Expression and Significance of Inflammatory Factors in Idiopathic Spinal Epidural Lipomatosis (Grade III).

BACKGROUND: Idiopathic spinal epidural lipomatosis (iSEL) is frequently associated with the utilization of steroids, endocrine disorders, obesity, and surgical interventions. Nevertheless, the pathogenesis of iSEL remains inadequately understood. The study aimed to investigate the contribution of inflammatory factors to idiopathic epidural lipomatosis. METHODS: Thirteen patients with iSEL (Grade III, iSEL group) and 12 patients with lumbar disc herniation (control group) who underwent unilateral biportal endoscopy from March 2020 to April 2023 were enrolled. Histological examination of adipose tissue was the performed to analyze expressions of pro-inflammatory cytokines (TNF-α, IL-1ß), and anti-inflammatory factors (arginase-1, IL-10) in serum and epidural adipose cells. RESULTS: Compared with the control group, the number of inflammatory cell infiltrations per field in HE-stained sections was significantly elevated, TNF-α and IL-1ß expression in adipocytes of epidural adipose tissue were markedly higher, and arginase-1 and IL-10 expression were significantly lower in the iSEL group (all p < 0.001). However, no statistically significant differences were observed in the serum level of TNF-α, IL-1ß, arginase-1, and IL-10 between the two groups (p = 0.963). In addition, there was also no significant disparity in adipocyte size between the two groups (p = 0.739). CONCLUSION: iSEL demonstrated elevated inflammatory cells and imbalance towards proinflammatory cytokines in adipocytes of epidural adipose tissue that may be associated with the pathogenesis of symptomatic iSEL. These data suggest that inflammatory response could be one of the mechanisms of iSEL. However, further multicenter epidemiological investigations and rigorous basic and clinical research are warranted to elucidate the specific etiology of iSEL.

Obesity is a risk factor for epidural lipomatosis: a meta-analysis.

PURPOSE: The purpose of this meta-analysis was to summarize the evidence of associations between obesity factors and spinal epidural lipomatosis (SEL) and to evaluate the strength and validity of these associations. METHODS: Electronic databases such as Wiley Online Library, PubMed, Embase, Cochrane Library were searched and manual retrieval of references, the time limit was from the establishment of the database to May 2020. Methodological quality evaluations of the included studies were assessed using the bias risk assessment tool recommended by the Cochrane Guidelines. The RevMan 5.3 software was used for meta-analysis. RESULTS: Finally, seven studies were included for meta-analysis, all of which were observational studies with mixed bias risk. These studies involved 807 patients, with an average age of 64 to 73.6 years, and 59.4 percent of the participants were male. The sample sizes for the included studies ranged from 28 to 288. The results of meta-analysis showed that high body mass index (BMI) was one of the factors affecting SEL (P < 0.01, MD 1.37, 95% CI [0.81, 1.92]). All reviews had a high risk of bias, and the most common source of bias was that there was no strict unified case diagnosis standard between researches, and some studies (four items) did not clearly describe the confounders that they controlled. CONCLUSIONS: We suggest that physicians should consider high BMI as a factor leading to SEL, and to control body weight actively should be considered as the preferred treatment strategy before surgical intervention is conducted.

MERRF syndrome (Myoclonic Epilepsy with Ragged Red Fibres) presenting with cervicothoracic lipomatosis.

INTRODUCTION: Patients with MERRF syndrome (Myoclonic Epilepsy with Ragged Red Fibres) usually present with encephalomyopathy. However, progressive, recurrent cervicothoracic lipomatosis may be rarely observed. CASE REPORT: The authors report 4 cases of MERRF syndrome associated with lipomatosis. In 3 patients, the diagnosis of MERRF syndrome was established on the basis of the clinical features of the lipomas and clinical interview revealing a personal or family history of lipomas and myopathy. DISCUSSION: In the presence of extensive spinal lipomatosis, the presence of other clinical signs of MERRF syndrome in the patient or the patient's family must be investigated. A diagnosis of MERRF syndrome can guide appropriate genetic counselling.

Benefits of bariatric surgery on spinal epidural lipomatosis: case report and literature review.

