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Evidence-based Practice and Quality Improvement

Hereditary hemorragic telangiectasia (HHT) or Osler-Weber-Rendu syndrome - a case report

1AP8-10

Santa, Barbara R.; Alves, J.; Araujo, R.; Freitas, S.; Paulino, A.; Vicente, F.

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European Journal of Anaesthesiology (EJA): June 2013 - Volume 30 - Issue - p 30-30
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Background: HHT is a rare autosomal-dominantly inherited vascular dysplasia that occurs in approximately 1-2/100.000 people, characterized by 3 of the following criteria: family history, recurrent epistaxis, mucocutaneous telangiectases and arteriovenous malformations (AVMs) in gastrointestinal tract, liver, lungs and central nervous system1. We report a successful clinical case of a patient with HHT and gastric angiodysplasia, submitted to a Total Gastrectomy and Roux-en-Y Esophagojejunostomy.

Case report: 48-year-old female, ASA II, family history of HHT, recurrent epistaxis, oral telangiectases, cerebral abscess medically treated 11years before and several gastric hemorrhagic episodes due to angiodysplasia. Pulmonary and cerebral AVMs were excluded preoperatively. Hemoglobin10,1g/dL; Platelets147x10 9/L. Balanced general anesthesia, with rapid sequence induction and orotracheal intubation with a number 7 tube was provided, with standard monitoring, laboratory evaluations, BIS and invasive arterial blood pressure. Filters applied in intravenous lines. Antibiotic prophylaxis administered. Volume controlled ventilation adapted for peak pressures around 15mmHg. The patient remained hemodynamically stable, with estimated blood losses of 500mL. 1U of RBC, 2U of FFP and platelets pool were administered. Analgesia and nausea and vomiting prophylaxis provided. Extubated after surgery and minimized aspiration. No postoperative complications were documented.

Discussion: Surgery is indicated in HHT with visceral AVMs. Airway protection, low pressure ventilation and early extubation in order to avoid exacerbation of intra-pulmonary shunt are some of the anesthetics specific considerations. No hemodynamic instability was observed, which may occur due to low systemic vascular resistance and unpredictable response to vasodilators/ pressors. Blood losses were the expectable, with no evidence of AVMs rupture or coagulopathy. There were no postoperative complications or signs of thromboembolism or infection, that can be favored due to lack of capillary matrix.1

References:

1. Peiffer KMZ,et al.AANA Journal 2009;77(2),115-118

Learning points: Being aware of the challenges presented in HHT patients is the first step to provide a safe and effective anesthesic technique. This involves specific interventions with regard to control of bleeding, maintaining adequate oxygenation and balancing hemodynamic values to optimize perfusion without compromising anesthesic death.

© 2013 European Society of Anaesthesiology