Lipomatosis of spinal epidural space, peritoneum, and renal sinus: a rare complication of long-term steroid therapy in a child with nephrotic syndrome.

Excessive visceral adipose tissue proliferation, resulting in diffuse lipomatosis, is a rare complication of long-term steroid therapy. A 10-year-old boy presented with severe radicular back pain with limitation of lower limb movements. He was diagnosed with steroid-resistant nephrotic syndrome and was on unregulated steroid therapy. Magnetic resonance imaging of the spine showed increased adipose tissue in the epidural space of the lumbo-sacral spine causing clumping of cauda equina nerve roots along with marked proliferation of fat in the renal sinus as well as peritoneum. He was started on pregabalin with tapering of steroids, following which there was a gradual decrease in pain and improvement of activity. Our patient had diffuse lipomatosis involving spinal epidural space, bilateral renal sinus, and peritoneum, secondary to steroid overuse. With the availability of advanced imaging techniques, the condition can be prevented by judicious and proper use of steroids with close follow-up for any untoward complications.

Orbital Lipomatosis: A Complication of Steroid Therapy in the Sweet Syndrome.

The description of a Sweet syndrome steroid dependant-induced orbital lipomatosis is reported. A 76-year-old-man with history of Sweet syndrome presented with severe bilateral proptosis (Hertel value, 25 mm) with decreased visual acuity and evoked potentials lengthened. A bilateral transpalpebral orbital decompression was performed by resection of intraorbital fat without bone removal. The surgery was uneventful. The volume of resected orbital fat was 15 ml for both sides. Proptosis reduction was 6 mm. Postoperative Hertel values were 19 mm, and evoked potentials were improved. The proptosis was managed successfully. Orbital lipectomy led to minimal sequelae and may be repeated if necessary in this case.

[Spinal epidural lipomatosis as a rare side effect in steroid-dependent Jo-1 antibody syndrome]. / Spinale epidurale Lipomatose als seltene Nebenwirkung bei steroidabhängigem Jo-1-Antikörper-Syndrom.

Spinal epidural lipomatosis (SEL) of the thoracic and lumbar spine is a rare entity, which leads to compression of the spinal canal. The exact pathogenesis is still unknown. It most commonly occurs in patients with long-term exogenous or endogenous glucocorticoid excess or morbid obesity but there are also idiopathic forms. The symptoms depend on the severity of the SEL and can manifest as clinically asymptomatic, non-specific back pain, radiculopathy up to spinal cord compression. The diagnosis is usually achieved by magnetic resonance imaging (MRI) of the affected spinal segments. The treatment varies between discontinuation of glucocorticoids, weight reduction up to multisegmental decompressive laminectomy. The following case report presents the findings of SEL in a patient with steroid-dependent Jo-1 antibody syndrome and provides a current literature review on this rare disease.

Small Bowel Lipomatosis: An Unusual Radiological Finding in Patients With Renal Cell Cancer on Pazopanib.

BACKGROUND/AIM: Treatment for advanced renal cell carcinoma (aRCC) comprises single agent or combinations of immune checkpoint inhibitors and/or anti-angiogenic agents. Pazopanib is a multitargeted anti-angiogenic tyrosine kinase inhibitor (TKI) approved as treatment for aRCC. We noted that a number of patients receiving pazopanib developed a radiological finding of small bowel lipomatosis. To evaluate the incidence of small bowel lipomatosis in patients with aRCC on treatment with pazopanib in comparison with other tyrosine kinase inhibitors. PATIENTS AND METHODS: We identified 12 out of 208 patients receiving pazopanib to have small bowel lipomatosis and compared their clinic-radiological findings with 314 patients with aRCC receiving other TKIs (sunitinib, cabozantinib, axitinib, and tivozanib). No patients receiving these TKIs developed small bowel lipomatosis. RESULTS: We compared the radiological findings in patients receiving pazopanib for aRCC. The presence of lipomatosis should not be considered as a clinically relevant finding in these patients. The presence of lipomatosis has no relation with the response to treatment to pazopanib and this is a unique finding seen only in patients on pazopanib. CONCLUSION: Small bowel lipomatosis is an occasional finding in patients with advanced renal cancer on pazopanib and is not seen with other TKIs.

Colonic mucosal pseudolipomatosis: disinfectant colitis?

