Spot diagnosis? Identify the deformity at wrist ? Madelung deformity

congenital dyschondrosis of the distal radial physis that leads to partial deficiency of growth of distal radial physis  excessive radial inclination and volar tilt ulnar carpal impaction Epidemiology occurs predominantly in adolescent females common in gymnasts Pathophysiology caused by disruption of the ulnar volar physis of the distal radius repetitive trauma or dysplastic arrest one hypothesis is due to tethering by Vickers ligament Vickers ligament is a fibrous band running from the distal radius to the lunate on the volar surface of the wrist (radio-lunate ligament) may be accompanied by anomalous palmar radiotriquetral ligament Genetics autosomal dominant Associated conditions Leri-Weill dyschondrosteosis rare genetic disorder caused by mutation in the SHOX gene   SHOX stands for short-statute homeobox-containing gene anatomically at the tip of the sex chromosome causes mesomelic dwarfism (short stature) associated Madelung's deformity of the forearm

Presentation

Symptoms most are asymptomatic until adolescence symptoms include symptoms of ulnar impaction median nerve irritation Physical exam leads to radial and volar displacement of hand  restricted forearm rotation

Imaging

Radiographs can see proximal synostosis characteristic undergrowth of the volar, ulnar corner of the radius increased radial inclination increased volar tilt MRI indications concern for pathologic Vickers ligament views thickening ligament from the distal radius to the lunate

Treatment

Nonoperative observation  indications  if asymptomatic  restricted activity indications activities with repetitive wrist impaction recommend cessation of weight-bearing activities until pain decreases  Operative physiolysis with release of Vickers ligament  indications wrist pain or decreased range of motion  efficacy of prophylactic release of Vickers ligament in mild deformity in skeletally immature patients unknown radial corrective osteomy +/- distal ulnar shortening osteotomy  indications wrist pain or decreased range of motion  cosmetic deformity functional limitations DRUJ arthroplasty indications highly controversial  painful DRUJ instability and limited supination/pronation significant deformity may require staged procedures

Techniques

Physiolysis and release of Vickers ligament approach volar approach to the distal radius technique release a pathologically thick ligament ligament approximately 0.5 to 1.0 cm in diameter bar resection and fat grafting in the physis  Corrective radial osteotomy +/- distal ulnar shortening osteotomy  goals restore mechanics of distal radius approach volar approach to the distal radius technique severe deformities may benefit from a staged procedure with initial distraction external fixation to avoid neurovascular stretching injury of a single procedure codome osteotomy allows correction of coronal and sagittal deformity

Complications

Incomplete physiolysis or premature growth arrest  Violation of radiocarpal or ulnocarpal joint  Incomplete deformity correction Recurrent deformity Nonunion of the osteotomy site Continued ulnar impaction (if radial osteotomy done alone)