Multiple anesthetic and obstetric challenges in a laboring patient with postural orthostatic tachycardia syndrome (POTS) : acquired hemophilia and a rare seizure disorder


Postural orthostatic tachycardia syndrome ; reflex seizure disorder ; acquired hemophilia ; pregnancy ; combined spinal epidural analgesia

Published online: Jun 28 2019

C.F. James (*), E.H. Burnett (**)

(*) Department of Anesthesiology and Perioperative Medicine, Mayo Clinic Florida 4500 San Pablo Road Jacksonville, Florida 32224
(**) Departments of Obstetrics and Gynecology and Anesthesiology, University of Florida Health-Jacksonville; 655 8th St W, Jacksonville, FL 32209


A 24 year old female Gravida 4 para 0212 presented in preterm labor with three rare co-morbidities, postural orthostatic tachycardia syndrome (POTS), acquired hemophilia, and a reflex seizure disorder. POTS is a rare type of autonomic dysfunction characterized by severe tachycardia and orthostatic intolerance and can be exacerbated by labor and delivery and further aggravated by a neuraxial block. Acquired hemophilia or factor VIII deficiency is a rare form of hemophilia and unlike congenital hemophilia presents during adulthood and was diagnosed during the patient’s previous pregnancy that resulted in a massive postpartum hemorrhage; requiring serial monitoring of Factor VIII activity and the availability of specific blood products during this admission. Reflex seizure disorder is a rare form of seizures and the patient’s seizures were elicited by repetitive sounds, which is a major concern in a typical noisy labor and delivery suite setting with fetal and maternal monitoring. A careful and planned induction of labor was arranged after collaboration with the obstetric and hematologic services, which included maintaining a quiet labor environment and cooperation with the hospital’s blood bank for the availability of blood products. Additionally, a tailored anesthetic during labor and delivery included low dose combined spinal epidural analgesia with emphasis on judicious epidural dosing limiting any hemodynamic changes while providing a near stress-free environment resulting in an uneventful outcome for both mother and baby.