Anaesthetic management of Osler–Weber–Rendu syndrome with coexisting congenital methaemoglobinaemia

Abstract

A 9-year-old cyanosed child suffering from Osler-Weber-Rendu syndrome with bilateral pulmonary arteriovenous malformations (PAVMs) was posted for cerebral angiography under general anaesthesia. Careful preanaesthetic evaluation led to the diagnosis of coexisting congenital methaemoglobinaemia. There is no previous report of Osler-Weber-Rendu syndrome coexisting with congenital methaemoglobinaemia. This report emphasizes that a second contributory cause of cyanosis must be suspected and meticulously looked for if the symptomatology in a patient cannot be explained by a single established diagnosis. Positive-pressure ventilation was associated with reduction in arterial oxygenation despite an increasing inspired oxygen concentration, which returned to preanaesthetic levels only after extubation and resumption of spontaneous respiration.

Anaesthetic management of parturients with hereditary haemorrhagic telangiectasia for caesarean section

“…Positive pressure ventilation may cause worsening of arterial oxygenation in patients with pulmonary AVMs. 45 Moreover, the stress response during induction of anaesthesia might contribute to increased blood flow to this abnormal vasculature with increased risk of rupture and haemorrhage. Intravenous injections pose the risk of air or drug precipitation acting as emboli that may traverse the shunt and enter the systemic circulation.…”

Fatal Systemic Embolism following Revision of Hip Arthroplasty: A Complication of Pulmonary Arteriovenous Malformation

“…13 However, positive pressure ventilation is thought to worsen oxygenation. 5,14 Discussion among the team relating to this case included the matter of prevention and whether or not the operation should have proceeded. Factors that were considered included whether the patient should have had a CTPA to assess the PAVM, as they are known to increase in size with increasing age, 7,8 and whether she would therefore have needed a work-up for embolisation of the PAVM.…”

Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

“…Indeed, a (non-pregnant) patient with known bilateral PAVMs and HHT underwent general anesthesia with worsening hypoxemia, which was attributed to either an increase in pulmonary vascular resistance due to positive pressure ventilation or possibly to a decrease in cardiac output (also as a result of positive pressure ventilation) leading to mixed venous desaturation and subsequent arterial desaturation. 46 Epidural analgesia has been used in labor and subsequent anesthesia for tubal ligation in a patient with HHT, PAVMs, and mitral regurgitation. 22 Epidural management was chosen primarily because of the valvular heart disease, but the authors speculated that it may have reduced surges in cardiac output and consequent distension of the PAVMs.…”