Congenital Tibial Pseudarthrosis

Excerpt

Paget gave the first description of congenital pseudarthrosis of the tibia (CPT) in 1891; it is characterized as a non-union of a tibial diaphyseal fracture that either develops spontaneously or after a history of trivial trauma in a previously dysplastic segment of the bone. It is associated with an increased tendency to re-fracture. It is usually considered one of the most commonly observed types of congenital pseudarthrosis. Anterolateral bowing, failure of the process of tubulation in early life, and cystic pre-fracture are a few of the common predisposing conditions associated with CPT.

The etiology of CPT remains controversial, and it revolves mainly around the decreased osteogenic capability of the pseudoarthrosis tissue. Around 38 to 90% of such cases are correlated with neurofibromatosis. A multimodality approach including genetic counseling is needed to screen for neurofibromatosis in all patients diagnosed with CPT.

In 60 to 90% of the patients with CPT, fibular abnormalities are present. Various types of classification systems have been suggested to cover the wide spectrum of this condition. Multiple treatment options are available for this disease, including both operative and non-operative options based on the severity of the condition.