Osteologie 2023; 32(01): 42-51
DOI: 10.1055/a-2010-7193
Review

Die chirurgische Therapie der Fibrösen Dysplasie

Surgical Intervention in Fibrous Dysplasia
Andreas Kurth
1   Zentrum für Orthopädie und Unfallchirurgie Marienhaus Klinikum Mainz, Mainz, Germany
› Institutsangaben

Zusammenfassung

Die fibröse Dysplasie ist eine seltene Knochenerkrankung mit einem breiten klinischen Spektrum von Erscheinungsformen bei Erwachsenen und Kindern. Die komplexe Veränderung des Knochens führt bei Kindern sehr häufig zu Frakturen und Deformitäten und im Erwachsenenalter zu chronischen, belastungsabhängigen Schmerzen. Trotz der Fortschritte im Verständnis der Pathogenese der Fibrösen Dysplasie ist ihre Behandlung nicht klar und immer noch umstritten. In den letzten drei Jahrzehnten hat es sowohl bei den chirurgischen als auch bei den medikamentösen Behandlungsmöglichkeiten Fortschritte gegeben. Das Ziel der Therapie muss es sein, Deformitäten, Frakturen und chronische Schmerzen zu vermeiden. In der Vergangenheit wurde die Fibröse Dysplasie ausschließlich chirurgisch behandelt, hauptsächlich durch eine Kürettage der Läsionen. Eine autologe Knochentransplantation hat sich als wenig erfolgreich herausgestellt, hingegen die Verwendung von allogenen kortikalen Spänen scheint vorteilhaft zu sein. Die Verwendung von intramedullären Osteosynthesen ist gegenüber Platten- und Schraubenosteosynthesen zu bevorzugen. Die Behandlung jedes Patienten muss individuell nach Rücksprache mit dem Patienten gestaltet werden. Antiresorptive Therapien haben in kleineren Untersuchungen Vorteile in Bezug auf Knochenschmerz und Frakturen erbracht.

Abstract

Surgical Intervention in Fibrous Dysplasia Fibrous dysplasia is a rare bone disease with a wide clinical spectrum of manifestations in adults and children. The complex alteration of bone very commonly results in fractures and deformities in children and chronic, weight-bearing pain in adults. Despite advances in understanding the pathogenesis of fibrous dysplasia, its treatment is not clear and is still controversial. Over the past three decades, there have been advances in both surgical and drug treatment options. The goal of therapy must be to prevent deformity, fracture, and chronic pain. In the past, fibrous dysplasia was treated exclusively surgically, mainly by curettage of the lesions. Autologous bone grafting has been shown to have little success, whereas the use of allogeneic cortical strout grafts appears to be beneficial. The use of intramedullary osteosyntheses is preferable to plate and screw osteosyntheses. The treatment of each patient must be individualized after consultation with the patient. Antiresorptive therapies have provided benefits in terms of bone pain and fractures in small studies.



Publikationsverlauf

Eingereicht: 02. November 2022

Angenommen: 09. Januar 2023

Artikel online veröffentlicht:
28. Februar 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • Literatur

