Congenital pseudoarthrosis of the tibia
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Congenital tibial pseudoarthrosis of the tibia describes abnormal bowing that can progress to a segment of bone loss simulating the appearance of a joint. The condition is usually apparent shortly after birth and is rarely diagnosed after the age of two.
Pathology
The aetiology is unclear, however, around 50% of cases are associated with neurofibromatosis type 1 (NF1). Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis.
Congenital ulnar psuedoarthrosispseudarthrosis andradial pseudoarthrosis can also occur, but much less frequently, and usually only in patients with NF1.
Radiographic features
- progressive bowing (usually anterolateral in the tibia)
- resorption of a short segment of bone
- usually at the site of maximal bowing
- usually between mid and distal one
third-third of the shaft
- angulation at the site of absent bone segment
- often mimics the appearance of a joint
- cupping of the bone proximal to the absent section
- sharpened narrow appearance of the distal bone
- often fracture or bowing of accompanying bone (fibula with tibia, radius with
ulnarulna)
-<p><strong>Congenital tibial pseudoarthrosis of the tibia</strong> describes abnormal bowing that can progress to a segment of bone loss simulating the appearance of a joint. The condition is usually apparent shortly after birth and is rarely diagnosed after the age of two.</p><h4>Pathology</h4><p>The aetiology is unclear, however, around 50% of cases are associated with <a href="/articles/neurofibromatosis-type-1">neurofibromatosis type 1 (NF1)</a>. Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis.</p><p><strong>C</strong>ongenital ulnar psuedoarthrosis and<strong> </strong>radial pseudoarthrosis can also occur, but much less frequently, and usually only in patients with NF1. </p><h4>Radiographic features</h4><ul>- +<p><strong>Congenital tibial pseudoarthrosis of the tibia</strong> describes abnormal bowing that can progress to a segment of bone loss simulating the appearance of a joint. The condition is usually apparent shortly after birth and is rarely diagnosed after the age of two.</p><h4>Pathology</h4><p>The aetiology is unclear, however, around 50% of cases are associated with <a href="/articles/neurofibromatosis-type-1">neurofibromatosis type 1 (NF1)</a>. Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis.</p><p><strong>C</strong>ongenital ulnar pseudarthrosis and<strong> </strong>radial pseudoarthrosis can also occur, but much less frequently, and usually only in patients with NF1. </p><h4>Radiographic features</h4><ul>
-<li>resorption of short segment of bone<ul>-<li>usually at site of maximal bowing</li>-<li>usually between mid and distal one third of shaft</li>- +<li>resorption of a short segment of bone<ul>
- +<li>usually at the site of maximal bowing</li>
- +<li>usually between mid and distal one-third of the shaft</li>
-<li>angulation at site of absent bone segment</li>-<li>often mimics appearance of a joint<ul>- +<li>angulation at the site of absent bone segment</li>
- +<li>often mimics the appearance of a joint<ul>
-<li>often fracture or bowing of accompanying bone (fibula with tibia, radius with ulnar)</li>- +<li>often fracture or bowing of accompanying bone (fibula with tibia, radius with ulna)</li>
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