[Congenital lipomatous hypoplasia of the exocrine pancreas in a child. Report of a new case].
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Seventy-four pediatric patients with parotid gland tumors were seen during a 52-year period. Seventy percent of the lesions were benign and 30% were malignant. When the primary treatment is either subtotal or total parotidectomy, the recurrence rate was 2.3%. The facial nerve should be dissected free of parotid parenchyma and preserved, except if the lesion was malignant--a situation in which the nerve might have to be sacrificed totally or in part to ensure adequate tumor removal. No patient died of a benign or malignant lesion in this series, and the morbidity after surgical removal of primary tumors of the parotid gland in children and infants was low.
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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.
We report for the first time to our knowledge long-lasting (4 hours) potentiation of single intubating dose of rocuronium by a single bolus of amikacin given 55 minutes later in a woman having no precipitating factor (renal failure, hepatic failure, ionic disorder, other drugs influencing neuromuscular function). This patient had received the same rocuronium dose one month sooner in similar circumstances (without aminoglycoside antibiotic drug) and had not presented any prolonged neuromuscular blockade at this time. Neuromuscular blockade should be monitored in every patient receiving aminoglycoside antibiotic with even a single intubating dose of neuromuscular blocking drug.
OBJECTIVE: To review the characteristics of Madelung's disease which is rare and unfamiliar to clinicians and to find the method of diagnosis and treatment. METHODS: Detailed clinical data of 7 patients with Madelung's disease were reviewed and analyzed. And related literatures were discussed together. RESULTS: All of 7 patients have excessive subcutaneous fat deposit predominantly around neck. One patients had the complication of central and peripheral neuropathy, One patients had the complication of glucose intolerance, One patients had the complication of chronic hepatopathy and hyperuricaemia. These 3 patients were associated with sleep apnea syndrome simultaneously. Another one patient was accompanied by autonomic nervous system disease only. Total neck lipectomy and abstinence from alcohol were performed on 5 patients. No recurrence was seen during a follow-up of 6 months to 5 years. One patient received partial lipectomy twice and relapsed again. All pathological results were nonencapsulated fat. One patient refused treatment. Six men patients were alcohol abusers. CONCLUSIONS: Madelung's disease is characterized by massive accumulation of nonencapsulated subcutaneous fat mainly located symmetrically in the neck. Chronic alcoholism may be a major risk factor. It may be associated with some internal diseases. Total neck lipectomy to improve figuration and relieve pressure and abstinence from alcohol may be the main effective therapy.
A 45-year-old man presented with intermittent abdominal pain for a month. Intestinal intussusception was diagnosed by the findings of abdominal ultrasonography and computed tomography. Intussusception was reduced by barium enema. Based on magnetic resonance imaging, we diagnosed intestinal intussusception due to multiple lipoma. At laparotomy, after successful reduction of the intussusception with Hutchinson's maneuver, an ileoceal resection was performed. Intussusception in adults is relatively rare and may be difficult to diagnose preoperatively. In the diagnosis of this disease, abdominal echo, CT, and MRI are useful.
Submucosal lipohyperplasia of the ileocecal valve (ICV) is reportedly a rarely diagnosed lesion of uncertain significance. Eight cases of ICV lipohyperplasia diagnosed in surgical specimens (seven resections, one biopsy) are reviewed: three cases were associated with right lower abdominal quadrant pain and ICV mass on barium enema or operative examination, two were associated with ICV mucosal acute inflammation and necrosis, and three were incidental in resections for cecal, appendiceal, and sigmoid neoplasia. To evaluate the frequency of ICV lipohyperplasia and any associated processes, a series of 51 autopsies was studied. Regarding lipohyperplasia in these valves, 10 (19.6%) were determined to have none, 14 (27.5%) were mild, 20 (39.2%) were moderate, and 7 (13.7%) were marked cases. Degree of lipohyperplasia correlated statistically with degree of cardiac right ventricular fatty infiltration (p = 0.0001), pancreatic fatty infiltration (p = 0.0314), and greater body weight of patient (p = 0.0009). No definite correlation was demonstrated with left ventricular, adrenal, or lymph node fatty infiltration, or with hepatic fatty change, body height, age of patient, or blood glucose. Various gastrointestinal symptoms and lesions accompanied lipohyperplasia, but no definite causal relationship was identified, except for one case of marked lipohyperplasia associated with marked mucosal necrosis and acute inflammation of ICV. In conclusion, ICV lipohyperplasia is a common finding that occasionally may be associated with clinical symptoms and other valve pathology. It correlates to some extent with right ventricular and pancreatic fatty infiltration and with greater body weight.
