Cases reported "Dilatation, Pathologic"

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1/213. Undiagnosed Mirizzi's syndrome: a word of caution for laparoscopic surgeons--a report of three cases and review of the literature.

    The mirizzi syndrome is often undiagnosed before surgery and can carry a high risk of iatrogenic damage to the common bile duct when encountered during open or, especially, laparoscopic surgery. Endoscopic management has recently been reported, but this treatment can be performed only when there is a high index of suspicion based on clinical criteria that the condition is present and therefore suggests the indication for endoscopic retrograde cholangiopancreatography (ERCP). This is not always the case. Consequently, in a considerable percentage of patients, the syndrome is discovered only after the bile ducts have been damaged during surgery. Three cases of Mirizzi's syndrome were observed in our experience of 896 laparoscopic cholecystectomies (0.3%). All patients were without typical symptoms, and the syndrome was unsuspected in spite of preoperative intravenous cholangiography. All patients required conversion to an open procedure, with two injuries of the common bile duct (a complete transection and a tear) being promptly repaired. We conclude that when this syndrome is suspected or found during surgery, the surgeon should follow these guidelines: (1) perform intraoperative cholangiography when possible, even through the gallbladder wall; and (2) dissect the gallbladder from above and, if necessary, open it to extract the stone. dissection of Calot's triangle should never be attempted. Great expertise is required to complete the operation laparoscopically. The reported cases in the literature refer to a high percentage of conversion, underlining the technical difficulties and making this syndrome, when undiagnosed and unsuspected, a real challenge for laparoscopic surgeons.
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2/213. Recurrent pancreatitis in a child with pancreas divisum. Endoscopic therapy of a Santorinicele.

    pancreas divisum is a rare congenital anomaly of the pancreatic ducts that has been implicated in pancreatitis. In addition, the finding of a Santorinicele, which is a cystic dilatation of the dorsal duct, suggests that there is an obstruction associated with a congenital or acquired weakness of the mucosa. We used an endoscopic technique to treat a child with recurrent pancreatitis who was found to have pancreas divisum and a large Santorinicele.
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3/213. Unilateral sensorineural hearing loss and its aetiology in childhood: the contribution of computerised tomography in aetiological diagnosis and management.

    OBJECTIVES: The objective of this study was to identify factors correlated with the CT outcome and to examine the contribution of the CT scan in the aetiological diagnosis and management of unilateral sensorineural hearing loss in childhood. methods: The records of 35 consecutively investigated patients by the audiology Department of Great Ormond Street Hospital between January 1996 and June 1998 were reviewed. The CT results, population sample characteristics, initiation of further investigations after the CT results and management decisions based on the CT results were tabulated and analysed. RESULTS: In a series of 35 consecutively investigated children with unilateral sensorineural hearing loss, 11 CT scans were identified as abnormal. The CT findings were: labyrinthitis ossificans (3), unilaterally dilated vestibular aqueduct (2), bilaterally dilated vestibular aqueduct (2), unilateral deformity of the cochlea ('Mondini') (1), unilateral severe labyrinthine dysplasia (1), unilateral markedly narrow internal acoustic meatus (1), bilaterally dilated lateral semicircular canals (1). The presence of progressive hearing loss was a significant predictor of abnormal CT outcome, while the severity of hearing loss was not. The CT scans offered valuable information regarding the aetiological diagnosis in all cases and, in addition, prompted the appropriate vestibular rehabilitation in three cases, further investigations in four (with dilated vestibular aqueduct) and hearing preservation counselling in two (bilateral DVA) (seven out of 35 = 20%). CONCLUSION: All children with unilateral sensorineural hearing loss should have a CT scan of the petrous pyramids/IAMs performed at some stage, as not only aetiology but also prognosis and management of these cases may be significantly influenced by the CT outcome.
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ranking = 1.9422299112319
keywords = duct, aqueduct
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4/213. Synchronous carcinoma of the gallbladder in a patient with intrahepatic bile duct carcinoma.

    An 83-year-old woman, diagnosed as having cholelithiasis, was admitted to the Department of Surgery, Nippon Medical School, with right hypochondrial pain. ultrasonography and computed tomography revealed a mass in the gallbladder fundus and a hypovascular tumor in the anterior segment of the liver. magnetic resonance imaging showed stenosis of the intrahepatic bile duct and dilatation of its proximal portion. She was diagnosed as having intrahepatic bile duct carcinoma combined with gallbladder carcinoma. At laparotomy, there was evidence of multiple peritoneal metastases and intraoperative histological examination of the gallbladder tumor revealed adenocarcinoma. Accordingly, only cholecystectomy and needle biopsy of the liver tumor was performed. Histological examination of the gallbladder revealed papillary adenocarcinoma invading the muscularis propria with medullary growth or intermediate stroma. There was no microvessel invasion, no perineural invasion and no lymph node involvement. On the other hand, the liver tumor was a cholangiocarcinoma with a well-differentiated tubular pattern. Therefore, this was a rare case of synchronous carcinoma of the gallbladder associated with intrahepatic bile duct carcinoma.
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5/213. Multiple gastrointestinal atresias with cystic dilatation of the biliary duct.

    A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.
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6/213. Maternally inherited diabetes and deafness (MIDD): unusual occult exocrine pancreatic manifestation in an affected German family.

