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Abstract: Lipomatous hypertrophy of the interatrial septum (LHIS) is a rare but benign cardiac tumor that can be found on cardiac imaging such as echocardiogram, or during surgery or an autopsy. Cardiac MRI is the best imaging modality to determine the borders of the tumor and its extension into the intraventricular septum and ventricular free wall. Patients require close monitoring because LHIS may cause right or left outflow tract obstruction or superior vena cava obstruction, requiring cardiac surgical intervention. This article describes a patient with LHIS who underwent cardiac surgery because of her increasing symptomatology., (Copyright © 2024 American Academy of Physician Associates.)

Rationale: Diffuse intestinal and mesenteric lipomatosis is a rare condition characterized by the overgrowth of adipose tissue in the intestines and mesentery. This case report aims to highlight the rare occurrence of chronic abdominal distention caused by this disease and its unique invasion into the muscle layer, which has not been previously reported., Patient Concerns: A 36-year-old woman with a 7-year history of abdominal distension was admitted to our hospital's Department of Gastrointestinal Surgery., Diagnose: Abdominal and pelvic computed tomography revealed diffuse small intestinal lipomatosis., Interventions: The patient underwent surgery. We performed an open-field ilectomy involving removal of all lipomatous intestines (250 cm)., Outcomes: During the surgery, diffuse nodular ileal and mesenteric lipomatosis was confirmed, characterized by the presence of multiple nodular lipomas within the submucosal and muscular layers. The surgical intervention involved the resection of 250 cm of the affected ileum, followed by jejunoileal anastomosis. Postoperative pathology confirmed the diagnosis, with lesions observed in both the submucosa and muscle layers. The patient showed significant improvement in symptoms, with normal intestinal function and weight gain observed over a 10-month follow-up period, and no signs of recurrence., Lessons: Diffuse intestinal and mesenteric lipomatosis can lead to long-term abdominal distension. Additionally, it may be involved in the muscle layer of the intestinal wall. Surgery is the primary treatment option for symptomatic intestinal lipomatosis., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Temporomandibular joint ankylosis (TMJA) secondary to congenital infiltrating lipomatosis of the face (CILF) is an exceptionally uncommon condition which is characterized by the involvement of unilateral facial soft tissues and bones. In some cases, the extensive exophytic bony growth in the temporomandibular joint region often extends toward the skull base and lies near adjacent vital structures. Only very few cases of TMJA associated with CILF have been reported in the literature. The authors report a case of a 36-year-old female with right TMJA secondary to CILF. The bony overgrowth in the right temporomandibular joint region was arising from a deformed right mandibular condyle, extending towards the ipsilateral temporal bone, greater wing of the sphenoid, skull base, and approaching the lateral limit of foramen ovale, and foramen spinosum. To prevent any damage to the skull base and adjacent vital structures and to achieve adequate mouth opening, the authors have performed a unique technique of subankylotic osteotomy for the release of TMJA, instead of conventional gap arthroplasty., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 by Mutaz B. Habal, MD.)

Background: Ventricular tachycardia (VT) recurrence rates remain high following ablation among patients with nonischemic cardiomyopathy (NICM)., Objectives: This study sought to define the prevalence of lipomatous metaplasia (LM) in patients with NICM and VT and its association with postablation VT recurrence., Methods: From patients who had ablation of left ventricular VT, we retrospectively identified 113 consecutive NICM patients with preprocedural contrast-enhanced cardiac computed tomography (CECT), from which LM was segmented. Nested within this cohort were 62 patients that prospectively underwent CECT and cardiac magnetic resonance from which myocardial border zone and dense late gadolinium enhancement (LGE) were segmented. A control arm of 30 NICM patients without VT with CECT was identified., Results: LM was identified among 57% of control patients without VT vs 83% of patients without VT recurrence and 100% of patients with VT recurrence following ablation. In multivariable analyses, LM extent was the only independent predictor of VT recurrence, with an adjusted HR per 1-g LM increase of 1.1 (P < 0.001). Patients with LM extent ≥2.5 g had 4.9-fold higher hazard of VT recurrence than those with LM <2.5 g (P < 0.001). In the nested cohort with 32 VT recurrences, LM extent was independently associated with VT recurrence after adjustment for border zone and LGE extent (HR per 1 g increase: 1.1; P = 0.036)., Conclusions: Myocardial LM is prevalent in patients with NICM of a variety of etiologies, and its extent is associated with postablation VT recurrence independent of the degree of fibrosis., Competing Interests: Funding Support and Author Disclosures The INFINITY study is funded by National Institutes of Health grant 1R01HL142893-01. Dr Xu is an American Heart Association postdoctoral fellow (23POST909139). The University of Pennsylvania Conflict of Interest Committee manages all commercial arrangements. Dr Marchlinski has served as consultant for Abbott Medical, Biosense Webster, Biotronik, and Medtronic Inc. Dr Nazarian has served as a consultant for CardioSolv and Circle CVI; and principal investigator for research funding from Biosense Webster, ImriCor, Siemens, ADAS software, and the U.S. National Institutes of Health. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2024 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Spinal epidural lipomatosis [SEL] is defined as an excessive accumulation of extradural normal adipose tissue. This condition may be idiopathic or acquired. Surgical decompression is considered the gold standard treatment in patients presenting with progressive neurologic deficit. We report a case of a 69-year-old male patient presented with neurogenic claudication and lower left limb radiculopathy. The magnetic resonance imaging (MRI) revealed a L5-S1 epidural compression sustained by a large epidural plaque whose signal was consistent with adipose tissue. A conservative approach, including weight loss attempt, revealed unsuccessful and the patient presented some months after the diagnosis at the emergency department with acute onset bilateral drop foot, more severe on the right side and urinary disturbances. Microsurgical decompression via L5 laminectomy and partial L4 laminectomy and fatty plaque debulking was performed. The post operative course was uneventful and at the two months post operative follow up the patient reported a significative improvement of the pre operative neurological signs and symptoms and of pain control. SEL deserves attention as an identifiable cause of radiculopathies, typically attributed to spinal stenosis, with a higher incidence than previously reported. Moreover cases of acute onset of cauda equina like syndrome were reported in patent affected by SEL, probably related to a local venous engorgement with stasis and edema. Therefore it should be considered as a possible diagnosis not only in patients complaining of stenosis-type symptoms but also in cases of acute neurological decline with cauda equina like syndrome, especially when provided with a "benign" imaging reading.

