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Pelvic lipomatosis presenting as uraemic encephalopathy.

Pelvic lipomatosis is a benign disorder of unknown aetiology, characterised by deposition of mature fat in the pelvis. It has a variable natural history, manifesting either with symptoms attributable to the lower urinary tract or varying degrees of renal failure. Owing to the mild, non-specific initial symptoms and slow progression, presentation is delayed and patients are often lost to follow-up, only to present with advanced renal failure later on. We report a case of a young patient with pelvic lipomatosis who, after an initial diagnosis, was lost to follow-up for several years and then presented with uraemic encephalopathy. The case highlights the importance of regular follow-up and an early intervention before irreversible changes occur.

Adult↗

Isolated symmetrical mediastinal lipomatosis.

Symmetrical mediatinal lipomatosis is a rare benign condition characterized by deposition of a large amount of mature adipose tissue within the mediastinum. Usually secondary to exogenous obesity, administration of steroids and Cushing's disease, it is more common in middle-aged males and is associated with alcohol abuse. Various other associations have been reported. CT and MRI can decisively diagnose this benign condition, which also helps in deciding treatment options. We present a case of a young female with a bizarre appearance on chest radiographs. Isolated, bilaterally symmetrical mediastinal lipomatosis was diagnosed on CT and MRI. The patient was thin and did not consume alcohol or steroids. Cushing's disease and other known associations were excluded.

Adult↗

[Symetric lipomatosis of the hands].

INTRODUCTION: We report a case of symmetric lipomatosis in an unusual palmary topography. CASE REPORT: A 56 year-old woman was referred for evaluation of scleroderma. The diagnosis was made 10 years earlier because of acrosclerosis of the distal phalanges of both hands. The disease began 2 months after administration of systemic steroids for severe asthma. Since then, she was followed up for her scleroderma every 2 years without any evidence of systematization of the disease. On examination, the first phalanges appeared protruding and podgy on the palms of both hands. On palpation, the consistence was suggestive of lipomas. Similar nodules were present on the back of both hands at the proximal part of the interdigital spaces. Tomodensitometry confirmed the lipomatous nature of nodules. DISCUSSION: This patient presented with a symmetric lipomatosis of a very unusual distal localization on both hands. This unusual aspect mimicked acrosclerosis. In this case report, chronology suggests that systemic steroids might have induced this lipohypertrophy.

Adrenal Cortex Hormones↗

[Pelvic lipomatosis. Diagnostic and therapeutic considerations apropos of 3 cases].

OBJECTIVE: We report three cases of pelvic lipomatosis, and from them we discuss its diagnostic and therapeutic considerations. METHODS/RESULTS: Three patients, 71, 65 and 61 years old respectively, presented with irritative lower urinary tract symptoms. Intravenous pyelogram (IVP) and CT scan were performed as diagnostic procedures. After diagnosis an endoscopic evaluation including biopsies was performed to rule out tumours. All three underwent Bricker-Wallace II type urinary diversion resulting in good control of irritative symptoms. During follow-up one patient presented with an infiltrative bladder tumour. CONCLUSION: Pelvic lipomatosis is a rare disease; although traditionally has been considered benign it can result in urinary tract obstruction with severe physiopathological consequences. IVU and CT-scan are valuable to confirm the diagnosis. Lower urinary tract symptoms are controlled with urinary diversion without cystectomy but potential malignization of the bladder lesions should make us reconsider treatment.

Aged↗

[Spinal extradural lipomatosis. Revision of 108 cases. Case induced by exogenous contribution of ACTH].

Authors present a male patient with Spinal Extradural Lipomatosis, previously treated of a cerebral astrocytoma with surgery and radiotherapy, after which he received ACTH for a long period of time. Clinical manifestations were rachialgia, paraparesia with pain and dysestesias in both lower extremities. Diagnosis was carried out by Magnetic Resonance imaging. After a progressive withdrawal of the treatment with ACTH, the patient achieved a complete recovery and neuroimaging studies showed the dissappearance of the compression caused by the lipomatosis. We carry out a revision of the literature showing data we consider of interest derived from the wide series subjected to study.

Adrenocorticotropic Hormone↗

[A histotopographic study of the pancreas in lipomatosis].

Histotopographic investigations of the pancreas in lipomatosis were carried out and results obtained were collated with clinical data. It was established that diminution of the exocrine parenchyma in this case may be quite considerable-in some cases only 9.9-12% of its amount remained. Despite the considerable diminishing of the exocrine parenchyma in lipomatosis, no clinical manifestations of the disorder of the exocrine function may be observed. Experimental data showed that loss of the exocrine parenchyma of the pancreas may be compensated in other parts of the gastro-intestinal tract, in the stomach in particular.

