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Journal of Clinical Neuroscience 15 (2008) 1323–1326 www.elsevier.com/locate/jocn
Review
Spinal epidural lipomatosis – A brief review Darweesh Al-Khawaja a,*, Kevin Seex a, Guy D. Eslick b,c a
Department of Neurosurgery, Wollongong Hospital, Loftus Street, Wollongong, New South Wales 2500, Australia b School of Public Health, The University of Sydney, Sydney, New South Wales, Australia c Department of Medicine, The University of Sydney, Nepean Hospital, Penrith, New South Wales, Australia Received 23 December 2007; accepted 12 March 2008
Abstract Spinal epidural lipomatosis (SEL) is a rare disorder characterized by overgrowth of fat in the extradural space, causing compression of the neural elements. It is frequently associated with the administration of exogenous steroids or elevation of endogenous steroids. We present two patients, both with epidural lipamotosis of idiopathic origin. One was 53-year-old man with progressive mid-thoracic and lower limb pain with associated weakness and neurogenic claudication due to thoracic epidural lipomatosis. The second was a 68-yearold male with lumbar epidural lipomatosis. Co-morbidities for the first patient included diabetes and obesity; however, there was no history of steroid administration. An MRI scan revealed thoracic cord compression, with significant stenosis at T4–T9. The second patient complained of progression of longstanding lumbar pain and claudicant symptoms. There was no history of steroid intake. An MRI revealed stenosis at L4–S1 and diffuse anterior lipomatosis. A literature review revealed 49 cases of idiopathic SEL and 62 of secondary SEL. We aimed to delineate the differences between these two relatively distinct entities including their anatomical distribution, clinical presentation and prognostic implications. Crown Copyright Ó 2008 Published by Elsevier Ltd. All rights reserved. Keywords: Epidural; Spinal lipomatosis; Steroids
1. Introduction
2. Methods
Spinal epidural lipomatosis (SEL) is a rare but well-recognized condition. It is defined as a pathological overgrowth of normal fat tissue in the extradural space. Steroid production (exogenous or endogenous) is the main aetiological factor associated with SEL; the first case of steroid-induced lipomatosis was reported by Lee et al.1 Other reported secondary causes include adrenal tumors, hypothyroidism, hyperprolactinemia and other endocrinopathies. Many cases have emerged without evidence of any clear predisposing factors. Badami et al.2 reported the first case in 1982 but Haddad in 1991 coined the term idiopathic SEL.3 We illustrate two patients with idiopathic SEL and review the literature.
2.1. Search strategy
*
Corresponding author. Tel.: +61 2 4225 1115; fax: +61 2 4225 1119. E-mail address:
[email protected] (D. Al-Khawaja).
The major electronic databases, MEDLINE, EMBASE, PubMed and Current Contents were searched (January 1966–July 2006). Additional manual searches were made using the reference lists from the selected articles to retrieve other papers relevant to the topic. There was no language restriction. The key words used included spinal, epidural, lipomatosis, and idiopathic. 3. Case reports 3.1. Case 1 3.1.1. Presentation and examination Our first patient was a 53-year-old male presenting with a 3 year history of mid-dorsal pain preventing him from
0967-5868/$ - see front matter Crown Copyright Ó 2008 Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2008.03.001
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oped mild left leg weakness, which recovered completely with physiotherapy. His dorsal pain, neurogenic claudication, and the myelopathic changes disappeared. 3.2. Case 2
Fig. 1. T1-weighted sagittal MRI of Case 1 showing the epidural lipomatosis compressing the upper thoracic spinal cord.
lying supine and a 1 year history of progressive bilateral lower-limb pain, weakness and neurogenic claudication, with incontinence developing 6 months prior to his presentation. He was a smoker and had diabetes but had no history of steroid administration. On physical examination, he was obese (body mass index (BMI) 33.9), had thoracic kyphosis and moderate mid-dorsal tenderness. There was pyramidal right leg weakness (power 3–4/5), decreased sensation over the L5 dermatome bilaterally and increased tone with bilateral upgoing plantar responses. Laboratory evaluation, including full blood count (FBC), electrolytes and creatinine (EUC), liver function tests, vitamin B12 and folate, urine analysis and culture, thyroid function tests, adrenocorticotropic hormone (ACTH) and cortisol, were all unremarkable. MRI demonstrated lipomatosis, of the thoracic spine with significant compression between T4 and T9 (Figs. 1 and 2). 3.1.2. Operation and outcome The patient underwent T4–T9 laminectomy. A thick layer of epidural fat was removed completely. He devel-
3.2.1. History and examination Our second patient was a 68-year-old male who complained of progression of longstanding lumbar pain and claudicant symptoms predominantly in the right anterior thigh and lateral lower leg. Symptoms were precipitated by standing longer than 10 minutes or walking greater than 200 m. He had a fall 14 years previously that resulted in a wedge fracture of L1 and subsequent mild lower back pain. He had diabetes (on oral hypoglycemics) and had a history of ischemic heart disease, which was treated with coronary stenting. He weighed 94 kg, had serum lipids within normal limits and no history of steroid intake. Examination revealed normal reflexes, power and sensation in the lower limbs and straight leg raising was negative. An MRI scan revealed small generalized posterior disc protrusions from L3–L4 to L5–S1. There was canal compromise, greatest at L4–L5 and L5–S1, due to a combination of disc disease and facet hypertrophy but also significant anterior lipomatosis. 3.2.2. Operation and outcome An L3–L5 laminectomy was performed without complications. Epidural fat was encountered ventral and dorsal to the thecal sac. It was removed using suction and bipolar diathermy. The patient’s symptoms resolved. 4. Discussion Our patients were classified as having idiopathic SEL as they were neither receiving steroids nor did they have any kind of endocrinopathy. We conducted a systematic lierature review1–63 to compare the two groups of SEL (idiopathic and secondary), notably their different anatomic distribution and prognostic significance (Table 1). Overall, most patients with SEL are obese, and more males than females are affected. The mean number of segments involved is similar in both groups (4–5 segments in
Fig. 2. TI-weighted axial MRI of Case 1 showing epidural lipomatosis compressing the spinal cord.
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Table 1 Characteristics and outcome data for 111 cases of spinal epidural lipomatosis (idiopathic vs secondary) Category
Distribution
Mean no. segments
Presentation
Management
Outcome
Idiopathic (49 cases; 90% male; 70% obese; mean age 46 years)
Thoracic (35%)
4.3
88% surgical; 12% conservative
50% full recovery
Lumbar (65%)
2
70% myelopathy, 5% paraplegia 60% pain (back/leg), 30% claudication, 30% leg weakness
88% surgical; 12% conservative
60% full recovery
Secondary (62 cases; 80% male; 70% obese; mean age: 44 years)
Thoracic (73%)
5
90% surgical; 10% conservative
Lumbar (35%)
2
15–20% full recovery 25% nil improvement 60% full recovery
50% myelopathy; 25% paraplegia 50% pain (back/leg), 10% claudication 25%, leg weakness
the thoracic spine and 2 segments in the lumbar spine). However, the distribution varied substantially, with secondary disease favoring the thoracic spine and idiopathic, the lumbar spine. The results of surgery were similar (60% full recovery) when the disease involved the lumbar spine in both idiopathic and secondary groups. However, in thoracic cases, the outcomes differed: the idiopathic group reported improved outcome response with 50% enjoying a full recovery whereas only 15–20% of those with secondary thoracic disease had a good outcome, possibly because 25% of patients in the secondary group initially presented with paraplegia. Surgical management was sought in almost 90% of cases. However, when patients had secondary lumbar disease only 65% underwent surgery, which suggests a greater role for conservative management in SEL in this location. In conclusion, SEL is a condition in which excess adipose tissue deposits in the spinal canal cause radiculopathy or spinal cord compression. It may be idiopathic or secondary to factors such as steroid excess or other endocrinopathies. Obesity seems an important factor in both groups. Although weight reduction seems beneficial, this is applicable only in those patients who present with mild symptoms but no significant signs. Surgical intervention seems the treatment of choice when patients present with abnormal neurological signs. Acknowledgement I would like to thank Dr. Tamadur Mahasneh for her help with data collection and literature review. Her contribution is highly appreciated for making this article successful. References 1. Lee M, Lekias J, Gubbay SS, et al. Spinal cord compression by extradural fat after renal transplantation. Med J Aust 1975;1:201–3. 2. Badami JP, Hinck VC. Symptomatic deposition of epidural fat in a morbidly obese woman. Am J Neuroradiol 1982;3:664–5. 3. Haddad SF, Hitchon PW, Godersky JC. Idiopathic and glucocorticoid-induced spinal epidural lipomatosis. J Neurosurg 1991;74: 38–42.
65% surgical; 35% conservative
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