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

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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.
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Citation: Dinger, T.F.; Eerikäinen,
M.S.; Michel, A.; Gembruch, O.;
Darkwah Oppong, M.; Chihi, M.;
Blau, T.; Uerschels, A.-K.; Pierscianek,
D.; Deuschl, C.; et al. A New
Subform? Fast-Progressing, Severe
Neurological Deterioration Caused
by Spinal Epidural Lipomatosis. J.
Clin. Med. 2022,11, 366. https://
doi.org/10.3390/jcm11020366
Academic Editors: Arash
Moghaddam and Raban Heller
Received: 12 December 2021
Accepted: 10 January 2022
Published: 12 January 2022
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4.0/).
Journal of
Clinical Medicine
Article
A New Subform? Fast-Progressing, Severe Neurological
Deterioration Caused by Spinal Epidural Lipomatosis
Thiemo Florin Dinger 1, * , Maija Susanna Eerikäinen 2, Anna Michel 1, Oliver Gembruch 1,
Marvin Darkwah Oppong 1, Mehdi Chihi 1, Tobias Blau 3, Anne-Kathrin Uerschels 1, Daniela Pierscianek 1,
Cornelius Deuschl 2, Ramazan Jabbarli 1, Ulrich Sure 1and Karsten Henning Wrede 1
1
Department of Neurosurgery and Spine Surgery, University Hospital of Essen, University of Duisburg-Essen,
47057 Duisburg, Germany; anna.michel@uk-essen.de (A.M.); oliver.gembruch@uk-essen.de (O.G.);
marvin.darkwahoppong@uk-essen.de (M.D.O.); Mehdi.Chihi@uk-essen.de (M.C.);
Ann-Kathrin.Uerschels@uk-essen.de (A.-K.U.); Daniela.Pierscianek@uk-essen.de (D.P.);
Ramazan.Jabbarli@uk-essen.de (R.J.); Ulrich.Sure@uk-essen.de (U.S.); Karsten.Wrede@uk-essen.de (K.H.W.)
2Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Hospital Essen,
University of Duisburg-Essen, 47057 Duisburg, Germany; maija.eerikaeinen@uk-essen.de (M.S.E.);
Cornelius.Deuschl@uk-essen.de (C.D.)
3Institute of Neuropathology, University Hospital of Essen, University of Duisburg-Essen,
47057 Duisburg, Germany; Tobias.Blau@uk-essen.de
*Correspondence: Thiemo-Florin.Dinger@uk-essen.de; Tel.: +49-201-723-1201
Abstract:
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.
Keywords:
acute paraparesis; spinal cord injury; spinal epidural lipomatosis; spine; pathophysiology;
neurological outcome; spinal surgery; atypical fat depositions
1. Introduction
Spinal epidural lipomatosis (SEL) is defined by pathological hypertrophy of epidural
benign fat cells leading to compression of neural structures [
1
]. As a consequence of ongoing
medical and socio-economic development in the western world, the present prevalence of
SEL (1 of 40 patients who underwent dedicated magnetic resonance imaging (MRI)) will
increase along with multimorbidity and metabolic diseases [
2
]. The exact pathophysiology
of SEL remains largely unknown. However, it is regarded as a consequence of a deranged
fat metabolism (e.g., caused by endogenous or exogenous steroid excess), leading to
atypical fat depositions [
1
]. Furthermore, venous congestion has been proposed as a
leading contributing etiological factor for the severe and acute SEL subform with rapidly
progressive symptoms [3,4].
