DEFORMIDADE DE MADELUNG PDF

If no improvement is made, a splint or brace can be used to keep the deviated arm straight. When none of the conservative treatments work surgical intervention is designated. Pediatrics[ edit ] Physiolysis Purpose of the treatment is the removal of the epiphysis that causes the abnormal growth of the wrist. This is done by making a small incision at the volar-radial side.

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The only effictive treatment for this disease is surgery. The patient showed noteworthy aesthetical improvement, without recurrence of the lipomatous deposits sofar. Keywords: Lipomatosis; lipodystrophy; symmetric benign lipomatosis. Since then, a little more than cases have been reported on in medical literature. This condition, of still unknown etiology, is characterized by a symmetrical deposition of adipose tissue along the neck, which causes the classical aspect called "horse collar" Otto Madelung 17 , , as well as on the parotid, retro auricular and submental regions hamster cheek , torso, deltoid and supraclavicular regions and in the proximal part of the upper limbs , pseudoathletic aspect , as well as the posterior cervical region buffalo hump Enzi 8 , The slow and progressive growth of the lipomatous masses brings about an aesthetical disfigurement, which often causes the patient to isolate himself or to go into depression Cavalcanti 3 , The lesion originates in the subcutaneous cellular tissue, penetrating into the muscular fascias or in spaces between the organs, apparently following the path of least resistance.

The lipomatous masses are firm and not encapsulated, mixing with the subcutaneous cellular tissue around them, and they are intimately related to the muscles, vessels and nerves. Microscopically, the lipomatous tissue differs from the adipose tissue because it shows more fibrous and vascular elements Enzi 7, 8 , Growth of lipomatous masses occurs through cellular multiplication similar to a neoplasia and not through hypertrophy of the preexisting cells Enzi 7 , It is believed that these patients presem a defect in the lipoliytical chain in a phase prior to the formation of intracellular AMPc Dorigo 5 , ; Enzi 7 , Klopstok et al.

These are associated not inconstant factors: ethylism, tabagism, hyperinsulinemia, hyperlipoproteinemia and hyperuricemia. However, there can be an occurence of mediastinal syndromes deriving from the compression of structures of the mediastinum by the lipomatous tissue, as well as sensorial, motor and autonomical neuropathies Enzi 8 , Diagnosis of this syndrome can be easily be done by observing the peculiar appearance of the patients; a computerized tomography and magnetic resonance are useful in deterrnining the extent of the masses as well as their relation to the vessels, nerves and muscles.

Differential diagnosis should include the various lesions of the subcutaneous cellular tissue, such as lipomas, angiodysplasias, neurofibromas, sarcomas, goiters, sialoadenitis, obesity and lymphatic tumors.

Several therapeutic methods have been suggested in an attempt to reduce the volume of lipomatous deposits 11, 14, 16, 23, 25, Leung et al. The patient, in this case, showed normallipolytic activities "in vitro" as well as "in viva". However, it is not certain whether lipolytical activitiesremain intact in this disease. Surgical excision, however, is the best treatment Dorigo 5 , ; Enzi 8 , ; Hoehn 9 , ; Hugo 10 et al.

Since there is no plan for cleaving the lesions and the normal adipose tissue, total resection of the deposits becomes impracticable. It is thus better to adopt a sculptural technique, in order to restore a relatively normal contouring while removing the largest possible part of the lipomatous tissue Hugo 10 , Despite the rigorous hemostasis and the use of postoperative drains, development of seromas and hematomas is rather common Hugo lO et al.

The use of liposuction in treating this syndrome was described by Carlin and Ratz 1 They considered this method as an ideal palliative, since it can be carried out under local anesthesia, with smaller cicatricial consequences and in as many phases as necessary. However, relapse of lipomatous masses is probable, just like in the surgical excision procedures. Although these masses were painless, they were progressively increasing in volume.

At the same time, he noticed a growth of masses on his face, his retro auricular and supradavicular areas, causing a slight change in his aspect.

The patient reported that he had not gained any weight during this period. He was a smoker for forty years thirty cigarettes per day and he was used to drinking one dose of alcoholic beverage per day in the last twenty years. No other diseases were reported. Patient was 1. The exam of the segments was normal, except for the presence of firmly consistent masses, with weak adherence to the deep painless planes along the pre, retroauricular, submento and cervical posterior regions, as well as the scapular regions, the lumbar region, the breasts and the scrotum.

Table II shows the surgical procedures performed. The disease begins in adults with and a previous history of alcoholism and tobaccoism Enzi 7 , ; Kodish 11 , ; Leung l4 , ; Lyon 16 , ; Moretti 20 et al. These factors were observed in our patient along with a progressive evolution of the lipomatous masses Enzi 8 , Surgical excision was chosen as it is the only effective method of treatment Dorigo 5 , ; Enzi 8 , ; Hoehn 9 , ; Hugo lO et al.

As referred to by Schuler 26 , the operations were laborious because of the infiltrative, fibrous and hypervascular nature of the lipomatous masses, which showed no plans of cleavage. Even though Penrose drains were used during two days after surgery, all operations formed seromas, requiring aspiration drainage. The anatomicopathological findings revealed adipose tissue with an increase in fibers and vessels, as reported by Enzi 8 , The postoperative result confirms the literature, showing that various interventions in series arenecessary, along with patience and skill of the surgeon in order to provide the patient a more harmonious appearance.

Even though the operation entails only temporary success, the aesthetical and psychological benefits obtained justify the operations. Multiple symmetric lipomatosis - treatment with liposuction. Different clinical types of lipomatosis. Multiple symmetric lipomatosis: a defectin adrnergic-timulated lipolisis II. South Med. ENZI G et al. Multiple symmetric lipomatosis: a defect in adrnergic-stimulated lipolysis. ENZI G. Multiple symmetric lipomatosis: an update clinical report.

Benign symmetrical lipomatosis: case report. Reconstr Surg. Benign symmetric lipomatosis: a case report. Benign symmetric lipomatosis: functional sympathetic denervation of adipose tissue and possible hypertrophy of Brown Fat. Familiar multiple lipomatosis. De ttadenolipotose symmetrique.

Paris, ; Multiple symmetric lipomatosis Launois - Bensaude Syndrome : effect of oral salbumatol. Adiposis and lipomatosis considered in reference to their constitutional relations and symptomatology. Uber den fetthah. Mendelian inheritance in man. Paris: Presse Med, ; Laryngeal involvement in benign symmetrical lipomatosis.

Brain Oxford. Multiple symmetric lipomatosis in a patient with Hurthle cell carcinoma of the thyroid: a case report. Benign syrnmetric lipomatosis Launois- Bensaude : report and review of the literature. Benign symmetric lipomatosis of the neck treated by liposuction: case reporto Scand. Hand Surg. Computed tomography in the evaluation of musculoskeletal neoplasms. Head Neck Surg. Launois Bensaude adenolipomatosis: case report.

Symmetrical lipodystrophy with hypothyroidism. IV - Gereral surgeon.

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