Services on Demand
Article
Indicators
Related links
- Cited by Google
- Similars in Google
Share
South African Journal of Surgery
On-line version ISSN 2078-5151
Print version ISSN 0038-2361
S. Afr. j. surg. vol.47 n.4 Cape Town Nov. 2009
CASE REPORT
Osteopetrosis - a challenge for the orthopaedic surgeon
Dieter CadoschI; Oliver P. GautschiII; Thomas BrockampIII; René ZellwegerIII
IM.D., PH.D.C; Department of Orthopaedic and Trauma Surgery, Royal Perth Hospital, Perth, and School of Anatomy and Human Biology, university of Western Australia, Australia
IIM.D; Department of Orthopaedic and Trauma Surgery, Royal Perth Hospital, Perth, and School of Anatomy and Human Biology, university of Western Australia, Australia
IIIM.D; Department of Orthopaedic and Trauma Surgery, Royal Perth Hospital, Perth, Australia
SUMMARY
Osteopetrosis (OP) is a rare heterogeneous group of inherited skeletal dysplasias characterised by osteoclast dysfunction, impaired bone resorption and poor bone remodelling. Three groups can be categorised on the basis of clinical findings. These include neurological symptoms, haematological abnormalities and renal tubular acidosis in the first group. Increased bone density, osteomyelitis and frequent fractures are the clinical findings in the second group, and the third group have normal life expectancy but may develop cranial nerve compression and osteomyelitis. Fractures in patients with OP are common and require appropriate pre-, peri- and postoperative management. The long bones are most frequently affected, fractures of the femoral neck and proximal (upper third) shaft being particularly common. This case report proposes possible operative fracture treatment in a patient with OP and highlights the potential perioperative pitfalls in this rare surgical population.
“Full text available only in PDF format”
REFERENCES
1. Tolar J, Teitelbaum SL, Orchard PJ. Osteopetrosis. Mechanisms of disease. N Engl J Med 2004; 351: 2839-2849. [ Links ]
2. Benichou OD, Laredo JD, de Vernejoul MC. Type II autosomal dominant osteopetrosis (Albers-Schönberg disease): clinical and radiological manifestations in 42 patients. Bone 2000; 26: 87-93. [ Links ]
3. Bollerslev J, Andersen PE. Radiological, biochemical and hereditary evidence of two types of autosomal dominant osteopetrosis. Bone 1998: 9: 7-13. [ Links ]
4. Kleinberg S. Osteopetrosis. Am J Surg 1954; 87: 50-62. [ Links ]
5. Hasenhuttl K. Osteopetrosis: review of the literature and comparative studies on a case with twenty-four years follow up. J Bone Joint Surg Am 1962; 44: 359-370. [ Links ]
6. Milgram JW, Jast YM. Osteopetrosis: a morphological study of twenty-one cases. J Bone Joint Surg Am 1982; 64: 912-929. [ Links ]
7. Chhabra A, Westerlund LE, Kline J, Mclaughlin R. Management of proximal femoral shaft fractures in osteopetrosis: a case series using internal fixation. Orthopedics 2005; 6: 587-592. [ Links ]
8. Yang BJ, Chen CF, Lien IN. Rehabilitation of left femoral subtrochanteric fracture in osteoporosis: a case report. Taiwan Yi Xue Hui Za Zhi 1980; 79: 1180-1187. [ Links ]
9. Gupta R, Gupta N. Femoral fractures in osteopetrosis: Case reports. J Trauma 2001; 51: 997-999. [ Links ]