Colonic pseudolipomatosis is rare and its pathogenesis is still unclear. A number of mechanisms, including mechanical injury during an endoscopic procedure or chemical injury by disinfectant, seem to contribute to its pathogenesis. In our endoscopy unit, pseudolipomatosis occurred in an epidemic pattern after changing the endoscopic disinfectant from 2% glutaraldehyde to peracetic acid compound to decrease the length of endoscope reprocessing time. We assumed that pseudolipomatosis could be a type of chemical colitis produced by the residual disinfectant solution that remained on the surface or in a channel of the endoscope after reprocessing. The aim of this report was to highlight a series of 12 cases of colonic pseudolipomatosis in order to describe the endoscopic and pathological features and discuss the harmful effect of disinfectants as a possible cause of pseudolipomatosis. To identify the cause of the lesions, we systematically reviewed each patient history and the endoscopic and histological features. From March 2004 to February 2005, 1276 colonoscopies were performed and 12 cases (0.94%) of colonic pseudolipomatosis were diagnosed at the Kangnam Sacred Heart Hospital of Hallym University. The pathogenesis of colonic pseudolipomatosis is not well-known, but our experience indicates the endoscopic disinfectant as the probable cause of pseudolipomatosis rather than either mechanical traumatic injury or intraluminal air pressure-related injury.

Progressive epidural lipomatosis with steroid use in severe refractory asthma.

BACKGROUND: Long-term immunosuppression with oral corticosteroids is frequently used to treat inflammatory diseases of the lung and is advocated in the management of some patients with asthma. METHODS: The authors describe the case of a 35-year-old man with severe refractory asthma who developed a slowly progressive thoracic spinal cord syndrome. RESULTS: Spinal imaging demonstrated the presence of spinal epidural lipomatosis, a rare complication of prolonged corticosteroid therapy, which is characterized by overgrowth of fat in the epidural space and neuronal compression. CONCLUSIONS: Spinal epidural lipomatosis should be considered in patients receiving long-term corticosteroid therapy who develop symptoms and signs suggestive of spinal cord compression.

Sudden paraplegia following epidural lipomatosis and thoracal compression fracture after long-term steroid therapy: a case report.

Sudden paraplegia secondary to the posterior spinal epidural compression and vertebral compression fracture as a complication in corticosteroid treatment is extremely rare. The authors presented a case 49-year-old man with chronic relapsing attack of Still's disease. After the identification of pathology, the surgical evacuation of lipid tissue and pedicle-based instrumentation showed therapeutic success. To the authors' knowledge, this is the first case showing both vertebral fracture and paraplegia that required urgent surgery in the follow-up Still's disease.

Steroid induced spinal epidural lipomatosis--case report and review of the literature.

Steroids are one of the most commonly prescribed medications for a variety of medical conditions, often long term. Spinal epidural lipomatosis (SEL) is a state of pathological fatty tissue overgrowth in the vertebral canal. It is a rare and dangerous complication of chronic steroid therapy that may lead to back pain, radiculopathy, or paraparesis. We describe a patient that was taking long term steroids and presented with progressively worsening weakness of the lower extremities. On the MRI scan, a long segment of unusual accumulation of fatty deposits in the posterior aspect of the spinal canal resulting in canal stenosis extending from C7 to the T10 level was observed. Despite an appropriate diagnosis and surgical intervention, his weakness did not resolve. We discuss the implications of this case in the primary care practice.

Substitution of corticosteroid with everolimus after lung transplantation: a pediatric case report.

We report the case of a 12 year-old lung transplant recipient, in whom compressive epidural lipomatosis secondary to corticosteroid prompted us to replace prednisone with everolimus. Discontinuing corticosteroid treatment after lung transplantation is associated with a risk of graft rejection despite concomitant immunosuppressive therapy with tacrolimus and mycophenolate mofetil. During a follow-up of 18 months with everolimus instead of prednisone, we did not observe graft rejection. In parallel, all symptoms related to epidural compression disappeared within a month.

[Steroid-induced spinal epidural lipomatosis in pediatric patients]. / Kortikosteroid-induzierte spinale epidurale Lipomatose bei pädiatrischen Patienten.

We describe three adolescent patients with chronic autoimmune disorders who developed back pain and, in two cases, spinal symptoms several months after initiating chronic treatment with glucocorticoids. In all cases, MRI showed extensive spinal epidural lipomatosis, a rare but classic untoward effect of chronic glucocorticoid therapy. Analysis of these three, as well as 11 other pediatric cases extracted from the international literature, revealed that spinal epidural lipomatosis manifests most commonly with back pain and within a mean of 1.3 years (range, 3 month-6.5 years) after initiation of therapy with corticosteroids. It frequently remits after reduction of the corticosteroid dose.

