Symptomatic thoracic epidural lipomatosis with syringomyelia: illustrative case

Lukasz Przepiorka Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland

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Katarzyna Wójtowicz Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland

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Przemysław Kunert Department of Neurosurgery, Medical University of Warsaw, Warsaw, Poland

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BACKGROUND

Spinal epidural lipomatosis is a rare but well-described disease. Here the authors present an extraordinary co-occurrence of spinal epidural lipomatosis with syringomyelia that did not improve after laminoplasty but required duroplasty as a definitive treatment.

OBSERVATIONS

This is the seventh case in the literature describing a co-occurrence of spinal epidural lipomatosis and syringomyelia. The pragmatic argument for a causative relationship between 2 phenomena is syrinxes reduction after surgery for spinal epidural lipomatosis. Additionally, duroplasty for spinal epidural lipomatosis in the setting of syringomyelia has not previously been reported. Duroplasty offers much better reconstruction of the subarachnoid space as compared with extradural decompression surgeries. It is one of the treatment options for spinal epidural lipomatosis. Last, most of the patients described in the literature with secondary syringomyelia were women with diabetes mellitus.

LESSONS

The co-occurrence of spinal epidural lipomatosis and syringomyelia is extremely rare. Neurological deficits in such cases respond well to surgical treatment. Accordingly, each case described in the literature resulted in syringomyelia reduction or resolution after surgery. Duroplasty may be necessary in selected cases of spinal epidural lipomatosis. Close follow-up and ongoing management of patients with epidural lipomatosis is therefore important.

ABBREVIATIONS

MRC = Medical Research Council; MRI = magnetic resonance imaging

BACKGROUND

Spinal epidural lipomatosis is a rare but well-described disease. Here the authors present an extraordinary co-occurrence of spinal epidural lipomatosis with syringomyelia that did not improve after laminoplasty but required duroplasty as a definitive treatment.

OBSERVATIONS

This is the seventh case in the literature describing a co-occurrence of spinal epidural lipomatosis and syringomyelia. The pragmatic argument for a causative relationship between 2 phenomena is syrinxes reduction after surgery for spinal epidural lipomatosis. Additionally, duroplasty for spinal epidural lipomatosis in the setting of syringomyelia has not previously been reported. Duroplasty offers much better reconstruction of the subarachnoid space as compared with extradural decompression surgeries. It is one of the treatment options for spinal epidural lipomatosis. Last, most of the patients described in the literature with secondary syringomyelia were women with diabetes mellitus.

LESSONS

The co-occurrence of spinal epidural lipomatosis and syringomyelia is extremely rare. Neurological deficits in such cases respond well to surgical treatment. Accordingly, each case described in the literature resulted in syringomyelia reduction or resolution after surgery. Duroplasty may be necessary in selected cases of spinal epidural lipomatosis. Close follow-up and ongoing management of patients with epidural lipomatosis is therefore important.

ABBREVIATIONS

MRC = Medical Research Council; MRI = magnetic resonance imaging

Spinal epidural lipomatosis is a rare, but well-described disease that most commonly occurs in middle-aged males.1 It is characterized by an abnormal, excessive distribution of adipose tissue (defined as thickness > 7 mm) in the spinal epidural space.2,3 It may be caused by endogenous or exogenous steroids, obesity, or surgery or can be idiopathic.4 Patients usually present with symptoms caused by compression of the spinal cord: myelopathy, paraplegia, radiculopathy, cauda equina syndrome, etc. Management may be conservative (e.g., weight loss, steroid reduction), especially when asymptomatic. However, in most cases, surgical decompression is warranted, with intended consequential improvement in neurological symptoms.5 Here we present an extraordinary co-occurrence of spinal epidural lipomatosis with syringomyelia that required unusual surgical treatment.

