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Management of the Parturient with Osler-Weber-Rendu
Abstract Number: T4C-6
Abstract Type: Case Report/Case Series
Introduction: Osler-Weber-Rendu syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant disorder characterized by arteriovenous malformations (AVMs) that develop in the skin, mucosa, and internal organs. This is a common genetic disorder that affects 1 in 5-8,000 people.1 Epistaxis is usually the presenting symptom. The penetrance is age-dependent and manifestations progress over time. Vascular malformations may affect the brain, lungs, liver, spinal cord and gastrointestinal tract.2 Pulmonary AVMs are likely to enlarge during pregnancy and may cause fatal hemorrhage.3
Case: A 30 y/o G5P4 Hispanic woman presented at term for an anesthesia consult prior to delivery. She had four prior uneventful spontaneous vaginal deliveries with labor epidurals. It had been 4 years since her last delivery. She had experienced epistaxis since youth and developed telangiectasias at her fingertips in her teens. Her symptoms had progressed since her last pregnancy with telangiectasias in her mouth and bleeding gums. The obstetrician ordered a chest CT to rule out pulmonary AVMs and a brain MRI to rule out cerebral AVMs when she presented for her consult. Both were negative. A lumbar MRI was ordered to assess for spinal AVMs which was also negative. She delivered vaginally with an epidural and had a postpartum tubal ligation two days later under spinal. Her hospital course was uneventful.
Discussion: Pregnancy in women with Osler-Weber-Rendu is high risk. Most of these pregnancies proceed normally; however, morbidity and mortality is increased due to potential pulmonary AVM hemorrhage, strokes and myocardial infarction. Recommendations for management of patients include pre-pregnancy screening and treatment for pulmonary AVMs and hospital admission for those parturients experiencing hemoptysis/sudden dyspnea. No pulmonary AVM screening is recommended for asymptomatic parturients. For patients with a family history of cerebral hemorrhage or cerebral symptoms a brain MRI is recommended. Also, exclusion of spinal AVMs by MRI during pregnancy should be done to allow for regional anesthesia. Other recommendations include antibiotic prophylaxis during delivery, avoiding prolonged second stage of labor (if cerebral AVM not excluded) and modified induction of general anesthesia to prevent hypertension with direct laryngoscopy.4
1. Chung MG. Hereditary hemorrhagic telangiectasia. Hands Clin Neurol. 2015;132:185-197.
2. Bari O, Cohen PR. Hereditary hemorrhagic telangiectasia and pregnancy: potential adverse events and pregnancy outcomes. Int J Womens Health. 2017;9:373-378.
3. Begbie ME,et al. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgrad Med J. 2003;79:18-24.
4. Shovlin CL,et al. Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services. BJOG. 2008;115:1108-1115.