Madelung Deformity in Skeletally Immature Patients: Morphologic Assessment Using Radiography, CT, and MRI

Cook, Paul A.; Yu, Joseph S.; Wiand, William; Lubbers, Lawrence M.; Coleman, Carl R.; Cook, Albert J.; Kean, John R.

doi:10.1097/00004728-199607000-00001

Cited by 49 publications

(26 citation statements)

References 16 publications

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“…In Turner syndrome multiple bone abnormalities were described [6]including Madelung deformity whose pathophysiology is still unknown [15]. On the basis of clinical experience, the frequency of Madelung deformity was estimated to be 7 or 8% [6, 16].…”

Section: Discussionmentioning

confidence: 99%

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Radiological Signs of Leri-Weill Dyschondrosteosis in Turner Syndrome

Binder¹,

Fritsch²,

Schweizer³

et al. 2001

Horm Res Paediatr

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Background/Aims: Leri-Weill dyschondrosteosis (LWD), a mesomelic short stature syndrome with Madelung deformity, was recently reported to be caused by SHOX (short stature homeobox-containing gene) haploinsufficiency. The loss of SHOX on Xp22.32, also called PHOG (pseudoautosomal homeobox-containing osteogenic gene), through structural aberrations of the X chromosome was also implicated in the short stature phenotype and some additional stigmata of Turner syndrome. The aim of this study was to systematically examine left-hand radiographs from Turner girls for the presence of signs of LWD. Methods: We retrospectively studied 168 left-hand radiographs from 54 patients with Turner syndrome (bone age >10.5 years) who were treated with rhGH and seen during the last 10 years in our clinic. For comparison, we analyzed 7 radiographs from 5 patients with LWD and 52 radiographs from 20 patients with GH deficiency. The shape of the distal radial epiphysis (triangularisation index = TI) and the carpal angle were quantitatively measured. In addition, we screened for the presence of a premature cleft fusion or an ulnar deviation of the articular surface of the distal radial epiphysis and for fourth metacarpal shortening. One of 54 Turner girls (2%) was affected with LWD and presented with Madelung deformity. Results: No milder forms of Madelung deformity were detected. However, there was a significant trend to a triangular shape of the distal radial epiphysis in Turner syndrome: the median TI was 2.7 in normal controls (range 1.8–3.7), 3.1 in Turner girls (range 2.0–6.3) (p < 0.001 against controls), and 6.0 in patients with LWD (range 3.5–11.0) (p < 0.001 against controls). Conclusions: The triangularisation index did not correlate with the carpal angle (median 122.5

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Section: Discussionmentioning

confidence: 99%

“…Therefore, LWD abnormalities of forearms, forelegs and the right wrist could not be detected. Unilateral Madelung deformity, however, is very rare and deformation of the distal radial epiphysis is a primary and initial sign in LWD [12, 15]. Therefore, it is unlikely that we missed any Turner girl with LWD.…”

Section: Discussionmentioning

confidence: 99%

Radiological Signs of Leri-Weill Dyschondrosteosis in Turner Syndrome

Binder¹,

Fritsch²,

Schweizer³

et al. 2001

Horm Res Paediatr

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“…2, 3, 4 and 5) with a corresponding "notch" seen in the distal medial radius at the ligament origin (Figs. 1, 2 and 6) [16,17]. The absence of a Vickers ligament distinguishes Madelung-type deformity from true Madelung deformity.…”

Section: Overviewmentioning

confidence: 98%

“…Although Vickers ligament is best demonstrated with magnetic resonance imaging (MRI), a characteristic flameshaped notch at the medial radial metaphysis can be seen on both radiographs and MRI at the origin of the ligament (Figs. 1, 2 and 6) [16,17]. This notch may have a small bony spur at its proximal margin.…”

Section: Vickers Ligamentmentioning

confidence: 99%

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Madelung deformity and Madelung-type deformities: a review of the clinical and radiological characteristics

et al. 2015

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Madelung deformity of the distal radius results from premature closure of the medial volar aspect of the distal radial physis, leading to increased volar tilt and increased inclination of the radial articular surface, triangulation of the carpus with proximal migration of the lunate and dorsal displacement of the distal ulna. The deformity is particularly common in Leri-Weill dyschondrosteosis, but it may also occur in isolation. True Madelung deformity can be differentiated from Madelung-type deformities by the presence of an anomalous radiolunate ligament (Vickers ligament). In this article, we will review the imaging characteristics of true Madelung deformity, including the common "distal radius" variant, the less common "entire radius" variant and "reverse" Madelung deformity. We will discuss the role of the Vickers ligament in disease pathogenesis and its use in differentiating true Madelung deformity from Madelung-type deformities arising from trauma or multiple hereditary exostoses. Surgical management of these patients will also be addressed.

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