題目

* Anterolateral Bowing and Congenital Pseudoarthrosis of the Tibia are congenital conditions, most commonly associated with Neurofibromatosis Type 1, that present with a bowing deformity of the tibial. Pathophysiology * Pathophysiology * pseudoarthrosis in not congenital, but in fact develops post-natally due to fracture non-union Treatment * The lesion does not self-resolve, and once fracture occurs there is low likelihood of spontaneous resolution * * Nonoperative * bracing in clamshell orthosis or patellar tendon bearing (PTB) orthosis * indications * children of ambulatory age (weight bearing) * bowing without pseudarthrosis or fracture * spontaneous remodeling is not expected * the goal is to prevent further bowing and fractures * osteotomy for bowing alone is contraindicated * technique * maintained until skeletal maturity * Operative * surgical fixation * indications * bowing with pseudarthrosis or fracture * amputation * indications * typically indicated after multiple failed surgical attempts at union * severe limb length discrepancy * dysfunctional angular deformity * Method- Syme or Boyd amputation