Kozin Scott H, Zlotolow Dan A
Department of Orthopaedic Surgery, Temple University, Philadelphia, PA; Shriners Hospitals for Children, Philadelphia, PA.
Department of Orthopaedic Surgery, Temple University, Philadelphia, PA; Upper Extremity Center of Excellence, Philadelphia, PA.
J Hand Surg Am. 2015 Oct;40(10):2090-8. doi: 10.1016/j.jhsa.2015.03.033. Epub 2015 Sep 1.
Madelung deformity of the wrist is more common in females and is often associated with Leri Weill dyschondrosteosis, a mesomelic form of dwarfism. Patients with Madelung deformity often report wrist deformity resulting from the prominence of the relatively long ulna. The typical Madelung deformity is associated with a Vickers ligament that creates a tether across the volar-ulnar radial physis that restricts growth across this segment. The distal radius deforms in the coronal (increasing radial inclination) and the sagittal (increasing volar tilt) planes. There is lunate subsidence and the proximal carpal row adapts to the deformity by forming an upside-down pyramid shape or triangle. Treatment depends on the age at presentation, degree of deformity, and magnitude of symptoms. Mild asymptomatic deformity warrants a period of nonsurgical management with serial x-ray examinations because the natural history is unpredictable. Many patients never require surgical intervention. Progressive deformity in the young child with considerable growth potential remaining requires release of Vickers ligament and radial physiolysis to prevent ongoing deterioration Concomitant ulnar epiphysiodesis may be necessary. Advanced asymptomatic deformity in older children with an unacceptable-appearing wrist or symptomatic deformity are indications for surgery. A dome osteotomy of the radius allows 3-dimensional correction of the deformity. Positive radiographic and clinical results after dome osteotomy have been reported.
腕部马德隆畸形在女性中更为常见,且常与勒里-韦尔软骨发育不全相关,后者是一种中肢短小型侏儒症。马德隆畸形患者常称腕部畸形是由相对较长的尺骨突出所致。典型的马德隆畸形与一条维氏韧带有关,该韧带在掌侧尺侧桡骨骺处形成一条束带,限制该节段的生长。桡骨远端在冠状面(桡侧倾斜增加)和矢状面(掌侧倾斜增加)发生变形。月骨下沉,近端腕骨排通过形成倒金字塔形或三角形来适应畸形。治疗取决于就诊时的年龄、畸形程度和症状严重程度。轻度无症状畸形需要进行一段时间的非手术治疗,并定期进行X线检查,因为其自然病程不可预测。许多患者从未需要手术干预。对于仍有相当生长潜力的幼儿,进行性畸形需要松解维氏韧带并进行桡骨骨骺松解术,以防止病情持续恶化,可能还需要同时进行尺骨骨骺阻滞术。对于年龄较大儿童出现的严重无症状畸形且腕部外观不可接受或有症状性畸形,均为手术指征。桡骨穹顶截骨术可对畸形进行三维矫正。已有报道称桡骨穹顶截骨术后影像学和临床结果良好。