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Three cases of pelvic lipomatosis are presented. Excretory urogram revealed characteristic elevation and elongation of urinary bladder base and relative hyperlucency of pelvic cavity. Associated varying degrees of hydronephrosis and hydroureters are seen secondary to distal ureteral obstruction. Barium enema showed elongation and elevation of rectosigmoid colon.
Pelvic lipomatosis and florid cystitis glandularis are rare. We report a case in which these two conditions were associated.
Pelvic lipomatosis is a rare condition which may be associated with obstructive uropathy. The radiologic features may be stimulated by pelvic malignancy and, in the past, surgery has often had to be performed for confirmation of the diagnosis. A case is described in which computed tomography (CT) provided definite proof that the pelvic organ displacement was due to excessive fat. It is considered that CT may obviate the need for further workup or surgery in these patients unless exploration is required for the relief of ureteric obstruction.
Benign symmetric acquired lipomatosis is a rare condition characterized by multiple, diffuse, subcutaneous collections of nonencapsulated mature adipose tissue. A thick and disfiguring deposit of fat is symmetrically distributed in the subcutaneous tissue of the neck, upper trunk and proximal portions of the upper extremities. The face, distal extremities, hands, and feet are characteristically spared. We describe a case in which only plantar involvement was present.
We describe a 2 1/2-year-old girl with encephalocraniocutaneous lipomatosis. The dysmorphologic manifestations in the skull, brain, skin, and eyes associated with a normal karyotype suggested the diagnosis of this rare neurocutaneous syndrome.
Spinal epidural lipomatosis (SEL) is defined as the deposition of loose fat tissue in the epidural space. Two cases are reported which illustrate the main features of this uncommon disease. Most frequently SEL is revealed by neurologic manifestations such as an acute chronic spinal cord compression. Corticosteroid therapy and obesity are the main factors able to induce the disease. The common location of the SEL at the thoracic spine is explained by the physiologic abundance of fat and by the relative narrowness of the spinal canal at this level. An osteoporotic vertebral crush fracture which reduces the diameter of the canal frequently reveals the SEL. MRI confirms the diagnosis of SEL and shows the extent in the spinal canal. The treatment of SEL is difficult. The decrease or the stop of corticosteroid therapy has allowed the recovery in a few cases with chronic neurologic symptoms. The risks of a surgical procedure are important in these patients and the medical or surgical treatment should be discussed taking into account the clinical symptoms of SEL, its location and extent, and the risks of recurrence if corticosteroid therapy must go on.
A patient undergoing chronic corticosteroid therapy presented with mesenteric lipomatosis that simulated malignant masses. Computerized tomography was instrumental in identifying these apparent tumors as benign fatty deposits. The spectrum of lipomatous abdominal lesions amenable to diagnosis by computerized tomography is reviewed.
Renal replacement lipomatosis (RRL) is the result of rare, usually unilateral, and severe atrophy and destruction of the renal parenchyma often caused by renal calculi. It may be associated with, sometimes, aging, atrophy, long-standing chronic inflammation and urinary infection, such as renal tuberculosis. We report magnetic resonance (MR) and computed tomography (CT) findings of our case, which has xanthogranulomatous pyelonephritis (XGP) and RRL additionally associated with nephrocutanous fistula.
AIMS: The aim of the present study was to investigate possible alterations in body composition and resting energy expenditure (REE) in type 1 multiple symmetric lipomatosis (MSL). SUBJECTS AND METHODS: Thirteen men aged from 40 to 78 years affected by type I MSL were compared with 13 healthy control subjects. Fat mass (FM) and fat-free mass (FFM) were determined by DEXA using both standard analysis and specifically for the lipomatous region. REE was measured by indirect calorimetry. RESULTS: FM was higher in MSL subjects at proximal arm level, but significantly lower at distal leg level than in controls (left 1.63+/-0.55 vs. 2.26+/-0.49 kg, P<0.05; right 1.63+/-0.53 vs. 2.40+/-0.54 kg, P<0.01). Arm FFM was similar in the two groups, while distal leg FFM was significantly lower in MSL cases (left: 7.8+/-1.3 vs. 8.7+/-0.8 kg, P<0.05; right: 8.0+/-1.5 vs. 9.2+/-0.9 kg, P<0.05). FFM strongly correlated with REE (r:0.86;P<0.001). REE, expressed as an absolute value and adjusted for FFM (1830+/-215 vs. 1675+/-120 kcal, P<0.05) was higher in MSL patients. CONCLUSION: In conclusion, MSL patients had a marked FFM and FM atrophy in the lower segments of the legs and an altered energy expenditure (hypermetabolism).
