Club hand

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Classification according to ICD-10
Q71.4 Longitudinal reduction defect of the radius: club hand (congenital), radial club hand
ICD-10 online (WHO version 2019)

A club hand is a congenital malformation of the forearm . This form of longitudinal hypoplasia can be caused by hypoplasia or aplasia of the radius and the first ray of the hand, so-called radial club hand ( radius aplasia ) or by hypo- or aplasia of the ulna as so-called ulnar club hand.

Epidemiology

Different expressions of the radial club hand. The spoke is progressively stunted until it is completely absent. The increasing bending of the hand is clearly recognizable.

The information on the frequency of a club hand fluctuates between 1: 100,000 and 11: 100,000 live births. The probability of a club hand is one to two orders of magnitude lower than with a club foot (approx. 1: 1000).

Depending on the author, the ratio between Manus vara and Manus valga is 3: 1 to 18: 1. The figures for the frequency of two-sided occurrence of a club hand also vary between 22 and 60%. By 1917, only about 200 cases had been described in the literature. In 1909, Adolf Stoffel (1880–1937) and his future wife Edda Stempel described the pathological changes in detail after the autopsy of nine children's corpses.

For those born between 1960 and 1962, the incidence of newborns with a radial club hand increased dramatically. The cause was the ingestion of Contergan (active ingredient: thalidomide) by the expectant mothers during pregnancy . The ratio of boys to girls was 2: 1 and with a double-sided club hand to one-sided it was around 4: 1.

etiology

The radial club hand occurs spontaneously in most cases. There are some older reports of familial accumulations, but today it is assumed that a club hand - as an independent malformation - is not hereditary. There are indeed some rare syndromes of genetic origin which, as one of several phenotypes, can lead to a congenital club hand.

The malposition of the hand is caused in the manus vara by a shortened or thinner, sometimes also by a missing radius (radius aplasia ) and in the manus valga by a shortened, thinner or missing ulna . Correspondingly, the affected hand deviates from the normal position in the radial or ulnar direction. Statistically, the radius is affected more often than the ulna, which is why there are more cases of manus vara than manus valga .

The malformation of the radius or ulna can be caused by a number of genetic syndromes. A radial club hand can include the Rothmund-Thomson syndrome , the TAR syndrome , the Holt-Oram syndrome , the De Toni-Fanconi syndrome , the rodent syndrome ( arcofacial dysostosis ), the VATER syndrome (V. : Vertebral defects , A: Anal atresia , T: Tracheal-esophageal fistula , E: Esophageal atresia , R: Radial and renal dysplasia ) and the Baller-Gerold syndrome . Ulnar club hand can, among other things through the proximal femoral focal deficiency , the Cornelia de Lange Syndrome , which Weyers-oligodactyly syndrome , which Akrokallosale syndrome (Schinzel syndrome), a ulnofibulare dysplasia and Pillay syndrome caused become.

When taking Contergan, the sensitive phase was between the 43rd and 45th day after the last menstruation . In addition to thalidomide, other noxious substances can lead to radial club hands during the embryonic phase. The alkylating agent busulfan produced in rats on 9 of gestation Ulnardefekte and on the 10th day of pregnancy radial defect in the rat embryo.

diagnosis

Congenital radius defect with missing thumb

A club hand is very noticeable in clinical appearance due to the shortened arm and the forearm bent inwards (radial club hand) or outwards (ulnar club hand). The deformity can vary greatly from person to person. For example, the elbow may be completely stiff ( ankylosis ) or show instability. The ulnar fingers may be absent and the remaining fingers of the affected arm may show syndactyly or camptodactyly . The skeletal misalignments and malformations of the hand, wrist and elbow joint and their extent are clearly visible in the X-ray image and are important for the selection of therapeutic measures.

The malformations are not limited to the forearm and hand bones. Soft tissue anomalies such as a missing ulnar artery or a radial artery are possible, in which case a central persistent artery is often present. A missing ulnar nerve is also often observed.

therapy

A club hand can be treated conservatively or surgically . Lighter forms of club hand are usually treated conservatively. The aim of this form of therapy is to counteract the contracture and thus align the affected hand in the longitudinal axis of the arm, and to compensate for the shortening of the arm. This is attempted from the patient's first day of life by fixing the arm with a plaster cast or splint. However, conservative treatment is often only successful in the case of minor deformities. Larger misalignments can usually only be corrected through surgical interventions. Corrections in the hand area can be made in the first year of life and vary widely from person to person. Separation of the fingers in syndactyly, rotational osteotomy , the removal of rudimentary bony parts, pollicization (formation of a thumb ) and transfer of finger bones are typical . Surgery on the forearm and elbow is usually not done before puberty to avoid endangering the growth plates. The club hand operation according to Walter Blauth is an exception . It can be done at pre-school age. The soft tissues on the wrist are severed and the ulna is bolted into the wrist. Through this correction of the misalignment and the simultaneous creation of a fibrous ankylosis, an attempt is made to increase the usability of the hand. Other interventions in the area of ​​the forearm and elbow are: excision of the fibro-cartilaginous ulna, radius-per-ulna fusion, ulnar lengthening osteotomy, corrective osteomy of the radius, radius head resection and repositioning osteotomy of the synostosed elbow.

literature

Individual evidence

  1. ↑ F. Hefti: Pediatric Orthopedics in Practice. Springer 1998, ISBN 3-540-61480-X .
  2. ^ AK Martini: Congenital malformations. (PDF; 3.2 MB) In: CJ Wirth: Orthopedics and orthopedic surgery: elbow, forearm, hand. Georg Thieme Verlag, 2003, ISBN 3-13-126211-7 , pp. 121-232.
  3. ↑ A. Stoffel and E. Stempel: Anatomical studies on the club hand. Verlag Enke, 1909, 157 pages, special print from the journal for orthopedic surgery. Volume 23, Number 1, 1909.
  4. ↑ a b G. Neff: The radial club hand - stigma or functional necessity? In: L. Zichner, MA Rauschmann, KD Thomann (editor): The Contergankatastrophe - A balance sheet after 40 years. Verlag Birkhäuser, 2005, ISBN 3-7985-1479-8
  5. ↑ a b W. Blauth: On the morphology and therapy of the radial club hand. Arch. Orthop. Accident chir. 65: 97-123 (1969). doi: 10.1007 / BF00416321
  6. ↑ L. van Maldergem: Baller-Gerold syndrome. In: PA Pagon et al. (Editor): GeneReviews. Seattle, University of Washington, 1993-2007, PMID 20301383
  7. ↑ J. Carls: Radiale Klumphand. (PDF; 82 kB) July 2008
  8. ↑ J. Carls: Ulnar Clubhand. (PDF; 45 kB) August 2008
  9. ^ T. Ogino: Congenital anomalies of the hand. The Asian perspective. In: Clinical orthopedics and related research number 323, February 1996, pp. 12-21, ISSN  0009-921X . PMID 8625568 .
  10. ↑ Dieter Buck-Gramcko : A life for hand surgery. Verlag Springer, 2007, pp. 5-8, ISBN 3-7985-1776-2 doi : 10.1007 / 978-3-7985-1777-6_2
  11. ^ H. Rössler and W. Rüther: Orthopedics and trauma surgery. Verlag Urban & Fischer, 2005, ISBN 3-437-44445-X , p. 59, limited preview in the Google book search