Hereditary Hemorrhagic Telangiectasia Case Study

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Background
Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia (HHT), is an autosomal dominant vascular dysplasia1, 2, 3. It is manifested by mucocutaneous telangiectases and arteriovenous malformations (AVM). Lesions can affect the nasopharynx, central nervous system, lungs, liver, gastrointestinal tract and conjunctiva. Recurrent epistaxis is the most common presentation. Diagnosis of HHT is made clinically on the basis of the Curaçao criteria: epistaxis, telangiectasia, visceral lesions and family history1. The prognosis varies, depending on the severity of symptoms. Treatment is focused on prevention of complications and supportive measures.1, 4, 5

Aims
The authors want to emphasize that this uncommon disease requires a carefully anaesthetic and surgical planning to guarantee the success of the procedure. We enhance regional anesthesia as a good option in some cases.

Case report
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Positive airway ventilation can exarcerbate shunting, so spontaneous ventilation should be maintained whenever possible.5
Patients with cerebral AVM require a careful hemodynamic management and increases in intracranial/systolic blood pressures must be avoided (especially during intubation/extubation).5
Patients with systemic AVM can have left-to-right shunting and a decreased systemic vascular resistance so anaesthetic induction should be careful and fluid status should be optimized before intervention. Their response to hypotension may be unpredictable and sometimes they do not respond to vasoconstrictor drugs.5
These patients are at a higher risk for endocarditis due to pulmonary AVM so prophylactic antibiotic is recommended before surgical or dental procedures.5
To prevent emboli formation filters should be placed in intravenous lines.5
Patients should be in a deep anaesthesia state at the extubation to avoid an increase in intracranial/systolic blood

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