Illustrative Case

Presentation

A 51-year-old female presented with a 6-year history of progressive paresthesia and paraplegia in her lower extremities. Her spinal magnetic resonance imaging (MRI) was significant for T2 to T10 epidural lipomatosis as well as a T2 to T3 syrinx (Fig. 1). Because of these symptoms, the patient underwent C7–T1 laminectomy with syrinx puncture and drainage to subdural space at an outside hospital. After that surgery, there was no improvement and her symptoms continued to progress. Her past medical history was significant for type 1 diabetes mellitus, hypercholesterolemia, epilepsy, depression, as well as a thyroidectomy for papillary thyroid cancer followed by radioiodine therapy.

FIG. 1.
FIG. 1.

Sagittal T2-weighted magnetic resonance (MR) images of the cervical spine (A) and with fat saturation (B) show syringomyelia and the upper part of spinal epidural lipomatosis. Axial T2-weighted MR images at the T2 and T5 levels (C and D). Sagittal T2-weighted MR images with fat saturation (E).

First Surgery

The patient underwent T4 and T10 vertebroplasty prior to surgical treatment. On admission, the patient had paraparesis of their lower limbs (grade 4 on the modified Medical Research Council [MRC] system) that was most pronounced in her feet, along with increased muscle tension, with loss of touch and temperature sensation from the T4 level. Her new MRI scan revealed increased intramedullary T2 hyperintensity surrounding the syrinx compared with her previous radiological study. As planned, she underwent T2 to T10 laminoplasty with fat removal. Histopathology confirmed adult adipose tissue in the epidural space.

After surgery the patient initially worsened neurologically; her paraparesis progressed, but this resolved with rehabilitation. The first follow-up MRI revealed mild reduction in intramedullary T2 hyperintensity surrounding the syrinx on the T2–T3 level.

She was discharged home on a walker with MRC grade 4 paraparesis in her lower extremities. She continued to slowly improve, with the ability to walk short distances unassisted. Unfortunately, this improvement was only temporary, and her primary symptoms of paraparesis and painful paresthesia returned. Spinal MRI after this deterioration was significant for an increased intramedullary T2 hyperintensity as compared with earlier postoperative imaging (Fig. 2).

FIG. 2.
FIG. 2.

Consecutive sagittal T2-weighted MR images 1 month (A and B) and 3 months (C and D) after the first surgery show an increase in intraparenchymal T2 hyperintensities.

Second Surgery

Because of her neurological deterioration, the patient was qualified to undergo a second surgery of duroplasty with fascia lata. Her postoperative course was uneventful, and thereafter she continued to improve neurologically postsurgery. Spinal MRI performed after the second surgery showed sufficient reconstruction of the subdural spinal space as well as almost complete syrinx resolution (Fig. 3).

FIG. 3.
FIG. 3.

Sagittal T2-weighted MR image after the second surgery (A) and with fat saturation (B). Axial T2-weighted MR image at the T7 level (C). A posterior view of 3-dimensional computed tomography reconstruction (D).

At the 1-year follow-up, she was able to walk, occasionally with the assistance of a cane. Painful paresthesia in the lower extremities had remarkably decreased, and the remaining symptoms are followed by pain management specialists.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

According to the best of our knowledge, this is the seventh case in the literature describing a co-occurrence of spinal epidural lipomatosis and syringomyelia (Table 1). The pragmatic argument for a causative relationship between 2 phenomena is the fact that all syrinxes were reduced or disappeared after the surgery for spinal epidural lipomatosis. The theoretical pathogenesis of syringomyelia in the setting of epidural lipomatosis goes beyond the scope of this brief communication. In short, it may have been caused in a mechanism of an extradural mass disturbing cerebrospinal fluid flow, pressure waves, or compromised venous circulation.6–9 Notwithstanding the mechanism, the indications for treatment were unequivocal due to the progressive neurological deterioration of the patient described herein.

TABLE 1.