J. Clin. Med. 2022,11, 366. https://doi.org/10.3390/jcm11020366 https://www.mdpi.com/journal/jcm
J. Clin. Med. 2022,11, 366 2 of 10
The correlation of corticosteroid usage and SEL is significant because patients who
require (chronic) corticosteroid treatment are often severely ill and represent a fragile
subpopulation [
5
]. Choosing optimal therapy is difficult, as general treatment guidelines
are pending. Physicians can only rely on case reports and reviews [
1
]. Furthermore, there
is even less knowledge available on how to treat the acute SEL cases [
6
]. When faced with
rapidly progressing neurological decline, physicians are thereby left with little choice but to
offer maximum therapy (i.e., surgery) [
7
]. With the current study, we analyzed all published
patients suffering from acute, severe SEL and additionally presented the experience gained
from treating a patient at our facility. By improving the knowledge about the patients’
characteristics, symptoms, therapy, and outcome, we hope that future decisions on how to
treat acute, severe SEL will be based on sounder evidence than currently possible.
2. Materials and Methods
2.1. Systematic Review, Search Strategy, and Acquisition of the SEL Cohort Data
The systematic review was performed according to the PRISMA guidelines [
8
]. PubMed,
Scopus, Web of Science, and Cochrane Library databases were searched to retrieve all
studies published before 7 November 2021 that reported on SEL patients with acute and
rapidly progressing severe symptoms. Severe symptoms were defined as either functional
(para-)plegia or fecal and urinary incontinence. The definition of ‘’acute onset with rapid
progression” was a new episode that worsened to a clinical presentation of ‘’severe symp-
toms” in
72 h. We used different combinations of the following keywords to select all
eligible studies: ‘’spinal epidural” (OR ‘’spinal”), “lipoma*” and ‘’fast *” (OR ‘’rapid*‘’ OR
‘’quick*‘’). The exact search terms are shown in Figure S1. After the exclusion of duplicate
records, TFD and AM independently screened the titles and abstracts (and, if necessary, the
full text) to assess the eligibility of the studies. Reference lists of relevant publications were
screened for additional studies. The publications’ language was restricted to English.
Studies were eligible for the review if they (1) reported at least one case with SEL, which
fulfilled the defined criteria of (2) a ‘’severe presentation”, (3) a ‘’rapid progression/acute
onset” and had no other contributing (spinal) pathologies (like acute vertebral fractures) and
(4) contained any data on demographic, clinical, radiographic, and anatomic characteristics
or therapy and outcome of SEL patients (Figure 1).
Based on the selected studies, we extracted and summarized all available data from
previously reported SEL patients with acute onset of severe symptoms. The patient cohort
was additionally screened by TFD to exclude potential double-listed cases. The quality of
the studies was addressed by OG and RJ using an adapted quality assessment score (QAS)
(Table S1), which has been described in detail previously [
9
]. All information was extracted
from case reports reducing the points given for “minimizing selection bias “to a minimum
of 2 points (e.g., prospective, consecutive studies would have received a maximum of
8 points).
J. Clin. Med. 2022,11, 366 3 of 10
Figure 1.
Flow chart of the systematic literature review. The inclusion and exclusion process of the
initial 711 studies identified by PubMed, Scopus, Web of Science, and Cochrane database searches
(Figure S1) are shown stepwise in the flow diagram. Abbreviation: SEL—spinal epidural lipomatosis.
2.2. Data Analysis
2.2.1. Data Collection
The following parameters of the patients and SEL were recorded for further analyses:
age at diagnosis, sex, medical history, history of SEL, neurological symptoms, modified
Carlson Comorbidity Index [
10
] (mCCI), spinal levels of SEL, treatment, histopathological
record, outcome (using the modified McCormick scale [
11
,
12
]), and time until severe
symptoms, until diagnosis, and until treatment. Data extraction was performed by TFD
and controlled by AM.
2.2.2. Study Endpoints and Statistical Analysis
The defined primary endpoint was the assessment of associations between the recorded
demographic, clinical, and anatomic characteristics of SEL patients and their outcomes.
With only eleven identified individuals, the data analysis was restricted to descriptive
analysis.
