Congenital constriction ring syndrome of the limbs; a prospective study of 23 Ghanaian patients

Abstract

Introduction: The congenital constriction ring syndrome is characterized by fibrous bands that encircle, strangle and even amputate parts of the foetus. It is a common condition amongst Ghanaian patients, butdata on it is quite scanty.Patients and Methods: A five year prospective study of patients presenting at a Plastic Surgical Clinic in Ghana with the characteristics of the congenital constriction ring syndrome was undertaken. The patients were examined clinically and the findings recorded. X-ray and clinical photographs of the affected limbs were taken. Treatment required several staged operations.Results: Twenty three patients made up of 15 males and 8 females were seen. The age at presentation ranged from nine days to 12 years with a mean age of 13.2 months. Thirty four limbs were affected, made upof seven right upper limbs, 10 left upper limbs, 10 right lower limbs and seven left lower limbs. In the upper limb malformations involved 61 digits and one forearm; in the lower limb malformations involved 38 toes, onefoot, eight legs, and one thigh. Four main types of lesions were found: constriction rings, intra-uterine amputations, acrosyndactyly, and simple syndactyly.Conclusion: The malformations of the syndrome affect the digits more often than other parts of the body; the central and longer digits being more at risk. Involvement of fingers is commoner than toes. Young mothers and first pregnancies are particularly at risk; the incidence of the syndrome can be reduced by improved antenatal medical care for young mothers, especially those in the lower socio-economic groups.Key words: Congenital constriction rings, acrosyndactyly, intra-uterineamputations.