Chitra Kumar wasn't aware of the small mass on the right side of her neck until a friend she was speaking with pointed it out. She quickly discovered that the soft, compressible mass — small and flesh-colored — appeared primarily when she spoke.

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Because the mass caused no pain or symptoms, Kumar wasn't overly concerned when her primary physician referred her to the Michigan Medicine Frankel Cardiovascular Center, where vascular medicine specialist Yogen Kanthi, M.D., and vascular surgeon Dawn Coleman, M.D., examined her last spring.

The diagnosis? An external jugular vein aneurysm/varix, a condition Coleman says is "extremely rare, with no known cause."

Coleman says 57-year-old Kumar had a history of asymptomatic right carotid artery stenosis and hypothyroidism.

"She had no neurologic or other symptoms associated with the soft, compressible, superficial mass, which measured 3 centimeters in diameter," says Coleman.

Although varicose veins in the lower extremities are common, similar venous malformations in the neck veins are very rare, says Kanthi.

"They are not related to age or gender, though jugular varices are more commonly diagnosed in children," he says, noting that although surgical indications are controversial, intervention is often recommended for risks of thromboembolism, theoretic risk of rupture and cosmetic reasons.

A treatment plan takes a turn

Resection is indicated for patients with symptoms or with enlargement that is evident on imaging, Coleman says. "A comprehensive analysis is needed to eliminate the possibility of malignancy as external jugular varix has been associated with hemangioendothelioma," vascular tissue that could be malignant.

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Coleman recommended Kumar's mass be surgically excised within the next month because of the risk of thromboembolism and cosmetic concerns.

But prior to surgery, Kumar awoke one morning to find the mass had changed significantly — no longer soft and compressible, but firm to the touch and measuring about 4.7 centimeters in diameter. Her external jugular vein aneurysm had ruptured, though she felt only minor discomfort. She was immediately scheduled for surgery, which involved a small neck incision, evacuation of hematoma from the rupture and ligation of her external jugular vein and branches.

Today, Kumar is fully recovered and has no physical limitations. She and her husband recently returned from a trip to Bohemian Switzerland National Park in the Czech Republic, where she easily managed a long mountain hike.

Says Coleman, "External jugular vein aneurysms like Chitra's are extremely rare venous malformations that can be complicated by rupture, but simple surgical excision can be performed safely."