Gelenkerhaltende Therapie bei Osteogenesis imperfecta

Zusammenfassung

Hintergrund Die Osteogenesis imperfecta (OI) ist mit einer Häufigkeit von ca. 1:20.000 eine Skeletterkrankung, mit der Pädiater und Orthopäden eher selten konfrontiert werden.

Methode Selektive Literaturrecherche in der Datenbank PubMed unter Berücksichtigung von Fallstudien und Reviews bis August 2022.

Ergebnisse Zwecks optimaler Therapieentscheidung für oder gegen eine operative Therapie bei OI ist eine interdisziplinäre Zusammenarbeit von Pädiatern und Orthopäden unabdingbar. Während bei Kleinkindern und der operativen Versorgung des Unterarmes häufig noch Titanelastische Nägel (TENs) ihren Einsatz finden, werden sobald der Durchmesser des Knochens es erlaubt im Wachstum primär Teleskopnägel bei der gelenkerhalten Versorgung der OI genutzt. Der hierbei weltweit mittlerweile am häufigste präferierte Teleskopnagel ist der Fassier-Duval (FD) Nagel. Bei ausgewachsenen Jugendlichen und Erwachsenen sind aufgrund der kleinen Markraum Durchmesser sogenannte „Custom made Implantate“ notwendig. Bei fehlender postoperativer primärer Belastungsstabilität ist eine Ruhigstellung indiziert, die zu einem weiteren Abbau des ohnehin schon atrophen Muskels bei dem OI-Patienten führt.

Schlussfolgerung Trotz Optimierung bzw. Entwicklung spezieller Implantate stellt die gelenkerhalte Versorgung der OI weiterhin eine Herausforderung für Patient und Arzt dar.

Abstract

Background Osteogenesis imperfecta (OI) is a skeletal disease with a frequency of about 1:20,000, which paediatricians and orthopaedic surgeons are rarely confronted with.

Method Selective literature search in the PubMed database including case studies and reviews up to August 2022.

Results Interdisciplinary cooperation between paediatricians and orthopaedic surgeons is essential for an optimal therapeutic decision for or against surgical treatment of OI. While titanium elastic nails (TENs) are still frequently used for the surgical treatment of the forearm in small children, telescopic nails are primarily used for the joint-preserving treatment of OI as soon as the diameter of the bone permits. The most commonly used telescopic nail is the Fassier-Duval (FD) nail. In adult adolescents and adults, so-called “custom made implants” are necessary due to the small medullary canal diameters. In the absence of postoperative primary load stability, immobilisation is indicated, which leads to further deterioration of the already atrophic muscle in the OI patient.

Conclusion Despite optimisation and the development of special implants, the joint-retaining treatment of OI continues to be a challenge for both patient and doctor.

Schlüsselwörter

Osteogenesis imperfecta - Therapie - Titanelastischer Nagel - Teleskopnagel - Custom Made Implantate

Key words

Osteogenesis imperfecta - therapy - telescopic nail - custom made implant - titanium elastic nail

Publikationsverlauf

Eingereicht: 22. September 2022

Angenommen: 16. November 2022

Artikel online veröffentlicht:
15. Dezember 2022

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