2.3. Image Analysis
According to Borréet al., anterior-posterior diameter ratios of epidural fat (EF) to the
dural sack (DuS) and the spinal canal (SpiC) were calculated using Centricity
Universal
viewer (GE, Healthcare, North Richland Hills, TX, USA) [13]. Likewise, cross-section area
ratios for EF vs. DuS and SpiC were calculated (see Equations (1)–(4) and Figure 2). For all
ratio calculations, only axial planes of maximum compression level were used.
Linear indexDuS/EF =
DDuS
DEF
(1)
J. Clin. Med. 2022,11, 366 4 of 10
Linear indexEF/S piC =
DEF
DSpiC
(2)
Area indexDuS/EF =
ADuS
AEF
(3)
Area indexEF/SpiC =
AEF
ASpiC
(4)
where D= anterior-posterior diameter, A= cross-sectional area, DuS = dural sack,
EF = epidural fat, and SpiC = spinal canal.
Figure 2.
Exemplary illustration of quantification of SEL extent compared (1) to the nerval structures
and (2) to the spinal canal. (
A
) The asymptomatic situation; (
B
) the symptomatic situation. Note
that in the asymptomatic situation, the spinal canal is already almost 70% occupied by the SEL; an
increase of 10% of the SEL thus results in functional paraplegia.
3. Results
Systematic Review of the Literature
Driven by the rapid progression and severity of symptoms, we felt the need to sys-
tematically identify all documented cases of acute, severe SEL to gather all accessible
information to improve the management of this SEL subform.
A total of 11 patients who suffered from acute, severe SEL could be identified through
the systematic review of the literature [
6
,
7
,
14
22
]. In addition, there was a prospectively
recorded case from our clinic (Supplementary Text and Figure S2), giving a total of
12 patients
for analysis (Table 1). Table S2 A lists the patients’ neurological symptoms
at admission. Ten out of the twelve patients suffered from functional paraplegia, and nine
suffered from at least incomplete cauda equina syndrome (bladder/rectal dysfunctions).
Altogether, half of the patients fulfilled both criteria of a severe neurological disturbance
(functional paraplegia and (incomplete) cauda equina syndrome).
J. Clin. Med. 2022,11, 366 5 of 10
Table 1. Summary of patients with acute, severe SEL, with the first eleven patients representing the cases identified by the systematic literature review.
ID Sex Age Medical History Steroids mCCI mMcCS Spinal
Levels Surgery Histo Complications Time [h] till
Ref. Year QAS *
ADM f/u Severe Diagnosis Treatment
1 M 45 Hypothyroidism,
obesity. X 0 IV IV 11 √ √ Death 48 48 N. r. Toshniwal et al.
[14]1987 28
2 F 62
CAD, phlebitis,
Raynaud’s
syndrome,
dermatomyositis.
0 V I 5 X No
“Rapidly
progres-
sive”
“Rapidly
progres-
sive”
N. r. Buthiau et al.
[15]1988 28
3 M 52
Atopic dermatitis,
CS with old
fracture of T7.
0 V - 4 X Death 0 12 23
Kaplan et al. [
16
]
1989 28
4 M 20 None. X 0 V V 4 √ √ No 12 96 120 Meisheri et al.
[17]1996 28
5 M 27
NHL, BMT, GvHD,
pneumonia, CS,
obesity.
2 IV - 19 X X Death “few
days”
“few
days” N.t. Resnick et al. [6] 2004 24
6 M 41 HIV, metastasized
NSCLC. 10 III II 9 X No 72 72 84 Vince et al. [18] 2005 30
7 M 60 CAD, Paget’s
disease. X 2 IV I 6 √ √ No 72 72 72
Oikonomou et al.
[19]2007 30
8 M 55 AHT. X 0 IV II 5 √ √ No 24 48 48 López-González
et al. [20]2008 30
9 M 49
DM I, hepatitis C,
i.v. heroin abuse,
tobacco (30py).
