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1/7. Efficacy of lipectomy and liposuction in the treatment of multiple symmetric lipomatosis.

    BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by enlarging, symmetric, nonencapsulated, fat deposits mainly on the neck and upper trunk. Liposuction and lipectomy, although palliative, are the treatments of choice, especially indicated when vital structures are compromised. OBJECTIVE: Our purpose was to evaluate the efficacy and safety of liposuction and lipectomy in the treatment of MSL. methods: We have examined two patients diagnosed with MSL who presented with symptoms derived from the compression of vascular, nervous, and/or respiratory tract structures. One was treated with lipectomy and the other with liposuction. RESULTS: A rapid resolution of the clinical symptoms was achieved with both therapies. The patient who was treated with lipectomy suffered from a compression of the left brachial plexus by scar tissue as an adverse effect, requiring a second surgical procedure. Liposuction only provoked a mild autoinvolutive hematoma in the other case. No clinical recurrences were observed at 3 and 2 years of follow-up respectively. CONCLUSIONS: We consider both lipectomy and liposuction as safe and effective techniques for the treatment of MSL patients. Although liposuction is usually associated with less adverse effects than lipectomy, location of the lipomas must be carefully considered before choosing one technique over another.
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2/7. abdominal wall and surgical scar endometriosis: results of magnetic resonance imaging.

    Scar endometriosis is a rare disease which is difficult to diagnose. The symptoms are nonspecific, typically involving abdominal wall pain at the time of menstruation. Clinical examination may reveal a painful nodule, if the scar involved is located on the abdominal wall, but is normal, when the lesion is located on the uterine scar. Other means of investigation (transvaginal ultrasonography, computed tomography) may be useful in case of lesions on the abdominal wall, or if the nodule is large, but give no specific results. The diagnosis is frequently made only after excision of the lesion. We report here 4 patients operated for scar endometriosis (two abdominal and two uterine scars) for whom MRI had suggested the diagnosis. Thanks to its very high spatial resolution, MRI enables very small lesions to be detected and can distinguish the hemorrhagic signal of endometriotic lesions. Furthermore, it performs better than the CT scan in detecting the limits between muscles and abdominal subcutaneous tissues.
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3/7. Clear cell carcinoma arising in a cesarean section scar. Report of a case with fine needle aspiration cytology.

    BACKGROUND: Clear cell carcinoma arising in a cesarean section scar is an extremely rare disease. To the best of our knowledge, there is no published report on the aspiration cytology. CASE: A 56-year-old woman presented with a mass in a cesarean section scar. Initially an abdominal desmoid was considered, but the features of fine needle aspiration (FNA) cytology suggested an adenocarcinoma. The resected tumor was histologically composed of clear cell carcinoma showing cystic, solid and papillary patterns. CONCLUSION: FNA cytology of masses arising in a surgical scar can be a useful tool in obtaining an accurate pathologic diagnosis of a malignant neoplasm.
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keywords = rare disease
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4/7. Cutaneous anthrax on eyelids.

    BACKGROUND: Ophthalmologists should be aware of the signs and symptoms of anthrax, although it is a rare disease in humans. We report our successful treatment of three patients with cutaneous lesions in the periorbital area. CASES: In this study, we report on the treatment of three female patients who were initially diagnosed as having preseptal cellulitis. OBSERVATIONS: Gram-positive robs were revealed in the microscopic examination of scrapings taken from the lesions. bacillus anthracis was found in only two of the three scraping-material cultures. Intravenous penicillin g was administered in all cases. Black and necrotic eschar, which is characteristic of anthrax, developed on the eyelids of all three patients during treatment. At the final examinations of the patients after the completion of treatment, we recognized the development of cicatrisation, lagophthalmos, and slight ectropion in the upper eyelid of the first patient, and, in the second patient, restriction of upper eyelid movement and development of a corneal scar from exposure keratopathy and ectropion. The cutaneous lesions healed without any eyelid pathology in the third patient. CONCLUSION: Although it is a rare disease in humans, anthrax should be considered in the differential diagnosis of preseptal and orbital cellulitis.
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keywords = rare disease
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5/7. Scar sarcoidosis in childhood: case report and review of the literature.

    sarcoidosis is a rare disease among children, manifesting differently in children below and above 4-5 years of age. Although the exact incidence and prevalence of childhood sarcoidosis is not known, the cutaneous involvement is frequent in both children and adults. Infiltration of old cutaneous scars with sarcoid granuloma in the active phase of disease, known as scar sarcoidosis is one of the uncommon cutaneous manifestations of sarcoidosis. We report a case of scar sarcoidosis in an 11-year-old child along with a brief review of the literature.
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keywords = rare disease
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6/7. Variegate porphyria. Clinical and laboratory features.

    Variegate porphyria (VP) is a rare disease infrequently diagnosed in the united states. The typical cutaneous signs of VP, including fragility, bullae, scarring, hypertrichosis, and photosensitivity, are indistinguishable from those of porphyria cutanea tarda (PCT). Inadequate laboratory evaluation of porphyrins present in, or improper interpretation of porphyrin test results from, biologic specimens from patients with VP may lead to inappropriate diagnosis and treatment. Clinical and laboratory data for three such cases of VP are presented. A plasma porphyrin fluorescence criterion which appears specific for patients with VP was observed in all three cases.
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keywords = rare disease
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7/7. Rare case of the utero-vesical fistula caused by intrauterine contraceptive device.

    The vesico-uterine fistula is a very rare disease. There have only been 150-200 causes. We are reporting on a case in which the chronic fistula was caused by an IUD having been placed 4 years ago and it "wandered" through the bladder. A 30 year-old patient in 1992 and IUD was inserted. She had gynecological controls twice, in 1993 last time. She has problem of urination very often. A cyclical bladder bleeding drew the attention to the disease. In ambulanterely performed cystoscopy we found an IUD perforating towards the interior of bladder in the borderline of its bottom and back wall and was situated in the bladder with its 3/4. We have removed it with forceps. After six weeks of expectation and strict observation did we want to manage the fistula after having consolidated the symptoms of the inflamed surroundings. During the operation we have noticed a wallment size mass of scar between the uterus and the bladder expanding to the height of the orifice of the uterus. The scarily fixed bladder has been separated from the cervix and the scarry wall of the fistula has been cut out. We have brained the cervix towards the vagina and then we've sutured the cervix and the bladder with Dexon 'O' treat, as well. We have interposed a surgical net between the cervix and the bladder followed by blood-clotting and peritonisation. We should take the follows into consideration: careful separation, fine operative technique, and strong well absorbing thread as well as trying to keep the organ. In our opinion the bioplast--interpositum used on our case maker the efficiency of the operation higher.
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keywords = rare disease
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