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PubMed1986

Familial multiple lipomatosis. Report of a case and a review of the literature.

Familial multiple lipomatosis is a hereditary syndrome of multiple lipomas occurring in a particular distribution. It is clinically distinct from multiple symmetric lipomatosis with which it is frequently confused in the dermatologic literature. A case of familial multiple lipomatosis is reported and a review of this condition is presented in an effort to resolve the confusion in the literature about familial multiple lipomatosis and multiple symmetric lipomatosis.

Adult↗

PubMed2000

A rare case of osteoporotic spine fracture associated with epidural lipomatosis causing paraplegia following long-term cortisone therapy.

Cushing's syndrome is frequently associated with osteoporosis. Therefore, the incidence of osteoporotic spine fractures is significant. They are a rare cause of paraplegic syndromes. Additionally, epidural lipomatosis may occur in those patients. The combination of both fracture and lipomatosis may cause neurological deficit. A case of a young patient suffering from drug-induced Cushing's syndrome is reported. She developed progressive paraplegia. Radiographs demonstrated kyphosis of the thoracic spine from T7 to T9 and pathologic fractures. Urgent operation was planned to stabilize and decompress the spinal cord in the area of the kyphosis. Fortunately, magnetic resonance imaging (MRI) was conducted first. It confirmed pathologic fractures of T7-9 but also showed massive epidural fat extending from the level of T1 to T9. As suspected, laminectomy alone in the area of the fracture proved to be insufficient, as shown by myelography during operation. For treatment of paraplegia in this case of symptomatic epidural lipomatosis, an expanded laminectomy was necessary to remove all the epidural fat. Having undergone this procedure, the patient is now recovering from paraplegia. Our experience suggests that care should be taken before operative treatment of patients with pathological fractures in combination with Cushing's syndrome. In addition to vertebral fractures, epidural lipomatosis has to be taken into consideration. Those patients with neurological deficits have to be treated by an extensive laminectomy.

Adult↗

PubMed2001

Assessment of right ventricular lipomatosis by histomorphometry in control adult autopsy cases.

A histomorphometry study was carried out to assess the degree of right ventricular lipomatosis in control autopsy cases and to evaluate if this was correlated with parameters such as sex, age, body mass index (BMI) and heart weight. A total of 70 adult cases were selected from cases of violent death between 1991 and 1999 and where autopsies were carried out in the Department of Pathology and Forensic Medicine in Garches. All cases with heart pathology, abnormal BMI or putrefaction were excluded. Cases with lung or liver pathology were also excluded. Furthermore, 10 adult autopsy cases who died suddenly of arrhythmogenic right ventricular cardiomyopathy (ARVC) were compared with 10 age and sex-matched control cases. Details on sex, age, BMI and heart weight were obtained from the post-mortem records. For each case one sample of the right front ventricular wall was fixed in 10% neutral saline-buffered formalin and one 5-microm-section was stained with haematoxylin and eosin. The Leica Quantimet 500 analysis system was used for the histomorphometrical study. The mean degree of lipomatosis was measured under blind conditions in the ventricular wall and epicardial fat was excluded. Covariance analysis and the Wilcoxon test were used for statistics. The mean age of the control population was 37.5 years, the sex ratio was 1.9:1 (male:female). The mean degree of lipomatosis was 17.03% and the degree of lipomatosis was significantly correlated with age (p = 0.0029) but not with sex, BMI and heart weight. There was a statistically significant increase in fat in ARVC cases compared with age and sex-matched controls (p < 0.001). Fat infiltration of the right ventricle could be an adipose involution due to an ageing process and heavy fat infiltration can be difficult to distinguish from ARVC. Our study suggests that fat infiltration is not essential for the post-mortem diagnosis of ARVC which also requires fibrosis and degenerating myocytes trapped within areas of fibrosis.

Adolescent↗

PubMed2005

Is epidural lipomatosis associated with abnormality of body fat distribution? A case report.

