Radial Dysplasia Reconstruction



Radial Dysplasia Reconstruction


Carley Vuillermin

Marybeth Ezaki

Scott N. Oishi





ANATOMY



  • The anatomic relations of the radial aspect of the wrist are altered due to the variable absence of the radius.



    • The higher the degree of radial dysplasia, the more divergent from normal anatomic relationships the findings will be. This is critically important when undertaking surgical intervention.








      Table 1 Bayne and Klug Classification of Radial Dysplasia

























      Type


      Radiograph


      Description


      I


      image


      Short distal radius; distal epiphysis present, delayed; mild radial deviation


      II


      image


      Defective growth proximal-distal epiphyses; radius in miniature


      III


      image


      Partial absence of radius; wrist unsupported


      IV


      image


      Total absence of radius


      Adapted from Bayne CG, Klug MS. Long-term review of the surgical treatment of radial deficiencies. J Hand Surg Am 1987;12(2):169-179.



    • A consistent but highly abnormal brachiocarpalis muscle has been described in thrombocytopenia-absent radius (TAR) syndrome.14 This muscle spans from just distal to the deltoid insertion directly into the radial side of the carpus and inserts as a broad aponeurotic fan into the carpus, joint capsule, and tendons of the radial wrist.


  • Many patients have associated thumb hypoplasia.10


  • Bayne and Klug2 have provided a classification based on radiographic findings (Table 1).



    • Several authors have proposed alterations to this classification in order to better describe the spectrum of presentation. James et al12 added N and 0 categories. N represents patients with a normal radius and carpus but hypoplastic thumb and 0 for patients with carpal abnormalities and
      normal radial length. Goldfarb et al9 proposed a type V for more proximal deficiencies.


PATHOGENESIS



  • Radial dysplasia develops during the period of embryogenesis. During this period, other organ systems are developing and may also be affected, as discussed later in this chapter.


NATURAL HISTORY



  • The natural history of patients with radial dysplasia clearly depends on the type of dysplasia present and the associated conditions.



    • Patients with isolated type I or II radial dysplasia usually do not require surgical intervention to address the wrist and radial deformity.


    • Patients with more severe dysplasia can frequently benefit from surgical intervention.


  • Many times radial dysplasia is part of a syndrome, and the associated sequelae clearly affect these patients more than the underlying radial dysplasia. The most common associations are with Holt-Oram syndrome, TAR syndrome, Fanconi anemia, and VACTERL (vertebral anomalies, anal atresia, cardiovascular anomalies, tracheoesophageal fistula, esophageal atresia, renal or radial anomalies, limb anomalies).10, 11


  • An association with several craniofacial syndromes has been well documented.8


  • No matter what procedure is used for treating the radial dysplasia, the patients all have a high incidence of recurrent deformity as they get older.2, 4, 17


PATIENT HISTORY AND PHYSICAL FINDINGS



  • The most significant finding is radial deviation at the wrist (FIG 1).


  • If the patient is older, the affected forearm will also be short.


  • Assessment of adjacent joints is essential. Frequently, there will be associated thumb hypoplasia or absence, and in more severe cases (especially Holt-Oram syndrome), the other digits may be stiff. Elbow range of motion is important and the ability to bring the hand to the mouth once the wrist is in a corrected position should be assessed. Radioulnar synostosis is also sometimes present especially in children with Holt-Oram syndrome.






    FIG 1A. Preoperative photo showing radial deviation of the wrist. B. Anteroposterior (AP) radiograph of the same child demonstrating type IV radial deficiency.


  • Because of its frequent association with systemic conditions, all patients require careful examination of their spine and cardiac, renal, and hematologic systems.


IMAGING AND OTHER DIAGNOSTIC STUDIES



  • Radiographs should be taken of both forearms to assess stage of radial dysplasia (see Table 1).


  • In addition, all patients warrant a workup for syndromes and associated conditions, such as Holt-Oram syndrome, Fanconi anemia, TAR syndrome, and VACTERL.



    • This may require echocardiogram, renal ultrasound, hematologic studies (complete blood count [CBC] and chromosomal fragility studies), and spinal evaluation.


    • Each treating physician should consider these associations and not assume they have already been worked up, especially if surgical care of the limb is contemplated.

Jul 22, 2016 | Posted by in ORTHOPEDIC | Comments Off on Radial Dysplasia Reconstruction
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