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The multiple manifestations of the encephalocraniocutaneous lipomatosis syndrome.

A case is reported of a recently described neurocutaneous syndrome, manifested by a noncommunicating right lateral ventricular cyst, a left-sided lipoma of the scalp with alopecia, right-sided multiple truncal lipomas, right-sided ocular manifestations, left leg hypertrophy, and seizures. The findings in cases of encephalocraniocutaneous lipomatosis are described to demonstrate the spectrum of this syndrome.

Adolescent↗

Spinal epidural lipomatosis: report of a case secondary to hypothyroidism and review of literature.

Epidural lipomatosis usually occurs as a complication of steroid therapy. This report includes the first description of a case secondary to hypothyroidism. Adults in all age groups can be affected and present with progressive signs and symptoms of spinal cord or cauda equina compression. The dorsal aspect of the thoracic spine is most commonly involved, usually in its total extent. Spine radiographs are normal and myelography poorly delineates the nature and extent of abnormality. While computed tomography can provide information about the nature of the spinal mass, magnetic resonance imaging is the investigation of choice. Extensive decompressive laminectomy is useful but the results are variable. Correction of underlying endocrine abnormality can reverse the process. The need for early diagnosis of this disorder is therefore emphasized.

Epidural Space↗

Congenital aggressive lipomatosis.

Three cases of congenital lipomatosis involving the thoracic region posteriorly are presented delineating the natural history of the disease and depicting underlying bone and soft tissue changes. The rib widening and pleural thickening appear to be related to increased intercostal vascularity feeding the overlying tumor, as delineated by angiography. This entity is not described in the radiologic literature.

Female↗

Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis.

In 1983 the Proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses after surgical interventions in the regions involved. Histology of the adipose tissue showed considerable anisocytosis and increased cell volume.

Abnormalities, Multiple↗

Epidural lipomatosis: case report and literature review.

We report a case of symptomatic epidural lipomatosis in a 36-year-old man following a heart lung transplant and 3.5 years of steroid medication. A review of the pertinent literature emphasises the importance of including this diagnosis in the differential diagnosis of patients receiving steroid medication or markedly obese patients with back pain or symptoms suggesting spinal cord or cauda equina compression.

Adult↗

Multiple symmetrical lipomatosis in the neck.

Multiple symmetrical lipomatosis (Madelung's disease) is a very rare condition that is commonest in countries bordering the Mediterranean. The disease is associated with symmetrical unencapsulated fat deposits over the neck and upper trunk. We present the first reported occurrence in the Chinese. Although lesions can initially mimic a head and neck malignancy, management is essentially symptomatic, with conservative removals done as indicated clinically or for cosmesis.

China↗

Ophthalmologic abnormalities in encephalocraniocutaneous lipomatosis.

Encephalocraniocutaneous lipomatosis (ECCL) is a sporadically occurring disorder that belongs to the group of neurocutaneous syndromes. Important characteristics of the case we present are: intracranial lipomas, a skull hamartoma, bilateral lipodermoids and jaw tumors (ossifying fibromas and compound odontomas). We propose four minimal criteria for the diagnosis of ECCL and review the ocular abnormalities reported to date.

Brain Diseases↗

Retroperitoneal lipomatosis--report of two cases with an unusual distribution.

Two cases of retroperitoneal lipomatosis with an unusual distribution are reported. In the first case the fat was confined to the perirectal soft tissues and the sigmoid mesentery, whereas in the second case it was peripancreatic and extended into the small bowel mesentery. The lipomatous nature of these retroperitoneal masses was suggested by the lucency of the fat on the plain abdominal roentgenograms. These cases were studied prior to the availability of computed tomography, so that the diagnoses were confirmed by surgical exploration, which may have been avoided by this currently available noninvasive technique.

Adult↗

Uneven lipomatosis: a pitfall in pancreatic sonography.

A marked difference in echogenicity between the anterior and posterior parts of the pancreatic head was demonstrated by pancreatic ultrasound in 4 patients. This finding could not be related to local pancreatic or peripancreatic pathology. In all patients the dorsal portion of the head, including the uncinate process, appeared less echogenic than the ventral part of the head, body, and tail. The level of demarcation was identical in all 4 cases, coinciding with the expected fusion line of the embryological dorsal and ventral pancreatic origin. Pathology available in 1 patient showed an uneven lipomatosis as an explanation for this hitherto unreported observation.

Aged↗

Ultrasonographic features of pelvic lipomatosis.

The authors describe the diagnostic ultrasound findings of pelvic lipomatosis in a patient presenting with lower extremity thrombophlebitis. In this condition, ultrasound shows the full urinary bladder to assume a tubular or "cigar" shape and to "float" in the pelvic fat with failure to distend normally to the pelvic side walls. Confirmatory computed tomography discloses normal muscular and osseous anatomy but an increase in radiolucent fat surrounding the pelvic viscera. Clinical and radiographic findings are reviewed, and a differential diagnosis is presented.

Adult↗