Synovial lipomatosis is a controversial entity of uncommon intraarticular joint lesions. Most studies focused on radiological findings and surgical resection modalities. Pathologic findings were limited to case reports. Detailed descriptions of the histopathologic features are lacking. We aim to highlight the salient histopathologic features of synovial lipomatosis. We performed a retrospective review study over 15 years retrieving cases of synovial lipomatosis. We found 5 patients (age range: 8-78, average age: 50 years, male to female ratio: 4:1) demonstrating unilateral synovial lipomatosis out of 241 (2.0%) patients with knee joint lesions. Four patients had osteoarthritis. One patient was obese. The resected specimens showed synovial hyperplasia, surface fibrin, hemosiderin pigment, microcalcifications, lymphoplasmacytic infiltrates, lymphoid aggregates, stromal fibrotic, degenerative, and metaplastic alterations shared by other synovitis related changes. Consistent features included abundant adipocytic components, surface adipocytes, prominent large vessel vasculatures, plasma cells, and dense perivascular lymphocytic cuff-like infiltrates. One patient demonstrated superimposed sarcoid-type epithelioid granulomas. A distinct fibrous band separated the lesional tissue from the underlying normal fatty tissue. Synovial lipomatosis predominates in older males with degenerative joint conditions with a second peak in children and adolescents. Obesity is not a risk factor. Some histopathologic features help distinguish synovial lipomatosis from look-alike joint lesions. The findings may suggest an angiolipomatous hamartomatous growth or an exaggerated synovial reaction inducing florid subsynovial stromal proliferative, metaplastic, and degenerative changes. Regardless of its etiopathogenesis, pathologists have a role in confirming the diagnosis and ensuring a complete arthroscopic synovectomy by assessing the presence of the fibrous septa., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Abstract: Synovial lipomatosis is a rare condition characterized by adipocyte proliferation within joint synovial tissue. It most commonly affects the knee and is typically intra-articular. Only 5 published case reports describe extra-articular synovial lipomatosis of the wrist. We present a case of a sexagenarian patient seen for his wrist arthropathy. His x-ray revealed pan-wrist arthritis and inflammatory soft tissue swelling. The patient was slated for a wrist fusion and Darrach procedure. Following the dorsal skin incision in the operating room, an unusual adipose mass was identified infiltrating all extensor compartments: midcarpal, radiocarpal, and distal radioulnar joints. The mass was excised and sent to pathology prior to proceeding with the slated surgery. Synovial lipomatosis was diagnosed postoperatively based on histopathology. Six weeks postoperatively, the wrist fusion had healed clinically and radiographically, and his pain had improved. There was no evidence of recurrence. Synovial lipomatosis is a rare entity that may imitate multiple other pathologies. It is possible that synovial lipomatosis may represent a secondary occurrence following degenerative articular disease or trauma in older patients. This is the first case report to date describing synovial lipomatosis of the wrist with extra-articular extension in the setting of pan-carpal wrist arthritis., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome with complex skin, eye, and central nervous system (CNS) symptoms. Diagnosis and treatment are challenging due to its rarity and diverse manifestations. It often involves issues like porencephalic cysts, cortical atrophy, and low-grade gliomas in the CNS, resulting in developmental delays. The spinal cord is frequently affected, leading to problems like medullary compression and radiculopathy, causing back pain and sensory/motor deficits. Surgical interventions are reserved for symptomatic cases to address hydrocephalus or alleviate spinal lipomas. This article reviews a case series to assess surgical risks and neurological outcomes., Case Series: We present a case series ECCL, focusing on the diffuse lipomatosis of the spinal cord and the intricate surgical procedures involved. A multi-stage surgical approach was adopted, with continuous neuromonitoring employed to safeguard motor pathways. We discuss clinical characteristics, imaging studies, and indications for neurosurgical interventions., Discussion: ECCL is a complex syndrome. Diagnosis is challenging and includes clinical evaluation, neuroimaging, and genetic testing. Treatment targets specific symptoms, often requiring surgery for issues like lipomas or cerebral cysts. Surgery involves laminectomies, spinal fusion, and motor pathway monitoring. Thorough follow-up is crucial due to potential CNS complications like low-grade gliomas. Hydrocephalus occurs in some cases, with endoscopic third ventriculostomy (ETV) preferred over ventriculoperitoneal shunt placement., Conclusion: Neurosurgery for ECCL is for symptomatic cases. ETV is preferred for hydrocephalus, while the treatment for lipoma is based on the presence of symptoms; the follow-up should assess growth and prevent deformities., (© 2024. The Author(s).)