X 2 N.r. N.r. 13 X No 12 12 12 Birmingham
et al. [7]2009 26
10 F 35
DM I, renal
disease,
endocarditis.
X 4 IV II 9 √ √ No
“wake up”
12 12 Stephenson et al.
[21]2014 28
11 M 69
Obesity, COPD,
DM II, AHT, hyper-
cholesterolemia.
X 4 II II 2 √ √ No “acute” N.r. N.r. Tardivo et al.
[22]2021 26
12 M 67
Adiposity, DM II,
alcohol abuse,
tobacco (40py),
NSCLC.
8 IV III 8 √ √
Wound
dehiscence,
death
24 72 72 Suppl. Materials 2022 32
Abbreviations: ADM–admission; AHT–arterial hypertension, BMT–bone marrow transplantation; CAD–coronary artery disease; CS–Cushing’s syndrome; DM–diabetes mellitus;
f/u–follow up; GvHD–graft versus host disease; mCCI–modified Charlson comorbidity index; mMcCS–modified McCormick scale; NHL–Non-Hodgkin lymphoma; n.r.–not reported;
NSCLC–non-small-cell lung carcinoma; n.t.–not treated; QAS–quality assessment score; Ref.–references; Suppl.–Supplementary. * See Table S1.
J. Clin. Med. 2022,11, 366 6 of 10
Ten out of 12 patients were male, and the median age was 50.5 years (range: 20 to
69 years). Five patients had a medical history of either endogenous or exogenous steroid
excess. Only three patients were obese (BMI > 30 kg/m
2
). The median mCCI score was two
(range: 0–10), with ten patients meeting the criteria of multimorbidity, and three patients
had a history of malignant neoplasm.
Regarding diagnostic management, all patients received laboratory workup. In five
centers, cerebrospinal fluid analysis was performed to screen for neurological disease
(Guillain-Barrésyndrome, transverse myelitis, etc.). To investigate the etiology, all centers
used imaging. Most of the centers used MRI (9/12), but in older studies, myelography was
an alternative (3/12). In some centers, a CT was performed prior to (3/12) or instead of an
MRI (post-myelography; 2/12) due to its better accessibility, especially in the past.
The number of involved spinal levels ranged from 4 to 19 (median of 7 levels), with
no publication quantifying axial extension of SEL nor compression of the spinal cord (see
Figure 2). All cases involved the thoracic spine, except for one solely lumbar SEL case,
while the cervical and lumbar spine were involved in one case. Figure 3depicts the extent
of SEL in case ID#12. In this particular case, imaging allowed comparison of the extent of
SEL in both the asymptomatic and symptomatic state (see Figure 2).
Figure 3.
Preoperative imaging. (
A
) Retrospectively, SEL was already present in the CT performed
during the diagnosis of lung cancer, 17 months earlier (sagittal and axial plane—at the level of T6).
(
B
) Between the diagnosis of lung cancer and the paraparesis, the volume of the SEL increased signifi-
cantly (preoperative CT in a sagittal and axial plane—at the level of T6) (see Figure 2).
(C) Emergent
MRI confirmed a fatty epidural tumor compressing the spinal cord (sagittal T1 TSE and axial T2 TSE
at the level of T6), causing myelopathy at the level of T7–9 as shown by a low, patchy T2-hyperintense
signal of the myelon at these levels. In addition, a general, atypical fat deposition pattern with an
increase of epidural, mediastinal, and subcutaneous fat as well as fatty muscle atrophy was observed
(
A
vs.
B
,
C
). The extent of SEL is highlighted in the sagittal planes by arrowheads and in the axial
planes by asterisks. The mass severely compressed the spinal cord, which was ventrally displaced.