Spinal epidural lipomatosis is an abnormal accumulation of unencapsulated epidural fat causing compression of the neural elements. It can be divided into idiopathic and secondary. Secondary is often associated with chronic steroid use and endocrinopathies. Idiopathic has been associated with obesity. We report a 48-year-old man with obesity and a history of chronic back pain who developed idiopathic spinal epidural lipomatosis diagnosed by magnetic resonance imaging, which subsequently resolved completely after sleeve gastroplasty over a 6-month follow-up period.

Lipomatoses.

Lipomatoses are benign proliferations of adipose tissue, often with typical distribution patterns, which usually occur without clear causes. In contrast to circumscribed lipomas, they develop diffusely and symmetrically and are not surrounded by a fibrous pseudocapsule. The most common form is benign symmetric lipomatosis (BSL; Launois-Bensaude syndrome), of which four types are distinguished based on the distribution pattern of hyperplastic adipose tissue. The etiology and pathogenesis of the disease are still largely unknown, although some forms appear to have a hereditary basis or to be associated with increased alcohol consumption. In some cases, mitochondrial dysfunctions have been detected. Lipomatoses may be solitary, but may also be associated with other symptoms or illnesses (comorbidity). Effective drug therapies are not known, but surgical procedures can alleviate the symptoms. Lipomatosis dolorosa, which can be distinguished from BSL, occurs predominantly in middle-aged women and is characterized by proliferation of adipose tissue and severe pain in the affected areas. Various differential diagnoses and concomitant illnesses need to be considered, in particular depression. There are no evidence-based recommendations for therapy. Repetitive lidocaine infusions can achieve temporary improvement. Recurrence after surgical intervention is not uncommon, but long-term success is also possible.

Lipoedematous scalp: is there an association with fatty infiltration of the parotid?

Lipoedematous scalp (LS) is an extremely rare condition characterised by a soft and boggy consistency in the scalp due to an increased layer of subcutaneous tissue.In this report, we present a case of LS in a 64-year-old Indian woman. Clinical examination revealed only vague boggy lumpiness involving the whole of occipital scalp extending to parietal scalp. MRI scalp showed diffuse fatty infiltration of the scalp, particularly at the posterior parietal and occipital convexity extending to both lateral aspects of the cranium, with homogeneous signal in keeping with fat. Incidentally MRI also found diffuse fatty infiltration of the parotids.The aethiopathogenesis of LS is still unknown, however it is believed that the hormone leptin could be the key hormone in the dysregulation of fat deposition and distribution. This case report highlights the subtle features with which these cases can present and explores the literature on reported cases of LS.

Lipomatosis arborescente. Presentación de un caso pediátrico / Arborescens lipomatosis in a young girl. A case report

La lipomatosis sinovial es una entidad, rara y benigna, de la membrana sinovial, que fue descrita por Albert Hoffa en 1904, y afecta con mayor frecuencia a la articulación de la rodilla, pudiendo presentarse en otras articulaciones. Ocurre en varones de mediana edad y, raramente, en niños. Siendo normalmente monoarticular, en algunos casos aparece en más de una articulación. Su etiología es desconocida y se habla de un proceso reactivo más que neoplásico. Esta enfermedad tiene una morfología radiológica, macroscópica y microscópica característicamente arborescente, por infiltración adiposa del tejido subsinovial, que es clave para su diagnóstico. Presentamos el caso de una niña de 12 años de edad, realizándose revisión de los hallazgos radiológicos y patológicos, así como su diagnóstico diferencial con otras entidades y su tratamiento (AU)

Synovial lipomatosis, described by Albert Hoffa in 1904, is a rare and benign entity of the synovial membrane. The knee joint is the most frequent site, although it can occur in other joints. Middle-aged men are most often affected; it is rarely found in children. It is normally monoarticular, although in some cases it appears in more than one joint. Of unknown etiology, it is thought to be a reactive, rather than a neoplastic, process. The pathology shows characteristic arborescent radiological, macroscopic and microscopic morphology due to adipose infiltration of sub-synovial tissue, which is essential for diagnosis. We report a case of synovial lipomatosis in a 12 year old girl and discuss the radiological and pathological findings, differential diagnosis and treatment (AU)

Spinal epidural lipomatosis - an easily ignored secondary intraspinal disorder in spinal kyphotic deformities.

