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Hereditary Hemorrhagic Telangiectasia Type I in Pregnancy
Abstract Number: RF2BB-160
Abstract Type: Case Report Case Series
Hereditary Hemorrhagic Telangiectasia Type I (HHT) is an autosomal dominant vascular disorder, which usually manifests as epistaxis, GI bleeding, pulmonary arteriovenous malformations (AVMs), and mucocutaneous telangiectasias. Serious complications such as paradoxical emboli through the right to left shunt created by pulmonary AVMs, hemorrhage and high output cardiac failure can develop in these patients. Prevalence is estimated at 1:5000 to 1:8000.
Our patient was a 27 year old G3P0 with a history of HHT and pulmonary AVMs which were coiled/ablated in 2003, 2009 and 2013. A CT scan in 2015, however, showed persistence of multiple pulmonary AVMs. She had significant hypoxia with a baseline oxygen saturation of 86% and an exertional saturation as low as 77%. The Maternal Fetal Medicine team wanted to deliver her by cesarean section (CS) at 37 weeks due to her worsening dyspnea and hypoxia. An MRI during pregnancy did not show any spinal AVMs or lumbar abnormalities, but did show vertebral body hemangiomas at T2, T6, T8, and T11. The patient underwent CS at 37 weeks with spinal anesthesia using 1.8mL of 0.75% PF bupivacaine with 15mcg fentanyl and 0.15mg morphine without incident. She was maintained on 3L by nasal cannula throughout the procedure and saturations ranged from 83-96%. She recovered well and was discharged home on POD#3.
While the safety of neuraxial anesthesia in patients with HHT is unclear, it is especially precarious in patients with spinal pathology. This patient was particularly challenging because not only was she at a high risk of neuraxial bleeding, she was also at a high risk of pulmonary complications. Although it was reassuring the spinal hemangiomas were in the vertebral bodies remote from the area of spinal placement and no large spinal AVMs were noted, it was unclear whether some smaller, abnormal vasculature may be present in the lumbar spine. The major concern was spinal bleeding, but this patient was also at increased risk of paradoxical embolism from her right to left shunting, and worsening hypoxemia due to increasing metabolic demands during pregnancy. This hypoxemia would not be amenable to supplemental oxygen due to her shunt pathology.
Even though no anesthetic was ideal, after multidisciplinary discussion, it was decided that a spinal would impart the lowest risk of complications. There is no evidence that the sympathectomy that occurs with a spinal would increase flow through a pulmonary AVM and result in further desaturation. In addition, a spinal had a lower risk of neuraxial bleeding because of the smaller needle size as compared to an epidural, and avoiding general anesthesia would be highly advantageous because of the high risk of prolonged post-operative intubation and pulmonary complications. It was believed the chances of the CS lasting longer than the duration of the spinal was unlikely and not worth the increased risk of an epidural.