Except for one patient (ID#5), who died soon after the diagnosis was revealed by
MRI [
6
], all other cases were treated surgically (see Figure 4A–C). Decompression was
performed in all these cases, with additional removal of the SEL in all surgical cases (ex-
emplarily Video S1), except for one study that did not report on SEL removal. In one
patient (ID#12), an additional dorsal fixation was performed to avoid instability caused by
multiple-level laminectomy. Regarding the time from first symptoms to surgical interven-
tion, there was a median of 60 h (range: 12–120 h). For four patients, no time until treatment
J. Clin. Med. 2022,11, 366 7 of 10
was reported. Seven cases reported a histopathological confirmation of the diagnosis (see
Figure 4D).
Figure 4.
Intraoperative and postoperative images. (
A
) Intraoperative view of the microsurgical
tumor removal. A section of SEL (yellowish mass) is lifted with an aspirator and a microsurgical punch
from dura (marked with *). (
B
) Overview of the operation field at the end of tumor removal and spinal
fixation, illustrating the extensions of the procedure. (
C
) Maximum intensity projection image of the
postoperative CT scan. (
D
) H and E stained tumor slice after histopathological preparation, allowing
the diagnosis of SEL with benign hypertrophic, unencapsulated fat cells (Bar represents 100 µm).
To analyze the outcome, the patients’ neurological status at admission versus follow-
up/discharge (f/u) was quantified and compared using the mMcCS (Table 1). For one
patient (ID#9), no outcome was reported [
7
]. Regarding the post-therapeutic recovery, two
patients (ID#6 and ID#11) improved by one point and another two patients (ID#8 & ID#10)
by two points in mMcCS. An improvement of three points and four points was observed
in two further patients, respectively (ID#2 and ID#7). Four patients did not recover at all
(ID#1, ID#3–5).
Additionally, the regained level of independence was analyzed (= mMcCS
II(-III)) to
estimate the “daily life” therapeutic benefit. Five patients did not regain independence, and
four of these patients died during hospitalization or during a period of less than
2.5 months
after treatment.
4. Discussion
These often multimorbid cases, with a rapidly progressing severe paraparesis due
to SEL, highlight the main problems when managing these patients. In contrast to the
sporadic clinical presentation of this subform, SEL typically shows a slow progression,
rarely causing a high level of impairment. Generally, the therapeutic recommendations
for SEL are based on case reports, case series, and reviews. Furthermore, no treatment
recommendations are available for the management of acute, severe SEL.
Clinical diagnosis of SEL is difficult, being a rare disease. There are many more
common causes for similar symptoms, including spinal disc herniation, inflammatory
disease of the spinal cord (e.g., transverse myelitis, multiple sclerosis, Guillain-Barré
syndrome), syringomyelia, and intraspinal tumors [
23
]. Therefore, the diagnosis can be
delayed or incorrect [
24
26
]. Thankfully, SEL can be easily detected in both CT and MRI as
a fat isodense (CT) or fat isointense (MRI) mass. By comparison, spinal angiolipoma is the
most difficult of the multiple differential diagnoses to distinguish in unenhanced images.
J. Clin. Med. 2022,11, 366 8 of 10
Furthermore, angiolipomas show vivid contrast enhancement, whereas SEL does not
enhance [
27
,
28
]. However, epidural fat tissue can easily be misinterpreted as an insignificant
finding in the imaging, especially when the patient has no symptoms. In our patient, the
pre-existing SEL showed significant growth over 17 months during the cancer therapy and
corticosteroid exposure. Therefore, it can be hypothesized that earlier appreciation of the
condition might have prevented the acute onset paraparesis and saved the patient from
extensive surgery. Consequently, it is crucial that the treating physicians, as well as the
radiologists, are aware of this diagnosis and the patient subgroup at risk.