Background: Facial infiltrating lipomatosis (FIL) is a rare condition characterized by congenital facial enlargement. Beyond its impact on physical appearance, FIL can also impair essential facial functions such as swallowing, chewing, vision, and breathing, imposing a substantial physiological and psychological burden. Currently, fewer than 80 cases of FIL have been reported, and the characteristics and management strategies for FIL remain unclear., Methods: We reviewed the clinical, surgical, and radiological records of 39 FIL patients who were treated at our center. Of these, genetic testing was performed for 21 patients., Results: Aberrant overgrowth involves subcutaneous fat, bones, muscles, glands, tongue, lips, and teeth. Epidermal nevi could be observed in the dermatomes innervated by the three branches of the trigeminal nerve, with the highest frequency seen in the dermatome of the mandibular branch. Four patients exhibited concurrent hemimegalencephaly (HMEG), with one case presenting HMEG on the opposite side of the FIL. Nineteen patients were confirmed to harbor the PIK3CA mutation. Thirty-three patients underwent surgical procedures, with a post resection recurrence rate of approximately 25%., Conclusions: A variety of maxillofacial structures may be involved in FIL. PIK3CA mutations are important pathogenic factors. Emerging targeted therapies could present an additional treatment avenue in the future. However, surgery currently remains the predominant treatment choice for FIL. The timing and modality of surgery should be individually customized, taking into account each patient's unique circumstances. Notably, there is a significant possibility of postoperative recurrence during childhood and adolescence, necessitating early strategic planning of disease management., (© 2024. The Author(s).)

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-1β), 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-1β 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-1β, 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.

Introduction: The aim of this study was to present the surgical technique and clinical outcomes of modified ileal conduit for pelvic lipomatosis (PL)., Methods: From 2020 to 2022, we prospectively enrolled 9 patients with PL undergoing modified ileal conduit. The patient characteristics, perioperative variables, and follow-up outcomes as well as the description of surgical technique were reported., Results: All 9 patients successfully completed the operation. Two patients had perioperative complications of Clavien-Dindo grade I. The mean operation time and bleeding volumes were 253 ± 51.4 min and 238.9 ± 196.9 mL, with a mean postoperative follow-up time of 13.0 ± 5.6 months. The postoperative 3-month and 1-year creatinine values were significantly decreased versus the preoperative (p = 0.006 and p = 0.024). The postoperative 3-month and 1-year estimated glomerular filtration rate values were significantly increased compared with those before operation (p = 0.0002 and p = 0.018). The separation value of left renal pelvis collection system after operation was significantly reduced compared with preoperative evaluation (p = 0.023 at 3 months and p = 0.042 at 1 year) and so was the right side (p = 0.019 and p = 0.023)., Conclusion: Modified ileal conduit is safe and feasible for PL. A large sample cohort with long-term follow-up is needed to evaluate the clinical outcomes of PL., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Syringomyelia associated with epidural lipomatosis is a rare finding. Only three published cases of epidural lipomatosis associated with syringomyelia exist in the literature., We report the case of a 46-year-old woman who presented with progressive myelopathy over an 18-month period. Imaging revealed significant thoracic spinal cord compression secondary to epidural lipomatosis from T3 to T8 with cephalad cervical syringomyelia extending from C7 to T1. Imaging was unremarkable for Chiari malformation or a craniospinal space-occupying lesion. A T2 to T8 laminoplasty was performed, removing excessive epidural adipose tissue to decompress the thoracic spinal cord. Postoperatively, the patient reported symptom improvement with complete symptom resolution at 3 months. Follow-up imaging at 3-months demonstrated thoracic spinal cord decompression with mild syrinx reduction. At two-year follow-up the patient remained asymptomatic with unchanged imaging., Syringomyelia in the setting epidural lipomatosis is a rare finding.

