Overt and subclinical baroreflex dysfunction following bilateral carotid body tumor resection: pathophysiology, diagnosis, and implications for management.

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Overt and subclinical baroreflex dysfunction following bilateral carotid body tumor resection: pathophysiology, diagnosis, and implications for management.

World Neurosurg. 2017 Feb 25;:

Authors: Ghali MG, Srinivasan VM, Hanna E, DeMonte F

Abstract
Carotid body paragangliomas are rare, usually benign, tumors arising from glomus cells of the carotid body. Bilateral involvement is present in ∼5% of sporadic cases and up to one-third of familial cases. In the majority of patients undergoing bilateral resection of carotid body tumors (CBTs), a condition known as baroreflex failure syndrome (BFS) develops following resection of the second tumor characterized by headache, anxiety, emotional lability, orthostatic lightheadedness, hypertension, and tachycardia. This is believed to result from damage to the carotid baroreceptor apparatus. Patients without overt cardiovascular abnormalities may have subclinical baroreceptor dysfunction evident only on specific testing, measuring HR and sympathetic nerve responses to baro-loading (eg., phenylephrine) and baro-unloading (e.g., Valsalva maneuver). Given the high incidence of BFS in patients undergoing bilateral resection of CBTs, it is suggested that operation be limited to unilateral resection of the dominant/symptomatic lesion and non-surgical intervention (i.e., embolization, radiotherapy) on the contralateral side. Alternatively, refinement of surgical technique to prevent injury to elements of the baroreceptor apparatus may prevent this unfortunate complication of bilateral tumor resection. We present a case of a 16 year old girl with bilateral jugular vagale and carotid body tumors who developed hypertension following surgical resection of her left jugular vagale tumor and worsening of hypertension concurrent with progression, eventually requiring intensity-modulated radiation therapy and a resection for significant progression of her left jugular vagale tumor. Our case illustrates the generalizability of BFS to patients with tumors involving the vagal baro-afferent fibers.

PMID: 28245992 [PubMed - as supplied by publisher]

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