Acute, fast-progressive SEL pathogenesis is barely understood, making it even more
challenging to treat. Some authors reported a venous stasis in the epidural venous plexus
with or without thrombosis as the main cause for acute worsening [
3
,
4
,
22
]. In contrast,
others hold the compression by the epidural fat responsible [
1
]. Exact knowledge of the
pathogenesis would have a significant impact on therapy, as the two above-mentioned
causes require different treatments. A better understanding of the general pathophysiology
would help differentiate which subgroup of patients might profit from a conservative
therapy, even when dealing with acute and severe symptoms. The current literature
suggests deranged (fat) metabolism, often associated with an increased endogenous or
exogenous corticosteroid excess, as a major risk factor for SEL [
1
,
23
]. In particular, a neuro-
sympatic auto-regulation of the fat metabolism is discussed, leading to an augmented fat
deposition in non-physiological locations [
29
]. In addition, for two patients included in
this review (ID#2 and 12), a systematic, augmented, atypical fat deposition was reported
(see Figure 3). We observed an increase in the mediastinal and subcutaneous fat as well
as fatty atrophy of the muscles. These observations support the hypothesis of a general
disturbance of fat metabolism in this SEL subform.
Regarding the treatment, all published cases of acute, fast-progressive, and severe
SEL without contraindication were treated surgically (representing maximum therapy),
presumably due to severe clinical presentation and lack of knowledge about the effective-
ness of non-surgical options (Table 1). Interestingly, two case reports demonstrated that
conservative treatment could result in a good outcome, even in patients suffering from
severe symptoms [
29
,
30
]. Lynch and colleagues reported on a case of severe paraparesis
in a patient taking corticosteroids for a recently diagnosed ulcerative colitis, with partial
recovery even after the first month after discontinuation of the treatment [
30
]. Bodelier et al.
reported on an acute, rapidly progressive paraparesis and parahypesthesia sub-T5 due to
severe SEL accompanied by a vertebral fracture in a patient suffering from an ectopic Cush-
ing’s syndrome. Severe steroid-induced osteopenia was, in this case, a contraindication
for back surgery. The patient underwent resection of the ACTH-producing tumor. In the
6-month follow-up after surgery, the patient showed a good recovery, with no difficulties
in his routine life, and he was able to walk, ride a bike, and work again [
29
]. Both articles
are of substantial importance, demonstrating that conservative management of patients
with severe symptoms might be a good alternative in well-chosen cases.
This knowledge offers a new perspective, considering the mono-directional decision-
making revealed by the systematic literature review (Table 1). The implication of surgery
has to be considered. The patients often undergo multiple-level decompression (T3–T9),
requiring additional dorsal fixation (see Video S1). In addition, the systematic literature
review revealed a median number of four involved spinal levels (Table 1), demonstrating a
high risk for instability in the case of surgical treatment.
However, it has to be acknowledged that the combination of major surgery and often
multimorbid SEL patients leads to high perioperative risk and potentially fatal compli-
cations [
1
]. Conclusively, in our systematic review, a median mCCI score of two (range:
0 to 10
) was observed, representing a subpopulation of impaired state of health, which has
been shown by others to correlate with a greater perioperative risk of complications and
mortality than a normal mCCI [
31
,
32
]. Therefore, it is not surprising that we observed
high mortality.
J. Clin. Med. 2022,11, 366 9 of 10
Nevertheless, it has to be mentioned that the information gathered here should be
interpreted with caution. Systematic reviews of the literature are susceptible to selection,
confounding, and information bias.
5. Conclusions
Overall, in our opinion, after the analysis of all cases, it can be stated that (I) acute,
severe SEL is a potential new subform and is still insufficiently understood; (II) multi-
morbidity, resulting in a higher likelihood of complications, including death, seems to be
overrepresented in these patients; (III) SEL should not be overlooked as a secondary finding
in imaging of patients with risk factors; (IV) conservative treatment might be considered
for well-chosen patients, as promising results have already been published.
Supplementary Materials:
The following are available online at https://www.mdpi.com/article/
10.3390/jcm11020366/s1, Supplementary Text: Case presentation, Figure S1: Systematic literature
search terms, Figure S2: Timeline summary of presented case, Table S1: Quality assessment score,
Table S2: Summary of neurological symptoms of the identified SEL cases, Video S1: Video summary
of microsurgical decompression and SEL removal.
