A New Subform? Fast-Progressing, Severe Neurological Deterioration Caused by Spinal Epidural Lipomatosis

Spinal epidural lipomatosis (SEL) is a rare condition caused by hypertrophic growth of epidural fat. The prevalence of SEL in the Western world is approximately 1 in 40 patients and is likely to increase due to current medical and socio-economic developments. Rarely, SEL can lead to rapid severe neurological deterioration. The pathophysiology, optimal treatment, and outcome of these patients remain unclear. This study aims to widen current knowledge about this “SEL subform” and to improve its clinical management. A systematic literature review according to the PRISMA guidelines using PubMed, Scopus, Web of Science, and Cochrane Library was used to identify publications before 7 November 2021 reporting on acute/rapidly progressing, severe SEL. The final analysis comprised 12 patients with acute, severe SEL. The majority of the patients were male (9/12) and multimorbid (10/12). SEL mainly affected the thoracic part of the spinal cord (11/12), extending a median number of 7 spinal levels (range: 4–19). Surgery was the only chosen therapy (11/12), except for one critically ill patient. Regarding the outcome, half of the patients regained independence (6/11; = modified McCormick Scale ≤ II). Acute, severe SEL is a rare condition, mainly affecting multimorbid patients. The prognosis is poor in nearly 50% of the patients, even with maximum therapy. Further research is needed to stratify patients for conservative or surgical treatment.

Spinal Epidural Lipomatosis Associated with Intrathecal Flow Voids: Demonstration of Engorged Veins Using Flat Panel Catheter Angiotomography

We present a case of a 54-year-old male with spinal epidural lipomatosis who had associated flow voids on magnetic resonance imaging with dilated intrathecal vessels. During spinal angiogram, 20s DynaCT (flat panel catheter angiotomography) was utilized to demonstrate the intrathecal engorged veins. Venous engorgement of epidural venous plexus has been previously described in epidural lipomatosis; however, dilated intrathecal perimedullary veins have not been demonstrated by imaging. We have described the utility of flat panel catheter angiotomography in understanding venous disorders in such patients.

Surgical management of thoracic myelopathy from long-segment epidural lipomatosis with skip hemilaminotomies: illustrative case

BACKGROUND Thoracic spinal epidural lipomatosis (SEL) involves the pathological overgrowth of histologically normal, unencapsulated adipose tissue that can compress the spinal cord and cause myelopathy. SEL has been associated with multiple medical conditions, including Scheuermann kyphosis (SK). Optimal treatment strategies for SEL, especially in the setting of a sagittal spinal deformity, remain unclear. OBSERVATIONS In this report, the authors discussed surgical management of a patient with thoracic SEL and SK using skip hemilaminotomies for resection of the epidural adipose tissue. To the authors’ knowledge, only one other report described a similar surgical technique in a patient who did not have a spinal deformity. LESSONS When conservative efforts fail, thoracic SEL may require surgical treatment. Surgical planning must account for co-medical conditions such as SK. The described approach involving skip laminotomies, which minimizes spine destabilization, is a viable option to treat SEL spanning multiple spinal segments. Prognosis after surgical treatment varies and is impacted by multiple factors, including severity of preoperative neurological deficits.

Histopathological and Cytogenetic Analysis of Epidural Adipose Tissue in Symptomatic Lumbar Epidural Lipomatosis

Lumbar epidural lipomatosis (LEL) is characterized by the abnormal accumulation of unencapsulated adipose tissue in the spinal epidural space. Symptomatic LEL occurs in middle aged and elderly patients, and it is most often seen in males. The purpose of this study was to elucidate the histopathological and cytogenetic characteristics of the epidural lipomatosis in patients with symptomatic LEL. Six patients undergoing decompressive spinal surgery (mean age, 69.4 years) were enrolled between 2013 and 2017. Three cases were steroid-induced and 3 cases were diagnosed as idiopathic LEL. We compared the differences in histological morphology between the subcutaneous fat tissue and epidural fat tissue in each patient. We also analyzed the karyotype of resected epidural lipomatous tissue using the G-band method. The epidural adipocytes were histologically more irregular and smaller compared with the subcutaneous adipocytes in all cases. The mean size of subcutaneous adipocytes and epidural adipocytes was 5,485.6 ± 2,584.1 µm2 and 2,705.4 ± 1,069.1 µm2, respectively. In cytogenetic analysis of the epidural adipocytes, loss of the Y chromosome (LOY) was found in all 6 cases. The mechanisms related to the development of LEL are not well understood. In this study, the size of the lipid component of epidural adipocytes was significantly smaller than that of subcutaneous adipocytes in LEL. Cytogenetically, LOY was frequently recognized. Although this may be an effect rather than a cause, LOY may be related to morphologic changes in and proliferation of adipocytes in LEL. LOY might partially contribute to the pathological mechanism or expression of LEL.

MRI Features and Clinical Significance of Spinal Epidural Lipomatosis: All You Should Know

: Spinal epidural lipomatosis (SEL) is defined as the abnormal accumulation of unencapsulated adipose tissue in the spinal epidural space. SEL can be asymptomatic or can cause a wide range of symptoms, the most common of which is neurogenic claudication. Several other neurological manifestations may also occur, above all myelopathy and radicular symptoms. The spinal level most frequently involved in patients with SEL is the lumbar one, followed by the thoracic one. Imaging plays a key role in disease assessment. MRI is considered the most effective and sensitive modality for diagnosing and staging SEL. Anyway, also CT scan can diagnose SEL. The diagnosis may be incidental (in mild-moderate disease) or may be taken into account in cases with neurological symptoms (in moderate-severe disease). There are some recognized risk factors for SEL, the most common of which are exogenous steroid use and obesity. Recent studies have found an association between SEL and obesity, hyperlipidemia and liver fat deposition. As a matter of fact, SEL can be considered the spinal hallmark of metabolic syndrome. Risk factors control represents the initial treatment strategy in patients with SEL (e.g. weight loss, steroid therapy suspension). Surgical decompression may be required when conservative treatment fails or when the patient develops acute/severe neurological symptoms.