Detaillierte Beitrags-Information
| Beitragstitel | Corrective osteotomy in X-linked vitamin D-resistant hypophosphatemic rickets (VDXLR) needs optimal metabolic control to be successful |
|---|---|
| Beitragscode | P098 |
| Autoren | |
| Präsentationsform | Poster |
| Themengebiete |
|
| Abstract |
Introduction X-linked vitamin D-resistant hypophosphatemic rickets (VDXLR) - initially described as Phosphatediabetes by Fanconi 1) - causes severe bone metabolism disturbance leading to rickets in childhood and persistent osteomalacia in adults. Metabolic improvement is achieved with oral supplementation of vitamin D (best if using active metabolites such as 1,25(OH)2D3) and phosphate. Historically, bone healing allegedly was poor until proven different by Fucentese et al. 2). As it is a rare disorder we wish to add one more case treated successfully after failed surgeries due to metabolic neglect. Patient Case: Male patient (45y) with VDXLR and a significant femoral varus and antecurvation deformity. During childhood he had repeated corrections using Ilizarov frames, treated at the age of 10 and unlocked intramedullary nails at the age of 20. At the age of 44 years he presented to us with bilateral shepards crook deformities (CCD varus-angle of 82° on the right and 84° on the left side) and with a procurvatum of 2° on the right and 8° on the left side. Imaging studies also revealed nails having perforated the femoral cortex bilaterally – due to the recurrent varus deformity of the proximal femur. The patient needed canes for support. After improving the metabolic situation with active vitamin D metabolites and phosphate – monitored by normalized phosphate hemostasis – staged osteotomies with intramedullary nail fixation achieved axial correction and full painfree weight bearing 12 weeks after the second operation. Primary bone healing occurred on both sides. Results There were no complications during the procedures or in the postoperative follow-up period. The osteotomies united uneventfully under adequate medical treatment of the VDXLR. There was a significant improvement with painfree and unrestricted function – 3 months after the last procedure the patient was ambulatory without crutches. Conclusion The most important prerequisite for successful operative correction of severe bony deformities in VDXLR is a normal metabolic condition. First line treatment comprises of active vitamin D metabolites and phosphate. Under these circumstances, subsequent corrective osteotomies show uncomplicated bony union. 1) Fanconi G.: Der frühinfantile nephrotische-glykosurische Zwergwuchs mit hypophosphatämischer Rachitis. Jahr. Kind. 147: 299-338 2) Fucentese et al.: Metabolic and orthopedic management of VDXLR; J Child Orthop (2008) 2: 285-291 |