Background: In contrast to osteoligamentous lumbar stenosis (LSS), outcome of surgical treatment for spinal epidural lipomatosis (SEL) is still not well defined. We present risk factors for SEL and clinical long-term outcome data after surgical treatment for patients with pure SEL and a mixed-type pathology with combined SEL and LSS (SEL+LSS) compared to patients with pure LSS., Methods: From our prospective institutional database, we identified all consecutive patients who were surgically treated for newly diagnosed SEL (n = 31) and SEL+LSS (n = 26) between 2018 and 2022. In addition, a matched control group of patients with pure LSS (n = 30) was compared. Microsurgical treatment aimed for posterior decompression of the spinal canal. Study endpoints were outcome data including clinical symptoms at presentation, MR-morphological analysis, evaluation of pain-free walking distance, pain perception by VAS-N/-R scales, and patient's satisfaction by determination of the Odom score., Results: Patients with osteoligamentous SEL were significantly more likely to suffer from obesity (body mass index (BMI) of 30.2 ± 5.5 kg/m 2 , p = 0.03), lumbar pain (p = 0.006), and to have received long-term steroid therapy (p = 0.01) compared to patients with SEL+LSS and LSS. In all three groups, posterior decompression of the spinal canal resulted in significant improvement of these symptoms. Patients with SEL had a significant increase in pain-free walking distance during the postoperative course, at discharge, and last follow-up (FU) (p < 0.0001), similar to patients with SEL+LSS and pure LSS. In addition, patients with pure SEL and SEL+LSS had a significant reduction in pain perception, represented by smaller values of VAS-N and -R postoperatively and at FU, similar to patients with pure LSS. In uni- and multivariate analysis, domination of lumbar pain and steroid long-term therapy were significant characteristic risk factors for SEL., Conclusions: Surgical treatment of pure SEL and SEL+LSS allows significant improvement in pain-free walking distance and pain perception immediately postoperatively and in long-term FU, similar to patients with pure LSS., (© 2023. The Author(s).)

Objective: To review the clinical research progress of spinal epidural lipomatosis (SEL)., Methods: The clinical studies on SEL at home and abroad in recent years were extensively reviewed, and the pathogenesis, clinical and imaging manifestations, and treatment status of SEL were summarized and analyzed., Results: SEL is a disease characterized by compression of the spinal cord and nerve roots due to abnormal accumulation of epidural adipose tissue in the spinal canal. Its prevalence and diagnosis rate are low and the pathogenesis is not fully understood. MRI is the most sensitive and specific diagnostic test for SEL. Surgical decompression and removal of excess adipose tissue are the only options for patients with acute SEL or those who have failed conservative management, and conservative management should be considered for other patients., Conclusion: SEL is a rare disease and related research still needs to be improved. In the future, high-quality, multi-center and large-sample studies will be of great significance for evaluating the choice of treatment methods and effectiveness of SEL patients.

Case: A 58-year-old man presented with lumbar spinal stenosis due to epidural lipomatosis. He underwent transsacral canal plasty (TSCP), in a manner similar to epidural adhesiolysis, which can be performed under local anesthesia. His leg pain improved dramatically in the year after surgery. Furthermore, magnetic resonance images during this time show neural decompression by reduction of the amount of epidural fat., Conclusion: TSCP was found not only to relieve pain but also to achieve neural decompression in this patient with spinal canal stenosis caused by lumbar epidural lipomatosis. This is the first report to demonstrate the usefulness of TSCP for spinal epidural lipomatosis., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/B975)., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

