Congenital Pseudarthrosis of the Tibia (CPT)

Key Points:

•    Congenital pseudarthrosis of the tibia is a rare and challenging condition to treat
•    CPT is a periosteal disease creating a fibrous hamartoma at the tibia and/or fibula
•    It is often associated with neurofibromatosis or fibrous dysplasia
•    There exists a constellation of typical radiographic findings and a range in severity
•    Successful treatment includes achieving union AND preventing re-fracture
•    Emerging evidence suggests benefit of cross-union of tibia and fibula
•    Must consider associated deformities in treatment plan (foot and ankle, leg length discrepancy)


Congenital pseudarthrosis of the tibia (CPT) involves nonunion of a tibial fracture occurring through dysplastic bone. It can occur spontaneously or as a result of minor trauma generally presenting before 2 years of age. The exact pathophysiology remains unclear but is known to involve pathologic periosteum resulting in abnormal bone turnover. CPT includes a range of clinical and radiographic presentations which may include pseudoarthrosis of the fibula. Many treatments have been proposed from amputation to reconstruction with varying success rates. The main complications of surgical treatment include failure to achieve union, deformity, and re-fracture. Newer-described techniques have shown promising results in terms of high success rates with minimal complications.


Clinical Findings:

Imaging Studies:

Fig 1. AP and Lateral XR of congenital pseudarthrosis of the tibia (CPT) with fibular involvement

•    Several classification systems exist based mainly on radiographic appearance and bone morphology.
•    Few guide management or predict outcome
•    Crawford (Crawford, 1986): focus on degree of tibial dysplasia and its morphology; most commonly used
•    Choi (Choi, COS 2011): main focus on fibula morphology and station. First to consider proximal fibular migration
•    El-Rosasy-Paley-Herzenberg (El-Rosasy, 2007): considers bone end morphology, mobility at pseudarthrosis site and history of previous surgery. First classification to guide treatment
•    Paley (Paley, 2019): considers integrity of tibia and fibula as well as their station in order to guide treatment


Fig 2. Intra-operative photograph of bone grafting after resection of hamartoma during cross-union technique for congenital pseudarthrosis of the tibia (CPT) with fibular involvemen
**Often a combination of the above (« Kitchen Sink »): VIDEO:  


(Dobbs, 2004) (Paley, 2021) 

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Top Contributors:

Caroline Tougas, MD