Rapid Development of Spinal Epidural Lipomatosis after Treatment of Metastatic Castration-Resistant Prostate Cancer with Second-Generation Androgen Receptor Antagonists.

BACKGROUND: Previous studies have described the association of spinal epidural lipomatosis with several conditions including chronic steroid therapy, Cushing's syndrome, obesity, Paget disease, and hypothyroidism. We present a report of rapid development of spinal epidural lipomatosis after treatment with second-generation anti-androgen therapy, a new strategy for treatment of metastatic castration-resistant prostate cancer that has been increasingly employed in the past few years. A comprehensive discussion of the underlying molecular networks involving androgen receptor blockage and adipocyte differentiation, as well as the clinical implications of such a phenomenon, are provided. CASE DESCRIPTION: We describe the clinical and radiological evolution of a 58-year-old male patient with metastatic prostate cancer, who developed new onset of rapidly progressing lumbosacral epidural lipomatosis with significant compression of the nerve roots of the cauda equina a few months after initiation of treatment with second-generation androgen receptor antagonists. CONCLUSIONS: The underlying pathophysiology of adipose tissue growth following the administration of anti-androgen therapy is discussed, with emphasis on both the canonical Wnt/ß-catenin pathway as well as in the Wnt-independent pathway involving direct activation of downstream transcription factors from the T-cell factor family by the androgen receptor. As second-generation androgen receptor antagonists have been increasingly used for treatment of castration-resistant stage metastatic prostate cancer, new onset of symptomatic epidural lipomatosis should be considered as a possible differential diagnosis, especially because the urinary symptoms of cauda equina compression may be improperly attributed to the primary prostate neoplasm.

Spinal Epidural Lipomatosis: A Rare Complication From Hormonal Therapy for Infantile Spasms.

BACKGROUND: Spinal epidural lipomatosis (SEL) represents pathologic overgrowth of extradural adipose tissue in the spinal canal that can result in spinal cord compression. SEL has been associated with excess corticosteroids, whether from exogenous steroid use or from excess endogenous steroids. Spinal epidural lipomatosis is rarely reported in children and has not been reported in association with hormonal therapy for infantile spasms. METHODS: We performed a detailed retrospective chart and literature review. RESULTS: We describe two children with symptomatic SEL associated with the use of high-dose hormone treatment for infantile spasms.

An unusual cause of paraparesis in a patient on chronic steroid therapy.

BACKGROUND/OBJECTIVE: Spinal epidural lipomatosis is the excessive deposition of unencapsulated fat in the epidural space. This is a rare disorder often associated with high levels of endogenous steroids or the administration of exogenous steroids. CASE DESCRIPTION: A 32-year-old man with congenital kyphosis treated with prednisolone daily for 5 months for interstitial lung disease developed compressive myelopathy. FINDINGS: Magnetic resonance imaging showed congenital kyphosis along with epidural lipomatosis compressing the cord. Cessation of steroid therapy was associated with improvement in the symptoms. CONCLUSIONS: Spinal epidural lipomatosis is a rare side effect of chronic steroid therapy that may occur with relatively short-term, low-dose regimens. In patients with congenital vertebral anomalies, spinal fat deposition may worsen the neurological status in an already compromised cord. Discontinuation of steroid therapy is beneficial; some patients may require surgical intervention for decompression.

[Precocious mediastinal lipomatosis: a rare complication of systemic corticosteroid therapy]. / Lipomatose médiastinale précoce: une complication exceptionnelle de la corticothérapie generale.

BACKGROUND: Corticosteroid-induced lipomatosis results from hypertrophy within adipose tissue; the condition is frequently asymptomatic and its incidence is underestimated. We report a case of mediastinal lipomatosis that is rare in terms of both site and presenting symptoms. CASE REPORT: A 46-year-old woman with no disease history other than obesity with a weight of 90 kg had been treated since 2002 for mixed connective tissue disease (profound lupus and dermatomyositis). She had been treated with oral corti costeroids (1 mg/kg/d). Two months after the start of treatment, she presented chest pains, resting dyspnea particularly aggravated in dorsal decubitus, chest edema in the subclavicular space and jugular turgescence. Chest x-ray revealed widening of all levels of the mediastinum. The chest CT scan showed lipomatosis throughout the entire mediastinum with no associated chest abnormalities or pericardial effusion. Rapid downward dosage adjustment ofcorticosteroids to 10 mg/d coupled with synthetic antimalarials resulted in gradual reduction of symptoms. The chest scan performed two months later short stabilization of the patient's mediastinal lipomatosis. DISCUSSION: The effects of long-term of glucocorticosteroid therapy are well-known, in particular Cushing's syndrome. Lipomatosis has been described more recently and affects different axial regions. Mediastinal localization is seen in 15% of patients treated. This presentation is less common than orbital and epidural localizations. Although often asymptomatic, as in our own report, it may present with worrying symptoms that pose real diagnostic problems. The diagnostic examinations of choice are CT scan or MRI. Regression following discontinuation or reduction of corticosteroids is inconsistent and often gradual.

