Συγγενής Ψευδάρθρωση Ωλένης

Συγγενής Ψευδάρθρωση Ωλένης

Congenital pseudoarthrosis of the ulna presents particular difficulties in its management and, like all congenital pseudoarthroses, it may require several operations which often have little improvement. The case of a young patient with congenital pseudoarthrosis of the ulna in the setting of neurofibromatosis is presented herein.
A young 10-year old boy underwent double osteotomy of his radius for deformity correction, but 2 years later the deformity recurred and he was reoperated. The second operation included a new radial osteotomy, iliac crest autograft and screw  fixation.  This  option  failed  and  he  was  revised  with  plate osteosynthesis.
Two years after the second operation the graft resorbed and the deformity recurred, associated with radial head dislocation and impingement on the humerus and with painful limited  flexion of the elbow as well.

Which  is  the  next  step?

The patient underwent subsequent mobilization with the use of an special external  fixator which  allows  the  pararel  translation  of  the  two  bones  of  the  forearm  and the proper alignment between the radius and the ulna was gradually achieved.
Thereafter, the patient was reoperated and any pathologic bone in the ulna was removed till healthy borders were reached; a free vascularized fibular graft was then placed in the gap using the fibular head as an ulna, in an attempt to reconstruct the distal radioulnar joint. Alignment of the bones of the forearm was finally accomplished after another radial osteotomy with concomitant reconstruction of the lateral ligamentous structures of the wrist (by  Secker  procedure),  and  reconstruction  of  the annular  ligament           with  the  use  a  tendinous  flap  of  the  triceps.
At the latest follow-up (18 years post the initial operation  and  12  years  after  the  fibula  transfer), the ulna appears clinically united, the patient has painless  full flexion and extension in the elbow, and although his pronation/ supination is mildly restricted, he has a  functional limb  permitting  full  activity.
Congenital pseudoarthrosis of the ulna is a surgical challenge and several factors should be taken into account. The  resection of the pathologic tissue from the ulna is very  important for definitive treatment and the proximal part of the fibula is a reliable solution for the reconstruction of the distal radioulnar joint.

(Η περίπτωση έχει παρουσιαστεί στο Πανευρωπαϊκό Συνέδριο Μικροχειρουργικής στη Γένοβα, Ιταλία, το 2010)

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