CASE REPORT: To report a case of epidural lipomatosis in a patient with abnormal adipose tissue distribution, glucose intolerance and mixed hyperlipidemia. A 63-year-old male patient presented with low back pain radiating to the left calf on standing and walking (walking distance <100 m). He weighed 97.5 kg, was 1.73 m tall (BMI 32.6 kg/m2) and had a waist circumference of 113 cm. He had a glucose intolerance after a 75-g glucose oral load test. CT-Myelography revealed voluminous epidural lipomatosis around L4-L5 and L5-S1. Low calorie diet and reduction in alcohol intake achieved a weight loss of 17.5 kg in 7 months (80 kg, BMI 25.8 kg/m2, waist circumference 94 cm) and dramatic improvement in low back pain, walking distance (>500 m) and reduction of lipomatosis on CT-scan. Our case suggests a relationship between central obesity phenotype and epidural lipomatosis. Specific insulin resistance treatment might be proposed for these patients if this hypothesis is confirmed in further studies.

Body Fat Distribution↗

PubMed1998

Lipomatosis of the ileum with volvulus: report of a case.

We herein present a patient with lipomatosis of the ileum including diverticulosis and volvulus. The patient presented with abdominal pain and vomiting. Preoperatively, we diagnosed lipomatosis with volvulus of the ileum based on the findings of abdominal ultrasonography (US) and computed tomography (CT). During surgery, the dilated ileum had rotated 720 degrees counterclockwise, and was found to contain lipomatosis and multiple diverticula. Although lipomatosis of the small bowel is extremely rare, it does show characteristic US and CT findings, thus making a preoperative diagnosis possible if this disease is included in the differential diagnosis.

Adult↗

PubMed2002

Combination of lumbar kyphosis, epidural lipomatosis, and perineural cyst as a cause of neurological deficit: a case report.

We describe the rare simultaneous occurrence of epidural lipomatosis and a perineural cyst at the same level, lumbar kyphosis, osteoporotic vertebral fractures, and neurological deficits. A 75-year-old corticosteroid-dependent female farmer presented with severe low back pain, progressive lumbar kyphosis, and inability to stand because of numbness and muscle weakness of both legs. Plain radiographs displayed markedly decreased bone density, significant lumbar kyphosis, and vertebral compression fractures of L2, L3, and L4. Magnetic resonance imaging of the lumbar spine revealed a perineural cyst at the L2-3 level, extensive epidural lipomatosis, and spinal canal stenosis. Laminectomy from L3 to L5 with resection of epidural fatty tissue restored her walking ability. We postulate that the osteoporotic fractures and epidural lipomatosis were induced by corticosteroid therapy. Preexisting degenerative lumbar kyphosis of the type commonly seen in elderly farmers could have promoted osteoporotic lumbar vertebral fractures at points where bending stress had been strongly exerted. The combination of a perineural cyst and epidural lipomatosis at the same level has not been reported previously.

Aged↗

PubMed1975

Pelvic lipomatosis: effect of diet.

Pelvic lipomatosis is a disease of dense infiltration of benign fatty tissue within the confines of the deep bony pelvis. A case is reported of an obese, hypertensive, thirty-nine-year-old black man with the typical clinical and radiographic features of pelvic lipomatosis. Prolonged fasting produced an extensive weight loss and profound resolution of his presenting symptoms, signs, and radiologic abnormalities. Subsequent regain of weight resulted in the reappearance of the disorder, confirming that pelvic lipomatosis may be a disease of accelerated and excessive fat deposition which can be reversed by dietary restriction. The literature pertaining to pelvic lipomatosis is reviewed in detail.

Adult↗

PubMed1991

Pelvic lipomatosis associated with cystitis glandularis and adenocarcinoma of the bladder.

Pelvic lipomatosis is a rare condition characterized by an overgrowth of normal fat in the perivesical and perirectal spaces. Cystitis glandularis, cystica or follicularis has been observed in 75% of the patients with pelvic lipomatosis. Although cystitis glandularis is widely regarded as premalignant few reports have documented its transition to adenocarcinoma. We describe a 41-year-old man with pelvic lipomatosis in whom primary adenocarcinoma of the bladder developed 6 years after a histological diagnosis of cystitis cystica and glandularis was established. To our knowledge this is the second case reported of pelvic lipomatosis associated with proliferative cystitis and adenocarcinoma of the bladder, indicating that intestinal metaplasia of the urothelium may be a precursor of malignancy in these patients.

Adenocarcinoma↗

PubMed1984

[Diffuse infiltrating lipomatosis. A subentity of intrapelvic fatty tumor].