Literature review on the co–occurrence of spinal epidural lipomatosis and syrinx

Case No.Authors & YearAge (yrs), SexComorbiditiesEpidural Lipomatosis & Syringomyelia DescriptionClinical PresentationManagementFollow-UpSyringomyelia Reduction?
1Citow & Kranzler, 20001354, MNoneLipomatosis from T1 to T10, syrinx from T1 to T3Progressive lower extremities stiffness & weakness, bowel & bladder urgencyT1 to T10 laminectomy w/ fat removalImprovement in strength & spasticityYes, complete
2Tucer et al., 20141427, MNoneLipomatosis from C7 to T5, syrinx from T6 to T9Weakness & contractions in legsT3 to T6 laminoplasty, excision of posterior wall of arachnoid cystImprovement in gait & the leg contractions at 2 mosYes, partial
3Sáez-Alegre et al., 20191242, FDM type 1Lipomatosis from T1 to T12, syrinx from C1 to T3Upper limb paresthesia, then lt hemiparesis & tongue paresthesia10 yrs of observation, when symptomatic: rt T1 to T12 unilat laminotomies w/ epidural fat removalProgressive motor improvement w/ persistence of sensory symptoms at 6 mosYes
4Arora & Leever, 20201548, FDM type 1, hypothyroidism, renal failure, kidney & pancreas transplantsLipomatosis from T1 to T7, syrinx from C7 to T1Bilat lower extremity numbness & paralysisT2 to T7 laminoplastyImprovement overall, near resolution of bladder incontinence, persisting painYes
5Martínez Santos et al., 20211031, FDM type 1, hyperlipidemia, & hypothyroidismLipomatosis from T2 to T12, syrinx from C3 to T2Progressive lower extremity weakness, spastic paraparesisT2 to T9 alternating hemilaminotomies w/ fat removalStrength significantly improved at 1 yrYes
6Alvarado et al., 20221646, FDM type 1 complicated by kidney & pancreas transplantation on chronic immunosuppressive steroid therapyLipomatosis from T3 to T8, syrinx from C7 to T1Progressive myelopathyT2 to T8 laminoplasty w/ fat removalAsymptomatic at 2 yrsYes, mild
7Current case52, FDM type 1, hypercholesterolemia, epilepsy, depression, thyroidectomy for papillary thyroid cancer followed by radioiodine therapyLipomatosis from T2 to T10, syrinx from T2 to T3Lower limbs paresthesia & paraparesisT2 to T10 laminoplasty w/ fat removal, then duroplasty w/ fascia lataNo motor nor sensory deficit in 1 yrYes

DM = diabetes mellitus.

Surgical Treatment of Spinal Epidural Lipomatosis

The role of surgery in spinal epidural lipomatosis is well established. Most authors describe spinal decompression via laminectomies, with some variations, such as the interesting method of alternating hemilaminotomies described recently by Martínez Santos et al.10 and Neal et al.11 However, the question remains: Why was the conventional laminoplasty not enough in our case?

Observations

In retrospect, we presume that, due to long-lasting epidural compression, the dural sac did not decompress properly during the first surgery. Duroplasty offers much better reconstruction of the subarachnoid space when compared with extradural decompression surgeries. In hindsight, we were probably too conservative during our first surgery. Nevertheless, we do not recommend duroplasty in each case of spinal epidural lipomatosis. Rather, we endorse a case-based approach. Notably, Sáez-Alegre et al.12 considered duroplasty but eventually dismissed this method due to the lack of evidence.

Duroplasty for spinal epidural lipomatosis in the setting of syringomyelia has not previously been reported. We noted several observations. First, although epidural lipomatosis is a rare condition, it is even rarer for patients to require a duroplasty as a definitive treatment. This highlights the unique nature of this case and underscores the importance of reporting it in the medical literature. Second, the first surgery provided temporary relief, but it was the addition of the duroplasty that ultimately resulted in the patient’s sustained improvement. This highlights the significance of the duroplasy and its potential value as a treatment option in similar cases. This could help guide clinical decision making and ultimately lead to better outcomes for patients with such rare phenomena.

Furthermore, we have 2 additional observations that are similar to those made by Martínez Santos et al.10 Interestingly, most of the patients exhibiting a coinciding syrinx and spinal epidural lipomatosis were women (5/7), which is different from the general spinal epidural lipomatosis population. Similarly, diabetes mellitus was present in most of these cases (5/7), of which each was female.

