Cases reported "Dilatation, Pathologic"

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1/13. Idiopathic localized hydromyelia: dilatation of the central canal of the spinal cord of probable congenital origin.

    Three adult patients are reported with asymptomatic localized widening of the central canal of the spinal cord. These patients were followed for a period of 24 years by imaging and/or clinical history and physical examination without evidence of signs or symptoms related to the spinal cord. This condition probably represents persistence into adult life of a fetal configuration of the central canal of the spinal cord. This process may be termed "idiopathic localized hydromyelia" to distinguish it from syringomyelia secondary to such causes as Chiari malformation, trauma, infection, or neoplasm.
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2/13. Aortic root dilation in apparent Lujan-Fryns syndrome.

    We present a patient and his maternal uncle who have a subaortic ventricular septal defect and aortic root dilation. They both have physical anomalies, characteristic behaviors, and cognitive disabilities that are consistent with the diagnosis of Lujan-Fryns syndrome (LFS). Although there have been 4 cases reported in the literature with heart findings, ventricular septal defect and aortic root dilation have not been previously reported in LFS. Differentiation between LFS and marfan syndrome (MS) is discussed. The pathophysiology of LFS as a connective tissue disorder is also considered.
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3/13. Segmental dilatation of the ileum as an unusual cause of gastrointestinal bleeding: report of one case.

    We report a case of segmental dilatation of the ileum in a 10-month-old male infant. Intermittent loose black-colored stool passage and normocytic anemia were noted at the initial visits to our hospital. There was no symptom or sign of intestinal obstruction such as abdominal distention or vomiting. On physical examination, he was found to be pale but his abdomen was soft and flat. Digital examination revealed brownish stool tinged with black-colored oil-like stool but no polyp. Laboratory studies excluded coagulopathy, hemolytic anemia and lead poisoning. During hospitalization, he was treated with nothing per mouth, intravascular fluids, ranitidine, and transfusion of packed red blood cells. All examinations including panendoscopy, technetium-99m (99mTc)-pertechnetate Meckel's diverticulum scan, and double contrast colon series revealed no organic lesion except that 99mTc-red blood cell bleeding scans showed abnormal bleeding in the small intestine. Because of his persistent gastrointestinal bleeding with unknown cause, we did an exploratory laparotomy when the patient was 13 months old and idiopathic segmental dilatation of the ileum was confirmed. The dilated segment is supposed to be idiopathic because of histologically proven normal muscle layers without ectopic tissue. This case suggests that segmental dilatation of the ileum can only present as gastrointestinal bleeding without intestinal obstruction.
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4/13. Congenital complete heart block: fetal management protocol, review of the literature, and report of the smallest successful pacemaker implantation.

    Fetuses with complete heart block have an increased mortality with most deaths occurring in utero or during infancy. The cardiac evaluation of these fetuses is difficult since the ventricular rate is low and the heart is dilated. We have implemented a strategy that includes the biophysical profile, which assesses fetal well-being, in combination with the cardiovascular profile that assesses cardiac function and the circulation. We present two cases of fetal complete heart block in which early delivery was recommended due to worsening cardiovascular profile scores. Biophysical profile scores were normal. Both babies were successfully treated, despite having risk factors that predicted poor outcomes. We hypothesize that our management protocol initiated intervention before fetal compromise, hydrops, and myocardial damage occurred. We recommend an evaluation of heart function in addition to an assessment of fetal well-being in fetuses with complete heart block. Early delivery should be considered if there is evidence of distress and/or deteriorating cardiac function.
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5/13. Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum.

    A 34-year-old man was admitted to our hospital with the chief complaints of back pain and epigastralgia. The physical examinations on admission disclosed no abdominal tumor. The serum concentration of total bilirubin was 1.4 mg/dl. The serum elastase-1 level was elevated to 526 ng/dl. Computed tomography showed a cystic lesion, 1 cm in diameter, in the head of the pancreas, without dilatation of the main pancreatic duct. Endoscopic retrograde cholangiopancreatography via the papilla of Vater and the accessory papilla revealed an enlarged ventral pancreatic duct and pancreas divisum. The preoperative diagnosis was mucin-producing pancreatic tumor in the ventral pancreas of a patient with pancreas divisum. A pylorus-preserving pancreatoduodenectomy was performed. The gross findings of the cut surface of the resected specimen disclosed mural nodules in the dilated duct of the ventral pancreas. A histological examination of the mural nodules in the ventral pancreas revealed mucin and intraductal papillary adenoma. Benign tumors associated with pancreas divisum are rare; to the best of our knowledge, only three cases have been reported. Although in these three patients the tumor developed in the dorsal pancreas, the tumor developed in the ventral pancreas in our patient.
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6/13. Left ventricular apical ballooning--a novel cardiac disease mimicking acute coronary syndrome: a case report in a Caucasian patient.

