Leri-Weill syndrome (dyschondrosteosis): A family study

Mohan, Vinay; Gupta, R. P.; Helmi, Khaled A; Marklund, Tage

doi:10.1016/0266-7681(88)90043-5

Cited by 10 publications

(5 citation statements)

References 10 publications

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“…4,5 It may be isolated or part of a syndrome such as dyschondrosteosis. 6 -8 It may be inherited with autosomal dominance and variable penetrance 9,10 or may be sporadic. 10 Clinical features are a dorsal prominence of the ulnar head and a certain degree of functional impairment.…”

mentioning

confidence: 99%

Isolated Wedge Osteotomy of the Ulna for Mild Madelung’s Deformity

Glard

Gay

Launay

et al. 2007

The Journal of Hand Surgery

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mentioning

confidence: 99%

Isolated Wedge Osteotomy of the Ulna for Mild Madelung’s Deformity

Glard

Gay

Launay

et al. 2007

The Journal of Hand Surgery

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“…In many cases there is a hereditary component with autosomal dominant inheritance of variable penetrance. [1][2][3][4] Several authors have reported the Madelung deformity as being one of the predominant features of dyschondrosteosis, 5-10 but this does not account for all cases and other bone dysplasias have also been associated with the condition. 1,11 The aetiology of the premature fusion of the growth plate is unclear and various causes have been suggested including the occupation or nutrition of the patient, [12][13][14][15][16] applied to the radius such as epiphysiodesis, corrective osteotomy and physiolysis; secondly, those applied to the ulna such as epiphysiodesis, excision of the head, shortening osteotomy and creation of a pseudarthrosis with or without fusion to the radius; and thirdly, combined techniques in which both bones are dealt with usually by one or other of the above methods.…”

mentioning

confidence: 99%

Osteotomy of the radius and ulna for the Madelung deformity

Reis

Katchburian

Faloppa

et al. 1998

The Journal of Bone and Joint Surgery. British volume

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Our results show statistically significant improvement in grip strength and range of movement of the wrist and forearm. Pain improved in 80% of the patients and 88% were satisfied with the appearance. One patient had a wound infection and another developed reflex sympathetic dystrophy. Two had some recurrence due to continued growth of the ulna and it is recommended that the procedure be delayed until skeletal maturity, or else combined with epiphysiodesis of the ulna.

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“…Two patients with Leri Weill syndrome, presented with dyschondrosteotic features of thick and bowed proximal radius, dorsal subluxation and tilt of radial head. 15,20 Vickers and Neilson described a thick fibrous structure that begins on the ulno-volar metaphyseal region of the radius and attaches to lunate and triangular fibrocartilage. 1 MRI helps in demonstrating this anomalous ligament.…”

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confidence: 99%

Early outcome in Madelung’s and Madelung like deformity of wrist in adolescent group

Gupta

Garg

2016

Int J Res Orthop

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Background: Madelung deformity is the result of bony and ligamentous dysplasia at the wrist that produces characteristic deformity and is usually recognized in late adolescence. Madelung like deformity can occur following trauma, infection or neoplasm. This esthetic deformity is encountered rarely in orthopaedic practice, when surgeon faces the challenging task to correct it using the best available technique. This study has been conducted to analyze the presentation, interventional procedures and their end result.Methods: We retrospectively analyze the clinical records, radiographs and functional outcome of patients who presented with Madelung and Madelung like deformity. Radiographic and clinical assessment was done. Surgery was considered for patients fitting into the inclusion criteria. Dorsolateral close wedge and palmar open wedge osteotomy of radius were done with or without ulnar procedure. Postoperative protocol was followed and periodic assessment was done. Mayo modified wrist score has been used to evaluate the pre-operative and post-operative scores.Results: The study enrolled sixteen affected wrists. Average age in our study was 14 years with an average follow-up of 12 months. Statistically significant increase in range of motion of wrist was achieved for in surgically treated patients. Significant change in pain score, grip strength, RAU° and carpal slip was noted. Postoperative wrist score was also significantly improved.Conclusions: Our study concludes that there is a definitive role of surgical intervention to improve the range of motion of wrist, grip strength and reduces pain which brings the patient back into the main stream of a functionally independent being.

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Leri-Weill syndrome (dyschondrosteosis): A family study

Abstract: Madelung's deformity due to Leri-Weill Syndrome (Dyschondrosteosis) is a rare condition. Six cases of this entity involving two generations in one family are reported in this communication. The differential diagnosis of the various causes of Madelung's deformity are briefly discussed.

Cited by 10 publications

References 10 publications

Isolated Wedge Osteotomy of the Ulna for Mild Madelung’s Deformity

Isolated Wedge Osteotomy of the Ulna for Mild Madelung’s Deformity

Osteotomy of the radius and ulna for the Madelung deformity

Early outcome in Madelung’s and Madelung like deformity of wrist in adolescent group

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