Cases reported "Constriction, Pathologic"

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1/319. Botulinum toxin: adjunctive treatment for posterior glottic synechiae.

    INTRODUCTION: Synechiae formation of the posterior glottis can result in tracheostomy dependence secondary to airway obstruction. Stenosis is caused by total or partial fixation of the vocal folds in adduction resulting from scar contracture. The treatment poses a management dilemma because of recurrent scar formation, made worse by mobility of the vocal folds. Although various treatment options from conservative endoscopic repair to open procedures have been proposed, the results are not satisfactory and patients often require multiple procedures. methods: We present the trial of a conservative approach that includes microscopic CO2 laser resection of the scar with concomitant botulinum toxin injection of the interarytenoid and thyroarytenoid muscles of the more mobile cord. This results in a temporary paresis of the adductor muscles and hence prevents overadduction in the posterior commissure during the postoperative healing period. STUDY DESIGN: We present the surgical technique and results in three patients who underwent the procedure. RESULTS: Treatment in all three patients was successful. CONCLUSIONS: The appropriate use of botulinum toxin may help improve the treatment outcome of posterior synechiae of the larynx without sacrificing any laryngeal components.
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2/319. The concurrence of ring constrictions in Adams-Oliver syndrome: additional evidence for vascular disruption as common pathogenetic mechanism.

    We report on a girl with congenital scalp and acral reduction limb defects, consistent with the diagnosis of Adams-Oliver syndrome. The presence of constriction rings makes the limb anomalies in this case similar to those seen in the amniotic band disruption sequence. Vascular disruption--with or without secondary amniotic rupture--may be responsible for the observed anomalies. Therefore we believe that the present observation adds further evidence for the hypothesis that the Adams-Oliver syndrome is a vascular disruption sequence.
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keywords = duct
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3/319. Autosomal recessive hydrocephalus due to congenital stenosis of the aqueduct of sylvius.

    Isolated hydrocephalus due to congenital stenosis of the aqueduct of Sylvius is almost always an X-linked recessive inherited condition. We describe a brother and sister with isolated hydrocephalus from congenital aqueductal stenosis. We believe that these two occurrences represent a rare autosomal recessive form of this abnormality. In assessing a first known occurrence of hydrocephalus with stenosis of the aqueduct of Sylvius in a family, the rare possibility of autosomal inheritance must be considered in genetic counselling.
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ranking = 41.494122519381
keywords = aqueduct, duct
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4/319. hepatopulmonary syndrome in inferior vena cava obstruction responding to cavoplasty.

    Reports show that hepatopulmonary syndrome mostly occurs in the setting of advanced hepatic dysfunction, with the associated vasoactive substance imbalance believed to be responsible for its pathogenesis. However, hepatopulmonary syndrome has also been reported in cases of mild hepatic dysfunction or noncirrhotic portal hypertension, indicating that portal hypertension also plays a part in the pathogenesis. liver transplantation remains the only therapeutic option of proven benefit. We describe 2 cases of hepatopulmonary syndrome in the setting of inferior vena cava (suprahepatic) obstruction, but with minimal hepatic dysfunction. After balloon cavoplasty, 1 patient showed, in addition to improvement of the features of hepatic outflow obstruction, significant reduction of dyspnea, cyanosis, and hypoxemia with arterial blood gas normalization within 2 weeks and intrapulmonary shunt reversal within 8 weeks. This implies that hemodynamic alterations (such as portal hypertension) independently contribute to the pathogenesis of hepatopulmonary syndrome in at least some of the cases. Therapies aimed at correcting these abnormal hemodynamics may be important in the treatment of this condition, especially when the hepatic functional status by itself does not warrant a liver transplant.
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keywords = duct
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5/319. subacute sclerosing panencephalitis in a case of aqueductal stenosis.