    The syndrome of "apical ballooning" consists of an acute onset of transient extensive akinesia of the apical portion of the left ventricle, without significant stenosis on the coronary angiogram, accompanied by chest symptoms, ECG changes, and a limited release of cardiac markers disproportionate to the extent of akinesia. So far, the vast majority of cases with this syndrome have been reported from Japanese patients and only a few cases of Caucasian patients have been described. Emotional or physical stress or other preceding triggering factors might play a key role in this cardiomyopathy, but the precise etiology remains unknown. We describe a case of "apical ballooning" in a white patient, who presented at our institution with this novel heart syndrome. Despite severe initial presentation, conservative medical management leads to good long term outcome in most patients.
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7/13. Caroli's disease associated with liver cirrhosis. An autopsy case.

    Caroli's disease is one of the rare congenital conditions associated with the cystic dilatation of intrahepatic bile ducts. This is a case report of a 41-year-old Japanese male who complained of jaundice and general fatigue at the age of 34 for the first time. He was clinically diagnosed as having Caroli's disease by physical examination and image analyses study. The patient died after seven years and three months from the onset of the disease on account of renal function impairment. An autopsy was performed, revealing cystic dilatation of the intrahepatic bile duct, associated with a cirrhotic liver and also evidence of portal hypertension, substantiated by esophageal varices and splenomegaly. The liver weighed approximately 2,200 g. A histological investigation revealed typical morphological evidence of cirrhotic glomerulopathy and tubular degeneration with the presence of calcium casts in the dilated tubuli. The lung revealed diffuse alveolar damage with partial organization associated with remarkable polymorphonuclear and macrophagic infiltration. In this paper, the pathogenesis of the cirrhotic change, biliary duct abnormality and potential malignant transformation in the liver are discussed in relation to Caroli's disease.
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8/13. Idiopathic myocarditis characterized by marked right ventricular dilatation. Report of two autopsy cases.

    We encountered two children with chronic idiopathic myocarditis accompanied by marked right ventricular dilatation, who died of progressive right heart failure. A definitive diagnosis was made by histological examination of the myocardium at autopsy. The patients were both boys, aged 7 years and 1 year and 4 months, and a number of identical features were evident upon physical and laboratory examinations. No heart murmur was heard, and gallop rhythm was noted in distant heart sounds. Electrocardiogram revealed intraventricular block, low voltage QRS complex, and ST-T abnormality. Two-dimensional Doppler echocardiogram and right ventriculogram showed marked dilatation and decreased contractility of the right ventricle as well as tricuspid regurgitation. Thinning of the wall and marked dilatation of the right ventricle were confirmed at autopsy. Our observations showed that chronic myocarditis associated with tricuspid regurgitation may readily lead to marked right ventricular dilatation even exceeding the degree of left ventricular dilatation. Idiopathic myocarditis associated with such unusual features is relatively rare, and may present problems in differentiation from other congenital heart diseases causing dilatation and dysfunction of the right ventricle.
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9/13. Dilated episcleral arteries--a significant physical finding in assessment of patients with cerebrovascular insufficiency.

    Dilated episcleral vessels associated with ipsilateral internal carotid artery occlusions have been previously reported though not widely appreciated. These ocular changes have been presumed to be manifestations of ocular ischemia. The authors have recently encountered this sign in seven patients and in none was there evidence of ocular ischemia. In addition to an ipsilateral internal carotid artery occlusion, arteriograms demonstrated that the major source of blood supply to the homolateral cerebral hemisphere was by retrograde flow through markedly enlarged ophthalmic arteries filled in retrograde fashion from dilated external carotid collateral channels in the orbit. This association of dilated episcleral arteries as a sign of increased orbital blood flow and the major source of collateral blood supply to the homolateral cerebral hemisphere has not been previously reported. We reemphasize the importance of a careful examination of the episcleral vessels in patients suspected of having internal carotid artery occlusions.
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ranking = 0.35121389486264
keywords = physical
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10/13. Pseudo-obstruction of the colon. A postoperative complication in orthopaedic patients.

    Pseudo-obstruction of the colon is a problem of bowel motility that predominantly involves the large intestine and that can mimic the clinical and radiographic features of mechanical obstruction of the colon. It must not be confused with the much more common and less dangerous complication of post-operative ileus. Although it is most often seen in elderly patients who are confined to bed, it can occur in younger patients. Cecal dilation can develop rapidly to dangerous proportions, and recognition by thorough physical examination and early abdominal radiographs is essential if operative intervention is to be avoided. Nasogastric suction, intravenous fluids, and frequent turning of the patient are often successful if begun early, but are unlikely to be of benefit once cecal dilation approaches fourteen centimeters on plain radiographs. In these advanced cases, tube cecostomy or colostomy may be the only way to avoid necrosis of the bowel wall, perforation, and sepsis.
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