Lessons

The co-occurrence of spinal epidural lipomatosis and syringomyelia is extremely rare. There is pragmatic proof that a syrinx is caused by lipomatosis, but the exact pathogenesis remains unknown. Neurological deficits caused by spinal epidural lipomatosis co-occurring with syringomyelia respond well to surgical treatment. Accordingly, each case described to this point in the literature resulted in syringomyelia reduction or resolution after the surgery for spinal epidural lipomatosis. Duroplasty may be necessary in the surgical treatment of selected cases of spinal epidural lipomatosis.

The additional lesson from this report lies in the importance of close follow-up and ongoing management for patients with epidural lipomatosis. Notably, close follow-up may be primarily clinical with periodic spinal MRI. Our findings are limited by the inherent nature of a case report of a very rare co-occurrence.

Disclosures

The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

Author Contributions

Conception and design: Kunert. Acquisition of data: Przepiorka, Wojtowicz. Analysis and interpretation of data: all authors. Drafting of the article: Przepiorka, Wojtowicz. Critically revising the article: all authors. Reviewed submitted version of the manuscript: Przepiorka, Wojtowicz. Approved the final version of the manuscript on behalf of all authors: Przepiorka. Administrative/technical/material support: Przepiorka, Wojtowicz. Study supervision: Przepiorka.

References

  • 1

    Alomari S, Lubelski D, Khalifeh JM, et al. Etiologies and outcomes of spinal epidural lipomatosis: systematic review of the literature and meta-analysis of reported cases. Clin Spine Surg. 2022;35(9):383387.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Robertson SC, Traynelis VC, Follett KA, Menezes AH. Idiopathic spinal epidural lipomatosis. Neurosurgery. 1997;41(1):6875.

  • 3

    Berlet R, Li D, Ruge J. Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case. J Neurosurg Case Lessons. 2023;5(8):15. CASE22556.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Kim K, Mendelis J, Cho W. Spinal epidural lipomatosis: a review of pathogenesis, characteristics, clinical presentation, and management. Global Spine J. 2019;9(6):658665.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Fassett DR, Schmidt MH. Spinal epidural lipomatosis: a review of its causes and recommendations for treatment. Neurosurg Focus. 2004;16(4):E11.

  • 6

    Koyanagi I, Iwasaki Y, Hida K, Houkin K. Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. Surg Neurol. 2005;63(4):350356.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Josephson A, Greitz D, Klason T, Olson L, Spenger C. A spinal thecal sac constriction model supports the theory that induced pressure gradients in the cord cause edema and cyst formation. Neurosurgery. 2001;48(3):636646.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Kyoshima K, Bogdanov EI. Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 2003;53(3):762769.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Klekamp J. The pathophysiology of syringomyelia—historical overview and current concept. Acta Neurochir (Wien). 2002;144(7):649664.

  • 10

    Martínez Santos JL, Saway B, Damon AC, Varma A. Multilevel hemilaminotomy windows for the surgical management of spinal epidural lipomatosis causing syringomyelia: technical note and literature review. World Neurosurg. 2021;148:412.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Neal MT, Patra DP, Lyons MK. Surgical management of thoracic myelopathy from long-segment epidural lipomatosis with skip hemilaminotomies: illustrative case. J Neurosurg Case Lessons. 2021;2(24):14. CASE21595.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Sáez-Alegre M, Pérez Lopez C, Giner García J, et al. Epidural lipomatosis and syringomyelia in adulthood: case report and literature review. World Neurosurg. 2019;129:341344.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Citow JS, Kranzler L. Thoracic epidural lipomatosis with associated syrinx: case report. Surg Neurol. 2000;53(6):589591.

  • 14

    Tucer B, Yilmaz MB, Ekici MA, Menku A, Koc K. Spinal arachnoid cysts associated with syringomyelia: a review of the literature and report of a case. Turk Neurosurg. 2014;24(4):606612.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Arora M, Leever JD. Thoracic epidural lipomatosis associated with syrinx: a case report. Radiol Case Rep. 2020;15(5):528530.

