1/9. Fibrous dysplasia of the temporal bone and maxillofacial region associated with cholesteatoma of the middle ear.Fibrous dysplasia of the temporal bone is a rare disease which may lead to progressive stenosis of the external auditory canal and the development of cholesteatoma. We present a case in which minimal symptoms were present despite a massive temporal bone fibrous dysplasia. cholesteatoma resulted most probably secondary to external auditory canal stenosis. Retroauricular fistula developed as a result of destructive effect of cholesteatoma, that influenced previous diagnosis and treatment of this clinically silent disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/9. Congenital pleuroperitoneal communication in a patient with pseudomyxoma peritonei.BACKGROUND AND OBJECTIVES: pseudomyxoma peritonei syndrome is a rare disease arising from a perforated appendiceal adenoma. The syndrome is characterized by progressive accumulation of mucinous ascites and tumor within the peritoneal cavity. Direct extension of pseudomyxoma peritonei to the pleural cavity is uncommon and has been associated with surgical penetration of the diaphragm at the time of cytoreduction. methods: We review the case of a patient who presented with mucoid peritoneal and pleural fluid consistent with spontaneous pleural spread of pseudomyxoma peritonei. RESULTS: Surgical exploration confirmed direct pleuroperitoneal communication by macroscopic diaphragmatic fenestration. CONCLUSIONS: This is a rare phenomenon. We outline a therapeutic approach to be applied when pleural involvement is suspected in patients with pseudomyxoma peritonei syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/9. A study of piriform sinus fistula cases.We have experienced five cases of piriform sinus fistula for the last 10 years. It is a relatively rare disease, and partly because of poor understanding of the disease, in one case infection had repeatedly recurred without being adequately treated for over 20 years, and in most cases there was a long time lapse before the diagnosis. In another case, it was difficult to image the fistula with contrast medium and fistulectomy was performed without identifying it on imaging. We have applied various devices to those cases where imaging of fistula was difficult, and achieved complete resection of fistula and have not observed recurrences of infection after resection.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/9. Congenital midline sinus of the upper lip. Report of a case.An extremely rare case of congenital midline sinus of the upper lip in a 12-year-old girl is presented. The sinus had a pit-like orifice in the midline philtral area with a duct extending 4 mm posterosuperiorly. It was excised by extraoral elliptical incision. Histopathological examination disclosed that the duct was covered by a stratified squamous epithelium with sebaceous glands. Twelve cases of this rare disease have previously been reported.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/9. tuberous sclerosis and associated pleuropulmonary lesions.tuberous sclerosis (TS) is a rare disease of the nervous system, being characterized by seizures, mental retardation and adenoma sebaceum. Concomitant pleuropulmonary lesions and spontaneous pneumothorax are extremely rare during the evolution of this disease. To date, only 19 cases of TS and spontaneous pneumothorax have been described in the literature. Here we present a case of TS and associated pleuropulmonary lesions with spontaneous pneumothorax in a 29-year-old female patient. Clinical, roentgenographic and histological aspects of this disease are commented upon.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/9. Recurrent suppurative thyroiditis due to pyriform sinus fistula: a case report.Acute suppurative thyroiditis is a rare disease, particularly in childhood. We present a case with recurrent acute suppurative thyroiditis due to a pyriform sinus fistula originating from the fourth branchial pouch. The typical symptoms of a pyriform sinus fistula are recurrent left-sided pain and swelling of the neck with signs of acute bacterial inflammation. diagnosis should be made by high resolution ultrasound, barium meal studies and endoscopic examination. During acute exacerbations treatment with antibiotics is indicated, but permanent cure can only be attained by complete fistulectomy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/9. portal vein aneurysm in the liver associated with multiple vascular malformations.portal vein aneurysm (PVA) includes focal dilatation of the portal vein, and was formerly thought to be a rare disease. We report a 46-year-old man with chronic aggressive hepatitis and intrahepatic portal vein aneurysm communicating with the hepatic vein. Hemangiomas in the liver and intracranial arteriovenous malformation (AVM) were also found. To our knowledge, this is the first report of a case of PVA in a patient with congenital intracranial AVM. As the PVA in this patient communicated with the hepatic vein, and as hemangiomas in the liver and intracranial AVM were also present, the pathogenesis in this patient seems to have been congenital anomaly of the vasculature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/9. Aortoesophageal fistula. Report of one case complicating foreing body ingestion with considerations about pathogenesis.Aorto-esophageal fistula is a very rare disease and few cases are reported. The most frequent ethiological factors are foreign body ingestion primary disease of esophagus or aorta and iatrogenic damages in particular endoscopical investigations. In this report a new case is described and a pathogenic explanation for the development of the disease is proposed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/9. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |