CONGENITAL CONSTRICTION BAND SYNDROME PATHOPHYSIOLOGY AND TREATMENT

The clinical manifestations of 88 children with congenital constrictio n band syndrome involvement of the hand were reviewed. Seventy-five of these children had evidence of digital or limb amputations, with 235 upper limb amputations and 138 lower limb amputations. In the hand, di gital amputations were most common in the index, middle, and ring fing ers, whereas in the foot, amputations of the hallux were most often no ted. Band indentation was often present at multiple levels. Proximal b ands may be associated with neural compression. Syndactyly was invaria bly associated with a proximal interdigital sinus or cleft and was fre quently associated with distal amputation. Examination of a 27-week ge station stillborn specimen having manifestations of congenital constri ction band syndrome demonstrated the intrauterine biologic response to band constriction. The variable clinical manifestations of congenital constriction band syndrome can best be explained as the response of t he growing, embryologically defined limb to intrauterine deformation o r band-induced compression and ischemia.