[Bilateral total hip prosthesis in osteopetrosis. Apropos of a case. Review of the literature].

PURPOSE OF THE STUDY

The authors report an original case of Albers-Schönberg's disease and describe its surgical treatment.

MATERIAL

Comparison between roentgenogram of the pelvis at the age of 9 and 46 years showed the original evolution of coxa vara in a case of osteopetrosis. A 46 year old male had severe pain and instability in both hips related to numerous problems consisting of: 1) on the right side: subtrochanteric femoral non-union after repeated fractures, head and neck femoral disappearance associated to dislocation. 2) on the left side: acetabular dysplasia, osteoarthritis and subtrochanteric femoral fracture that had fusionned. Total hip arthroplasty was performed on both hips.

RESULT

Clinical result was good in both hips (PMA score = 6-6-4) at the latest follow-up (four years on the left side and three years and four months on the right side). The surgical procedures were difficult because of the hardness and brittle character of the bony substance and because of non unions and deformities. Special care was required to avoid shattering femoral diaphysis and false routes during creation of a new medullary canal.

CONCLUSION

Total hip prosthesis represents a functionnal salvage procedure in patients with Albers-Schönberg's disease.