Madelung's deformity.

نویسندگان

  • J Muthukrishnan
  • A Verma
  • K D Modi
چکیده

A 21-yr old lady presented with primary amenorrhea and short stature for evaluation. She had a wide carrying angle (Cubitus valgus) and a typical deformity at the wrist joint (Fig. 1) which was evaluated. There were no other dysmorphic features. She was found to have high serum FSH and low serum estradiol with streak gonads on ultrasound. Karyotype was 45 XO. She was diagnosed to have Turner’s syndrome and the deformity of the wrist is typical of this condition known as Madelung’s deformity (Bayonet deformity). It is a skeletal deformity of the wrist characterized by acute angular configuration and palmar displacement of the proximal row of carpal bones leading to the hand appearing like a ‘rifle bayonet’. Its pathogenesis is linked to the haploinsufficiency of the SHOX (short stature homeobox) gene. 1,2 It is associated with Turner’s syndrome (10% of cases)2 and Chondrodysplasia of the distal radial epiphysis, or Leri-Layani-Weill syndrome (dyschondrosteosis).2,3

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منابع مشابه

Sex-influenced expression of Madelung's deformity in a family of dyschondrosteosis.

Dyschondrosteosis is a mesomelic form of short stature which occurs in conjunction with a characteristic wrist deformity. Madelung's deformity. A family with dyschondrosteosis had an affected father and two daughters. The affected females had dyschondrosteosis and Madelung's deformity, while the affected males had dyschondrosteosis, but no Madelung's deformity. All affected members had arthralg...

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Reverse wedge osteotomy of the distal radius in Madelung's deformity.

Madelung's deformity results from a growth defect in the palmar and ulnar region of the distal radius. It presents as an excessively inclined radial joint surface, inducing "spontaneous progressive palmar subluxation of the wrist". The principle of reverse wedge osteotomy (RWO) consists in the reorientation of the radial joint surface by taking a circumferential bone wedge, the base of which is...

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Correction of Madelung's deformity by the Ilizarov technique.

We present our experience with correction of Madelung's deformity by the Ilizarov technique. Seven patients (eight deformities) were treated by osteotomy of the radius with subsequent lengthening and angular correction. They were reviewed at a mean of 30 months (1.5 to 5.5 years). At the time of operation their mean age was 19 years (9 to 44). At follow-up all were free from pain and supination...

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Madelung deformity--the hallmark of dyschondrosteosis.

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عنوان ژورنال:
  • The Journal of the Association of Physicians of India

دوره 54  شماره 

صفحات  -

تاریخ انتشار 1952