Literature ReviewOvert and Subclinical Baroreflex Dysfunction After Bilateral Carotid Body Tumor Resection: Pathophysiology, Diagnosis, and Implications for Management
Introduction
Carotid body tumors (CBTs) are (typically) benign, slow-growing tumors arising from glomus cells, with an annual incidence of 1.6 per 10,000.1 Most synthesize (and some secrete) catecholamines. Some show nodal or distant metastasis.2, 3 Proto-oncogene mutations involving c-myc, bcl-2, and c-jun,4 among others, have been described, as well as germline mutations in succinate dehydrogenase.5 CBTs represent the most common type of paraganglioma and 5% present bilaterally.6 In familial cases (typically autosomal dominant), bilaterality affects 26%–33% of patients.7, 8 Bilateral resection may cause the baroreflex failure syndrome (BFS), with reported prevalence of ∼66%.9
Section snippets
Methods
Case studies and series of bilateral CBTs and BFS were identified through a comprehensive literature search in the PubMed database.
Case Description
A 16-year-old girl presented with postural dizziness/lightheadedness, hoarseness, chronic cough, and dysphagia. On examination, she had a blood pressure (BP) of 120/80 mm Hg (right arm, sitting), a heart rate (HR) of 64 bpm, and evidence of neuropathies of the left vagus, accessory, and hypoglossal nerves with vocal cord paresis, atrophy of the sternocleidomastoid and trapezius muscles, and atrophy and fasciculations of the left side of the tongue. Arteriography identified bilateral glomus
Baroreflex Dysfunction
BFS has been reported by several investigators after bilateral CBT resection or carotid endarterectomy, neck trauma/irradiation, and brainstem stroke. In cases of bilateral CBTs, this typically follows resection of the second tumor, but not in all patients.10
Baroreceptors are stretch receptors concentrated in the carotid sinus and aortic arch and also located in the great vessels from heart to skull base.11, 12 Carotid and aortic arch baroreceptors relay via the carotid sinus (branch of cranial
Conclusions
Bilateral resection of CBTs frequently causes overt or subclinical baroreflex dysfunction, as a result of injury to the carotid baroreceptor apparatus. These patients show headache, anxiety, or emotional lability, as well as abnormalities of resting ABP, increased ABP variability, tachycardia, orthostasis, and episodes of hypotension. In some cases, baroreflex dysfunction improves, perhaps through compensation by aortic or other extracarotid baroreceptors. However, in many patients, BFS
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Operative management of symptomatic, metachronous carotid body tumors involving the skull base and its neurological sequelae
2021, Journal of Vascular Surgery Cases and Innovative TechniquesCitation Excerpt :Furthermore, unilateral hypoglossal nerve paralysis has minimal effect on speech and swallowing as the contralateral nerve is enough for this function, despite the expected hemitongue atrophy. BFS is a well-documented sequela of bilateral CBT resection due to unopposed sympathetic brainstem signals from bilateral baroreceptor denervation.10,11 Headache, anxiety, hypertension, and tachycardia classically characterize this morbidity,2,8,10-12 and its spectrum ranges from hypertensive crisis to volatile hypertension and orthostatic tachycardia.3
Blood Pressure Management in Afferent Baroreflex Failure: JACC Review Topic of the Week
2019, Journal of the American College of CardiologyCitation Excerpt :In some cases, radiation injury also produces carotid artery stenosis that contributes to the symptomatology. Afferent baroreflex failure can also result from bilateral resection of neck tumors, most commonly carotid body paragangliomas, with damage to the carotid sinus nerve (17–19). It can also be caused by familial dysautonomia (hereditary sensory and autonomic neuropathy type 3), a rare congenital disease characterized by developmental failure of afferent neurons, affecting individuals of Ashkenazi Jewish ancestry (20).
Surgical Management of Bilateral Carotid Body Tumors
2019, Annals of Vascular SurgeryCitation Excerpt :Its main function is to monitor blood arterial oxygen partial pressure (PaO2) and arterial carbon dioxide partial pressure (PaCO2) as well as pH changes in the arterial blood. On the other side, carotid baroreceptors are located in the carotid sinus, and they regulate arterial blood pressure (ABP).8,10 CBTs are rare and are considered to have an incidence of 1-2 per 100,000 patients, accounting for 0.012% of all body tumors.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.