    The mitochondrial (mt) 3243 dna mutation is an underlying cause of maternally inherited diabetes and deafness (MIDD) syndrome and the syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS). We report an affected German MIDD pedigree with maternal lineage over three generations. The index patient, her mother, her maternal aunt and her maternal grandmother all suffered from diabetes and premature hearing loss and were positive on testing for the mt 3243 dna mutation. The 27-year-old index patient had a history of grand mal seizures. As sequela of abdominal ultrasound and confirmed by magnetic resonance cholangio-pancreaticography, she was diagnosed with chronic pancreatitis with pancreatic calcifications and pancreatic duct dilation, although she was completely asymptomatic and with no signs of steatorrhoea. She did not have gallstones and the common bile duct was normal. A possible etiopathogenic pathway for pancreatitis could be a suppressive effect of the mt 3243 mutation on the oxidative phosphorylation in affected mitochondria. Although pancreatitis and pancreatic dysfunction in association with the mt 3243 mutation, especially in patients with comorbidity of MELAS and diabetes, has previously been described as a rare manifestation, this case is specific because of the discrepancy of advanced morphological pancreatic alterations and complete lack of pancreatogenic symptoms.
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7/213. Aqueduct stenosis due to venous ectasia with a dural arteriovenous fistula.

    We report aqueduct compression by venous ectasia in a 65-year-old man with a dural arteriovenous fistula in the posterior cranial fossa draining into a superior vermian vein. Conventional and phase-contrast MRI showed the aqueduct stenosis and the causative dilated vein.
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ranking = 2.6281532741546
keywords = duct, aqueduct
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8/213. Dilated bile duct in patients receiving narcotic substitution: an early report.

    Narcotic substitution is now widely used. morphine can induce a spasm of the sphincter of oddi but dilation of bile duct has been reported only in an anecdotal case. In June 1995, we observed a first case of dilation of the common bile duct without organic obstacle in a hepatitis c virus (HCV)-infected patient who was under narcotic substitution, suggesting a causal relationship. We conducted a prospective study to evaluate the precise prevalence of bile duct abnormalities related to narcotic substitution in active intravenous drug or ex-intravenous drug users referred to our liver unit for histologic evaluation of HCV infection. We conducted a prospective study in a 30-month period of 334 HCV-infected patients, including 36 receiving narcotic substitution with methadone or buprenorphine. biliary tract was analyzed by ultrasonography and by endoscopy ultrasound in cases of bile duct abnormalities. Of the 36 patients under narcotic substitution, 3 (8.3%) had asymptomatic dilated bile duct without organic obstacle--defined as a common bile duct > or =9 mm--compared to 1 of 298 (0.03%; p < 0.001) of those who did not receive substitution. Narcotic substitution may lead to bile duct dilation that does not require invasive diagnosis procedures.
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9/213. Late onset X-linked hydrocephalus with normal cerebrospinal fluid pressure.

    A family with X-linked hydrocephalus with normal cerebrospinal fluid (CSF) pressure and in which three brothers and a grandson of case 1, a proband, were affected is reported. The symptoms at onset were epileptic attacks that started in adulthood in the three brothers and at the age of 6 years in the grandson. In the three brothers, from 10 to 27 years after the onset of epileptic episodes, disorganization of intelligence and psychiatric deterioration were gradually noticed by their families. At the same time, they showed occasional urinary incontinence. brain computed tomography (CT) scans revealed dilatation of the ventricular systems. Based on the results of the measurement of CSF pressure and radioactive-iodinated human serum albumin (RISA)-cysternography, two of the brothers were diagnosed as having normal pressure hydrocephalus (NPH), and they were treated neurosurgically. However, no obvious improvement in clinical symptoms was observed. Although the grandson had shown normal psychomotor development during his early childhood, temporal epilepsy and temper tantrums started at the age of 6 years. Computed tomography-scanning revealed dilatation of the ventricular system similar to the other three cases at the age of 8 years. With the diagnosis of NPH, the patient underwent a shunt operation, which resulted in no obvious effects. As it is reasonable to surmise that the pathological gene would have been transferred via the daughter of the proband to the grandson, it is suggested that the inheritance manner might be X-linked recessive. The cases presented here are different from the cases of hydrocephalus due to stenosis of the aqueduct Sylvius (HSAS) and other types of X-linked hydrocephalus reported previously in terms of the age of onset, course, symptoms, and CT findings. Thus, it is suggested that the present cases might be a new type of X-linked hydrocephalus.
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ranking = 0.64740997041064
keywords = duct, aqueduct
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10/213. Medial indentation of the duodenal sweep by common bile duct dilatation.

    The dilated common bile duct has long been recognized as a cause for a smooth, tubular impression across the duodenal bulb or immediate postbulbar duodenum. Only scattered references suggest that a smooth indentation on the medial aspect of the descending duodenum might also be due to an enlarged, tortuous common duct. Three cases of this condition are reported. The dilated common duct impression can mimic a pancreatic mass. While computed tomography, ultrasonography, or transhepatic cholangiography readily suggest the true diagnosis, potential pitfalls in patient management are possible when the first radiographic procedure is an upper gastrointestinal series.
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