Patterns of Presentation and Outcome of Management of Congenital Pesudarthrosis of the Tibia

Abstract

Background: Congenital pseudarthrosis of the tibia (CPT) is a rare paediatric orthopaedics condition characterized by bowing of the tibia with spontaneous fracture which does not heal easily. It is sometimes associated with Neurofibromatosis. It has many classification schemes and various methods of treatment.

Objectives: To study the patterns of presentation, demographic characteristic and outcome of management of CPT.

Patients and Methods: This is retrospective case series study, records and follow up notes of all patients of CPT who presented to and managed at Soba University Hospital between 2003 and 2014 were reviewed, data were collected and analysed.

Results: there were 37 patients with CPT, 19 males and 18 females. Their ages at
presentation ranged between 1month and 11 year, mean of 3.8 years. Neurofibromatosis was associated in 37.8% of the cases. The most common deformity was anterolateral bowing of the tibia seen in over 90% of the cases. In 54% of cases the treatment was surgical and 90% of these had intramedullary rush pin and free fibular grafting. In 88% complete healing could be attained. In two cases there was non-union.

Conclusion: Congenital Pseudarthrosis of the tibia is a rare pathology we have reported 37cases. One third of the patients had Neurofibromatosis. Majority of cases had anterolateral bowing. Associated fibular pseudarthrosis is very rare. If they present before fracture they can be protected by bracing. Intramedullary rush pin with bone grafting is easy, cost effective with union achieved in over 80% of cases.

Key words: Bowing of the tibia, spontaneous fracture, neurofibromatosis, Sudan