Introduction A 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)
QUESTIONS 1 of 2 1 2 Previous Next (OBQ10.59) Which of the following skeletal dysplasias is caused by a sex linked mutation of the short stature homeobox (SHOX) gene? Review Topic QID: 3147 1 Cleidocranial dysplasia 16% (540/3461) 2 Leri-Weil dyschondrosteosis 49% (1696/3461) 3 Pseudoachondroplasia 14% (477/3461) 4 Ellis-van Creveld (EVC) syndrome 13% (451/3461) 5 Achondroplasia 8% (270/3461) ML 4 Select Answer to see Preferred Response PREFERRED RESPONSE 2 (OBQ08.90) Madelung's deformity of the distal radius is caused by which of the following? Review Topic QID: 476 1 Premature fusion of the distal radial ulnar joint 4% (99/2700) 2 Physeal growth mismatch between the distal radius and ulna 16% (427/2700) 3 Nutritional deficiency affecting the physeal zone of provisional calcification 1% (31/2700) 4 Impaired growth of the volar and ulnar aspect of the distal radial physis 78% (2093/2700) 5 Unrecognized trauma 1% (39/2700) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 4
Login to View Community Videos Login to View Community Videos Frontiers in Upper Extremity Surgery - 2016 Madelungs - Michael J. Garcia, MD (Frontiers #14, 2016) Michael Garcia Hand - Madelung's Deformity 2/17/2017 550 views
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