ABSTRACT
Objective
To ascertain presentation of constriction band syndrome and its association with other congenital anomalies seen at our institution.
Patients and Methods
This retrospective study was conducted at the department of Plastic and Reconstructive Surgery, Hayatabad Medical Complex, Peshawar from January 2000 to June 2009. Twenty seven patients with constriction band syndrome were included in the study. All were admitted through the outpatient department. The location and degree of each constricting ring and associated anomalies were recorded. Surgery was performed in stages with an average interval of 3 to 6 months.
Results
Out of 27 patients, 11 were males and 16 were females. Age range was 1-18 years (mean 8.19 years). Upper limbs involvement was seen in 15 patients on the right side and 16 on the left side, with total number of 31 digits involved on the right and 38 digits on the left side respectively. The most commonly involved digits were middle and ring finger. Lower limbs were involved in 8 patients on the right side and 11 patients on the left side. Nine toes were involved on the right side and 11 on the left side. Most frequently involved toes were second and third toe. Intrauterine amputations were more common on the right side. The most common associated anomalies were syndactyly, acrosyndactyly, hypoplastic phalanges, lymphedema and Talipes Equino Varus.
Conclusion
Constriction band syndrome had varied presentation and is cause of morbidity in the newborn. We noted three new associated conditions in this syndrome previously not reported. These were strawberry hemangioma of the face, polydactyly and congenital first web contracture increasing the list of associated anomalies in constriction band syndrome. The syndrome and its complications are amenable to corrective surgery with good results. Early intervention is needed for a successful outcome. (Rawal Med J 2010;35: ).
Key words
Constriction band syndrome, syndactyly, polydactyly.
INTRODUCTION
Constriction band syndrome is an uncommon congenital abnormality with multiple disfiguring and disabling manifestations.1-4 Constriction bands, also called congenital rings, Streeter dysplasia, amniotic bands and annular defects are anomalous bands that encircle, either partially or completely a digit or an extremity.5 A wide spectrum of clinical deformities are encountered ranging from simple ring constrictions, minor digital defects to major craniofacial and visceral defects.2-4,6 This syndrome was first recognized by Portal7 in 1685. It occurs in approximately 1 in 1200 to 1 in 15000 live births.4,6-10 Upper and lower extremities malformations are the most common and consist of asymmetric digital ring constrictions, distal atrophy, congenital intrauterine amputations, and acrosyndactyly and lymph edema.2,4,5,7,11 Inheritance is sporadic, although association with other anomalies, such as club feet or cleft lip and palate has been described.9 Patterson classifi
Key words: Constriction band syndrome, syndactyly, polydactyly.
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