Non-familial visceral myopathy: clinical and pathologic features of degenerative leiomyopathy.
Degenerative leiomyopathy (DL) is a distinctive form of acquired degenerative visceral myopathy of uncertain etiology that occurs largely in Africa and results in intestinal pseudo-obstruction (IP). In this review of 39 patients from the Western Cape region of South Africa, the mean age at presentation was 9.5 years (range 6 months to 16 years). Characteristic clinical features included a chronic, insidious history of repeated attacks of abdominal distension, abdominal 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 radiologic 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.
Moore
S W
SW
Department of Paediatric Surgery, Faculty of Medicine, University of Stellenbosch, Tygerberg 7505, South Africa.
Schneider
J W
JW
Kaschula
R D C
RD
D015746:Abdominal Pain / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007418:Intestinal Pseudo-Obstruction / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D008297:Male; D008854:Microscopy, Electron; D009130:Muscle, Smooth / Q000473:pathology*; D012189:Retrospective Studies; D013019:South Africa / Q000453:epidemiology; D014839:Vomiting / Q000209:etiology