Madelung deformity
| Classification according to ICD-10 | |
|---|---|
| Q74.0 | Other congenital malformations of the upper extremity (s) and shoulder girdle Madelung deformity |
| ICD-10 online (WHO version 2019) | |
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The Madelung deformity is a growth disorder of the forearm that is associated with a characteristic deformity. It was first comprehensively described in 1878 by the German surgeon Otto Wilhelm Madelung , after whom Madelung's hand deformity and Madelung's fat throat were named. However, there have been at least six descriptions of the misalignment before, including a. by Guillaume Dupuytren .
Pathogenesis
It is a dysostosis , which is caused by a disturbed course of the enchondral ossification . During growth there is a lag of the distal radius - metaphysis , especially at the ulnar and dorsal side, so the spoke grows bent after volar and ulnar. This makes the ulna longer than the radius.
In addition, in idiopathic Madelung deformity, there is an abnormally rigid ligament structure ( Vickers ligament ) between the proximal wrist and the distal end of the spoke, which is responsible for a subluxation of the proximal carpal row. This band, up to 5–7 mm thick, was first described by Vickers and Nielsen in 1989 and is found in almost all idiopathic Madelung deformities. It arises in a small pit on the volar-ulnar side of the radial metaphysis, which is usually clearly visible in the X-ray, and runs under the pronator quadratus muscle to the lunar bone , where it inserts volarly at the original insertion of the radiolunar ligament, and to the triangular fibro-cartilaginous complex between the ulna and wrist. The ligament consists of fibrotic, fibrous cartilage and hyaline cartilage structures.
The Enchondromatosis can lead a Madelung's deformity to the image.
The idiopathic Madelung's deformity is very rare, accounting for less than 2% of all childhood hand deformities from. In two out of three cases, the deformity is found on both forearms. Girls and women are about four times more likely to be affected. There is often a familial cluster, often with an autosomal dominant inheritance pattern.
Madelung deformity is more common in girls with Ullrich-Turner syndrome ( prevalence 7.5%).
In patients with Léri Weill's dyschondrosteosis, Madelung's deformity is almost always found; the cause here is a deficit of the SHOX protein ( short stature homeobox-containing gene ), s. Also mesomelic dysplasia of the Langer type , in which comparable deformities are less common.
Clinical appearance
The ulna protrudes on the dorsal side of the wrist and the radius is broadly curved in the volar and ulnar direction. This leads to the so-called bayonet misalignment of the hand with subluxation of the wrist.
In the case of Madelung deformity, the mobility of the wrist, which is tilted in a volar and radial direction, and the rotation of the forearm in supination and pronation are restricted. The dorsiflexion and the abduction ulnar be hindered by bony inhibition.
Madelung deformity does not appear until adolescence, it is not present in childhood (although it is often referred to as a congenital deformity).
Complaints only appear in late adolescence, they are often indicated by the prominent cubit, and are mostly stress-dependent. Sometimes only the misalignment leads to the diagnosis without any symptoms.
X-rays of the wrist show a severe misalignment of the distal radius joint surface, which is tilted ulnar and volar. The ulna is excessively long ( ulna-plus variant ) The gap between the spoke and ulna can be greatly enlarged and look wedge-shaped ( chevron carpus ), so that the lunar bone slips into it and becomes wedge-shaped. The remaining carpal bones are subluxated on the volar side. Very rarely there is also an inverse Madelung deformity , in which the radius is bent dorsally instead of volar and the proximal carpal row subluxes on the dorsal side of the hand.
therapy
After the end of growth, surgical therapy should be considered in the event of restricted mobility and pain. In the past, shortening of the ulna was performed, or an operation according to Suave-Kapangi-Lowenstein with distal radioulnar arthrodesis and distal ulna osteotomy, but now mainly a corrective osteotomy of the distal radius is performed as the site of the actual deformity. Ulna shortening is only performed if the ulna is excessively long, and only in connection with a corrective radius osteotomy.
In addition, it has become the standard to cut the Vickers tape, and this cutting is probably the decisive step in obtaining a pain-free wrist, while the radius osteotomy mainly corrects the deformity. In Harley's technique, which was presented in 2006, a metaphyseal arched corrective osteotomy is performed via an anterior (anterior) access , which enables a three-dimensional correction by tilting the distal fragment, together with a severing ( release ) of the volar ligamentous apparatus. The osteotomy is held in place with two radially inserted transcutaneous Steinmann pins and the arm is immobilized in the upper arm cast for six weeks.
A Texas case series with 19 patients operated on using the Harley technique and 31 operated wrists showed consistently good to excellent results after a mean age of eleven. The remaining reduction in forearm rotation is compensated for by increased rotation in the wrist itself, due to the existing joint laxity , and is rarely a problem.
The operations should be performed in specialized pediatric hand surgery centers. Surgery complications are rare; carpal tunnel syndrome has been described, and ulnar nerve compression has been described when an inverse Madelung deformity is corrected . In addition, a recurrence can occur, especially in young operated patients.
Particularly in children with Léri-Weill dyschondrosteosis, it is recommended that Langenskjöld's epiphysolysis be performed at an early stage with an adipose tissue interposal on the affected ulnar-volar side of the distal radius growth plate with severing of the Vickers band.
In Madelung deformity, avascular osteonecrosis of the wedge-shaped deformed moonbone (lunate malacia ) has occasionally been observed. In the long term, the Madelung deformity can lead to osteoarthritis of the wrist, although no studies on the long-term course are available.
literature
- Paul Holenstein, Pierre Buchs: The Madelung's deformity - expression of a dyschondrosteosis (11 cases). Orthopedics magazine
- CS Ranawat, J. DeFiore, LR Straub: Madelung's deformity. An end-result study of surgical treatment (13 cases). The Journal of Bone & Joint Surgery [Am] 57-A (1975), pp. 772-775.
- John Anthony Herring: Tachdijan's Pediatric Orthopedics , 3rd Edition, WB Saunders Company, Philadelphia 2002, Volume 1, pp. 416-422.
Individual evidence
- ↑ X-ray of the right forearm
- ↑ Leidenberger F et al .: Clinical endocrinology for gynecologists. 4th edition. Springer Medizin Verlag, Heidelberg 2009
- ↑ Suzanne Steinman, Scott Oishi, Janith Mills, Patricia Bush, Lesley Wheeler, Marybeth Ezaki: Volar Ligament release and distal radial dome osteotomy for the correction of Madelung deformity: Long-term follow-up . The Journal of Bone & Joint Surgery 2013, Volume 95-Am, Issue 13, July 3, 2013, pages 1198-1204; doi: 10.2106 / JBJS.L.00714 .
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