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Neuraxial Analgesia for Labor and Delivery of Parturient with Osler-Weber-Rendu Syndrome
Abstract Number: SU-90
Abstract Type: Case Report/Case Series
Hereditary Hemorrhagic telangiectasia (HHT) or Osler-Weber-Rendu Syndrome is a rare, autosomal dominant disease, characterized by multisystem Arterio Venous Malformations (AVMs) and bleeding. Physiologic and hormonal changes of pregnancy in the presence of multisystem AVMs may increase maternal morbidity and mortality. We describe a case in which the risk of spinal AVMs present unique challenges and anesthetic considerations for neuraxial placement in an obstetric patient with HHT (1, 2).
A 28 year old G2P1 with a history of HHT presented at an estimated gestational age of 38 weeks in spontaneous labor. The patient was diagnosed with HHT at the age of 8y and has a family history of HHT. Prior to pregnancy, she had intracranial AVMs treated with gamma knife radiosurgery. Patient had a normal Echocardiogram and MRI (MRA and MRV) of chest during current pregnancy. She did not have any imaging studies of her spine. Upon request and evaluation, an epidural catheter was placed uneventfully. Analgesia was obtained with a continuous epidural infusion of 0.125% Bupivacaine and 2mcg/ml fentanyl titrated to provide a bilateral T10 sensory blockade without significant motor blockade. The patient had an uncomplicated vaginal delivery. Neurochecks were performed throughout labor and continued for 12 hours after epidural catheter removal.
The incidence of spinal AVMs in HHT is low but hormonal changes in pregnancy can further weaken abnormal small vasculature. Therefore, neuraxial technique in an obstetric patient with HHT presents concern for epidural and spinal hematoma with resulting spinal cord compression and potentially long-term neurological sequelae. It is prudent to screen for epidural and spinal AVMs prior to neuraxial, however there is no evidence to support mandatory screening. When the presence or absence of epidural or spinal AVMs is unknown, as in our case report, we recommend the following:
1. Avoidance of medications that can impair coagulation such as NSAIDs.
2. Low concentration local anesthetic to minimizing motor blockade while providing adequate analgesia.
3. Careful monitoring for neurologic deficits after neuraxial placement into the postpartum period after the epidural catheter has been removed.
1. de Gussem, Els M. MD et. al., ‘Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia’. Obstet Gynecol: 2014 Mar;123(3):514-20
2. Edgcombe, Hilary FRCA ,Lomax, Suzi FRCA, ‘Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia’. Can J Anesth, 2009, 56:374–384
3. Begbie M, Wallace G, Shovlin C. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): a view from the 21st century. Postgraduate Medical Journal. 2003;79(927):18-24. doi:10.1136/pmj.79.927.18