BACKGROUND: A previous study reported a high prevalence of spinal epidural lipomatosis (SEL) in patients with Scheuermann kyphosis (SK) and suggested that it may play a role in the pathogenesis of this disease. According to our observation, however, SEL occurs in other spinal kyphotic deformities as well. The aim of this study was to test the hypothesis that SEL commonly occurs in patients with different types of kyphotic deformities as a secondary intraspinal disorder. METHODS: MR images of 16 patients with congenital kyphosis (CK), 40 patients with SK, 13 patients with tuberculotic kyphosis (TK), and 69 age- and sex-matched controls were retrospectively evaluated. The body mass index (BMI), kyphosis Cobb angle, and sagittal diameters of spinal epidural fat (EF) and the dural sac (DS) in the apical region (EFA, DSA) and non-kyphotic region (EFN, DSN) were measured. The EF ratios at the apical vertebral level (EFRA) and in the non-kyphotic region (EFRN) were calculated as EF / (EF + DS). RESULTS: EFA and EFRA were significantly higher among patients with CK, SK, and TK than among controls (P < 0.05). Seven CK patients (43.8%), 8 SK patients (20.0%), and 11 TK patients (84.6%) fulfilled the diagnostic criteria for SEL, while only 6.3, 2.5, and 0% of patients in the control groups did (P = 0.019, 0.014, and < 0.001, respectively). Spearman's correlation analysis showed statistically significant correlations between the kyphosis Cobb angle and the amount of EF in all three patient groups. CONCLUSIONS: SEL is a common secondary intraspinal disorder in different types of kyphotic deformities, and surgeons should pay increased attention to this intraspinal anomaly because excessive EF may compress the spinal cord and cause neurological deficits.

MRI Screening in Operative Scheuermann Kyphosis: Is it Necessary?

STUDY DESIGN: Patients with preoperative spine magnetic resonance imaging (MRI) studies from a prospective multicenter study of operative adolescent Scheuermann kyphosis (SK). OBJECTIVES: To investigate the usefulness of MRI screening in operative planning for SK surgeries. SUMMARY OF BACKGROUND DATA: Neural axis abnormalities in operative SK have not been previously studied with MRI screening, despite its use. METHODS: One orthopedic surgeon and two radiologists evaluated all images retrospectively. Radiographs were evaluated for kyphosis apex and magnitude. MRIs were evaluated for spinal cord abnormalities, epidural lipomatosis, location and number of vertebral wedging, Schmorl nodes and posterior disc herniations, frequency of spondylolysis, etc. The relationship of these pathologies to the kyphosis apex was explored. This group was compared to a surgical SK group without preoperative MRIs. RESULTS: Eighty-six patients with MRIs, mean age 16.3 years, 64% male, and a mean preoperative kyphosis of 75.9° were evaluated. There were 17 spinal cord abnormalities. Low-lying conus was found in 2 patients, and syrinx in 15 (no Chiari malformations). Epidural lipomatosis was found in 49 patients, average of 5.7 levels. Anterior vertebral wedging occurred in all (mean 4.7 levels). Posterior disc herniations averaged 5.2 levels/patient and 1.8 levels caudad to the apex. Spondylolysis was reported in 8.1%. Four cases (4.7%) had the operative plan changed as a result of the preoperative MRI: two due to neural compression, one due to disc herniation and one due to a spinal cord draped over the apex. Thirty-one patients did not receive an MRI; there were no significant differences between the two groups. The rate of postoperative neurologic change was 3.5% in the MRI group and 3.2% in the no-MRI group. CONCLUSIONS: Based on 4.7% of cases requiring a change in the operative plan as a result of preoperative MRI, the authors recommend considering performing screening MRI in operative SK patients.

Lipogranuloma after facial cosmetic procedures.

