Naslov
Management of weak bone orthopaedic problems in patients with benign tumours, Ollier disease, fibrous dysplasia and renal osteodystrophy

Autori
Antičević, Darko

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, prošireni sažetak, ostalo

Izvornik
Hand-outs of afternoon EPOS Symposium / Scientific, Committee - Helsinki, 2012

Skup
European Paediatric Orthopaedic Society 31st Annual Meeting - Afternoon Symposium on "Weak Bone Diseases"

Mjesto i datum
Helsinki, Finska, 18.04.2012. - 21.04.2012

Vrsta sudjelovanja
Pozvano predavanje

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
weak bone, orthopaedic management

Sažetak
Management of weak bone orthopaedic problems in patients with benign tumors, Ollier disease, fibrous dysplasia and renal osteodystrophy Some benign bone tumors and tumor-like lesions (UBC, NoF, ABC, mono-ostotic fibrous dysplasia) could produce local bone weaknes leading to pathological fracture. Other disease (Ollier's disease, renal osteodystrophy) cause more generalized bone softening disorders. In case of pathologic fracture orthopaedic surgeons are faced with three problems: First, to diagnose and to treat underlying disease. Second, to reduce and to stabilize fracture under circumstances of weak and low bone stock. Third, to avoid possible complications, notably AVN and growth arrest. Benign tumors and tumor-like lesions. Pathological fracture in patients UBC and ABC is more likely in high-stress region of lower extremities i.e. proximal femur. In the case of pathological fracture at any location one should be certain about correct diagnosis of underlying disease that is causing bone weakness. Sometimes cystic-like lesions could be misleading (1). There are several choices of implants that could be used in case of pathological fracture due to UBC or ABC. When one is considering surgery, size and location of UBC/ABC are of utmost importance. NoF is not a tumor, it is disorder of developmental nature with bone that normally ossify is replaced with fibrous connective tissue. NoF involving of more than 50% of the diameter of the bone (in both projections) has higher risk of pathological fracture. In high-risk regions like proximal femur and distal tibia prophylactic internal fixation may be warranted. However, for most i.e. smaller NoF lesions conservative management is advised. Ollier's disease (multiple enchondromatosis). In 1899. French surgeon Luis L. Ollier (1830- 1900) described multiple enchondromatosis under name of dyschondroplasia. This is a rare non- familial skeletal disorder (prevalence 1/100.000) with clinical manifestation in the 1st decade of life. It is characterized with asymmetric distribution of cartilage metaphyseal lesions of tubular bones. Orthopaedic clinical problems include angular deformity and shortening of involved the bone, with possible pathologic fracture (2). Clinical manifestation of Ollier's disease is extremely variable and prognosis is not easy to assess. There is no medical treatment and surgery is indicated in patients with axial deformity, LLD and pathological fracture. Ilizarov ex.-fix. without or in combination with intramedullary nails (FEN) is recommended (3). In the mild forms observation is advised. However, risk of malignancy is present in 25-30% of patients, later in life - about 40ies (4). Multiple enchnodroma with soft tissue vascular anomalies are known as Maffucci's syndrome. Occult malignant non-skeletal (brain, abdomen) lesions are very frequent in Maffucci's syndrome patients and periodic surveillance is mandatory. It is uncertain, weather are mutations of single gen or in combination (germ line and/or somatic) responsible for disorder of cell proliferation or apoptosis in Ollier's disease and Maffucci's syndrome. Epiphyseal- metaphyseal enchnodromatosis is described as new clinical entity due to fact growth plate arrest and joint incongruity in each of eight presented patients (Gabos&Bowen, JBJS-Am, 1998.). Fibrous Dysplasia (FD). Fibrous dysplasia is a benign fibro-osseous disorder of bone characterized by replacement of normal lamellar cancellous bone with abnormal immature fibrous tissue. This genetic but not-inherited disease may be presented in one (monoostotic) or several (polyostotic) bones. Polyostotic form can be in one (monomelic) or multiple (polymelic) limbs. Clinical triad i.e. polyostotic FD associated with café-au-lait skin hyper-pigmentation and hyperfunctioning endocrinopathies are characteristic features for patients with McCune-Albright syndrome (MAS). Phosphaturia is often associated with FD and MAS, and it is contributing factor for higher incidence of fractures. Monostotic form occurs more frequent in 75% to 80% of patients. The most common locations of lesion are ribs, proximal femur, tibia and cranio-facial bones with symptoms occurring in late childhood or adolescence, but usually patients are asymptomatic. In polyostotic form skeletal changes are usually more severe presenting with pain, deformity and LLD. Plain radiographs show cystic lesions in medullar region with thinning of cortex. Diagnostic delay and pitfalls are frequent in monostotic forms. Bone scan shows increase uptake within lesion. Asymptomatic monostotic FD has usually benign natural history except in high-risk region i.e. proximal femur where microfractures can lead to typical shepherd’s crook deformity. Surgery in FD and MAS is indicated depending on size and location of the lesion. Extended curettage and grafting (cortical bone grafts) and internal fixation are required to heal the lesion and correct deformity. However, recurrence of FD occurs rather frequently and monitoring of the lesion is mandatory up to 5 years. Renal osteodystrophy (ROD). End-stage renal failure (ESRF) in children will result with numerous musculoskeletal manifestation of distorted bone mineral homeostasis. Despite advances in peritoneal and haemodialysis, allograft renal transplantation many patients will develop ROD and approximately one third of patients will develop musculo-skeletal deformities. Those deformities are the most common genu valgum, followed by ankle valgum/varum, epiphyseolysis (often bilateral), coxa vara, pathological fracture, bowing of long bones, osteonecrosis, brown tumor, scoliosis and pes equinovarus. Many younger patients in whom ESRF develop before 3 years of age will have short stature and significant muscular weakness. Bilateral genu valgum and bilateral slipped capital femoral epiphysis (SCFE) are common orthopaedic problems that require treatment. Stabilisation of the patient’s metabolic profile should be obtained pre-operatively. Treatment of genu valgum has gained evolution from more extensive surgery i.e. corrective distal femoral/proximal tibial osteotomy and angular plate fixation to epiphyseal stapling with (rh) growth hormone (8) and Ilizarov method (9). Contemporary treatment of genu valgum in patients with ROD is facilitated with guided growth tension band concept. The goal of treatment in patients with SCFE is prevention of progression of slippage and closure of the physis. This could be obtained by standard method of single screw fixation, although metabolic control of ROD is essential. In case of pathological fracture in lower extremities intramedullary fixation is treatment of choice. List of selected references: 1. Shrader MW, Schwab JH, Shaughnessy WJ, Jacofsky DJ. Pathologic femoral neck fractures in children. Am J Orthop 2009 ; 38:83-6. 2. Shapiro F. Ollier's disease. An assessment of angular deformity, shortening and pathological fracture in twenty-one patients. J Bone Joint Surg (Am) 1982 ; 64:95-103. 3. Popkov D, Journeau P, Popkov A, Haumont T, Lascombes, P. Ollier's disease limb lengthennig: Should intramedullary nailing be combined with circular external fixation? Orthop Traumatol Surg Res 2010 ; 96:348-53. 4. Liu J, Hudkins PG, Swee RG, Unni KK. Bone sarcomas associated with Ollier's disease. Cancer 1987 ; 59:1376-85. 5. Laville J-M. Flexible Intramedullary nailing (FIN) and bone weakness. Chap. 21 in: Lascombes P. Flexible intramedullary nailing in children, Springer-Verlag, Berlin-Heidelberg, 2010:p.263-77. 6. Ippolito E, Bray EW, Corsi A, De Maio F, Exner UG, Gerhon Robey P, et al. Natural history and treatment of fibrous dysplasia of bone: a multicenter clinicopathologic study promoted by the European Pediatric Orthopaedic Society. J Pediatr Orthop B 2003 ; 12:155-77. 7. DiCaprio MR, Enneking WF. Fibrous dysplasia. Pathophysiology, evaluation and treatment. J Bone Joint Surg (Am) 2005 ; 87:1848- 64. 8. Omoloja AA, Kumar K, Crawford AH, Strife CF. Epiphyseal stapling and recombinant human growth hormone for correction of genu valgum in children with chronic renal insufficiency. J Pediatr Orthop 2003 ; 23:639-42. 9. Bar-On E, Horesh Z, Katz K, Weigl DM, Becker T, Cleper R, Krause I, Davidovits M. Correction of lower limb deformities in children with renal osteodystrophy by Ilizarov method. J Pediatr Orthop 2008 ; 28:747-51.

Izvorni jezik
Engleski

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