Extradural lipomatosis presenting with paraplegia.
M Deogaonkar, A Goel, K Tingare, H Dahiwadkar, R Nagpal
Department of Neurosurgery, KEM Hospital, Parel, Mumbai.
Department of Neurosurgery, KEM Hospital, Parel, Mumbai.
An unusual case with spinal extradural lipomatosis in a non-obese and otherwise healthy man is reported. The patient presented with a history of weakness of legs which progressed to paraplegia over a 40 day period.
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Deogaonkar M, Goel A, Tingare K, Dahiwadkar H, Nagpal R. Extradural lipomatosis presenting with paraplegia. J Postgrad Med 1995;41:85-6
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Deogaonkar M, Goel A, Tingare K, Dahiwadkar H, Nagpal R. Extradural lipomatosis presenting with paraplegia. J Postgrad Med [serial online] 1995 [cited 2019 Nov 12 ];41:85-6
Available from: http://www.jpgmonline.com/text.asp?1995/41/3/85/485
Extradural lipomatosis is a pathological overgrowth of normal fat. Most of the reported cases of spinal lipomatosis are usually associated with long-term steroid administration or obesity,. Idiopathic extradural lipomatosis is very rare, and our case belongs to this particular group. The usual presentation is with pain, paraesthesiae and/or paraparesis. In the literature search, we failed to come across any report in which a patient with lipomatosis presented with paraplegia.
A 20-year-old male patient presented with a 40 day history of progressive numbness and tingling paraesthesiae in the legs with weakness of both legs and incontinence of urine and stools. He had moderate grade fever for 2 days prior to the onset of symptoms. He had become paraplegic 2 days prior to admission. When admitted, he had flaccid paraplegia with a complete sensory loss below the level of the 4th thoracic dermatome. A lumbar myelogram was unsuccessful due to extradural extravasation of the contrast. Myelogram following lateral cervical injection of the contrast revealed block to caudal flow of contrast at 67 thoracic level. The block suggested a posterior extradural lesion. The patient was taken up for emergency surgery. The presence of a thick layer of extradural fat (much more than usual) from 5th to 9th thoracic levels was an unexpected finding. A wide laminectomy with as much excision of the lipomatous tissue as possible was carried out. At the end of the excision, the dural tube was pulsatile. Histo-logical examination confirmed the presence of normal fat [Figure:2]. During the 10 day postoperative period in the hospital, he showed no recovery. He has unfortunately not returned for a followup evaluation, Discussion
Histologically, extradural lipomatosis is a condition which involves excessive growth of normal extradural fat. Lee et al first reported a case of spinal lipomatosis in 1975. Since then, there have been 44 cases reported in the literature. The condition has been more frequently reported in males, the mean age being 42 years. The dorsal canal is most commonly affected, followed by the lumbosacral region. In our patient, the fat deposition was posterior to the dura as in most reported cases,,,. Thirty six of the reported patients were on therapeutic steroids for various conditions like immunocompromised states, Graves disease and dermatomyositis,. The exact mode of abnormal growth response of normal extradural fat to exogenous steroids is not clear. There are six reports wherein the extradural lipomatosis was associated with obesity,. One patient had hypothyroidism, where generalised fat deposition occurs due to suppressed lipolysis. Our literature survey revealed only one earlier patient with 'idiopathic' spinal lipomatosis.
Patients usually present with localised, chronic back pain. Slowly progressive symptoms similar to those in spinal canal stenosis have been frequently seen. Our patient was unique in that he had rapid progression of weakness in the legs and became paraplegic within a 40 day period. Acute symptomatology of this kind with rapid progression to paraplegia, in this relatively benign condition is very unusual. Such a clinical course and presentation has not been reported earlier. The differential diagnosis includes spinal tumours, abscess, haematoma and thoracic disc prolapse. As our patient had history of fever prior to onset of neurological symptoms, an extradural abscess was the first diagnosis. Magnetic resonance imaging is the investigation of choice as it may suggest the histological nature of the compressive lesion and provides a clear image of the extent of the pathology,. Myelography could only reveal the level of the block and its extradural nature. When taken up for surgery, the diagnosis in order of priority were an extradural abscess, clot, extradural neoplasm and tuberculous granuloma. In these patients, plain Xrays are always normal. An alternative to MRI is a computerised tomographic (CT) myelography. Both MRI and CT were not available in house, hence the need to do the old fashioned, butreliable myelography. There have been reports in the literature wherein there have been favourable responses to conservative treatment with weight reduction in obese patients, and to reduction of steroid dosage in cases secondary to corticosteroid administration. Most patients, how ever, have undergone wide laminectomies with excision of the extradural fat. The results have been favourable. The patient being reported failed to show any improvement in response to this form of surgery, during his short postoperative stay. We have been unsuccessful in getting him back for a follow up evaluation.
An unusually rapid progressive paraplegia in a patient with thoracic spinal extradural lipomatosis is being reported. Absence of the known predisposing factor is an additional very unusual feature, having been reported only once before. This extradural lipomatosis will have to be added to the list of the differential diagnosis of extradural spinal cord compression, albeit low down.
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