HHT is manifested by mucocutaneous telangiectases and arteriovenous malformations AVMs in different parts of body. Patient received tranexamic acid 1 gm slow I. Maintenance of anesthesia was done with sevoflurane, nitrous oxide and oxygen, and atracurium as a muscle relaxant. Investigation revealed Hb 6. Visit for more related articles at Archives of Medicine.
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In Rendu [ 9 ] first noted the association between hereditary epistaxis and telangiectasia in a 52 years old man. YAG laser [ 52 ]. Patient was reversed by using standard reversal techniques with neostigmine and glycopyrollate. Location Lesions Indications of management Management Nose Telangectasia, AVMs Recurrent epistaxis Sclerotherapy with sodium tetradecyl sulphate[ 46 ], submucosal radiofrequency [ 47 ], Bevacizumab [ 48 ], Septal mucosal dermoplasty [ 3949 ], Embolisation of external carotid artery branches[ 50 ], Skin Telangiectasia Pain [ 2 ], cosmesis[ 4 ] Cauterization, hypertonic saline sclerotherapy, dye laser treatment [ 51 ].
Maintenance of anesthesia was done with sevoflurane, nitrous oxide and oxygen, and atracurium as a muscle relaxant.
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Sudden change in blood pressure may cause bleeding from AVMs anywhere in the body, most serious of which is from cerebral AVM. All Published work is licensed under a Creative Commons Attribution 4. Histopathology of HHT lesions show many layers of smooth muscle cells without elastic fibers and very frequently arterioles directly communicating with smooth muscle cells.
Patients with HHT presenting with continuous bleeding pose a serious problem to the anaesthesiologists. Patients with pulmonary involvement are at high risk of developing cerebral thrombotic and embolic events including stroke, brain abscess, or transient ischemic attacks due to right-to-left shunting [ 1424 ].
Management include blood transfusion, antifibrinolytics and surgical hemostasis. Sclerotherapy with sodium tetradecyl sulphate[ 46 ], submucosal radiofrequency [ 47 ], Bevacizumab [ 48 ], Septal mucosal dermoplasty [ 3949 ], Embolisation of external carotid artery branches[ 50 ].
Intravenous access may be difficult. HHT is a rare systemic fibro vascular dysplasia [ 6 ] with incidence varying from 1 in 5, to 10, [ 7 ] to 1 to 2 in 1,00, [ 6 ]. Introduction Osler-Weber-Rendu disease OWRD or Hereditary Hemorrhagic Telangiectasia HHT is a rare autosomal dominant disorder that causes muco-cutanesous and visceral vascular dysplasia and results in increased tendency for bleeding [ 1 – 4 ].
Endoscopic view of the angiofibroma.
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The 4 clinical diagnostic criteria are as follows:. He was stabilised with ml hydroxyl-ethyl starch till blood products were made available, followed by 3 unit packed cell and 4 unit FFP transfusion. Intraoperatively controlled hypotension should be used to reduce bleeding. Gastric distension may occur from ingested blood and may cause reflux and aspiration during induction.
Epistaxis is the most common symptom of HHT and mucocutaneous telangiectasia manczia most common sign [ 5 ]. V infusion to control the bleeding, glycopyrrolate to reduce secretions, pantoprazole as antiulcer prophylaxis and Ondansetron to reduce reflux.
As a result telangiectases are very sensitive to slight trauma and friction.
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Anesthesia strategy should include rapid sequence induction and controlled hypotension. A urinary catheter was inserted and orogastric tube aspiration was done.
Osler [ 10 ] in and Weber [ 11 ] in further elaborated the association between hemorrhagic lesions in skin and mucous membranes and its familial inheritance. Antifibrinolytics including tranexamic acid [ 3435 ] and aminocaproic acid [ 36 ] have been used with success skni control epistaxis.
In unstable patient presenting with severe bleeding focus should be jandzia to simultaneous resuscitation and hemostasis.
Pre-existing anemia due to recurrent bleeding is common and sudden decompensation may lead to heart failure.
Patients with HHT may present with variety of symptoms and management differs accordingly. Intraoperatively controlled hypotension was induced with sevoflurane and propofol boluses to maintain systolic blood pressure between 90 to mmHg. Uncontrolled bleeding may occur from skin lesions during patient positioning and transport. Lesions can affect the nasopharynx, central nervous system CNSlung, liver, and spleen, as well as the urinary tract, gastrointestinal GI tract, conjunctiva, trunk, arms, and fingers [ 213 ].
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He had been diagnosed previously as HHT due to presence of vascular malformation in the nose and paranasal sinuses by endoscopy and stomach by upper GI skkn.
Cauterization, hypertonic saline sclerotherapy, dye laser treatment [ 51 ]. Classic triad of presentation include telangiectases of the skin and mucous membranes, epistaxis, and a positive family history. As the bleeding occurs from malformed vessels standard coagulation tests will reveal no abnormality.