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Der Orthopäde

, Volume 36, Issue 3, pp 281–292 | Cite as

Arthrogryposis multiplex congenita

  • K. ParschEmail author
  • S. Pietrzak
CME Weiterbildung • Zertifizierte Fortbildung

Zusammenfassung

Von 1975–2004 wurden 38 Kinder mit Arthrogryposis multiplex congenita betreut. Die angeborenen Gelenkkontrakturen verlangen oft aufwändige Eingriffe. Die Aussichten für selbstständiges Gehen sind bei der distalen Arthrogrypose groß. Dagegen bleiben bei Amyoplasie zeitlebens Hilfsmittel notwendig. Ziel aller Habilitationsanstrengungen sind selbstbewusste Erwachsene, die trotz ihrer Einschränkungen ihr Leben meistern. Die Hüftsituation variiert von einer weitgehend normalen Hüfte bis zur teratologischen Luxation. Deren operative Behandlung erreicht begrenzte Vorteile, aber kaum verbesserte Beweglichkeit. Die Kniekontrakturen werden aktiv behandelt, um eine verbesserte Sitz-, Steh- und Gehfähigkeit zu erreichen. Der häufige arthrogrypotische Klumpfuß verlangt ebenso wie der arthrogryposebedingte Schaukelfuß aufwändige konservative und operative Hilfen. Die Ellenbogenstreckkontraktur wird nach erfolgloser konservativer Therapie auf einer Seite operativ behandelt. Für die Schulter-, Hand- und Fingerkontrakturen existieren geringe konservative und kaum operative Verbesserungsmöglichkeiten.

Schlüsselwörter

Arthrogrypose Teratologische Hüftluxation Kniekontrakturen Arthrogrypotischer Klumpfuß Ellenbogenstreckkontraktur 

Congenital multiple arthrogryposis

Abstract

From 1975 to 2004 a total of 38 children handicapped by congenital multiple arthrogryposis were cared for. The congenital joint contractures demand a major effort in terms of surgical reconstruction. In the case of distal arthrogryposis the chances that patients will be able to walk without help are good, while those with amyoplasia are likely to be dependent on mobility aids throughout their lives. The ultimate goal of treatment for patients is to develop into self-confident adults who can cope with life despite their handicaps. The hip in arthrogryposis shows variable forms of pathology, ranging from the almost normal hip to hip contractures with dislocation. Its treatment has some limited advantages, but hardly improves mobility. The knee contractures are actively treated to allow patients to sit, stand and walk better. The club foot and the rocker-bottom foot need sophisticated conservative and operative treatments. If conservative manipulation of bilateral extension contractures of the elbow fails operative treatment is carried out on the dominant side. For shoulder, hand and finger contractures conservative manipulation brings about little improvement, and surgical approaches help hardly at all.

Keywords

Arthrogryposis Teratologic hip dislocation Knee contracture Arthrogrypotic clubfoot Elbow extension contracture 

Notes

Interessenkonflikt

Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen. Die Präsentation des Themas ist unabhängig und die Darstellung der Inhalte produktneutral.

