Congenital Pseudarthrosis of the Tibia: Retrospective Review of 23 Cases

Twenty-three patients were treated at Santa Casa de Sao Paulo Medical School from 1993 to the present. All of them were classified Boyd type II and all were associated with neurofibromatosis. Their ages ranged from 1 to 13 years. The number of previous surgeries ranged from none to nine. The shortening of the affected leg was from 0 to 8 cm.

Treatment involved 1) removal of all diseased bone and periosteum (until normal structures), and 2) vascularized fibular graft from the contralateral leg, harvested as described by Gilbert, and using the island flap as a monitor. When the shortening was larger than 2 cm, previous lengthening with a Wagner device was carried out in eight patients and with an Ilizarov apparatus in nine. In three cases, further angular correction with osteotomy and AO plate in the transplanted hypertrophied fibula was carried out.

The follow-up ranged from 1 to 12 years. Bone union was achieved within 10 weeks, except in 2 cases in which infection and thrombosis occurred, requiring transfer of the ipsilateral fibula to provide union in 6 months. All patients walk without a brace and eight use shoe compensation of 1 to 4 cm.