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Complex Delivery of a Parturient with Von Hipple Lindau Disease and Pheochromocytoma
Abstract Number: FCA-540
Abstract Type: Case Report Case Series
A 27yo G3P0020 with PMH of Von-Hipple Lindau (VHL) and Chiari malformation had prenatal care transferred to Grady Hospital at 34wks gestation for delivery planning of this complex patient. The patient has a strong family history of VHL disease and was officially diagnosed in 2016 with both VHL and Chiari malformation. On presentation to our maternal fetal medicine (MFM) clinic she expressed symptoms of palpitations with heart rate range of 100-170 bpm, hypertension, and occasional dizziness. Due to the nature of VHL disease, a MRI Brain-Abdomen-Thorax-Lumbar Spine was performed to evaluate the extent of her Chiari malformation and to rule out potential vascular hemangiomas in her lumbar region. Imaging revealed mild cerebellar tonsillar ectopia with normal CSF flow, multiple pancreatic and renal cysts which are hallmarks of VHL presentation, and no vascular malformations. Although imaging was reassuring with no visible adrenal mass, her symptomatology was concerning for a pheochromocytoma. Further workup revealed elevated plasma and urine metanephrines with a suspected microscopic pheochromocytoma. A multidisciplinary team including MFM, obstetric anesthesiology, neurology, and endocrinology is used to manage this patient. In this case report, we will discuss the dilemma of when to administer appropriate alpha blockade and the potential maternal and fetal complications associated; the pros and cons of both vaginal and cesarean delivery methods in a parturient with VHL and pheochromocytoma; and the choice of anesthesia for both delivery methods and potential neurological complications. This patient’s delivery is scheduled on February 12, 2019. Our current plans include a primary elective cesarean section at 38weeks under epidural anesthesia. There are limited case reports and evidence to help guide the management of a pregnant patient with VHL complicated by microscopic pheochromocytoma, so we hope to discuss our experience with this complex patient and outline the key factors to mitigate maternal and fetal morbidity.
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2. Penney, D.J. “Arnold-Chiari malformation and pregnancy”. International Journal of Obstetric Anesthesia 10.2(2000):139 – 141.
3. Hayden, Gephart, Kalanithi, Chou. “Von Hippel-Lindau disease in pregnancy: A brief review.” Journal of Clinical Neuroscience 16 (2009) 611–613.