Chapter 1 Spinal Epidural Lipomatosis: A Rare Clinical Entitiy

Transcription

1 Chapter 1 Spinal Epidural Lipomatosis: A Rare Clinical Entitiy Schäfer VS 1,2 * 1 Department of Internal Medicine I, Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Germany 2 Acura Rheumatology Center Rhineland Palatinate, Germany * Corresponding Author: Schäfer VS, Department of Internal Medicine I, Division of Rheumatology and Clinical Immunology, University Medical Center of the Johannes Gutenberg University Mainz, Germany, valentin.s.schaefer@ hotmail.de First Published September 25, 2017 Copyright: 2017 Schäfer VS. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source. 2

2 Abstract Spinal epidural lipomatosis (SEL) is characterized by an excessive accumulation of fat within the epidural space. Most commonly the thoracic and lumbar spine is affected. SEL is a rare entity, which can lead to compression of the spinal canal. The exact pathogenesis is still unknown. Many entities like exogenous or endogenous glucocorticoid excess, injections of glucocorticoids, hypothyreosis, obesity and others are reported to be linked to the development of SEL, but there are idiopathic forms as well. The symptoms depend on the severity of the SEL and may present clinically unapparent or as non-specific back pain, radiculopathies up to spinal cord compression syndrome. The diagnosis is usually made by MRI of the affected spinal segment. The treatment varies between discontinuation of glucocorticoids and weight reduction. In the case of progressive neurological symptoms due to spinal cord or root compression, surgical spinal decompression is indicated. Spinal Epidural Lipomatosis Spinal epidural lipomatosis (SEL) is a rare disease entity. The most frequent cause is exogenous long-term administration of glucocorticoids (usually mg prednisone equivalent / day) [1]. An exact time interval, in which the occurrence is to be expected, cannot be defined on the basis of the available literature (data from 3

3 months to several years), an association with the glucocorticoid dose administration is certainly to be expected. Other rather rare causes are summarized in table 1. Typically, male patients of middle age are affected (mean age 43 years) [1,2]. SEL is predominantly encountered in the thoracic and lumbar spine, affection of the cervical spine has not yet been described in the literature [3]. Endogenous as well as exogenous excess of glucocorticoids leads to a cushingoidal fat redistribution in the body and presumably to a hypertrophy of the epidural fat tissue [3]. Histological analyses show interestingly no relevant difference to ordinary subcutaneous fat tissue [2]. The clinical manifestations are manifold and can range from nonspecific lumbago, radicular symptoms [4], increasing lower limb paresis [5] up to Cauda equina syndrome [6]. The beginning of the symptoms is usually creeping with slow progression [3]. The diagnosis of a SEL is made by MRI of the affected spinal segments. The epidural adipose tissue displays as hyperintense in T1- and T2-weighted sequences with possible compression of the spinal cord, in the lumbar region possibly leading to Cauda equina syndrome or isolated nerve root affection.figure one to three displays typical MRI findings in SEL. In the lumbosacral region typically a so-called Ysign of the dural sac in axial sections is visible [7]. 4

4 Based on new data it is assumed that due to the increasing frequency of obesity also the incidence of the SEL increases. For this reason, SEL should be included in the differential-diagnostic considerations in typical clinical presentation and glucocorticoid medication [8]. Depending on the seriousness of the symptoms, a weight reduction as well as a reduction of the glucocorticoid dose must be urgently sought, which often leads to a reduction in the symptoms [1,5,9,10]. Robertson et al. [5] recommend a weight reduction of at least 15 kilograms. In the case of progressive neurological symptoms due to spinal cord or root compression, surgical spinal decompression is indicated [11], which, according to Fessler et al. [1], is subject to a one-year mortality rate of up to 22% due to postoperative complications. There are currently no data on the risk of recurrence. Conclusion for the Clinic -- In the case of steroid-dependent patients with lower back pain and radical symptoms, SEL should be considered after exclusion of other causes. -- The diagnosis is performed by means of an MRI of the affected spinal cord segments. -- Late identification can lead to Cauda-equina syndrome. -- Glucocorticoids are absolutely contraindicated in SEL. 5

5 Figure 1: T2-weighted MRI, transversal section, at height of the lumbar vertebral body 3/4. Epidural space narrowing with fat-signal epidurally (arrow) affecting the posterior subarachnoid space. 6

6 Figure 2: Sagittal MRI SURVEY of the spine from cervical vertebral body 5 to thoracic vertebral body 12 with an increase of the epidural fat tissue (arrows). 7

