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Anesthetic management dilemma in a pregnant patient with hereditary hemorrhagic telangiectasia
Abstract Number: F-63
Abstract Type: Case Report/Case Series
Introduction: We report a case of a pregnant patient with Hereditary Hemorrhagic Telangiectasia (HHT) also known as Rendu-Osler-Weber syndrome presenting for delivery. HHT is characterized by recurrent epistaxis, mucocutaneous telangiectasias and arteriovenous malformations (AVMs) most commonly affecting the central nervous system, the gastrointestinal and respiratory system . HHT can increase the risk of serious complications during pregnancy . Case presentation: A 31-year-old female, pregnant at 32 weeks was admitted for medical optimization due fetal intrauterine growth restriction. Past medical history included morbid obesity (BMI 49), HHT, diabetes mellitus type 2, asthma, chronic back pain with prescription opioid dependence. On airway exam she had a Mallampati classification III. Due to pregnancy and HHT she had a higher risk of bleeding with airway manipulation. A magnetic resonance imaging study was suggested to the patient to identify potential cerebral, spinal or pulmonary AVMs, however the patient refused. That left us with the dilemma of doing a spinal anesthetic risking rupture of an undiagnosed spinal AVM or perform general anesthesia in a patient with a potentially difficult airway. We elected epidural anesthesia due to the low estimated incidence, about 1%, of spinal AVMs in this population  and due to the fact that our patient had a non-reassuring airway. Delivery was uneventful she was discharged on post-op day 2. Discussion: HHT can complicate pregnancy and delivery and can pose a management dilemma for both the obstetric team and the anesthesiologist. Although theoretically a lot of catastrophic complications can happen including intracerebral bleeding, hemothorax, massive hemoptysis, heart failure and paralysis after puncture of spinal AVM, most women with HHT have an uneventful pregnancy and delivery . In one of the few studies examining the outcomes of pregnancy in women with HHT, the most common delivery complication was uterine hemorrhage . There is limited data regarding the optimal management of these patients; therefore, the obstetric and anesthetic plan should be tailored on a case-by-case basis taking into consideration the risks of AVMs in the brain, lungs and spine.
References: 1. Govani, F.S. and C.L. Shovlin, Hereditary haemorrhagic telangiectasia: a clinical and scientific review. Eur J Hum Genet, 2009. 17(7): p. 860-71. 2. de Gussem, E.M., et al., Outcomes of pregnancy in women with hereditary hemorrhagic telangiectasia. Obstet Gynecol, 2014. 123(3): p. 514-20. 3. Faughnan, M.E., et al., International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia. J Med Genet, 2011. 48(2): p. 73-87. 4. Lomax, S. and H. Edgcombe, Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia. Can J Anaesth, 2009. 56(5): p. 374-84.