  • 1 Recklinghausen V.. Die fibrose oder deformierende Ostitis, die Osteomalacie und die osteoplastische Carcinose in ihren gegenseitigen Beziehungen. Festschrift Rudolf Virchow zum 13. Berlin, Germany: Georg Reimer Verlag.; 1891
  • 2 Monge J, Kricun M, Radovcic J, Radovcic D, Mann A, Frayer DW.. Fibrous dysplasia in a 120,000+ year old Neandertal from Krapina, Croatia. PloS One 2013; 8: e64539
  • 3 McCune DJ.. Osteitis fibrosa cystica: the case of a nine-year-old girl who also exhibits precocious puberty, multiple pigmentation of the skin and hyperthyroidism. American journal of diseases of children 1936; 52: 743-744
  • 4 Albright FBA, Hampton AO, Smith P.. Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precocious puberty in females: report of 5 cases. The New England journal of medicine. 1937; 216 (17): 727–746. Lichtenstein L. Polyostotic fibrous dysplasia. Arch Surg 1938; 36: 874-898
  • 5 Lichtenstein LJH.. Fibrous dysplasia of bone: a condition affecting one, several or many bones,graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extraskeletal abnormalities. Arch Path 1942; 33: 777-816
  • 6 Siegal GP, Dal Cin P, Araujo ES.. Fibrous Dysplasia. In: Fletcher CD, Unni KK, Mertens F, eds. Pathology and Genetics of Tumours of Soft Tissue and Bone. Vol 1. Lyon: AIRC Press;; 2002
  • 7 Chapurlat RD, Gensburger D, Jimenez-Andrade JM, Ghilardi JR, Kelly M, Mantyh P.. Pathophysiology and medical treatment of pain in fibrous dysplasia of bone. Orphanet journal of rare diseases 2012; 7: S3
  • 8 Lee JS, FitzGibbon EJ, Chen YR. et al. Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet journal of rare diseases 2012; 7: S2
  • 9 Michael T, Collins MR, Paolo Bianco. Fibrous Dysplasia. In: Rosen CJ, ed. Primer on the metabolic bone diseases and disorders of mineral metabolism. Vol Eight. John Wiley & Sons, Inc; 2013
  • 10 Hart ES, Kelly MH, Brillante B. et al. Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome. Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research 2007; 22: 1468-1474
  • 11 DiCaprio MR, Enneking WF.. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. The Journal of bone and joint surgery. American volume 2005; 87: 1848-1864
  • 12 Ladermann A, Stern R, Ceroni D, De Coulon G, Taylor S, Kaelin A.. Unusual radiologic presentation of monostotic fibrous dysplasia. Orthopedics 2008; 31: 282
  • 13 Parekh SG, Donthineni-Rao R, Ricchetti E, Lackman RD.. Fibrous dysplasia. J Am Acad Orthop Surg 2004; 12: 305-313
  • 14 Wood A, Raman R, Jhobta A, Dhiman DS, Seam RK.. Normal technetium-99m-MDP uptake in fibrous dysplasia of the hip. Hellenic journal of nuclear medicine 2009; 12: 72-73
  • 15 Utz JA, Kransdorf MJ, Jelinek JS, Moser RP, Berrey BH.. MR appearance of fibrous dysplasia. Journal of computer assisted tomography 1989; 13: 845-851
  • 16 Kransdorf MJ, Moser RP, Gilkey FW.. Fibrous dysplasia. Radiographics: a review publication of the Radiological Society of North America, Inc 1990; 10: 519-537
  • 17 Shah ZK, Peh WC, Koh WL, Shek TW.. Magnetic resonance imaging appearances of fibrous dysplasia. The British journal of radiology 2005; 78: 1104-1115
  • 18 Guille JT, Kumar SJ, MacEwen GD.. Fibrous dysplasia of the proximal part of the femur. Long-term results of curettage and bone-grafting and mechanical realignment. The Journal of bone and joint surgery. American volume 1998; 80: 648-658
  • 19 Enneking WF, Gearen PF.. Fibrous dysplasia of the femoral neck. Treatment by cortical bone-grafting. The Journal of bone and joint surgery. American volume 1986; 68: 1415-1422
  • 20 Leet AI, Boyce AM, Ibrahim KA, Wientroub S, Kushner H, Collins MT.. Bone-Grafting in Polyostotic Fibrous Dysplasia. The Journal of bone and joint surgery. American volume 2016; 98: 211-219
  • 21 Liens D, Delmas PD, Meunier PJ.. Long-term effects of intravenous pamidronate in fibrous dysplasia of bone. Lancet 1994; 343: 953-954
  • 22 Lane JM, Khan SN, O’Connor WJ. et al. Bisphosphonate therapy in fibrous dysplasia. Clinical orthopaedics and related research 2001; 382: 6-12
  • 23 Boyce AM, Kelly MH, Brillante BA. et al. A randomized, double blind, placebo-controlled trial of alendronate treatment for fibrous dysplasia of bone. The Journal of clinical endocrinology and metabolism 2014; 99: 4133-4140
  • 24 Majoor BC, Appelman-Dijkstra NM, Fiocco M, van de Sande MA, Dijkstra PS, Hamdy NA.. Outcome of Long-Term Bisphosphonate Therapy in McCune-Albright Syndrome and Polyostotic Fibrous Dysplasia. J Bone Miner Res 2017; 32: 264-276 DOI: 10.1002/jbmr.2999.. Epub 2016 Nov