Study Design: This was a systematic review of the literature., Objective: The aim was to examine the contemporary demographics, etiological factors, treatment options and outcomes of spinal epidural lipomatosis (SEL) in adults., Summary of Background Data: SEL is primarily seen in obese patients as well as those on steroid therapy. Much regarding the etiology and treatment outcomes of SEL is unknown., Methods: We reviewed Ovid MEDLINE, PubMed, SCOPUS, and Google Scholars databases from 1990 through August 2020 to identify cases of SEL. Data collected included patient characteristics, disease associations, level of pathology, treatment, and clinical outcomes., Results: Ninety articles (145 individual cases) were included in the analysis. The median age was 54 years and 79% were males. Obesity-associated SEL constituted the largest proportion (52%) of our cohort. 22% of SEL cases were related to steroid use, while 26% cases were considered to be idiopathic. Lumbosacral SEL was the most frequently reported level of disease (68.9%), followed by the thoracic level (26.2%). The mean age of cases who underwent surgical intervention was 55 years, as compared with 48 years in those who received conservative management ( P =0.03). 95% of patients reported some degree of symptomatic improvement regardless of the treatment modality. Logistic regression suggested a possible superior outcome associated with those undergoing surgical treatment., Conclusion: In contrast to historical comparisons, contemporary articles support that obesity has become the major contributing factor for SEL. Logistic regression of the existing cases suggests that there may be a role for surgical intervention in select patients., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background and Importance: Spinal epidural lipomatosis is a rare condition commonly associated with chronic corticosteroid use and obesity that involves deposition of adipose tissue in the epidural space of the spinal canal. This accumulation of adipose tissue may cause compression of the spinal cord and/or nerve roots and result in compressive symptoms such as myelopathy or radiculopathy. Spinal involvement is usually confined to either the thoracic or lumbar spine but can infrequently affect both regions. Depending on pre-existing conditions, treatment options include weight loss and discontinuation of exogenous steroid use, both of which have been shown to be effective therapeutic methods. Surgical decompression may be useful for appropriately selected patients in whom conservative therapy has failed or who experience acute neurological deterioration, although this is rarely indicated., Clinical Presentation: In this study, we describe a patient receiving long-term corticosteroid therapy who presented with symptomatic epidural lipomatosis that involved the thoracic and lumbar spine. She was treated with decompression by continuous T3-L5 hemilaminectomies performed through 5 small incisions of alternating laterality. After surgery, the patient experienced clinical improvement and was able to return to her baseline., Conclusion: We illustrate a successful spinal decompression of extensive epidural lipomatosis through a less-invasive surgical approach using several small incisions to accomplish uninterrupted hemilaminectomies. This alternative approach to a standard continuous incision can be considered in cases of extensive spinal epidural lipomatosis in patients with multiple medical comorbidities in whom wound healing is believed to be an issue and for whom minimizing blood loss is crucial., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Madelung's disease is a rare benign systemic lipomatosis, which often presents in the head, neck and upper trunk regions. The appearance of symmetrical, excessive adipose tissue in the subcutaneous layer is its clinical characteristic. Orbital involvement is unusual with only a few cases reported previously. In this study, we describe the clinical and radiological features of Madelung's disease in the orbits. A 42-year-old man with alcohol addiction presented with chronic bilateral masses of the lower eyelids and proptosis. Computed tomography (CT) scan showed excessive symmetrical non-encapsulated fat deposition in the orbital fat, lower eyelids, salivary glands, subcutaneous tissue along the neck and under the sternocleidomastoid muscles and supraclavicular areas bilaterally. Histopathological study of the orbital masses revealed mature adipose tissue interspersed with thin fibrous septae. He developed recurrent lipomatosis 1 year after surgical excision.

Background: Spinal epidural lipomatosis (SEL) is characterized by symptomatic neurogenic compression from adipose tissue in the spinal canal. The question arises whether patients with morbid obesity have higher volumes of epidural adipose tissue (EAT) in their lumbar spinal canal compared with patients with a normal weight, and to what extent this decreases after bariatric surgery., Methods: In this explorative study the lumbar EAT volume was assessed in 25 patients with morbid obesity (body mass index [BMI] >40) using available lumbar magnetic resonance imaging (MRI) prior to their bariatric surgery. An age- and sex-matched control group (n = 25) of patients with a normal weight (BMI 20-25) was used for comparison. Participants from the obesity group underwent a postoperative control MRI., Results: The mean volume of EAT per MRI slice of the group of patients with obesity was significantly higher than for the group of patients with normal weight (mean: 83.3 ± 30.7 mm 3 vs. mean 56.5 ± 25 mm 3 ; P < 0.001). Fifteen participants with obesity (15 of 25) agreed to undergo a follow-up MRI. There was a significant decrease in EAT volume per MRI slice for these 15 participants (mean 82 ± 25.5 mm 3 vs. 46 ± 20.0 mm 3 ; P < 0.001) over time up to 3.6 (range: 1.2-6.0) years after bariatric surgery., Conclusions: Patients with obesity have significantly larger volumes of EAT in comparison with patients with normal weight. After bariatric surgery, a significant weight loss coincided with a significant volumetric reduction of this adipose tissue in the spinal canal. Future prospective studies in patients with symptomatic SEL may elucidate whether decreases in EAT volume influence concurrent neurogenic claudication symptoms., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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 the 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., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Rationale: Lumbar spinal epidural lipomatosis (SEL) is a rare condition characterized by excessive overgrowth of extradural fat within the lumbar spinal canal. Surgical decompression is commonly performed to treat symptomatic SELs. Fenestration or laminectomy with epidural fat debulking was a routine surgical procedure according to the literature, that may be causing postoperative lumbar instability. In the present study, we presented a brief report of lumbar SEL and introduced another surgical approach., Patient Concerns: A 55-year-old man complained of severe low back pain and right leg radicular pain for a year, accompanied by neurogenic intermittent claudication. He received a variety of conservative treatments, including non-steroidal anti-inflammatory drugs, acupuncture, and physical therapy. However, his pain did not diminish. Finally, a posterior epidural mass in the dorsal spine extending from the L3 to L5 level, which caused dural sac compression was found on lumbar magnetic resonance imaging. This mass was homogeneously hyperintense in both T1W1 and T2W1 images, suggestive of epidural fat accumulation., Diagnoses: Lumbar SEL., Interventions: The patient underwent lumbar laminectomy, epidural fat debulking, and spinous process-vertebral plate in situ replantations., Outcomes: The patient presented with complete recovery of radiculopathy and low back pain after surgery. Postoperative magnetic resonance imaging showed that the increased adipose tissue disappeared, and the dural sac compression was relieved. A computed tomography scan revealed the lumbar lamina in situ. He was able to walk normally and remained relatively asymptomatic for 12 months after the operation at the last follow-up visit., Lessons: Lumbar laminotomy and replantation provide an ideal option to treat lumbar SEL because it can achieve sufficient and effective decompression, simultaneously reconstruct the anatomy of the spinal canal, and reduce the risk of iatrogenic lumbar instability., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

