Sarwar H. Orakzai MD, James T. Devries MD, Robert C. Griffin, Salvatore P. Costa MD, Robert Palac MD, Richard J. Powell MD.

From the vascular laboratory of Dartmouth-Hitchcock Medical Center, Dartmouth School of Medicine,

Lebanon, NH

Vascular Ultrasound Case Presentation

• CC: Left sided neck mass

• HPI: 43 y female presented with enlarging left sided neck mass

• Left sided neck mass present for year but stable in size

• Overall the last few months, the mass slowly increased in size

• Continued growth despite course of antibiotics

Case

• ROS: no dysphagia, odynophagia, flushing

• + intermittent dizziness and heart racing

• PMH: Nasal allergies

• SH: works in a neurology office in medical records, smokes ½ ppd, ocassional alcohol use

• FH: Father has premature CAD, Mother has DM , no malignancies

Case

• Meds: zyrtec 10mg qd

zantac prn

MVI 1 tab qd

Case

• Afebrile HR 95 BP 130/89 RR 18 sats 98% on RA

• HEENT: 4cm pulsatile left sided neck mass below the angle of the jaw, no movement on swallowing, + bruit

• On right side a smaller mass in upper neck was noted, no bruit

• CVS: S1,S2 audible, no m/r/g

• Resp: CTA bilaterally

• Neuro: CN II-XII intact

Examination

Left CCA

Left ECA and ICA

Left ICA with mass

Left ICA and ECA with mass

Left ICA and ECA with mass

Left ICA and ECA with mass

Right sided tumor (mid neck)

CT scan

Case

• Considering the vascularity of the mass, it was decided to perform preoperative embolization followed by surgical resection

• Successful embolization of left sided tumor by vascular surgery

• Followed by resection of left sided CBT by ENT within 48 hours

Angiogram

• Post op course uncomplicated except for mild hoarseness and dysphagia

• Both hoarseness and dysphagia resolved soon

• Plan to resect right sided CBT in the future

Case Managment

• Paragangliomas are rare tumors that arise from neural crest cells associated with autonomic ganglia

• Can secrete neuropeptides and catecholamines

• Carotid body (CB) is the largest paraganglion in the head and neck and is located at the carotid artery bifurcation

Carotid Body Tumor

• Upon stimulation, the CB releases neurotransmitters that activate the sensory fibers to increase ventilation rate

• CB thus protects the rest of the internal organs from hypoxic damage

• Carotid body tumors (CBT) are the most frequent type of head and neck paragangliomas, accounting for 65% of these tumors

Carotid Body Tumor

• Typically presents in the 4th or 5th decades of life

• Painless, gradually enlarging neck mass at the level of the carotid bifurcation

• Limited mobility in the vertical direction but mobile in the lateral plane (Fontaine sign)

Carotid Body Tumor

• CBT can cause compression of carotid arteries, cranial nerves, sympathetic chain and other neighboring structures

• Leading to pain, hoarseness, dysphagia, Horner's syndrome, and other neurological symptoms

• Functional hormone secretion is less common with head and neck paragangliomas

Carotid Body Tumor

• CBTs may be familial

• Familial CBT are associated with mutations in the succinate dehydrogenase complex.

• Chronic hypoxia in patients with emphysema or in patients living at high altitudes can also lead to hyperplasia/neoplasia of the chief cells

Carotid Body Tumor

• Bilateral CBT may be seen in 30% of familial cases but are rare in sporadic cases

• CBT may be malignant in 5-10% of cases

• Histologically, chief cells are arranged in a characteristic pseudoalveolar pattern referred to as “cell balls” (zellballen)

Carotid Body Tumor

• Evaluation of CBT usually includes ultrasound, CT, MRI/MRA and angiography

• Ultrasound is usually the first diagnostic modality

• Solid, slightly heterogenous mass is detected at the level of carotid bifurcation

• Broadening of the bifurcation with shifting of ICA posteriorly and laterally and the ECA anteriorly and medially

Diagnosis

• Color and power doppler demosntrate the characteristic increased vascularity of the tumor

• Ultrasound also excludes the presence of lymph nodes, thyroid or brachial cysts

• Also useful for follow up of CBT and screening of familial cases

Cerebrovascular US

• CT usually shows a homogeneous mass with intense contrast enhancement and displacement of the carotid bifurcation by the mass

• MRI/MRA provides superior definition of the relationship of CBT to adjacent vascular and skull base structures

• MR/MRA shows intense homogeneous contrast enhancement and the classic “salt and pepper" appearance reflecting signal voids intermixed with regions of focally intense signal intensity

CT and MR/MRA

• Angiography is used for better evaluation of tumor vascularity, tumor invasion of blood vessels and adequacy of the intracranial circulation if internal carotid artery sacrifice is necessary

• It also serves to preoperatively embolize the dominant blood supply to the tumor, thus reducing intraoperative profuse bleeding.

Angiography

• For large tumors with symptoms, surgical resection is usually the treatment of choice

• CBTs have a rich blood supply, and preoperative embolization of the main arterial supply helps to reduce bleeding and other complications

• Surgery may be complicated by postoperative cranial nerve dysfunction and other neurological complications

Management

• Bilateral excision of CB tumors may cause the baroreflex failure syndrome

• Characterized by hypertension in the first 24-72 h after surgery followed by labile hypertension/hypotension, headaches, emotional instability, and palpitations

• Long term follow-up is important following resection to evaluate for recurrence

Management

Thank You