  • 16

    Alvarado AM, Masood Z, Woodrow S. Cervical syringomyelia with caudal thoracic epidural lipomatosis: case report and literature review. Int J Neurosci. 2022;9:17.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • Collapse
  • Expand
  • FIG. 1.

    Sagittal T2-weighted magnetic resonance (MR) images of the cervical spine (A) and with fat saturation (B) show syringomyelia and the upper part of spinal epidural lipomatosis. Axial T2-weighted MR images at the T2 and T5 levels (C and D). Sagittal T2-weighted MR images with fat saturation (E).

  • FIG. 2.

    Consecutive sagittal T2-weighted MR images 1 month (A and B) and 3 months (C and D) after the first surgery show an increase in intraparenchymal T2 hyperintensities.

  • FIG. 3.

    Sagittal T2-weighted MR image after the second surgery (A) and with fat saturation (B). Axial T2-weighted MR image at the T7 level (C). A posterior view of 3-dimensional computed tomography reconstruction (D).

  • 1

    Alomari S, Lubelski D, Khalifeh JM, et al. Etiologies and outcomes of spinal epidural lipomatosis: systematic review of the literature and meta-analysis of reported cases. Clin Spine Surg. 2022;35(9):383387.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Robertson SC, Traynelis VC, Follett KA, Menezes AH. Idiopathic spinal epidural lipomatosis. Neurosurgery. 1997;41(1):6875.

  • 3

    Berlet R, Li D, Ruge J. Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case. J Neurosurg Case Lessons. 2023;5(8):15. CASE22556.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4

    Kim K, Mendelis J, Cho W. Spinal epidural lipomatosis: a review of pathogenesis, characteristics, clinical presentation, and management. Global Spine J. 2019;9(6):658665.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Fassett DR, Schmidt MH. Spinal epidural lipomatosis: a review of its causes and recommendations for treatment. Neurosurg Focus. 2004;16(4):E11.

  • 6

    Koyanagi I, Iwasaki Y, Hida K, Houkin K. Clinical features and pathomechanisms of syringomyelia associated with spinal arachnoiditis. Surg Neurol. 2005;63(4):350356.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Josephson A, Greitz D, Klason T, Olson L, Spenger C. A spinal thecal sac constriction model supports the theory that induced pressure gradients in the cord cause edema and cyst formation. Neurosurgery. 2001;48(3):636646.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8

    Kyoshima K, Bogdanov EI. Spontaneous resolution of syringomyelia: report of two cases and review of the literature. Neurosurgery. 2003;53(3):762769.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Klekamp J. The pathophysiology of syringomyelia—historical overview and current concept. Acta Neurochir (Wien). 2002;144(7):649664.

  • 10

    Martínez Santos JL, Saway B, Damon AC, Varma A. Multilevel hemilaminotomy windows for the surgical management of spinal epidural lipomatosis causing syringomyelia: technical note and literature review. World Neurosurg. 2021;148:412.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    Neal MT, Patra DP, Lyons MK. Surgical management of thoracic myelopathy from long-segment epidural lipomatosis with skip hemilaminotomies: illustrative case. J Neurosurg Case Lessons. 2021;2(24):14. CASE21595.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12

    Sáez-Alegre M, Pérez Lopez C, Giner García J, et al. Epidural lipomatosis and syringomyelia in adulthood: case report and literature review. World Neurosurg. 2019;129:341344.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13

    Citow JS, Kranzler L. Thoracic epidural lipomatosis with associated syrinx: case report. Surg Neurol. 2000;53(6):589591.

  • 14

    Tucer B, Yilmaz MB, Ekici MA, Menku A, Koc K. Spinal arachnoid cysts associated with syringomyelia: a review of the literature and report of a case. Turk Neurosurg. 2014;24(4):606612.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Arora M, Leever JD. Thoracic epidural lipomatosis associated with syrinx: a case report. Radiol Case Rep. 2020;15(5):528530.

  • 16

    Alvarado AM, Masood Z, Woodrow S. Cervical syringomyelia with caudal thoracic epidural lipomatosis: case report and literature review. Int J Neurosci. 2022;9:17.

    • PubMed
    • Search Google Scholar
    • Export Citation

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