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A new way of scanning the kidney by ultrasonography is the posterior oblique longitudinal scan. This provides new information about renal anatomy, especially the renal aspect of the parenchyma and the hilus in its vertical axis. It supplies useful diagnostic information particularly in pseudotumours, hydronephrosis, and lipomatosis.
Sonographic examinations of 17 patients revealed 21 kidneys with anechoic sonolucent areas within the renal sinus. Computerized tomography was obtained in 3 patients and the mass effects were proven to be multilocular parapelvic cysts. These parapelvic cysts simulated renal sinus lipomatosis.
Madelung-Launois-Bensaude lipomatosis is a rare disease characterized by an enormous accumulation of fat, mainly in the region of the neck. A case is presented to illustrate the course of the disease and its clinical features. In particular, the necessity for a bilateral conservative neck dissection is emphasized to avoid early recurrence.
Madelung's disease, also known as benign symmetrical lipomatosis, is a rare proliferative disorder of unknown etiology that was first mentioned by Brodie in 1846. Characterized by multiple symmetrical deposits of unencapsulated fat in the head and neck region, the disease is most common in middle-aged men with a history of alcohol abuse. The only effective therapy in cases of dyspnea and dysphagia, indicating the necessity of treatment, is the surgical resection of the adipose tissue. The authors report on the evaluation and therapy in a case of giant Madelung's disease.
INTRODUCTION: The aim of this study was to correlate structural, histomorphological damage of the salivary gland with scintigraphic findings during fractioned radiotherapy. METHODS: The head and neck area of 27 WAG/RijH rats was irradiated with (60)Co-gamma-rays (60 Gy/30f/6 weeks). A port-system was implanted and (99m)Tc-pertechnetat applied at different stages of irradiation (0, 16, 30, 46, 60 Gy and 6 months post irradiation). RESULTS: After the application of 16 Gy an intra- and extra-cellular oedema developed in the salivary glands. The progressive vacuolisation (30 Gy) passed over into lipomatosis (46 Gy) and necrosis (60 Gy) in the parotid and mandibular glands. Six months after irradiation treatment, the chronic histomorphological damage corresponded to stage II according to Seifert. The corresponding loss in gland function was 13% (16 Gy); 26% (30 Gy); 57% (46 Gy); 75% (60 Gy) and 66.5% (6 months post irradiation). CONCLUSION: This animal model demonstrates the correlation between histomorphological and scintigraphic findings.
In the case of a 49 year old patient, a weight gain of 37 kg occurred during the first weeks of the year 2003, accompanied by a painless increase in the diameter of the upper arms and thighs. This process did spontaneously cease in the summer of 2003, but weight reduction could not be achieved. The diagnosis of multiple symmetrical lipomatosis, also known as Launois-Bensaude syndrome, the first authors to describe the condition in detail in 1898, could be established due to the unique appearance of the patient. Typical features of the disease are the accumulation of multiple lipomata in the shoulder girdle, upper arms, thorax and thighs, whereas the face, the forearms and the shanks are typically excluded. The etiology of the disease is obscure, dietetic intervention is futile, surgical approaches are liposuction or excision of the lipomata. Cessation of alcohol consumption may also be helpful, since the condition is typically associated with present or past alcohol abuse. The disease is usually reported to be rare, but there is reason enough to assume, that it is frequently misdiagnosed as simple truncal obesity.
Benign Symmetric Lipomatosis (Madelung's disease) is a rare disease, characterized by massive fatty deposits in the neck, the shoulders, and the upper extremities. The deformity is associated with chronic alcohol use, malignant tumors of the upper airways, neuropathy, diabetes mellitus, hyperlipidemia, and other metabolic disorders. Although the deformity is prone to recurrence, surgical removal via lipectomy or liposuction provides the only way of palliation. This paper describes the treatment of a 51-year-old man with a history of alcoholism and liver cirrhosis. He reported masses in his cervical and facial regions that had gradually enlarged over a period of 6 years. He also developed respiratory symptoms due to the fatty compression of his upper airways. Our experience with ultrasound-assisted liposuction for the palliative treatment of this disease is reported.
Liposuction has become the most frequent aesthetic procedure, and its indications continue to expand to different areas of plastic surgery. In this article the authors present their experience with liposuction in the treatment of nine cases termed "atypical," for not being purely aesthetic. Included are four congenital lipodystrophies-occult spinal disraphism, osteogenesis imperfecta, congenital lipomatosis difusa, and Klinefelter syndrome-and five acquired ones-posttraumatic lipoma, posttraumatic asymmetry, insulinic hypertrophic lipodystrophy, adiposis dolorosa, and TRAM flap. Particular features of each lipodystrophy are reviewed and details of fat distribution and density are described. We also review some specific details of the technique. On the other hand, we wish to point out the importance of recognizing and correctly diagnosing some specific lipodystrophies that are part of defined clinical patterns and that require an integral therapeutic approach.
When renal echograms are made, the central renal pyelocalyceal complex (CRPC) is seen as a strongly echogenic oval confluence surrounded by echo-poor renal parenchyma. Fragmentation of the CRPC is abnormal, and this fragmentation is widely known in association with urinary obstruction. But, there are a variety of nonobstructive causes for dispersion of the CRPC; renal duplication, postobstructive atrophy, chronic pyelonephritis, renal sinus lipomatosis, nephrocalcinosis, and renal cystic disease. Patterns of CRPC fragmentation may be correlated with renal parenchymal status to determine the cause of abnormal renal echograms.
