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1/5. The use of a nasal obturator in hereditary haemorrhagic telangiectasia: an alternative to Young's procedure.

    epistaxis associated with hereditary haemorrhagic telangiectasia (HHT) is a challenging condition. Septodermoplasty, electrocautery and laser treatment often provide short-term relief only. The surgical closure of the nostrils ('Young's procedure') represents a longer term solution but has the disadvantage of causing permanent complete nasal obstruction. A Silastic obturator has the advantage of being a non-surgical intervention and allows temporary relief of the nasal obstruction.We present the cases of three patientswith HHT who used the obturator with good clinical effect, suggesting that this is a realistic non-surgical alternative to Young's procedure for patients with HHT.
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2/5. Bleeding gastric telangiectasia. Complication of Raynaud's phenomenon, esophageal motor dysfunction, sclerodactyly and telangiectasia (rest) syndrome.

    anemia and skin telangiectasia were the main presenting features of a patient with Raynaud's phenomenon, esophageal motor dysfunction, sclerodactyly and telangiectasia (rest) syndrome. Diffuse gastric telangiectasia with chronic intermittent blood loss were responsible for the anemia. The multisystemic and progressive character of this collagenopathy in our patient is described and the endoscopic picture is presented. Management difficulties and treatment alternatives are discussed.
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3/5. Surgical management of life-threatening epistaxis in Osler-Weber-Rendu disease.

    Hereditary hemorrhagic telangiectasia is a systemic disorder of blood vessels. It often leads to severe epistaxis that is refractory to conventional therapy. Three patients with severe epistaxis caused by hereditary hemorrhagic telangiectasia unresponsive to laser coagulation and to septal dermoplasty and requiring multiple transfusions underwent extirpation of nasal mucosa through a rhinotomy approach, with a median forehead flap and split-thickness skin graft coverage of the mucosal defects over the floor and lateral walls of the nose. All patients had reduced frequency and severity of bleeding without any need for transfusions during follow-up periods of 6 months, 3 years, and 5 years, respectively. forehead flaps resulted in minimal morbidity. Partial nasal obstruction and a forehead scar were the main complaints. Surgical technique, complications, and alternative therapy are discussed.
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4/5. Endometrial ablation for severe menorrhagia in a patient with hereditary hemorrhagic Telangiectasia. A case report.

    BACKGROUND: Hereditary hemorrhagic telangiectasia is a rare, inherited disease characterized by abnormal visceral and superficial blood vessel anastomoses. These telangiectasias predispose the patient to a lifelong history of recurrent bleeding for the nasal and gastrointestinal mucosa. Less commonly involved organs include the liver, brain and lung. To date there is no cure for this disease. Management requires many palliative minor surgical procedures to stop actively bleeding sites. Major surgery is often contraindicated in these patients due do coexisting medical sequelae of their underlying disease. CASE: menorrhagia was diagnosed in a 42 year-old multipara with known history of hereditary hemorrhagic telangiectasia. The bleeding was unresponsive to hormonal therapy. Substantial preexisting conditions, including profound anemia, history of multiple strokes, a seizure disorder and ventricular arrhythmias, precluded major surgical intervention, including hysterectomy. Serial injections of leuprolide acetate injections were followed by hysteroscopic "rollerball" electrocoagulation of the endometrium under regional anesthesia. On long-term follow up, the patient was cured of her menorrhagia. CONCLUSION: Endometrial ablation provides patients who have significant medical complications with an effective, minimally invasive alternative to hysterectomy for control of menorrhagia.
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5/5. liver transplantation resolves the hyperdynamic circulation in hereditary hemorrhagic telangiectasia with hepatic involvement.

    BACKGROUND & AIMS: Hepatic involvement in hereditary hemorrhagic telangiectasia is common but often asymptomatic. However, in some cases, the vascular lesions that involve the liver may lead to high-output cardiac failure and pulmonary hypertension that is predominant over hepatobiliary manifestations. liver transplantation and treatment of these complications are described and discussed in this article. methods: Three patients with hereditary hemorrhagic telangiectasia and hepatic involvement received transplants. They had pulmonary hypertension and chronic right-sided heart failure caused by disseminated intrahepatic telangiectasias with shunts between the hepatic artery and hepatic veins or portal vein. Left-to-right intrahepatic shunt output was estimated to range between 51% and 57.5% of cardiac output. RESULTS: Hyperdynamic circulation disappeared after liver transplantation in all patients. Results of computed tomography and right-sided heart catheterization performed 6 months later were normal. Follow-up periods currently are 65, 53, and 29 months, and each patient continues to be asymptomatic. CONCLUSIONS: This report suggests that liver transplantation can be considered as an alternative and successful curative treatment that may prevent the irreversible evolution of cardiopulmonary disease.
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