Ptosis and orbital fat prolapse after posterior sub-Tenon's capsule triamcinolone injection.

PURPOSE: To describe the occurrence of orbital fat prolapse and blepharoptosis after posterior sub-Tenon (PST) triamcinolone injection. DESIGN: Retrospective review of consecutive case series. PARTICIPANTS: Patients with ptosis and orbital fat herniation after PST triamcinolone injection. METHODS: Charts of all patients with ptosis and orbital fat herniation presenting after PST triamcinolone injection to the oculoplastics service of the Cole Eye Institute between 1999 and 2003 were reviewed. Charts were reviewed for patient age, indication, dates of injections, time to patient complaint or time to referral for ptosis, and marginal reflex distance (MRD1). MAIN OUTCOME MEASURES: Ptosis and orbital fat herniation after PST triamcinolone injection. RESULTS: Eleven patients with a history of ipsilateral PST triamcinolone injections were seen with ptosis and orbital fat prolapse. Ten charts were available for review. Mean patient age was 64 years (range, 45-78 years). Patients underwent 1 to 9 ipsilateral injections, and 2 patients underwent bilateral injections. Patients were seen for ptosis evaluation on average 22.5 months (range, 3-56 months) after the initial injection, and 6.6 months (range, 0-20 months) after the most recent injection. All patients demonstrated significant orbital fat prolapse in conjunction with statistically significant ptosis (P = 0.016). Tissue was obtained in 3 cases. Histologic findings in 1 case showed orbital fat infiltrated by histiocytes that seemed to contain phagocytosed material. CONCLUSIONS: Posterior sub-Tenon triamcinolone injection may cause ptosis associated with orbital fat prolapse. This finding may be a relatively common complication of PST triamcinolone injection. We recommend counseling patients about this risk before PST triamcinolone injection.

Do glucocorticoids cause spinal epidural lipomatosis? When endocrinology and spinal surgery meet.

Here, we report pathogenetic aspects of spinal epidural lipomatosis (SEL) based on a literature review. SEL is a rare entity but can cause significant morbidity. Its symptoms can be identical to those of more common disorders such as vertebral and disc disease, and cord lesions (for example, transverse myelitis, multiple sclerosis and syringomyelia). Therefore, it often goes undiagnosed. In addition, SEL occurs in patients on glucocorticoid therapy, which can lead to myopathy, thereby mimicking the motor symptoms of SEL. Glucocorticoids seem to play a major role in the development of SEL, although idiopathic SEL has also been reported. The latter occurs almost exclusively in obese individuals who may have concurrent hypercortisolism. Once clinically suspected, SEL is best diagnosed by magnetic resonance imaging (MRI). Treatment of SEL is directed at reducing body weight in patients with idiopathic SEL, and at decreasing glucocorticoid excess in patients with endogenous or exogenous hypercortisolism. In severe cases, decompressive laminectomy might become necessary to alleviate the neurological symptoms caused by spinal cord compression.

Spinal epidural lipomatosis: case reports, literature review and meta-analysis.

BACKGROUND CONTEXT: Symptomatic spinal epidural lipomatosis (SEL), a rare cause of spinal cord compression, has most often been associated with exogenous steroid use. PURPOSE: Identify four associations with SEL, correlate the associated groups with level of disease and compare treatment with outcome data in these groups. STUDY DESIGN/SETTING: Case reports of three patients and analysis of 104 cases from the literature. PATIENT SAMPLE: Three patients from the senior author's practice. OUTCOME MEASURES: Not applicable. METHODS: The authors report three new cases of SEL not associated with steroid use. They review all available English literature and present a table of all 104 reported cases. RESULTS: The clinical course of three new patients is reported. CONCLUSIONS: Associated conditions are exogenous steroid use, obesity, endogenous steroid excess, and some remain idiopathic. Although SEL is a rare condition, our review of the literature reveals many more reported cases than previously thought. With increased awareness of this condition and improved imaging techniques, further studies of this disease should be undertaken.