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Spinal epidural lipomatosis with acute and rapidly progressive neurological deterioration – systematic literature review, meta-analysis, and case report
Spinale epidurale Lipomatose mit akuter und schnell fortschreitender neurologischen Verschlechterung – systematische Literaturübersicht, Metaanalyse und ein Fallbericht
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Veröffentlicht: | 4. Juni 2021 |
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Objective: Spinal epidural lipomatosis (SEL) is a rare condition defined by hypertrophic growth of epidural fat. The incidence of SEL in the Western world is likely to increase due to the current medical and socio-demographic development. Unfortunately, little is known about this disease, and choosing the best therapy is difficult, especially when facing a rare clinical presentation: “Acute onset SEL with severe neurological deterioration.” With this study, we aim to widen current knowledge about this SEL subform and improve its clinical/therapeutic management.
Methods: We performed a systematic literature search using PubMed, Scopus, Web of Science, and Cochrane Library to identify all original articles published before November 04th, 2020, reporting on acute/rapidly-progressing, severe SEL (≤72 h from first symptoms to severe disability). Additionally, according to CARE guidelines, we report on the diagnostic workup, decision-making, surgical treatment, and outcome of a 67 years old male suffering from acute functional paraplegia caused by SEL.
Results: Of 509 identified unique records, the final analysis comprised 11 patients with acute, severe SEL reported in 10 publications (published between 1987 and 2014). The majority of the patients were male (9/11) and multimorbid (9/11; quantified by a median Charlson Comorbidity Index [CCI] score of 2). The SEL mainly affected the thoracic part of the spinal cord (11/11, with one case, also involving the cervical and the lumbar spine, respectively) and extending a median number of 4 spinal levels (range: 4-19). Surgery was the only chosen therapy (10/11) except for a critically-ill patient who died shortly after the diagnosis. Regarding the outcome, only half of the patients regained independence (5/10; = mMcCS ≤ II).
Conclusion: Acute, severe SEL is a rare condition, which seems to affect mainly multimorbid patients. The prognosis is devastating in nearly 50% of the patients, even when they received maximum therapy. Further research is needed to differentiate patients who are better managed conservatively from those requiring surgery.
Figure 1 [Fig. 1]