7 Figure 3: Sagittal T1- and T2-weighted MRI of the cervico-thoracic transition with moderate (T2) and strong (T1) epidural hyperintensity at the beginning of thoracic vertebral body 1 (arrows), due to an increase of epidural adipose tissue. Table 1: Possible causes of spinal epidural lipomatosis. Etiologies Exogenous glucocorticosteroids, ca. 75% [12-14] Epidural glucocorticosteroid injections [15,16] Endogenous Cushing syndrom [9] Hypothyreosis [17] Hypophysal tumors [3] Obesity [4] Idiopathic [6,17-20] 8

8 References 1. Fessler RG, Johnson DL, Brown FD, Erickson RK, Reid SA, et al. Epidural lipomatosis in steroidtreated patients. Spine. 1992; 17: Fassett DR, Schmidt MH. Spinal epidural lipomatosis: a review of its causes and recommendations for treatment. Neurosurgical focus. 2004; 16: E Artner J, Leucht F, Cakir B, Reichel H, Lattig F. [Spinal epidural lipomatosis]. Der Orthopade. 2012; 41: Qasho R, Ramundo OE, Maraglino C, Lunardi P, Ricci G. Epidural lipomatosis with lumbar radiculopathy in one obese patient. Case report and review of the literature. Neurosurgical review. 1997; 20: Robertson SC, Traynelis VC, Follett KA, Menezes AH. Idiopathic spinal epidural lipomatosis. Neurosurgery. 1997; 41: Lisai P, Doria C, Crissantu L, Meloni GB, Conti M, et al. Cauda equina syndrome secondary to idiopathic spinal epidural lipomatosis. Spine. 2001; 26: Kuhn MJ, Youssef HT, Swan TL, Swenson LC. Lumbar epidural lipomatosis: the Y sign of thecal sac compression. Computerized medical imaging and graphics : the official journal of the Computerized Medical Imaging Society. 1994; 18:

9 8. Fogel GR, Cunningham PY, Esses SI. Spinal epidural lipomatosis: case reports, literature review and meta-analysis. The spine journal : official journal of the North American Spine Society. 2005; 5: Chapman PH, Martuza RL, Poletti CE, Karchmer AW. Symptomatic spinal epidural lipomatosis associated with Cushing s syndrome. Neurosurgery. 1981; 8: Haddad SF, Hitchon PW, Godersky JC. Idiopathic and glucocorticoid-induced spinal epidural lipomatosis. Journal of neurosurgery. 1991; 74: Sato M, Yamashita K, Aoki Y, Hiroshima K. Idiopathic spinal epidural lipomatosis. Case report and review of literature. Clinical orthopaedics and related research. 1995: Ebright JR, Stellini MA, Tselis AC. Spinal epidural lipomatosis in a human immunodeficiency viruspositive patient receiving steroids and protease inhibitor therapy. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America. 2001; 32: E Kotilainen E, Hohenthal U, Karhu J, Kotilainen P. Spinal epidural lipomatosis caused by corticosteroid treatment in ulcerative colitis. European journal of internal medicine. 2006; 17: Moller JC, Cron RQ, Young DW, Girschick HJ, Levy DM, et al. Corticosteroid-induced spinal 10

10 epidural lipomatosis in the pediatric age group: report of a new case and updated analysis of the literature. Pediatric rheumatology online journal. 2011; 9: Sandberg DI, Lavyne MH. Symptomatic spinal epidural lipomatosis after local epidural corticosteroid injections: case report. Neurosurgery. 1999; 45: Tok CH, Kaur S, Gangi A. Symptomatic spinal epidural lipomatosis after a single local epidural steroid injection. Cardiovascular and interventional radiology. 2011; 34: S Toshniwal PK, Glick RP. Spinal epidural lipomatosis: report of a case secondary to hypothyroidism and review of literature. Journal of neurology. 1987; 234: Bednar DA, Esses SI, Kucharczyk W. Symptomatic lumbar epidural lipomatosis in a normal male. A unique case report. Spine. 1990; 15: Payer M, Van Schaeybroeck P, Reverdin A, May D. Idiopathic symptomatic epidural lipomatosis of the lumbar spine. Acta neurochirurgica. 2003; 145: Stern JD, Quint DJ, Sweasey TA, Hoff JT. Spinal epidural lipomatosis: two new idiopathic cases and a review of the literature. Journal of spinal disorders. 1994; 7: