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More than meets the eye? Anesthetic management of a parturient with PHACE syndrome for cesarean delivery
Abstract Number: T-63
Abstract Type: Case Report/Case Series
Introduction:An association between cervicofacial hemangioma and intracranial arterial abnormalities was first described by Pascual-Castroviejo in 1978. This association was thereafter coined ‘PHACE syndrome’ by Frieden in 1996. Features include posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. A paucity of data exists in the literature regarding the anesthetic management of these patients,especially in the parturient.
Case presentation:A 25-year-old nulliparous woman at 39 2/7 weeks of gestation presented to our labor and delivery unit for scheduled elective primary cesarean delivery. Her pregnancy had been uncomplicated. Past medical history was significant for moyamoya disease (MMD) which was diagnosed at age 19 following a 4 month episode of recurrent headaches, nausea, vomiting and blurred vision. Surgical history was significant for 27 facial reconstructive surgeries due to infantile hemangiomas, including laser ablation of subglottic and epiglottic hemangiomas as well as tracheostomy. Although assisted vaginal delivery was offered to the patient, she ultimately decided to proceed with elective cesarean delivery. Upon anesthesia consultation she was alert, cooperative, and neurologically intact. Further history elicited intermittent dysphagia attributed to her prior epiglottic procedures, although she denied history of aspiration or recurrent pneumonia. Airway exam revealed a Mallampati II which improved to I with phonation, small mouth opening, normal thyromental distance, normal tongue, ability to prognath, and full range of neck motion. Scarring was present over the lower lip, right mandible, chin, and neck; tracheostomy scar was also visible.
On the day of surgery, an epidural was placed, and following placement of a radial arterial line, her epidural was slowly dosed with a total of 15mL alkalinized 2% lidocaine . Hemodynamic stability was achieved with 1800 mL of Lactated ringers and a total of 350 mcg of phenylephrine. The patient remained cooperative and comfortable throughout the perioperative period and did not require intravenous sedation. Both mother and baby did well and were discharged home on postoperative day 3.
Discussion: PHACE syndrome is a diagnosis which is likely under-recognized in the general population. Clinical implications are important for the anesthesiologist, and include the determination of candidacy for neuraxial techniques, potential for difficult airway, understanding of hemodynamic goals in light of neurovascular disease, cardiovascular concerns, and recognition of endocrine abnormalities. Appropriate evaluation of syndrome features should be performed, and these findings should guide the management of these patients.
Freiden IJ et al. Arch Dermatol 1996, 132:307-311
Metry D et al. Pediatrics 2009 Nov;124(5):1447-56
Leffert LR et al. Anesthesiology 2013 Sept;119(3):703-18