Literatur

  1. 1.
    Akazawa H, Oda K, Mitani S et al. (1998) Surgical management of hip dislocation in children with arthrogryposis multiplex congenital. J Bone Joint Surg Br 80: 636–640CrossRefPubMedGoogle Scholar
  2. 2.
    Asif S, Umer M, Beg R, Umar M (2004) Operative treatment of bilateral hip dislocation in children with arthrogryposis multiplex congenita. J Orthop Surg 12: 4–9Google Scholar
  3. 3.
    Axt MW, Niethard FU, Döderlein L, Weber M (1997) Principles of treatment of the upper extremity in arthrogryposis multiplex congenita Type I. J Pediatr Orthop Part B 6: 179–185Google Scholar
  4. 4.
    Beals RK (2005) The distal arthrogryposes. Clin Orthop Rel Res 435: 203–210Google Scholar
  5. 5.
    Brunner R, Hefti F, Tgetgel JD (1997) Arthrogrypotic joint contracture at the knee and the foot: correction with a circular frame. J Pediatr Orthop Part B 6: 192–197Google Scholar
  6. 6.
    Carlson WO, Speck GJ, Vicari V, Wenger D (1985) Arthrogryposis multiplex congenital. A long-term follow-up study. Clin Orthop Rel Res 194: 115–123Google Scholar
  7. 7.
    Choi IH, YangMS, Chung CY et al. (2001) The treatment of recurrent arthrogrypotic club foot in children by the Ilizarow merthod. J Bone Joint Surg Br 83: 731–737CrossRefPubMedGoogle Scholar
  8. 8.
    Clarren SK, Hall JG (1983) Neuropathologic findings in the spinal cords of 10 infants with arthrogryposis. J Neurol Sci 58: 89–102CrossRefPubMedGoogle Scholar
  9. 9.
    DelBello DA, Watts HG (1996) Distal femoral extension osteotomy for knee flexion contracture in patients with arthrogryposis. J Pediatr Orthop 16: 122–126PubMedGoogle Scholar
  10. 10.
    Drummond DS, Cruess RL (1978) The management of the foot and ankle in arthrogryposis multiplex congenital J Bone Joint Surg Br 60: 96–99Google Scholar
  11. 11.
    Eulert J (1984) Klinik und Behandlung der Arthrogryposis multiplex congenital Untere Extremität. Zschr Orthop 122: 661–669PubMedGoogle Scholar
  12. 12.
    Fahey MJ, Hall JG (1990) A retrospective study of pregnancy complications among 828 cases of arthrogryposis. Genet Counsel 1: 3–11Google Scholar
  13. 13.
    Francillon MR (1971) Arthrogrypotikerschicksale. Zschr Orthop 109: 678–709Google Scholar
  14. 14.
    Freeman EA, Sheldon JH (1938) Cranio-carpotarsal dystrophy. An undescribed congenital malformation. Arch Dis Child 13: 277–283Google Scholar
  15. 15.
    Fucs P, Svartman C, de Assumpcao RMC, Lima Verde SR (2005) Quadricepsplasty in arthrogryposis (amyoplasia): long-term follow-up. J Pediatr Orthop part B 14: 219–224Google Scholar
  16. 16.
    Green ADL, Fixsen JA, Lloyd-Roberts FC (1984) Talectomy for arthrogryposis multiplex congenita. J Bone Joint Surg Br 66: 697–699PubMedGoogle Scholar
  17. 17.
    Grill F (1990) Das arthrogrypotische Hüftgelenk. Zschr Orthop 128: 384–390PubMedGoogle Scholar
  18. 18.
    Grill F, Franke J (1987) The Ilizarow distractor for the correction of relapsed or neglected clubfoot. J Bone Joint Surg Br 69: 593–597PubMedGoogle Scholar
  19. 19.
    Gruel CR, Birch JG, Roach JW, Herring JA (1986) Teratological dislocation of the hip. J Pediatr Orthop 6: 693–702PubMedGoogle Scholar
  20. 20.
    Guidera KJ, Drennan JC (1985) Foot and ankle deformities in arthrogryposis multiplex congenital. Clin Orthop Rel Res 194: 93–98Google Scholar
  21. 21.
    Hall JG (1997) Arthrogryposis multiplex congenital: Etiology, genetics, classification, diagnostic approach, and general aspects. J Pediatr Orthop Part B 6: 159–166Google Scholar
  22. 22.
    Hoffer MM, Swank S, Eastman F et al. (1983) Ambulation in severe arthrogryposis. J Pediatr Orthop 3: 293–296PubMedGoogle Scholar
  23. 23.
    Menelaus MB (1971) Talectomy for equinovarus deformity in arthrogryposis and spina bifida. J Bone Joint Surg Br 53: 468–473PubMedGoogle Scholar
  24. 24.
    Murray C, Fixsen JA (1997) Management of knee deformity in classical arthrogryposis multiplex congenita (amyoplasia congenita) J Pediatr Orthop part B 6: 186–191Google Scholar
  25. 25.
    Niki H, Staheli LT, Mosca VS (1997) Management of clubfoot deformity in amyplasia. J Pediatr Orthop 17: 803–807CrossRefPubMedGoogle Scholar
  26. 26.
    Otto AW (1841) Monstrum humanum extremitatibus incurvatis.. in Vratislaviae Museum. Anatomico-Pathologieum Breslau 322. Zitiert in „Classic“ Clin Orthop Rel Res (1985) 194: 4–5Google Scholar
  27. 27.
    Ponseti IV (ed) (1996) Congenital clubfoot. Fundamentals of treatment. Oxford University Press, OxfordGoogle Scholar
  28. 28.
    Rompe G (ed) (1968) Die Arthrogryposis multiplex congenita und ihre Differentialdiagnose. Georg Thieme, StuttgartGoogle Scholar
  29. 29.
    Sarwark JF, MacEwen GD, Scott CI (1990) Amyoplasia (A common form of arthrogryposis). J Bone Joint Surg Am 72: 456–459Google Scholar
  30. 30.
    Simonian PT, Staheli LT (1995) Periarticular fractures after manipulation of knee contractures in children. J Pediatr Orthop 15: 288–291PubMedGoogle Scholar
  31. 31.
    Södergard J, Ryöppy S (1990) The knee in arthrogryposis multiplex congenital. J Pediatr Orthop 10: 177–182PubMedGoogle Scholar
  32. 32.
    Södergard J, Hakamies-Blomquist L, Sainio K et al. (1997) Arthrogryposis multiplex congenital: Perinatal and electromyographic findings, disability, and psychological outcome. J Pediatr Orthop Part B 6: 167–171Google Scholar
  33. 33.
    Szöke G, Staheli LT, Jaffe K, Hall JG (1996) Medial-approach open reduction of hip dislocation in amyoplasia-type arthrogryposis. J Pediatr Orthop 16: 127–130PubMedGoogle Scholar
  34. 34.
    Staheli LT, Chew DE, Elliott JS, Mosca VS (1987) Management of hip dislocations in children with arthrogryposis. J Pediatr Orthop 7: 681–685PubMedGoogle Scholar
  35. 35.
    Yau PWP, Chow W, Li YH, Leong JCY (2002) Twenty year follow-up of hip problems in arthrogryposis multiplex congenital. J Pediatr Orthop 22: 359–363CrossRefPubMedGoogle Scholar
  36. 36.
    Zukunft-Huber B (Hrsg) (2005). Der kleine Fuß ganz groß. Dreidimensionale manuelle Fußtherapie bei kindlichen Fußfehlstellungen. Elsevier: Urban & Fischer München, JenaGoogle Scholar

Copyright information

© Springer Medizin Verlag 2007

Authors and Affiliations

  1. 1.Weinbergweg 68StuttgartDeutschland
  2. 2.Klinika Orthopdii CMKPOtwockPolen

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