We present a case of a man with a background of myasthenia gravis who presented with a neck lump, which was diagnosed as thyrolipomatosis in continuity with a very large thymolipoma. Following removal of these lesions, the patient's myaesthenic symptoms improved. While thymolipomas are often seen in the context of myasthenia gravis, thyrolipomatosis is a rare entity and to our knowledge the concurrent finding of both lesions with myasthenia gravis has never been reported. We highlight the important imaging features of both entities and the clinical importance of recognising them.

Background: Spinal epidural lipomatosis is a rare cause of lumbar spinal stenosis. While conservative therapy is applicable for most of cases, surgical intervention is necessary for severe ones. This is the first time we apply this modified technique to this disease., Case Presentation: The case is a 53-year-old man. He is 175 cm tall and weighs 102 kg (body mass index 33.3 kg/cm 2 ), presenting with low back pain and bilateral legs pain and numbness. Radiological examination showed severe lumbar spinal stenosis resulting from adipose hyperplasia, combined with hyperosteogeny and hypertrophy of ligaments, which are common etiological factors. Posterior decompression, internal fixation and a modified articular fusion technique was performed on this patient, and regular follow-up that up o 22 months showed outstanding clinical outcomes., Conclusions: A suitable style of posterior lumbar fusion should be considered to especially severe case with lumbar spinal stenosis and idiopathic spinal epidural lipomatosis.

Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article.

Objective: Lumbar epidural lipomatosis is a rare condition defined as the excessive accumulation of epidural fat (EF). We herein investigated the indicators of the posterior compression factors, EF and yellow ligament, to identify the clinical features of lumbar epidural lipomatosis., Methods: Five hundred consecutive patients who underwent posterior lumbar decompression surgery for lumbar spinal stenosis (LSS) were retrospectively reviewed. The EF/SC-L index (the ratio of the anteroposterior length of the EF to that of the spinal canal [SC]) was evaluated at the spinal level that exhibited maximum dural tube compression. The participants were divided into 3 groups: grade I, EF/SC-L index ≤50%; grade II, EF/SC-L index 51%-74%; grade III, EF/SC-L index ≥75%. EF/SC-A (the ratio of the cross-sectional area of EF to that of SC) and YL/SC-A (the ratio of the cross-sectional area of yellow ligament [YL] to that of SC) were calculated. The clinical outcomes were assessed according to the Japan Orthopaedic Association scale for lumbar disease., Results: EF/SC-L exhibited a significantly positive correlation with EF/SC-A (r = 0.82, P < 0.001), and a negative correlation with YL/SC-A (r = -0.71, P < 0.001). The Japan Orthopaedic Association score recovery rate was 56.7 ± 22.6 in the case-matched control group, 34.5 ± 31.2 in the grade II group (P < 0.001), and 39.6 ± 24.9 in the grade III group (P = 0.032)., Conclusions: The EF/SC-L index is a simple and reliable indicator to quantitatively evaluate posterior compression in patients with LSS. As the accumulation of EF is associated with worse operative outcomes, the EF/SC-L index should be considered when planning lumbar decompression surgery for patients with LSS., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Purpose: Spinal epidural lipomatosis (SEL) is defined as an abnormal and extensive accumulation of unencapsulated adipose tissue within the spinal epidural space. To date, there is a lack of high-level evidence studies reporting the outcome of surgical treatment of symptomatic SEL in patients with lumbar spinal stenosis (LSS). The aim was to compare clinical outcomes in patients with symptomatic LSS with and without SEL who underwent decompression surgery alone at the 12- and 24-month follow-up., Methods: One hundred and eighty-three patients met the inclusion criteria, of which 14 had mainly SEL on at least one level operated in addition to possible degenerative changes on other levels and 169 degenerative LSS only. The main outcomes were pain (Spinal Stenosis Measure (SSM) symptoms), disability (SSM function), and quality of life [EQ-5D-3L summary index (SI)] at 24-month follow-up, and minimal clinically important difference (MCID) in SSM symptoms, SSM function, and EQ-5D-3L SI., Results: The multiple regression linear models showed that SEL was associated with worse SSM symptoms (p = 0.045) and EQ-5D-3L SI scores (p = 0.026) at 24-month follow-up, but not with worse SSM function scores. Further, depression (in all models) was negatively associated with better clinical outcomes at 24-month follow-up. In the outcomes SSM symptoms and EQ-5D-3L SI, distinctly more patients in the classical LSS group reached MCID than in the SEL group (71.3% and 62.3% vs. 50.0% and 42.9%)., Conclusions: Our study demonstrated that decompression alone surgery was associated with significant improvement in disability in both groups at 2 years, but not in pain and quality of life in patients with SEL.

