Congenital constriction ring syndrome of the limbs: A prospective study of 16 cases

Abstract

Background: The congenital constriction ring syndrome is characterised by fi brous bands that encircle, strangle and even amputate parts of the foetus. It is a common
condition amongst Ghanaian patients, but data on it is quite scanty. Materials and Methods: A 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 fi ndings recorded. An x-ray and clinical photograph of the affected limbs was taken. Treatment required several staged operations. Surgical correction of the constriction ring was done by excision and Z-plasty to prevent or alleviate lymphoedema, separation of distally fused digits and skin grafting of defect. Results: Sixteen
patients made up of 10 males and six females were seen. The age at presentation ranged from nine days to 12 years with a mean age of 14.6 months. Twenty-two limbs were affected, made up of four right upper limbs, six left upper limbs, seven right lower limbs and fi ve left lower limbs. In the upper limb malformations involved 42 digits; in the lower limb malformations involved 33 toes, one foot and fi ve legs. Four main types of lesions
were found: constriction rings, intrauterine amputations, acrosyndactyly, and simple syndactyly. Conclusion: Congenital constriction ring syndrome is of uncertain
aetiology and could cause morbidity in the newborn. The syndrome and its complications are amenable to corrective surgery with good results. Early intervention is desirable for a successful outcome.

Keywords: Acrosyndactyly, amputations, congenital
constriction rings

African Journal of Paediatric Surgery Vol. 5 (2) 2008: pp. 79-83