Osler-Weber-Rendu syndrome: an anaesthetic challenge?

Abstract

Osler-Weber-Rendu syndrome, or hereditary haemorrhagic telangiectasia (HHT), is an autosomal dominant vascular dysplasia characterised by mucocutaneous telangiectases and arteriovenous malformations (AVMs). Diagnosis is clinical and treatment is supportive. The authors demonstrate a safe anaesthetic approach for a patient with HHT. A 53-year-old woman with a left trochanteric fracture was scheduled for urgent orthopaedic surgery. She was diagnosed as having HHT and presented with recurrent epistaxis, telangiectases and gastrointestinal AVMs. She had undergone a nasal dermoseptoplasty 4 weeks earlier, with total nasal occlusion. Surgery was undertaken with a lumbar and sacral plexus block performed with neurostimulation. She was sedated and spontaneous ventilation was maintained. The procedure was completed without complications. Anaesthetic management of patients with HHT is a challenge. The patients should be carefully studied before surgery, to reduce perioperative morbidity and mortality. Regional anaesthesia may be a good alternative to general anaesthesia, but the use of neuroaxial techniques is still controversial.

Hypoxemia without Respiratory Distress: Hereditary Hemorrhagic Telangiectasia in a Child

“…4 Because of its unique pathophysiology, when an asymptomatic patient with HHT undergoes a surgical procedure with anesthesia, care is taken to reduce the risk of air embolization, rupture of possible AVMs, endocarditis, and death during the procedure. [7][8][9][10] Although inheritance is autosomal dominant, first-degree relatives are often unaware of their diagnosis because the clinical findings can be subtle and seen in the general population. 2 This may be especially true in pediatric patients, as the development of clinical signs progresses with age, and the disease may not have manifested itself.…”