiomyopathy causing intestinal obstructi on clinically manifests in young indigenous African children as massiv e megacolon without aganglionosis. Eighteen children have been seen ov er a 16 year period from a localized geographic area. There were 10 ma les and 8 females having a mean age of 9.5 years at presentation and a mean duration of symptoms of 4-3 years. The youngest was 6 months old . All had progressive abdominal distension and infrequent stooling but 11 had intermittent diarrhoea and 9 had colicky abdominal pain. Gross gaseous distension of the large bowel with extension into small intes tine occurred in 9 and this extended into stomach and oesophagus in 4. Biopsy of the dilated, thin walled bowel showed smooth muscle degener ation and necrosis with replacement by fibrous tissue. Neuronal cells of Auerbach's plexus tend to be displaced into the circular layer of s mooth muscle with mild inflammatory changes. Some small arteries show medial fibrosis with subintimal fibroblastic proliferation. Acetylchol inesterase and immunohistochemical staining with neural and muscle mar kers are within normal limits. One child died while 14 have been maint ained on prokinetic agents, low residue diets, laxatives and enemas. N ine children have required surgical intervention or whom 4 had volvulu s and 3 adhesive bowel obstruction. Degenerative leiomyopathy is a dis tinctive entity with classical clinical and histological features. The aetiology is still obscure.