Congenital Pseudarthrosis of the Tibia: Management and Complications

Shah, Hitesh (2012) Congenital Pseudarthrosis of the Tibia: Management and Complications. Indian Journal of Orthopaedics, 46 (6). pp. 616-626.

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Abstract

Congenital pseudarthrosis of the tibia (CPT) is a rare pathology, which is usually associated with neurofibromatosis type I. The natural history of the disease is extremely unfavorable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously. It is challenging to treat effectively this difficult condition and its possible complications. Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture. The key to get primary union is to excise hamartomatous tissue and pathological periosteum. Age at surgery, status of fibula, associated shortening, and deformities of leg and ankle play significant role in primary union and residual challenges after primary healing. Unfortunately, none of invasive and noninvasive methods have proven their superiority. Surgical options such as intramedullary nailing, vascularized fibula graft, and external fixator, have shown equivocal success rate in achieving primary union although they are often associated with acceptable results. Amputation must be reserved for failed reconstruction, severe limb length discrepancy and gross deformities of leg and ankle. Distinct advantages, complications, and limitation of each primary treatment as well as strategies to deal with potential complications have been described. Each child with CPT must be followed up till skeletal maturity to identify and rectify residual problems after primary healing

Item Type: Article
Uncontrolled Keywords: Congenital pseudarthrosis of the tibia; complications; children; surgery;treatment
Subjects: Medicine > KMC Manipal > Orthopaedics
Depositing User: KMC Manipal
Date Deposited: 10 Sep 2013 04:08
Last Modified: 10 Sep 2013 04:08
URI: http://eprints.manipal.edu/id/eprint/137096

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