Mobile encapsulated lipomas are a benign neoplastic process with pronounced mobility in the subcutaneous plane. Characteristic clinical and histologic features differentiate this distinctive entity from similar reactive adipose processes.
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10 cases of lipofibromatous hypertrophy of the main nerves in the upper limb in 7 female and 3 male patients are described. In 1 case at the level of the middle third of the upper arm and in 6 cases at the level of the wrist and neighboring areas. In 2 patients these pathological changes were found in the ulnar nerve, in 1 case the hypertrophy was limited to the upper arm, in the other it extended all along the extremity from below the axilla to the wrist. Finally, in 1 case a 20 cm long hypertrophy was found at the level of the deep branch of the radial nerve (posterior interosseus nerve) in the lower arm below the elbow. The fibrous and lipomatous tissue snugly surrounds the fascicles and cannot be separated from them without damaging them, even if the finest microsurgical techniques are used. As the disease has been diagnosed in children as young as five years a congenital etiology has to be considered. The condition is distinct though from the disease of DEJERINE-SOTTAS or from that of von RECKLINGHAUSEN. The hypertrophic nerve is progressively submitted to a chronic compression in the naturally narrow passages (carpal tunnel, ulnar grove at the elbow, arcade of FROHSE) or it becomes painful and paretic after even minor trauma which leads probably to local oedema, intraneural bleeding and epineural compression. An external and internal decompression has to be carried out. Resection or defatting of the hypertrophic fascicles, however, should be avoided. In 2 of our cases the disease was anamnestically present for 45-50 years and clinically followed in another case for 18 years. The disease does not seem progressive. These cases show, that this disorder, which first appeared in the medical literature in 1964 and have been described by several authors in the median nerve, exists as well in the two other main nerves of the upper extremity.
Macrodactylia fibrolipomatosis is a rare congenital anomaly or neonatal form of enlargement of the digits. An infant with this malady affecting the left foot is described by the author. Initial observation, followed by appropriate surgical intervention, consisted of amputation and debulking procedures. Improved functional and cosmetic appearance is the desired result.
Localized soft tissue and bone overgrowth may be the result of many different causes and will vary in severity, from involvement of a single digit to one half of the body. There are a variety of causes, including chronic lymphedema; lymphangiomatous malformations; neurofibromatosis; vascular malformations including the Klippel-Trenaunay-Weber syndrome; macrodystrophia lipomatosa; multiple enchondromatosis and Maffucci's syndrome. More recently, the Bannayan syndrome and the Proteus syndrome have been documented among the causes. Unexplained hemihypertrophy may also occur in association with intra-abdominal neoplasms. The various entities are reviewed and their radiological features described and illustrated.
The authors present four cases. The first case is a diaphragmatic-mediastinal, the second one is a cervical-mediastinal one, the third case has a buttock, the last one is a case of hip-thigh. Their ages are one, eight and three years old and the fourth case is seven days old. They appear in axilla, neck, thoracic wall and fat prevertebral tissue, in below three year old children. Despite their benignity, the tumors grow very fast. The complete extirpation is fundamental to avoid relapses, which are presented approximately in a 15 for 100 of the cases. Mutilations are not necessary, since it is a benign tumor. Finally, the authors amply review the literature. They differentiate the concepts of lipoma, hibernoma and lipoblastoma.
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When lipomas reach 4 cm or more in size or are multiple, liposuction becomes an excellent alternative to excisional surgery. The basic concept is that fat lobules are bluntly broken down or curetted by a cannula and then removed through the cannula's aperture by a vacuum force generated from a suction machine. There are few minor sequelae, and these are rarely of a prolonged nature. The benefits of liposuction are decreased surgical time, a shorter scar, a better final contour, and the ability to remove several lipomas through one opening. In addition, liposuction surgery is a useful modality for the removal of lipomas because of the low recurrence rate and minimal malignant potential of these tumors.