We present a rare case of symptomatic cystic lumbar spinal epidural lipomatosis that required surgical treatment via a minimally invasive tubular laminectomy. To our knowledge this is the first pathology confirmed report of compressive cystic lumbar spinal epidural lipomatosis available in literature., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Competing Interests: Die Autoren geben an, dass kein Interessenkonflikt besteht.

Background Context: Spinal epidural lipomatosis (SEL) results from excess lumbar epidural fat (EF) accumulation that compresses the cauda equina or nerve roots. Guidelines for the therapeutic management of SEL are not currently available., Purpose: To elucidate the efficacy of lumbar decompression surgery in SEL., Study Design: Multicenter retrospective study., Patient Sample: A total of 288 consecutive patients who underwent posterior lumbar spinous-splitting decompression surgery for lumbar spinal canal stenosis and followed up greater than 2 years at participating institutions were retrospectively reviewed., Outcome Measures: Japanese Orthopedic Association Back Pain Evaluation Questionnaire (JOABPEQ) and Roland-Morris Disability Questionnaire (RDQ)., Methods: Participants were divided into two groups according to the ratio of EF to anteroposterior diameter of the spinal canal (EF/SC-L) at the spinal level with maximum dural tube compression. Patients with EF/SC-L of ≥0.6 and <0.6 were defined as those with SEL and non-SEL, respectively. We assessed whether surgical treatment was "effective" or "not effective" using the JOABPEQ based on the following: an increase of ≥20 points in the postoperative score compared with the preoperative score, or a preoperative score <90 with a postoperative score ≥90 points. We constructed a multiple Poisson regression model by adjusting for confounding factors, and determined estimated relative risk (RR) for "not effective" with surgical treatment using the JOABPEQ. Additionally, we selected age-, sex-, BMI-, and decompression levels-matched patients with non-SEL and compared the frequency of "not effective" between SEL patients (n=60) and non-SEL patients (n=60)., Results: Analysis using the RDQ and JOABPEQ showed that the 1- and 2-year postoperative scores were significantly better than the preoperative scores in the both groups. Multivariable Poisson regression analysis demonstrated that SEL was significantly associated with "not effective" for decompression surgery in the 1-year postoperative outcomes of walking ability ([RR] 1.5, 95% confidence interval [CI] 1.0-2.2) and social life (RR 1.3, 95% CI 1.0-1.8) and the 2-year postoperative outcomes of walking ability (RR 1.6, 95% CI 1.2-2.3). Matching analysis showed that SEL was significantly associated with "not effective" with lumbar decompression surgery in the 2-year postoperative outcomes of walking ability (p=.02)., Conclusions: Patients with SEL exhibited significant improvements in surgical outcomes at 1 and 2 years postoperatively. However, compared with the non-SEL group, the efficacy of posterior lumbar spinous-splitting decompression surgery was worse in the SEL group, especially for walking ability. These results indicate that EF accumulation should be considered when planning treatment for patients with lumbar spinal canal stenosis and estimating the efficacy of lumbar decompression surgery., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: To investigate the clinical, imaging and pathological features of congenital infiltrating lipomatosis of the face (CILF) and to discuss whether it is a subtype of hemifacial hyperplasia (HH)., Methods: Sixteen patients diagnosed with CILF were included in this study. All patients had undergone panoramic radiography and spiral CT examinations. Thirteen patients received biopsy, surgery treatment and pathological examination. The clinical documentation and imaging data were retrospectively reviewed., Results: The cheeks (14/16), parotid glands (12/16), tongues (9/16), masticatory muscles (8/16) and the lips (7/16) were the most frequently affected soft tissue organs. The maxilla (14/16), zygoma (13/16), mandible (13/16) were involved among the maxillofacial bones. Dental malformations included macrodontia (8/16), poor formation of the roots (7/16), accelerated tooth germ development or premature eruption of permanent teeth (7/16) and missing of the permanent teeth (4/16). All malformations were restricted to one side of the face and did not trespass the middle line. Pathologically, CILF was featured by the diffuse infiltration of redundant mature adipose tissue into the tissue of the affected organ., Conclusion: CILF is a congenital developmental facial malformation characterized by infiltration of nonencapsulated, mature adipose tissue, resulting in facial soft and hard tissue hypertrophy and dental malformations in hemifacial structures. CILF could be considered as a subtype of HH., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Objective: To describe a minimally invasive decompression technique for symptomatic spinal epidural lipomatosis using percutaneous biportal endoscopic surgery., Methods: In this report, we describe the indirect decompressive effect that was gained by partial laminotomy and ligamentum flavectomy under biportal endoscopic view. Direct neural decompression was then performed by removal of proliferated fat. We described the technical process and compared pre- and postoperative radiating leg pain, life quality, and a radiologic grading system of neural compression., Results: This technique was performed successfully in 3 patients with idiopathic spinal epidural lipomatosis. Radiating pain was reduced, and functional disability and radiologic compression were improved. Postoperative instability and surgical complications related to the procedure were not observed., Conclusions: Percutaneous spinal endoscopy is a minimally invasive muscle-preserving technique for spinal lipomatosis that achieves neural decompression directly by lipoma removal and indirectly by partial bone and ligament removal., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Lipomatosis of nerve (LN) is one part of the spectrum of adipose lesions of nerve. Nerve-territory overgrowth is present in approximately 62% of cases. Given the wide variability in published reports, there is substantial need to understand the results of surgical treatment and outcomes in this disorder., Methods: Raw data from the published systematic review of LN were used for this analysis. PubMed and Google Scholar databases were also screened for any additional papers. The cases were sorted into 2 groups: (1) definite LN cases and (2) probable LN cases (lacked definite proof of LN diagnosis). For statistical analysis, P-value <0.05 was considered statistically significant., Results: The total number of 486 definite and 160 probable LN cases (646 cases combined) was included for analysis. The most commonly performed procedure was nerve decompression in both definite (n = 104; 21.4%) and combined definite and probable LN groups (n = 107; 16.6%). Improvement of symptoms was most often reported after nerve decompression (n = 52). A soft-tissue debulking procedure had the highest association with improvement (odds ratio 144.6, 95% confidence interval: 13.8-1516.2, P <0.001) in the definite LN group., Conclusions: Treatment options for LN ranges widely, although notable consistencies exist. The most conservative procedure involving nerve decompression was the most commonly performed procedure, with reported good outcomes. In contrast, worsening of symptoms was most commonly reported when nerve resection was performed. All treatment modalities were associated with improvement compared with no treatment. Diagnostic biopsy should be avoided., (Copyright © 2019 Elsevier Inc. All rights reserved.)