Launois-Bensaude syndrome, otherwise known as Madelung's disease, is a rare disorder characterized by fatty accumulations of the upper trunk, neck and head, causing serious cosmetic deformity and neck immobility. We report a case of a patient with Launois-Bensaude syndrome who required 4 different airway management strategies during his hospitalization for postoperative complications after an elective surgical excision of a severe neck lipomatosis. Anesthesiologists who treat patients with Madelung's disease should be aware of 2 major problems: difficult airway and increased frequency of postoperative bleeding. A proper choice of airway management technique is therefore necessary, including a safe intubation and extubation plan to reduce the consequences of airway complications.
A case of congenital parotid lipomatosis in an infant is reported and literature is reviewed. This rare condition in children presents as gradually increasing parotid swelling, which is difficult to diagnose preoperatively as this condition is not considered in the differential diagnosis of a parotid mass. Complete excision with superficial or total parotidectomy with preservation of facial nerve is the treatment of choice.
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.
The neurocutaneous pattern syndromes are a group of disorders characterized by congenital abnormalities involving both the skin and the nervous system for which no identifiable cause has been isolated.(1) Ophthalmologic manifestations of these syndromes are common. These rare syndromes often have overlapping ophthalmic and systemic findings. Examples include encephalocraniocutaneous lipomatosis (ECCL), oculocerebrocutaneous syndrome (OCC), and linear nevus sebaceous syndrome (LNS). Clarifying the diagnostic criteria for these syndromes is a worthy goal because it will help elucidate the phenotypic spectrum of these poorly understood diseases as well as possibly facilitate genetic counseling. In this short report, a case of ECCL is used to illustrate the clinical manifestations of neurocutaneous syndromes.
Two cases of 'sabre sheath' trachea in combination with mediastinal lipomatosis are reported. The initial chest radiographs in both cases were interpreted as showing a mediastinal tumour causing tracheal compression. The correct diagnosis was subsequently made using computed tomography (CT). It is important to be aware of these benign conditions when interpreting chest radiographs, particularly in cases of suspected mediastinal pathology.
Multiple Symmetric Lipomatosis (MSL) is a rare disorder of lipid metabolism which is mainly seen in Mediterranean and eastern European populations, which results in massive fat accumulation mainly around the neck and back. The main differential diagnosis lies between MSL and the fat accumulation of Cushing's disease, and liposarcoma. This case demonstrates that MR imaging is a valuable aid to the diagnosis and treatment of this disease by giving excellent definition of soft tissue and vascular structures, allowing accurate assessment and preoperative planning of the disease.
A case of parotid lipomatosis is reported, and the literature is reviewed. This rare condition presents as a slow growing parotid mass, which may be diagnosed preoperatively using computed tomography or magnetic resonance imaging. Long-term clinical follow-up is important because apparently complete surgical excision may be complicated by recurrence.
We report a case of pelvic lipomatosis with ureteral obstruction and vesicoureteral reflux. In addition to computerized tomography, nuclear magnetic resonance imaging provided definite and graphic proof of the diagnosis. Operative treatment consisted of ureteroileocystostomy.
Pelvic lipomatosis is uncommon in female subjects. The symptoms and signs are vague in all patients but radiologic studies, especially pelvic computerized tomography, should provide data for the diagnosis and obviate operative intervention. All patients should have careful followup to rule out obstructive uropathy.