Amputation Outcomes in Congenital Pseudarthrosis of the Tibia.

BACKGROUND: Successful radiographic union in the treatment of congenital pseudarthrosis of the tibia (CPT) may be complicated by persistent pain, recurrent fracture, and poor function necessitating further intervention, including amputation. The long-term functional, radiographic, and clinical outcomes of patients who have undergone amputation as treatment for CPT are unknown.

METHODS: A retrospective study of patients with a diagnosis of CPT secondary to neurofibromatosis and eventual treatment with amputation were included. Clinical and radiographic data, including initial Crawford classification, type of amputation, subsequent operative procedures, and evidence of radiographic healing were collected.

RESULTS: A total of 17 patients with a mean age of 4.5 years (range, 0.7 to 9.2 y) at the time of amputation met inclusion criteria. Clinical follow-up averaged 11.1 years (range, 2.1 to 18.4 y), with radiographic follow-up averaging 9.1 years (range, 2.1 to 16.4 y). The mean number of surgeries before amputation was 2.2 procedures. Four patients underwent amputation as the primary procedure (3 Boyd, 1 below knee amputation (BKA)). At the time of amputation, a Boyd amputation was performed in 13 patients with stabilization of the pseudoarthrosis achieved with retrograde Rush rodding of the tibia and local autograft. A transtibial amputation (BKA) was performed in 4.After the Boyd procedure, 4 of the 13 patients (31%) demonstrated persistent nonunion of the pseudoarthrosis and required secondary procedures to gain union. At the most recent follow-up, 12 of 13 patients demonstrated successful radiographic healing of the pseudoarthrosis. Two patients, one for persistent pain and the other for refracture, were later converted to a BKA during the late teen years. All patients functioned well with the use of prosthetic devices.

CONCLUSIONS: Union of the pseudoarthrosis occurred in >90% of cases following amputation. However, secondary procedures were required in 13 of the 17 patients (76%). Early amputation in the treatment of CPT provides a stable extremity and potential for a high level of function with the use of an adequate prosthesis.

LEVEL OF EVIDENCE: Level IV-case series.