The aim of this study was to report the experience and long-term efficacy of a novel surgical treatment for pelvic lipomatosis (PL) using a combination of pelvic fat mass extirpation and ureteral reimplantation.Data of 8 patients with PL who underwent pelvic fat mass extirpation and ureteral reimplantation at our hospital from September 2010 to March 2018 were retrospectively reviewed. Demographics, serum creatinine level, radiographic changes, perioperative complications, and patient-reported outcomes were evaluated.Surgeries were performed successfully without severe perioperative complications in all 8 patients. Median operating time was 150 minutes with a median estimated blood loss of 75 mL. Patients were discharged after a median of 8.5 postoperative days. Imaging studies at the first follow-up revealed varying extents of alleviation of hydronephrosis and 3 patients' urinary symptoms were gradually relieved after surgery. During a median follow-up of 48.5 months (range, 10-100 months), all patients exhibited excellent surgical outcomes without evidence of disease progression, except 1 patient who underwent radical cystectomy with Bricker ileal conduit surgery due to hydronephrosis recurrence in the 49th postoperative month.Based on these cases, pelvic fat mass extirpation and ureteral reimplantation is a safe and effective surgical treatment for PL.

Background: Symptomatic spinal epidural lipomatosis (SSEL) is characterized by hypertrophy of adipose tissue within the spinal canal and consequent neural compromise. The exact pathogenesis remains enigmatic. The authors describe a retrospective case series, define the full clinical spectrum, and discuss possible pathogenetic mechanisms., Methods: The medical notes and imaging of 9 patients with SSEL undergoing surgery from 2008-2018 were analyzed. Seven patients presented secondary to lumbosacral spinal epidural lipomatosis (SEL); 3 patients with chronic incomplete cauda equina syndrome (CES), 3 patients with acute CES (including a 25-week gravid patient and a 40-year-old patient with intravenous leiomyomatosis, both of whom had mild SEL) and 1 patient with chronic lumbar radiculopathy. In addition, 2 patients presented with progressive myelopathy secondary to thoracic SEL., Results: Patients presenting with acute CES had a mean age of 37 years (range 23-49 years) and mean extradural fat (EF)-to-spinal canal (SC) ratio of 47% (range 41%-58%), in comparison with patients with chronic CES; mean age 61 years (range 58-65 years) and EF:SC ratio 72% (range 65%-80%). Patients underwent laminectomy and resection of EF at compressive levels. All patients with CES experienced complete resolution of symptoms at follow-up (range 1-48 months)., Conclusions: The clinician should be astute to the radiologic features of SEL, particularly in patients presenting with CES in the absence of acute disk herniation. The outcome of patients with CES and SEL after surgery is excellent regardless of symptom duration. Venous impedance related to increased body mass index and EF deposition may play the predominant role in addition to mechanical compression in the pathogenesis of SSEL., (Crown Copyright © 2019. Published by Elsevier Inc. All rights reserved.)