Sickle cell disease (or drepanocytosis) is a hemoglobinopathy characterized by an increase in viscosity and adhesivity of the typically sickle-shaped erythrocytes. The pathological osteo-articular involvement in the course of drepanocytosis is secondary to the avascular necrosis of the bone marrow, caused by vaso-occlusive episodes in the microcirculation during acute painful crises. Osteoporosis and extramedullary hematopoiesis are also consequences of the disease. The involvement of the spine is common, with clinical features ranging from simple changes in spinal morphology ("fish-mouth" appearance) up to vertebral bodies fractures with kyphotic deformity. In the presence of vertebral fracture, treatment options listed in the literature are conservative (rest, symptomatic therapy, orthosis), because of the high incidence of intra- and perioperative complications (acute respiratory syndrome, vaso-occlusive crisis…), in addition to the increased rate of implant failure. We report here a case of a young man affected by multiple pathological symptomatic vertebral fractures at the thoraco-lumbar junction, secondary to a well controlled but severe form of sickle cell disease. We decided to treat the patient surgically because of a worsening and potentially invalidating kyphosis deformity. We describe the surgical procedure and the management of a later complication consisting in the collapse of the osteoporotic vertebra below the instrumentation that required a surgical revision. Although a conservative approach is most frequently indicated, we believe that the surgical option should be considered when a clinical worsening occurs in a young patient with otherwise well-controlled disease.
|Number of pages||5|
|Journal||European Review for Medical and Pharmacological Sciences|
|Publication status||Published - 2014|
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