Madelung deformity is due to premature closure or defective development of the ulnar third of the distal epiphysis of the radius. This deformity results in a radial shaft that is bowed with increased interosseous space and dorsal subluxation of the distal radioulnar joint. It often occurs as rare congenital deformity and does not usually manifest until years. It may also be seen as an acquired consequence of trauma to the growth plate, e. The congenital form has autosomal dominant inheritance with variable penetrance. Madelung deformity should not be confused with Madelung-Launois-Bensaude syndrome , also referred to as Madelung disease, which is characterized by lipomatous accumulation in the subcutaneous regions of the neck, upper thorax and upper limbs 6.
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SNIP measures contextual citation impact by wighting citations based on the total number of citations in a subject field. The patient is a year-old woman who presented nocturnal paresthesia lasting for 6 months on both hands, but predominantly right dominant hand.
Upon examination, there was a solid tumor on the posterior side of both wrists Fig. The hemogram and blood chemistry were normal, as well as the neurophysiological study of the median and ulnar nerves.
Panel A shows the ulnar aspect of both wrists, with a prominence that corresponds to the ulnar head, better seen on the left wrist. Panel B shows a lateral view of the left wrist.
The posteroanterior wrist X-rays Fig. The lateral projection Fig. Posteroanterior X-ray of both wrists. Otto Wihelm Madelung described the deformity carrying his name for the first time in Our patient presented slowly increasing pain, becoming intense at 6 months.
In fact, the main symptoms were nocturnal paresthesia, motivated by an entrapment of the median nerve in the carpal tunnel. These symptoms, associated to Madelung's deformity, have been previously described by other authors. Madelung deformity is the consequence of the disorganized growth of the radial epiphysis, leading to arching of the radius, a premature fusion of the epiphysis and a delay in the development of the ulnar part and the anterior part of the distal radial epiphysis.
These alterations cause an inclined radial distal joint surface to the anterior and ulnar area, which leads to an anterior translation of the hand and the wrist, and a dorsal dislocation of the ulna in its distal posterior part. Initial medical treatment consists normally of analgesics, non-steroidal anti-inflammatory drugs and muscle relaxants, which are not very effective.
Splints may be employed to avoid excessive joint mobilization. In cases such as ours, in which the most bothersome symptoms are derived from a median nerve compression in the carpal tunnel, a local infiltration of steroid may significantly alleviate nocturnal paresthesia in a few days.
Surgical treatment must be considered based on 4 factors 1 : a patient age and the possibility of distal ulnar growth; b severity of deformity; c severity of symptoms, and d clinical and radiographic findings. The objectives of surgical treatment are: alleviation of pain, esthetic correction of the deformity and improvement of joint motion, the latter being the most difficult to attain.
The authors state that no experiments were performed on persons or animals for this study. The authors state that they have followed their workplace protocols regarding the publication of patient data and all patients included in the study have received enough information and have given their written informed consent to participate in the study.
This document is in the possession of the corresponding author. The authors have no conflicts of interest. Deformidad de Madelung. Reumatol Clin. ISSN: Open Access Option.
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This item has received. Article information. Show more Show less. Clinical Case The patient is a year-old woman who presented nocturnal paresthesia lasting for 6 months on both hands, but predominantly right dominant hand.
Lateral X-ray of both wrists. Madelung deformity. Medscape Reference [updated Sekiya, P. Jebson, D. Hereditary disorders with maladies of the wrist and elbow.
Iowa Orthop J, 17 , pp. Ly-Pen, J. Med Clin Barc , , pp. Luchetti, A. Mingione, M. Monteleone, G. Carpal tunnel syndrome in Madelung's deformity.
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[Clinical and Molecular Study in a Family With Multiple Osteochondromatosis]
We present two cases of a family with the diagnosis of multiple osteochondromatosis, which was confirmed by molecular study with nonsense in heterozygosis mutation c. GlnStop in the EXT1 gene. In these cases, the Madelung deformity was presented in one patient as an uncommon finding and chondrosarcoma as a feared complication in the other case, highlighting intrafamilial variation, which is why individual and interdisciplinary evaluation is recommended. In addition, before a genetic entity should provide adequate and timely family genetic counseling to all its members. GlnStop en el gen EXT1. Keywords: EXT1; Multiple osteochondromatosis; clinical; genetic counseling.
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