AN APPROACH TO THE MANAGEMENT OF CONGENITAL PSEUDARTHROSIS OF THE TIBIA TREATED SURGICALLY: AN INSTITUTIONAL REVIEW

Background: Congenital pseudarthrosis of the tibia (CPT) is a rare dysplastic disorder of the tibial diaphysis, quoted to affect approximately 1 in 140,000 to 1 in 250,000 live births, and is strongly associated with neurofibromatosis type 1 (NF1). It remains one of the most treatment-resistant conditions in paediatric orthopaedics: union is frequently achieved only after several operations, refracture is common, and a proportion of children still come to amputation. Data from Indian government tertiary centres, where children often present late and after prior treatment elsewhere, are limited. Materials and Methods: We performed a descriptive longitudinal institutional review of five consecutive children with CPT managed in the Department of Orthopaedics of a government tertiary care teaching hospital in Kolkata between 2022 and 2024. All children presenting to the orthopaedic out-patient department with CPT were included; children with active infection at the affected site, whose guardians declined consent for a planned staged multiple-operation programme, or who were unfit for anaesthesia because of medical comorbidity were excluded. The core operative strategy was excision of the hamartomatous pseudarthrosis, correction of deformity, autologous fibular and iliac crest grafting and, where the tibia could not be reconstituted, tibialisation of the fibula. Children were reviewed at 6 and 12 weeks, at 6 and 12 months and yearly thereafter, and were mobilised in a protective leg guard. The mean follow-up was 3 years. Outcome variables were weight-bearing ability, radiological signs of union, clinical assessment of union, range of motion of the knee and ankle, and infection. Results: Five children were treated (male: female 3 : 2). Café-au-lait macules consistent with NF1 were present in three of five children. Each child underwent between two and eight operative procedures. Satisfactory bony union was achieved in four of five children; in one child union could not be obtained despite eight procedures and a below-knee amputation was performed at the parents’ request. Three children were bearing weight satisfactorily at latest review, one child awaited further surgery, and the knee and ankle range of motion was preserved in the children who united. Residual shortening of 2–3 cm was managed with a shoe raise. There was no deep infection, and no refracture occurred after union during follow-up. Conclusion: Staged surgical reconstruction based on radical excision of the pseudarthrosis and tibialisation of the fibula is a viable option for CPT in a resource-constrained tertiary care setting and produced union in four of five children. Families must be counselled at the outset that treatment is prolonged, that several operations and several graft harvest sites are likely, and that protection of the united segment must continue for years because of the risk of refracture. Correcting the primary problems early, before secondary deformities become fixed, remains the central objective of treatment.