Nineteen patients with various types of lipoma in the pelvis are described. Clinical findings, radiological examinations, and follow-up studies constitute a particular type of lipoma, diffuse infiltrating lipomatosis; this can be distinguished from other fatty tumours because of its localisation, extent, therapeutic results and long course. Computed tomography enables us to differentiate simple lipomatosis pelvis from diffuse infiltrating lipomatosis. Histological examination is essential for excluding a liposarcoma. Growth or malignant change of diffuse infiltrating lipomatosis is most easily recognized by computed tomographic serial observation.

Adult↗

PubMed1981

Lipomatosis and true lipomas of the ileocecal valve.

Two patients with lipomatosis and two patients with true lipoma of the ileocecal valve are described. The symptomatology, radiological appearances, pathological findings and treatment are discussed. True lipomas of the ileocecal valve should be differentiated from the more commonly occurring lipomatosis or lipohyperplasia. The rare true lipomas have a demarcating capsule around the fatty tissue and are confined to only one of the ileocecal lips. The lipomatosis, on the other hand, is characterized by a diffus fatty deposition in the submucosa of the valve with no encapsulation. Lipomatosis as well as true lipomas may appear as rounded, smoothly outlined and sharply demarcated masses on barium enema examination. Occasionally, these filling defects may present problems of differential diagnosis with respect to malignant involvement of the ileocecal region. A correct preoperative diagnosis, however, is important to prevent unnecessary or too radical surgical treatment.

Aged↗

PubMed1999

Symptomatic spinal epidural lipomatosis after local epidural corticosteroid injections: case report.

OBJECTIVE AND IMPORTANCE: Spinal epidural lipomatosis, which causes symptomatic compression of neural elements, is a well known but uncommon complication of Cushing's syndrome. Spinal epidural lipomatosis has been reported frequently in association with chronic systemic corticosteroid therapy, but thus far only one case has been attributed to local epidural corticosteroid injections. CLINICAL PRESENTATION: We report another case of symptomatic spinal epidural lipomatosis after epidural corticosteroid injections. This is the first such case documented by magnetic resonance imaging and confirmed with surgical exploration. INTERVENTION: The patient's symptoms of neurogenic claudication resolved after lumbar laminectomy in the region of previous corticosteroid injections and the removal of epidural fat, which was compressing the thecal sac. CONCLUSION: This case should alert clinicians that epidural lipomatosis, which causes symptomatic thecal sac compression, is a possible complication, not only of systemic glucocorticoid therapy, but also of local epidural corticosteroid injections.

Aged↗

PubMed2004

Epidural steroid injections in the treatment of symptomatic lumbar spinal stenosis associated with epidural lipomatosis.

Epidural lipomatosis has been implicated as a cause or contributor of symptomatic lumbar spinal stenosis. Although epidural steroid injections have been very successful for symptomatic treatment of spinal stenosis; their role in treatment of symptomatic stenosis secondary to epidural lipomatosis is unclear. A review literature (MEDLINE, PubMed) found no reports justifying the use of steroids. We present two patients with lumbar epidural lipomatosis causing or contributing to symptomatic spinal stenosis. Both patients presented with unilateral lower limb radicular symptoms unrelieved with conservative measures such as medications and physical therapy. They were treated with a single transforaminal epidural steroid injection at the symptomatic level. Both had 80-85% pain relief. These reports suggest a beneficial role of epidural steroid injections for patients with symptomatic lumbar spinal stenosis caused by or contributing to epidural lipomatosis.

Aged↗

PubMed1978

Lipomatosis of the pancreas in autopsy material and its relation to age and overweight.

Lipomatosis, regarded as deposition of fat cells in the pancreas, was investigated and graded into four groups in 394 consecutive autopsies. In all necropsies except one fat cells were present; gr. 1:51%, gr. 2:26%, gr. 3:15% and gr. 4:8%. Age as well as overweight was significantly correlated to the grade of lipomatosis. The number of necropsies with gr. 3--4 lipomatosis was significantly less in the group with long terminal illnes, indicating that the presence of fat cells in the pancreas is to some extent reversible. No evidence of pancreatic disease was mentioned in the clinical records of the patients with gr. 3--4 lipomatosis.