Aged↗

[Epidural lipomatosis secondary to corticotherapy].

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. A precocious diagnosis is important because it influences the clinical evolution and the therapeutics option. The computed tomography (CT) and magnetic resonance imaging (MRI) are very important to the diagnosis. Authors report a case of a mediastinal and epidural lipomatosis in a man receiving corticoid therapy, with symptoms consistent with acute respiratory failure and progressive paraparesis.

Adrenal Cortex Hormones↗

Polygonal deformation of the dural sac in lumbar epidural lipomatosis: anatomic explanation by the presence of meningovertebral ligaments.

BACKGROUND AND PURPOSE: In patients with epidural lipomatosis, axial lumbar spine CT and MR images occasionally reveal a geometric, polygonal or stellar, shape of the dural sac. The purpose of this study was to define the anatomic structures responsible for this radiologic appearance. We hypothesized that meningovertebral ligaments could anchor the dura mater to the osteofibrous walls of the spinal canal and account for the geometric deformation of the dural sac. METHODS: The epidural spaces were examined in 15 adult cadaveric and seven aborted fetal lumbar spines. For macroscopic studies, 70 adult vertebral segments were separated and dissected by removing the extradural fat. For microscopic examination, axial histologic sections were obtained from 35 fetal and five adult undissected vertebral segments. RESULTS: Meningovertebral ligaments were observed in the median, paramedian, and lateral aspects of the anterior and posterior epidural spaces of both adult and fetal lumbar spines. These ligaments anchor the outer surface of the dura mater to the osteofibrous walls of the lumbar canal. They may form an irregular longitudinal septum partitioning the epidural space. Histologic examination demonstrated the fibroelastic composition of these ligaments and suggested their possible perivascular morphogenetic origin. CONCLUSION: The morphologic and topographic features of the meningovertebral ligaments explain the polygonal, stellar, or Y-shaped deformation of the dural sac observed on axial CT and MR images in patients with lumbar epidural lipomatosis.

Aborted Fetus↗

Spinal epidural lipomatosis with thoracic osteoporotic compression fracture causing paraplegia.

Spinal epidural lipomatosis (SEL) frequently occurs as a result of long-term steroid administration for various disorders, and patients often present with osteoporosis. Acute paraplegia in patients with extensive thoracic SEL is rare. We report a case of acute paraplegia caused by osteoporotic compression fracture with extensive thoracic SEL in a 44-year-old man with rheumatoid arthritis who had received steroid therapy for 4 years. He presented initially with abdominal distension and weakness of lower limbs, and a sudden onset of paraplegia with complete motor and sensory loss below the T6 level ensued. Plain radiographs showed an osteoporotic compression fracture of the T6 vertebra. Magnetic resonance imaging showed osteoporotic compression fractures of the T5 and T6 vertebrae and SEL from T2 to T10 vertebrae. Decompressive laminectomy with epidural fat debulking was performed, and the pathology was confirmed as epidural lipomatosis. His neurological condition showed no improvement below the T6 level 3 months after surgery. Osteoporotic compression fracture is a risk factor for acute paraplegia in patients with thoracic SEL and decompressive surgery should be performed without delay.

Acute Disease↗

Lipomatosis of the parotid gland in children.

Parotid lipomatosis is extremely rare in children. Only 4 cases have previously been reported in the English language medical literature. Surgical excision is frequently complicated by recurrence. We report, a fifth case, on a 5-month-old girl with rapidly progressive parotid lipomatosis. Emphasis is laid on the importance of preserving the unusually delicate tumor capsule to prevent tissue spillage and recurrence. The creation of an appropriate cleavage between the mass and the expanded skin with sparse subcutaneous fat, safeguarding the tumor capsule on one side and the skin blood supply on the other, represents a rewarding technical challenge.

Biopsy, Needle↗

[Adenolipomas, lipomatosis and amyloidosis of the thyroid gland].

Adipose tissue in the thyroid gland was observed in 7 biopsies and 2 autopsies: in 3 cases of solitary adenolipoma, 3 cases of multiple adenolipoma, 1 case of pure lipomatosis and 2 cases of lipomatosis with multiple adenolipomas combined with amyloidosis of lobules as well of adenomas in one bioptic case. Pathogenesis of this unusual phenomenon was discussed.

Adenoma↗

[Enlarged heart caused by massive subepicardial lipomatosis with idiopathic dilatation of the right cavities].