Computed tomography (CT) was used to distinguish between pelvic lipomatosis and idiopathic inferior vena caval thrombosis as a cause of a pear-shaped bladder seen on intravenous urography. The findings on CT were explicit in explaining the patient's clinical problem.
There is a broad spectrum of lipomatous tumors that involve soft tissues including ordinary lipomas, variants of lipomas, heterotopic lipomas, lipomatosis, hibernomas and liposarcomas. The recognition of these tumors is important to avoid diagnostic pitfalls. The MR imaging appearance of the lesion, including location, shape and internal architecture, suggests the diagnosis of lipomatous tumors. The signal intensity on T1-, T2-, fat-suppressed T2-weighted (FS-T2) images or short tau inversion recovery (STIR), T1- and fat-suppressed T1-weighted images after Gd-DTPA administration can be a useful aid in distinguishing between biologically different types of lipomatous tumors. The imaging features are often characteristic and reflect the histological pattern of the tumors. We present an overview of the lipomatous tumors and we correlate MR imaging appearance with histological findings.
OBJECTIVE: Description of a patient with a rare syndrome leading to the partial overgrowth of craniofacial bones. CASE REPORT: In a 17-year-old female patient with severe hyperostoses of the craniofacial bones asymmetric overgrowth of the right upper and left lower limbs, linear hyperpigmentation of the right arm and a history of regional lipomatosis were found. These manifestations were suggestive of Proteus syndrome. The management of the craniofacial involvement is described and a literature overview presented. CONCLUSION: Proteus syndrome is a rare overgrowth syndrome probably related to a somatic mutation that involves craniofacial structures in about 30% of patients. Early diagnosis is required for adequate interdisciplinary treatment.
Fishman syndrome, also known as encephalocraniocutaneous lipomatosis (ECCL), is a rare, congenital neurocutaneous syndrome characterized by unilateral skin, eye, and brain abnormalities. Epileptic seizures and developmental delay are usually present. We report the clinical, radiological, and, for the first time, neurophysiological findings in a 24-year-old woman diagnosed with ECCL who was evaluated for epilepsy surgery. Functional magnetic resonance imaging revealed transfer of memory and language functions to the nonaffected hemisphere, providing evidence that functional reorganization and restoration of cognitive functions may occur in the context of extensive malformations, such as neurocutaneous syndromes.
Highly active antiretroviral therapy (HAART) can induce a characteristic lipodystrophy syndrome of peripheral fat wasting and central adiposity. HIV-1 protease inhibitors are generally believed to be the causal agents, although the syndrome has also been observed with protease-inhibitor-sparing regimens. Here, we postulate that the mitochondrial toxicity of the nucleoside-analogue reverse-transcriptase inhibitors plays an essential part in the development of this lipodystrophy, similar to the role of mitochondrial defects in the development of multiple symmetrical lipomatosis.
A case of Madelung's disease (benign multiple symmetrical lipomatosis, Launois-Bensaude syndrome) is described. The characteristic clinical features, associations with chronic alcoholism and a review of the current literature is reported.
Numerous studies have been devoted to the various histopathologic changes of the surface of the tongue. The muscles of the tongue and their changes in the process of ageing as well as in neurogenic atrophies, however, have not been systematically examined so far. Enlarging upon previous studies, an autopsy material of 170 tongues from all age groups was histologically examined in order to identify the steps in the development of age-related atrophy. These are characterized by a histometrically documented atrophy of the musculature with progressive lipomatosis. Fatty infiltration starts in the 2nd/3rd decade at three sites of predilection: body, root and tip of the tongue in the region of the anterior lingual gland (NUHN). The age-dependent atrophy and the known clinical behaviour of neurogenic atrophies of the tongue are correlated with the histopathology of three typical forms: a neurogenic myasthenic atrophy of the tongue with high-grade symmetrical fatty replacement as an expression of a progressive myasthenic denervation; a lipomatous atrophy of the tongue as a late stage of poliomyelitis and long-term treatment in the "Iron Lung", and a lipomatous hemiatrophy due to posttraumatic extracranial hypoglossus paresis. The paretic part of this tongue consists of 85-90% fatty tissue. The different degrees of muscular atrophy of the tongue are largely compensated by metaplasia of fatty tissue in the perimysium following muscular degeneration (in the sense of a fatty replacement). With the exception of general and severe myasthenic atrophy, size, form and function of the tongue fail to show any significant changes in age-related atrophy and hemiatrophy.
We describe a case of hemifacial hyperplasia due to a combination of lipoma and lipomatosis. The contribution of MRI to the diagnosis of hemifacial hyperplasia and to monitoring growth and development in this condition is demonstrated.
Multiple symmetrical lipomatosis (MSL) is a rare syndrome characterized by symmetric unencapsulated lipomas around the axial region and frequently associated with neurological involvement, particularly myopathy and neuropathy (Klopstock et al., 1994). Here we present a typical image of a patient with MSL, explaining this disease for neurologists who might care for these patients in an average day at the neuromuscular consultation unit.