  • 25 Papapoulos S, Lippuner K, Roux C. et al. The effect of 8 or 5 years of denosumab treatment in postmenopausal women with osteoporosis: results from the FREEDOM Extension study. Osteoporosis international: a journal established as result of cooperation between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA 2015; 26: 2773-2783
  • 26 Majoor BCJ, Papapoulos SE, Dijkstra PDS, Fiocco M, Hamdy NAT, Appelman-Dijkstra NM.. Denosumab in Patients With Fibrous Dysplasia Previously Treated With Bisphosphonates. J Clin Endocrinol Metab 2019; 104: 6069-6078 DOI: 10.1210/jc.2018-02543..
  • 27 Enneking WF, Gearen PF.. Fibrous dysplasia of the femoral neck. Treatment by cortical bone-grafting. The Journal of bone and joint surgery. American volume 1986; 68: 1415-1422
  • 28 George B, Abudu A, Grimer RJ, Carter SR, Tillman RM.. The treatment of benign lesions of the proximal femur with non-vascularised autologous fibular strut grafts. The Journal of bone and joint surgery. British volume 2008; 90: 648-651
  • 29 DiCaprio MR, Enneking WF.. Fibrous dysplasia. Pathophysiology, evaluation, and treatment. The Journal of bone and joint surgery. American volume. 2005 87. 1848-1864
  • 30 Majoor BC, Peeters-Boef MJ, van de Sande MA, Appelman-Dijkstra NM, Hamdy NA, Dijkstra PD.. What Is the Role of Allogeneic Cortical Strut Grafts in the Treatment of Fibrous Dysplasia of the Proximal Femur?. Clinical orthopaedics and related research. 2016
  • 31 Leet AI, Boyce AM, Ibrahim KA, Wientroub S, Kushner H, Collins MT.. Bone-Grafting in Polyostotic Fibrous Dysplasia. The Journal of bone and joint surgery. American volume 2016; 98: 211-219
  • 32 Majoor BCJ, Leithner A, van de Sande MAJ, Appelman-Dijkstra NM, Hamdy NAT, Dijkstra PDS.. Individualized approach to the surgical management of fibrous dysplasia of the proximal femur. Orphanet J Rare Dis 2018; 13: 72 DOI: 10.1186/s13023-018-0805-714..
  • 33 Al-Mouazzen L, Rajakulendran K, Ahad N.. Fibrous dysplasia, shepherd’s crook deformity and an intracapsular femoral neck fracture. Strategies in trauma and limb reconstruction 2013; 8: 187-191
  • 34 Freeman BH, Bray EW, Meyer LC.. Multiple osteotomies with Zickel nail fixation for polyostotic fibrous dysplasia involving the proximal part of the femur. The Journal of bone and joint surgery. American volume 1987; 69: 691-698
  • 35 Stanton RP, Ippolito E, Springfield D, Lindaman L, Wientroub S, Leet A.. The surgical management of fibrous dysplasia of bone. Orphanet journal of rare diseases 2012; 7: S1
  • 36 Stanton RP, Diamond L.. Surgical management of fibrous dysplasia in McCune-Albright syndrome. Pediatric endocrinology reviews : PER 2007; 4: 446-452
  • 37 Kushare IV, Colo D, Bakhshi H, Dormans JP.. Fibrous dysplasia of the proximal femur: surgical management options and outcomes. Journal of children’s orthopaedics 2014; 8: 505-511
  • 38 Ippolito E, Farsetti P, Valentini MB, Potenza V.. Two-stage surgical treatment of complex femoral deformities with severe coxa vara in polyostotic fibrous dysplasia. The Journal of bone and joint surgery. American volume 2015; 97: 119-125
  • 39 Li W, Huang X, Ye Z. et al. Valgus osteotomy in combination with dynamic hip screw fixation for fibrous dysplasia with shepherd’s crook deformity. Archives of orthopaedic and trauma surgery 2013; 133: 147-152
  • 40 Tong Z, Zhang W, Jiao N, Wang K, Chen B, Yang T.. Surgical treatment of fibrous dysplasia in the proximal femur. Experimental and therapeutic medicine 2013; 5: 1355-1358
  • 41 Yang L, Jing Y, Hong D, Chong-Qi T.. Valgus osteotomy combined with intramedullary nail for Shepherd’s crook deformity in fibrous dysplasia: 14 femurs with a minimum of 4 years follow-up. Archives of orthopaedic and trauma surgery 2010; 130: 497-502
  • 42 Benedetti Valentini M, Ippolito E, Catellani F, Farsetti P.. Internal fixation after fracture or osteotomy of the femur in young children with polyostotic fibrous dysplasia. Journal of pediatric orthopedics. Part B 2015; 24: 291-295
  • 43 Jung ST, Chung JY, Seo HY, Bae BH, Lim KY.. Multiple osteotomies and intramedullary nailing with neck cross-pinning for shepherd’s crook deformity in polyostotic fibrous dysplasia: 7 femurs with a minimum of 2 years follow-up. Acta orthopaedica 2006; 77: 469-473
  • 44 Stanton RP, Ippolito E, Springfield D, Lindaman L, Wientroub S, Leet A.. The surgical management of fibrous dysplasia of bone. Orphanet journal of rare diseases 2012; 7: S1