OBJECTIVE: Lipogranuloma is a rare inflammatory reactive process in the dermis and subcutis. We present a summary of the 6 cases of lipogranuloma after facial cosmetic procedures. STUDY DESIGN: We performed a retrospective review including patient demographic data, clinical symptoms, appearance on computed tomography, treatment, pathology results, and history of facial cosmetic procedures. RESULTS: In most cases, the nodules were painless and showed no significant growth. Computed tomography revealed ill-defined swellings in the buccal fat pad with heterogeneous density. Histopathological examinations revealed numerous variable-sized empty microcysts surrounded by abundant lymphocytes and foamy macrophages, the characteristic features of lipogranuloma. On further questioning, all of the patients revealed that they had undergone some form of facial cosmetic procedure in the preceding months to years. Among the 6 cases, facial autologous fat injection may have been the main cause of lipogranuloma. CONCLUSION: Lipogranulomas can develop months to years after facial cosmetic procedures distant from the injection sites. A thorough understanding of the patient's medical history and the clinical and histopathologic characteristics of lipogranuloma are necessary to make a definite diagnosis and allow appropriate treatment.

Gastroduodenal Lipomatosis in Familial Multiple Lipomatosis.

OBJECTIVE: To present a case of gastroduodenal lipomatosis associated with familial multiple lipomatosis (FML). CLINICAL PRESENTATION AND INTERVENTION: A 58-year-old male presented with FML that manifested as multiple, painless, subcutaneous lipomas on his body; his mother had subcutaneous lipoma without a diagnosis of gastroduodenal lipomatosis. His lipid profile was normal. Abdominal computed tomography showed multiple, submucosal, polypoid lesions (of uniform density) of fat in the stomach and duodenum, and a small, similar lesion in the ileum. CONCLUSION: This case shows that gastrointestinal lipomatosis can manifest as FML.

Encephalocraniocutaneous Lipomatosis Without Ocular Malformations.

BACKGROUND: Encephalocraniocutaneous lipomatosis is a rare congenital neurocutaneous syndrome resulting from ectomesodermal dysgenesis and characterized by unique hairless scalp lesions in the form of nevus psiloliparus, ipsilateral ocular malformations, and central nervous system anomalies. According to the 2009 diagnostic criteria proposed by Moog et al., ocular abnormalities are supposed to be the most consistent feature of encephalocraniocutaneous lipomatosis. PATIENT DESCRIPTION: We describe an 18-year-old girl with most of the central nervous system manifestations of encephalocraniocutaneous lipomatosis, major skin alterations including nevus psiloliparus, but no ocular involvement. CONCLUSION: Our patient suggests more variability in clinical features and a more complex genetic/embryonic etiology of encephalocraniocutaneous lipomatosis.

Potential Involvement of Obesity-Associated Chronic Inflammation in the Pathogenesis of Idiopathic Spinal Epidural Lipomatosis.

STUDY DESIGN: Multicenter case-control study. OBJECTIVE: To characterize the pathogenesis of idiopathic spinal epidural lipomatosis (SEL). SUMMARY OF BACKGROUND DATA: SEL is often associated with the history of steroid use or endocrine disorders; however, the pathogenesis of idiopathic SEL remains poorly understood. METHODS: Sixteen patients who underwent lumbar decompression surgery due to severe idiopathic SEL were included in the study (L group, 15 men and 1 woman; mean age, 71.5 yrs). Fifteen patients without SEL, who underwent decompression surgery for lumbar canal stenosis, were selected as controls (C group, 14 men and 1 woman; mean age, 70.3 yrs). The following parameters were analyzed in these two groups: body mass index (BMI), medical history, histology, the size of adipocytes in the epidural fat (EF) tissues, and the expression level of the transcripts for adiponectin, leptin, tumor necrosis factor-α (TNF-α), interleukin (IL)-1ß, IL-6, and IL-8. RESULTS: The mean BMI of the L group was significantly higher than that of the C group (29.1 vs. 25.2 kg/m, P = 0.006), and there was a significant correlation between BMI and the width of EF in both groups. The average adipocyte size in the EF was significantly larger in the L group than in the C group (2846.8 vs. 1699.0 µm, P = 0.017). Furthermore, the expression levels of the transcripts for TNF-α and IL-1ß in the L group were significantly higher than those in the C group [2.59-fold increase (P = 0.023) and 2.60-fold increase (P = 0.015), respectively]. CONCLUSION: Our data suggest that the pathogenesis of idiopathic SEL is associated with obesity. In addition, the increased expression of two major inflammatory cytokines in the EF in the L group may indicate that SEL is causally related to chronic inflammation. LEVEL OF EVIDENCE: 3.