The case is reported of a man of 48 with known longstanding gross cardiomegaly which was completely asymptomatic. Angiocardiography and coronary arteriography showed dilatation of the right side of the heart, and especially of the auricle. In addition, the ventricle was separated from the diphragm by a transparent non-fluid area. At operation, gross dilatation of the right atrium was confirmed, but no causative lesion could be found; there was also marked lipomatosis which involved particularly the area beneath the right ventricle. It is difficult to classify this most unusual case as either a classical dilatation of the right atrium, idiopathic or secondary, or as a cardiac lipoma or lipomatosis.

Angiocardiography↗

[Ileo-ileal and ileocecal invagination due to intestinal lipomatosis].

Intestinal lipomatosis is a rare entity and few cases have been reported in the literature. The condition is usually asymptomatic. Symptomatic cases usually present as obstruction or, less frequently, as bleeding. Intestinal barium studies, ultrasonography and computed tomography are useful diagnostic techniques. We present the case of a 47-year-old man with no relevant medical history who presented with intestinal obstruction of several months' duration. Complementary investigations yielded a diagnosis of intestinal obstruction due to ileocecal invagination secondary to endoluminal tumors of the ileum. Surgery and pathological analysis revealed the latter to be intestinal lipomatosis. This rare clinical entity has been associated with diverticulosis and intestinal volvulus.

Digestive System Surgical Procedures↗

Lipoma arborescens (diffuse articular lipomatosis).

Lipoma arborescens (LA) (diffuse articular lipomatosis, synovial lipomatosis, Hoffa disease) is a rare intra-articular lesion of unknown etiology. This article presents three patients who had LA, which was diagnosed in the knee in two patients and in the wrist of the third patient. Details of the clinical and histomorphological examination and treatment, in addition to a review of the literature, are discussed. The article concludes that in patients with a slow increase in painless swelling of the joints, unresolving articular pain with or without limited motion, or intermittent effusions following a minor trauma, LA should be considered in the differential diagnosis. It should be remembered that LA occurs in joints other than the knee, such as the elbow, shoulder, and wrist. Although recommended surgery involves arthrotomy and synovectomy, arthroscopic synovectomy may be a useful treatment modality, particularly in the larger joints as the recurrence rate is low.

Adipose Tissue↗

[Myopathy-lipomatosis associated with A8344G mitochondrial DNA mutation].

We report the clinical features of two unrelated patients, a 51-year-old woman and a 54-year-old man, presenting proximal myopathy with lipomatosis. In both patients, muscle biopsies showed numerous ragged-red fibers. Molecular analysis were performed with denaturating gradient gel electrophoresis (DGGE) on muscle, blood, hair, buccal and urinary cells. The A8344G mutation of the tRNA-lysine gene of the mitochondrial DNA was detected in all tissues at high levels (more than 80 p cent). None of the patients had a contributive family history, and signs of central nervous system involvement were absent. These observations confirm that lipomatosis may be encountered in mitochondrial disorders and is tightly associated with the A8344G mutation.

Adenine↗

Benign symmetric lipomatosis in a patient with severe scalp psoriasis.

Benign symmetric lipomatosis, or Madelung's disease, is a rare condition of undetermined cause characterized by symmetrical subcutaneous masses that consist of normal adipose tissue and are located predominantly in the head and neck areas. A case of benign symmetric lipomatosis in a patient with unusual psoriasis and excessive alcohol intake is presented.

Humans↗

[Pelvic lipomatosis associated with glandular cystitis. Report of two cases].

The authors report two cases of pelvic lipomatosis associated with glandular cystitis. This is a rare disease with variable and nonspecific clinical features. Complementary investigations useful for the diagnosis of pelvic lipomatosis are CT and especially MRI, intravenous urography and biopsies. The clinical features, radiological findings and therapeutic approach are discussed in the light of a review of the literature.

Adult↗

Benign symmetric lipomatosis with axonal neuropathy and abnormalities in specific mitochondrial tRNA regions.

Benign symmetric lipomatosis, also called Madelung's disease, is characterized by lipomata and fatty infiltrations. Involvement of the nervous system has occasionally been described, mitochondrial dysfunctions have been suggested. We report a 55 year old male suffering from benign symmetric lipomatosis with associated axonal neuropathy and hyperlipoproteinemia. He showed a remarkable phenotype of neuropathy i.e. no sensory disturbance, ubiquitous fasciculations and muscle cramps, furthermore reduced COX activity and abnormalities in specific mitochondrial tRNA regions.

Axons↗