Author Contributions:
T.F.D. wrote the manuscript, performed the systematic literature review, and
processed the case report. M.S.E. and C.D. performed the radiological workup. They also refined the
radiological quantification system to measure the compression caused by the SEL. A.M. and O.G.
assisted with the systematic literature search/review and analysis. M.D.O. and M.C. assisted with
creating the Figures, Tables, and Supplementary Materials, including video editing. T.B. performed
the neuropathological workup of the case. Additionally, he reviewed the neuropathological informa-
tion gained by the systematic literature review. A.-K.U., D.P., R.J., and U.S. co-supervised the project
and assisted in manuscript writing. K.H.W. initiated and supervised the entire project and was the
surgeon in charge of the presented case report. All authors have read and agreed to the published
version of the manuscript.
Funding:
This manuscript was supported by the Open-Access Fund of the Medical Library of the
University of Duisburg-Essen, Germany.
Institutional Review Board Statement: Not applicable.
Informed Consent Statement:
Written informed consent for publication was obtained from the
patients to publish this paper.
Data Availability Statement:
The data presented in this study are available on request from the
corresponding author. The data are not publicly available because of sensitive patient information.
Acknowledgments:
The authors would like to thank Ruth Arnold for her constructive criticism and
proofreading of the manuscript.
Conflicts of Interest:
Outside the submitted work, Karsten Henning Wrede received personal fees
from Biogen for expert opinions on aneurysms and vestibular schwannomas. All the other authors
report no conflict.
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Study design: Narrative review of available literature. Objective: To summarize current trends in pathogenesis and management of spinal epidural lipomatosis (SEL) and suggest areas where more research would be of benefit. Methods: The available literature relevant to SEL was reviewed. PubMed, Medline, OVID, EMBASE, Cochrane, and Google Scholar were used to review the literature. Institutional review board approval is not applicable for this study. Results: This article clearly summarizes current trends in the pathogenesis and management of SEL. Conclusions: Possible etiologies of SEL include exogenous steroid use, endogenous steroid hormonal disease, obesity, surgery induced, and idiopathic disease. Comorbidities such as acquired immunodeficiency syndrome and Scheuermann's disease have also been implicated in the pathogenesis of SEL. Steroid-induced SEL seems to have a proclivity for the thoracic region of the spine and has a higher incidence of paraplegia when compared with other forms. Several treatment modalities exist for SEL and are dictated by the underlying cause of the disorder. These include weight reduction, cessation of steroid medications, treatment of underlying endocrine abnormalities, and surgical decompression. Conservative treatments generally aim to decrease the thickness of adipose tissue in the epidural space, but the majority of patients tend to undergo surgical decompression to relieve neurologic symptoms. Surgical decompression provides a statistically significant reduction in symptoms, but postoperative mortality is high, influenced primarily by the patient's preoperative comorbidities. Physicians should consider the underlying cause of SEL in a given patient before pursuing specific treatment modalities, but alarm symptoms, such as the development of acute cauda equina syndrome, should likely be treated with urgent surgical decompression.
