Osler-Weber-Rendu syndrome: an anaesthetic challenge?

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.