    A 14-year-old boy presented with history of deterioration of scholastic performance, altered behaviour, difficulty in walking and repeated falls since last 2-3 months. He had history of delayed milestones and at 11 months developed febrile rashes. On investigations, MRI showed compensated hydrocephalus with aqueductal stenosis. EEG suggested subacute sclerosing panencephalitis (SSPE). CSF was positive for measles antibody titre. This case of aqueductal stenosis with SSPE is very interesting for its diagnostic dilemma.
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ranking = 35.566390730898
keywords = aqueduct, duct
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6/319. Radiologic findings of mirizzi syndrome with emphasis on MRI.

    We have reported a case of mirizzi syndrome preoperatively diagnosed using MR cholangiopancreatography. MRCP and T2-weighted image using a single-shot fast spin-echo sequence accurately depicted all components of mirizzi syndrome, including impacted stone in the neck of the gallbladder compressing the common hepatic duct and wall-thickening of the gallbladder without any evidence of malignancy. The combination of MRCP and T2-weighted image can be counted on to replace conventional modalities of diagnosing mirizzi syndrome without any loss of diagnostic accuracy.
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keywords = duct
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7/319. A case of nephrogenic diabetes insipidus caused by obstructive uropathy due to prostate cancer.

    Nephrogenic diabetes insipidus (DI) secondary to chronic urinary tract obstruction is a rare disease. The exact cause is unknown but it is likely that increased collecting duct pressures cause damage to the tubular epithelium, resulting in insensitivity to the action of arginine-vasopressin (AVP). A 77-year-old man complaining of polyuria and polydipsia was treated with alpha glucosidase inhibitor under the impression of polyuria due to diabetes mellitus. But his symptoms did not improve. water deprivation and AVP administration study revealed that the patient had nephrogenic DI. urinary tract obstruction due to an enlarged prostate was suggested as a principal cause of nephrogenic DI. The patient underwent transurethral resection of the prostate and bilateral subcapsular orchiectomy. After surgery, the urine osmolarity was normalized and the patient became symptom-free. We report a case of nephrogenic DI due to obstructive uropathy which was cured by surgery eliminating obstruction.
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ranking = 0.33333333333333
keywords = duct
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8/319. Aqueductal stenosis and hydrocephalus in an infant due to aspergillus infection.

    Aqueductal stenosis is a common cause of hydrocephalus during infancy. We report on an infant born with aplasia cutis congenita at the scalp vertex and hypoplastic left heart syndrome developing systemic aspergillosis after cardiac surgery. The infant died at the age of 76 days despite systemic antimycotic therapy with a combination of flucytosine and amphotericin b. Therapy started at post-operative day 17 and was also applied intrathecally. Post-mortem examination revealed meningitis, multiple brain aspergillomas and microabscesses with focal ependymitis, focal bronchopneumonia, and necrotizing enterocolitis. One of the brain aspergillomas was located close to the aqueduct causing an aqueductal stenosis and an obstructive hydrocephalus. Histologically, aspergillus hyphae could only be detected in the aspergilloma of the aqueduct. To the best of our knowledge, this is the first reported case of an aqueductal stenosis caused by an aspergilloma.
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ranking = 25.377593820598
keywords = aqueduct, duct
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9/319. granular cell tumor at the hepatic duct confluence mimicking Klatskin tumor. A report of two cases and a review of the literature.

    BACKGROUND: Granular cell tumors are rare tumors most often located in the oral cavity, skin or subcutaneous tissue. The occurrence of this tumor in the biliary tree is extremely rare. methods: Two patients are described presenting with biliary obstruction due to a tumor at the hepatic duct confluence. One patient is a 38-year-old white male with concomitant cutaneous granular cell tumors, and the other a 50-year-old white female. RESULTS: Hilar excision was performed in both patients. Histopathology of the tumors revealed a proliferation of cells with granular cytoplasm, diagnosed as granular cell tumor. CONCLUSION: At preoperative examination, hilar granular cell tumors are difficult to differentiate from cholangiocarcinoma, sclerosing cholangitis or more common benign biliary tumors. Treatment consists of surgical excision after which prognosis is favorable.
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ranking = 1.6666666666667
keywords = duct
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10/319. 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 = 13.188796910299
keywords = aqueduct, duct
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