Congenital deficiency of the tibia: A report on 22 cases

Congenital deficiency of the tibia (tibial hemimelia, aplasia, or dysplasia ) is very rare, with an incidence of approximately 1 per 1 million live bir ths. The disorder usually is accompanied by other congenital anomalies on t he same limb or other parts of the body. A report is made of 22 limbs obser ved in 18 patients from 1970 to 1987 at San Juan de Dies Hospital in Caraca s, Venezuela, with more than a 5-year follow-up. The report includes 11 gir ls and 7 boys, with observations of 11 left and 11 right tibiae. Familial a ntecedents were present in four patients: brother and sister and two brothe rs. According to the classification by Jones, Barnes, and Lloyd-Roberts, th ere were nine cases of type Ia, four of type Ib, three of type II, two of t ype III, and four of type TV. The foot of the affected limb was normal in o nly 12 cases. Treatment, always surgical, consisted of T/K amputation in 10 cases of types Ia and Tb, fibula pro tibia and B/K amputation in I case of type II, Syme amputations in 2 cases of type III, and astragalectomy and c losure of diastasis in 4 cases of type TV deformity. Patients in five cases refused treatment. The sooner the amputation is performed, the easier and faster the rehabilitation and adaptation to the prosthesis. An early amputa tion is accepted by the child as a "congenital amputation.".