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Background: Spinal epidural lipomatosis (SEL) refers to an excessive accumulation of fat within the epidural space. It can be idiopathic or secondary, resulting in significant morbidity. The prevalence of SEL, including idiopathic and secondary SEL, and its respective risk factors are poorly defined. We therefore sought to: (1) assess the prevalence of SEL among patients who underwent a dedicated Magnetic Resonance Imaging (MRI) scan of the spine -including: incidental SEL (i.e. SEL without any spine-related symptoms), SEL with spine-related symptoms, and symptomatic SEL (i.e. with symptoms specific for SEL), and (2) assess factors associated with overall SEL and subgroups. In addition, we assessed differences between SEL subgroups. Methods: We reviewed the records of 28,902 patients, aged 18 years and older with a spine MRI (2004 to 2015) at two tertiary care centers. We identified SEL cases by searching radiology reports for spinal epidural lipomatosis, including synonyms and misspellings. Prevalence numbers were calculated as a percentage of the total number of patients. We used multivariate logistic regression analysis to identify factors associated with overall SEL and subgroups. Results: The prevalence of overall SEL was 2.5% (731/28,902): incidental SEL 0.6% (168/28,902), SEL with symptoms 1.8% (526/28,902), and symptomatic SEL 0.1% (37/28,902). Factors associated with overall SEL in multivariate analysis were: older age (OR: 1.01 95%CI: 1.01 - 1.02, p < 0.001), higher Modified Charlson Comorbidity Index (OR: 1.10, 95%CI: 1.07 - 1.13, p < 0.001), male sex (OR: 2.01, 95%CI: 1.71 - 2.37, p < 0.001), BMI > 30 (OR: 2.59, 95%CI: 1.97 - 3.41, p < 0.001), Black/African American race (OR: 1.66, 95%CI: 1.24 - 2.23, p = 0.001), systemic corticosteroid use (OR: 2.59, 95%CI: 1.69 - 3.99, p < 0.001), and epidural corticosteroid injections (OR: 3.48, 95%CI: 2.82 - 4.30, p < 0.001). Conclusions: We found that about 1 in 40 patients undergoing a spine MRI had SEL; 23% of whom with no symptoms, 72% with spine-related symptoms, and 5% with symptoms specific for SEL. Our data help identify patients that might warrant an increased index of suspicion for SEL.
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Systemic glucocorticoids are an essential therapy for a range of conditions, but their multiple side effects can produce significant morbidity for patients. The objective of this review is to discuss these side effects while addressing 3 questions: 1) What dose and duration of glucocorticoid therapy should prompt concern for individual side effects?; 2) How should clinicians counsel patients about these complications?; and 3) How can these problems be prevented or managed? To accomplish these objectives, we have created a series of tables and algorithms based on a review of relevant data to guide counseling, prophylaxis, and management of 11 glucocorticoid side effects. The first article in this 4-part continuing medical education series begins with a review of glucocorticoid pharmacology followed by a discussion of bone health (ie, osteoporosis and osteonecrosis).
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Objectives: The purpose of this study was to evaluate the correlation between the dilatation of the spinal epidural venous plexus (SEVP) and the amount of epidural fat. Methods: Between January 2007 and January 2012, 116 patients with prominent epidural fat and 116 control subjects without prominent epidural fat were included in this study. On the lumbar magnetic resonance (MR) images, we graded the amount of epidural fat and counted the number of vertebrae to determine the longitudinal extent of the epidural fat. We evaluated and classified the dilatation of the spinal epidural venous plexus and the degree of central canal stenosis. Results: SEVP dilatation significantly differed between the group with prominent epidural fat and the control group (p < 0.0001). Dilatation of the anterior epidural veins was seen in all subjects with dilatation of the SEVP. In the group with prominent epidural fat, 80 patients (69%) showed dilatation of the posterior epidural veins. The longitudinal extent of the prominent epidural fat was significantly associated with the grade of SEVP dilatation. The epidural fat grade and the sum of the epidural fat grades of all levels of the lumbar spine with prominent epidural fat showed a positive correlation with the grade of central canal stenosis (r = 0.421 & r = 0.347, respectively, p < 0.0001). Conclusion: The dilatation of epidural veins was statistically significant in patients with prominent epidural fat. The detection of SEVP dilatation on MR images may be helpful for spine surgery involving the epidural space. Advances in knowledge: Due to the risk of bleeding, the detection of SEVP dilatation on MR imaging may be helpful when considering decompression surgery with a posterior approach for spinal stenosis caused by prominent epidural fat.