Adult↗

PubMed1986

Multiple symmetrical lipomatosis in a patient with Hürthle cell carcinoma of the thyroid: a case report.

Multiple symmetrical lipomatosis is a rare disorder of fat distribution which generally affects middle age men of Mediterranean origin. Hürthle cell carcinoma is an uncommon variety of follicular carcinoma which invades locally and metastasizes hematogenously to bone, lung, and other distant organs. This report describes a patient who developed multiple symmetrical lipomatosis after undergoing total thyroidectomy for Hürthle cell carcinoma of the thyroid. Type IV hyperlipidemia, hyperuricemia, frequent ethanol usage, and macrocytosis were features previously associated with multiple symmetrical lipomatosis which were present in this patient. Fourteen years after the initial surgery, metastatic Hürthle cell carcinoma was detected in the superior mediastinum. The location of the metastasis and the long disease free interval are unusual features of this case. A possible association between Hürthle cell carcinoma and multiple symmetrical lipomatosis is considered.

Carcinoma↗

PubMed1978

Different clinical types of lipomatosis. Case report.

Following a brief review of the literature, three characteristic case histories are reported in order to demonstrate the different appearances of lipomatosis. According to the survey, grouping into three main types is suggested: 1) Diffuse lipomatosis without any sharp demarcation from the surrounding soft tissue, and a tendency to recurrence; 2) Diffuse, symmetrical lipomatosis mainly located to the neck region, and 3) Multiple lipomatosis with well-defined, encapsulated, small lipomas which never recur following removal.

Adult↗

PubMed1999

Endoscopic retrograde pancreatographic findings of pancreatic lipomatosis.

Pancreatic lipomatosis is characterized by fatty infiltration or replacement of the pancreas, and has been associated with many conditions. We recently experienced two cases of pancreatic lipomatosis in patients with pancreatic pseudocyst and a case of lipomatosis in diabetes mellitus. In these patients, abrupt obstruction of the main pancreatic duct with smooth tapering is a typical endoscopic retrograde pancreatography (ERP) finding of pancreatic lipomatosis and must be differentiated with pancreatic carcinoma.

Adult↗

PubMed1991

[Pelvic lipomatosis associated with cystitis glandularis--case report].

Pelvic lipomatosis is a rare benign disease of unknown etiology characterized by the proliferation of infiltrating fatty tissue within the deep bony pelvis. These fatty tissue may enclose the pelvic and retroperitoneal organs and various degrees of luminal compression and obstruction may happen. There is no typical presenting symptoms of pelvic lipomatosis. The major diagnostic methods are radiological examinations. CT scan is the definitive test to confirm the diagnosis. Open biopsy may be necessary when the diagnosis is inconclusive. Various treatment modalities have been tried, but only the diet therapy and complete ablation of fatty tissue ever reported to be successful. Cystitis glandularis, a rare proliferative inflammatory disease of the bladder, is reported to occur concurrently with pelvic lipomatosis in a patient. Herein we report a similar case. A middle-aged male patient was admitted with the chief complaint of painless gross hematuria for two weeks. After admission a series of radiological examinations and endoscopic bladder biopsy were performed. He was proved to be a rare case of pelvic lipomatosis associated with cystitis glandularis.

Adult↗

PubMed1984

[Benign symmetrical lipomatosis in women. Association with alcoholic hepatopathy].

Benign symmetric lipomatosis or Launois-Bensaude syndrome is a condition characterized by diffuse, unencapsulated, painless growth of lipomatous tissue with a peculiar symmetric topology occurring predominantly in middle-aged men. In the present study a short survey of the clinical manifestations, differential diagnosis, current pathophysiologic theories, and therapy of this disease is given. From the literature published since 1950 112 observations are reviewed with respect to alcohol abuse in the premorbid period and other coexisting illnesses. Of the patients 70% were heavy drinkers and in over 45% significant liver damage was present. In addition, two rare cases of benign symmetric lipomatosis in women, aged 42 and 49 years, respectively, are presented. Both were alcoholics with hepatic sequelae. The possible existence of a female morphological subtype of benign symmetric lipomatosis is ventilated and its identity with Touraine-Renault's segmental lipomatosis of the trunk suspected.

Adult↗