1/6. Idiopathic symptomatic epidural lipomatosis of the lumbar spine.BACKGROUND: Symptomatic spinal epidural lipomatosis (SEL) of the lumbar spine is a rare disease, often associated with steroid overload. Idiopathic lipomatosis is even much less frequent. signs and symptoms depend upon the level and degree of nerve root compression. diagnosis is best based on MRI. Weight reduction can be curative, however after failure of medical treatment or in severe cases surgical decompression should be performed. METHOD: Four patients with severe symptoms of lumbar spinal epidural lipomatosis were treated by surgical decompression. Patient history and neurological examination are described, diagnostic imaging is demonstrated, surgical treatment and outcome are documented. Different surgical techniques including laminectomy, interlaminar fenestration and lateral recess decompression were applied and are discussed. FINDINGS: All four patients improved after surgery. No surgical complications were observed. Even though limited to four cases this is the second largest series of operated idiopathic spinal epidural lipomatosis. INTERPRETATION: Surgical decompression was effective in improving symptoms in severe lumbar idiopathic spinal epidural lipomatosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/6. Fatal acute pulmonary embolism in a patient with pelvic lipomatosis after surgery performed after transatlantic airplane travel.We describe a case of a 37-yr-old patient who traveled from europe to the United States and succumbed to a massive pulmonary embolism 6 days after elective pelvic surgery despite routine postoperative thrombotic prophylaxis. In retrospect, he was likely to have developed a deep venous thrombosis during the transatlantic trip to our hospital. Anesthesiologists and other physicians involved in perioperative management need to be aware of the prevalence of venous thromboembolism in patients with a history of recent prolonged air travel. This is particularly true in tertiary referral centers, where patients with rare diseases may have a major surgical intervention within days of prolonged air travel.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/6. Lipedematous alopecia of the scalp.Lipedematous scalp is a rare disorder, mainly described in adult African-American females. We report 2 adult caucasian males with lipedematous scalp associated with androgenetic alopecia. patients were studied by dermoscopy and histopathology; they were treated with finasteride 1 mg. In our patients, lipedematous scalp affected the occipital and the vertex areas and pathologically exhibited mild edema and thickening of the adipose subcutaneous layer. At videodermoscopy, lipedematous scalp areas showed linear areas of teleangiectasia within the scalp creases, possibly caused by compression of the superficial blood capillaries by the increased volume of the subcutaneous fat layer within the thickened scalp. finasteride at a dose of 1 mg per day for 1 year induced mild improvement of androgenetic alopecia in one patient and stabilization of the disease in the other. The lipedematous scalps remain unchanged. Lipedematous scalp is apparently a rare disease even though the condition is probably underdiagnosed. As a matter of fact, we diagnosed lipedematous scalp in our patients during a clinical examination for androgenetic alopecia, which was the patients' complaint. The association of lipedematous scalp and androgenetic alopecia in our two patients appears to be coincidental.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/6. Pelvic lipomatosis with cystitis cystica, cystitis glandularis and adenocarcinoma of the bladder: first reported case.Pelvic lipomatosis is a rare disease which may cause obstructive uropathy. It has been reported with cystitis cystica and cystitis glandularis. cystitis cystica and cystitis glandularis have been reported previously as progressing to adenocarcinoma of the bladder. The first reported case of pelvic lipomatosis, cystitis cystica, cystitis glandularis and adenocarcinoma of the bladder is presented and the literature reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/6. Madelung's disease (benign symmetric lipomatosis).Madelung's disease, benign symmetric lipomatosis, is a rare disease of undetermined cause characterized by symmetric deposits of diffuse adipose tissue on the cheeks, the neck, and the upper trunk. This article outlines our experience with four patients and presents a case report of a specifically remarkable affectation that agrees with previous data linking male alcoholics with this disease. The treatment in all cases was palliative surgical removal of excess fat from the neck and paracervical regions.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/6. The use of an ultrasonic assisted lipectomy device for the treatment of obstructive pelvic lipomatosis.Pelvic lipomatosis is a rare disease of unknown etiology characterized by overgrowth of pelvic fat. We describe a 60-year-old man with pelvic and retroperitoneal lipomatosis causing severe urinary obstruction with massive hydroureteronephrosis. The diagnostic procedure and the management of the patient is described, emphasizing the value of the Ultrasonic Assisted lipectomy device in performing ureterolysis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |