A rare case of osteoporotic spine fracture associated with epidural lipomatosis causing paraplegia following long-term cortisone therapy

Abstract

Cushing's syndrome is frequently associated with osteoporosis. Therefore, the incidence of osteoporotic spine fractures is significant. They are a rare cause of paraplegic syndromes. Additionally, epidural lipomatosis may occur in those patients. The combination of both fracture and lipomatosis may cause neurological deficit. A case of a young patient suffering from drug-induced Cushing's syndrome is reported. She developed progressive paraplegia. Radiographs demonstrated kyphosis of the thoracic spine from T7 to T9 and pathologic fractures. Urgent operation was planned to stabilize and decompress the spinal cord in the area of the kyphosis. Fortunately, magnetic resonance imaging (MRI) was conducted first. It confirmed pathologic fractures of T7-9 but also showed massive epidural fat extending from the level of T1 to T9. As suspected, laminectomy alone in the area of the fracture proved to be insufficient, as shown by myelography during operation. For treatment of paraplegia in this case of symptomatic epidural lipomatosis, an expanded laminectomy was necessary to remove all the epidural fat. Having undergone this procedure, the patient is now recovering from paraplegia. Our experience suggests that care should be taken before operative treatment of patients with pathological fractures in combination with Cushing's syndrome. In addition to vertebral fractures, epidural lipomatosis has to be taken into consideration. Those patients with neurological deficits have to be treated by an extensive laminectomy.

Spinal epidural lipomatosis in children with renal diseases receiving steroid therapy

“…There was no significant difference in the cumulative dosage of prednisone per kilogram between patients with SSEL and patients without SSEL in our study. Meanwhile in only 8 of 56 patients (14.3%), with the exception of our 5 patients, [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25] has the cumulative dosage of prednisone per kilogram been described (250, 293, 328, 347, 409, 418, 780, 1,480 mg/ kg, respectively). In 9 of 13 patients (69.2%), including our patients, SSEL occurred within a prednisone dosage of about 400 mg/kg.…”

“…This report is the first on the frequency of SSEL in children. The small number of published reports may be the result of this condition being overlooked or misinterpreted as vertebral compression fractures on plain film X-ray evaluation or steroid-induced myopathy, because SSEL is often associated with vertebral compression fractures [18 of 29 patients (62%)] [1,4,5,6,7,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25] (Table 2). Since MRI became available, we have performed spinal MRI for patients who complained of back pain or numbness as possible symptoms of early-stage SEL, and detected 5 patients with SSEL.…”

“…In 9 of 13 patients (69.2%), including our patients, SSEL occurred within a prednisone dosage of about 400 mg/kg. The cumulative dosage of prednisone described in 55 patients [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25] was between 2.2 and 357.7 g (57.4€76.7 g). Of the 55 patients, 9 (16.4%) had more than 100 g and 10 of 55 patients (18.2%) had less than 10 g. The cumulative dosage varies very widely and therefore may not be an important risk factor for SSEL.…”

“…Hierholzer et al [8] described 33 patients with SSEL in a literature review in 1996. We have identified another 29 patients, including 3 French and 2 Japanese reports [1,4,5,6,7,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25], and we therefore review a total of 62 patients, including 12 children. The underlying diseases were 10 bronchial asthma, 8 nephrotic syndrome, 7 renal transplantation, 5 heart transplantation, 4 ulcerative colitis, and others.…”

Severity of presentation is associated with time to recovery in spinal epidural lipomatosis

“…In the 17 surgical patients with available data, the time course of patients' neurological deterioration was noted in days [6][7][8]10,11,13,14,17,18,20,23,24,[26][27][28][29][30][32][33][34]36,37]. The duration of symptoms refers to the period of time between when the patient first reported noticing symptoms and their definitive surgery.…”