Jugular phlebectasia: A cause of neck swelling—Case report

Souza, Andréa Mv; Guerra, Ângela Francisca Marques; Lima, Tânia Mara Assis; Duprat, André de Campos

doi:10.1016/j.pedex.2008.03.002

Cited by 3 publications

(3 citation statements)

References 8 publications

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“…In our review of the literature, we did not find an association of jugular phlebectasia with Vernet syndrome. A case report of jugular phlebectasia, hoarseness, and SCM weakness in an otherwise‐healthy 10 year‐old South American girl does suggest this association [6]; however, the authors did not define it as Vernet syndrome [6]. We believe ours is the first published case of Vernet syndrome occurring spontaneously as the result of IJVP.…”

Section: Discussionmentioning

confidence: 73%

“…Although the Valsalva maneuver is the most useful clinical sign used to diagnose the dilation of IJV, CT, Doppler sonogram, and MRI are the most useful imaging studies to further investigate IJVP [5‐7]. In our case, a head and neck MRI (with and without contrast) was supplemented with CT and CT venogram to obtain a more thorough evaluation of the vascular anomaly.…”

Section: Discussionmentioning

confidence: 99%

“…The right IJV normally has a greater diameter and cross-sectional area than the left, with a median area of 160 mm 2 (range 108-235 mm 2 ) [4]; however, internal jugular venous phlebectasia (IJVP) is described as a rare vascular entity resulting in an anomalously dilated IJV, primarily of the right side, and can be diagnosed by physical examination findings and imaging [2,[4][5][6][7]. The finding is usually incidental, and although a cause for IJVP is not known, intrathoracic causes have been suggested, such as anomalous reduplication of the jugular vein, pressure over the brachiocephalic vein, and compression by the scalene muscles [5].…”

Section: Introductionmentioning

confidence: 99%

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A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia

Daley

Colliver

2014

PM&R

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A 36-year-old woman presented with right shoulder weakness after a left parotid tumor resection. The overall clinical presentation included severe paralysis and atrophy of the right sternocleidomastoid and upper trapezius, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. A diagnosis of right-sided Vernet syndrome (cranial nerve IX, X, XI lesions) was made, presumably from compression of cranial nerves by internal jugular vein phlebectasia. To our knowledge, this is the first case report of spontaneous Vernet syndrome associated with internal jugular vein phlebectasia in the absence of other lesions of the jugular foramen.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia

Daley

Colliver

2014

PM&R

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Neck masses due to internal jugular vein phlebectasia: Frequency in Menkes disease and literature review of 85 pediatric subjects

Stevens

Price

Marko

et al. 2020

American J of Med Genetics Pt A

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Classic Menkes disease is a rare X-linked recessive disorder of copper metabolism caused by pathogenic variants in the copper transporter gene, ATP7A. Untreated affected individuals suffer failure to thrive and neurodevelopmental delays that begin at 6-8 weeks of age and progress inexorably to death, often within 3 years. Subcutaneous injections of Copper Histidinate (US Food and Drug Administration IND #34,166, Orphan product designation #12-3663) are associated with improved survival and neurological outcomes, especially when commenced within a month of birth. We previously identified internal jugular vein phlebectasia (IJP) in four Menkes disease subjects. This feature and other connective tissue abnormalities appear to be consequences of deficient activity of lysyl oxidase, a copper-dependent enzyme. Here, we report results from a prospective study of IJP based on 178 neck ultrasounds in 66 Menkes subjects obtained between November 2007 and March 2018.Nine patients met the criterion for IJP (one or more cross-sectional area measurements exceeding 2.2 cm 2 ) and five subjects had clinically apparent neck masses that enlarged over time. Our prospective results suggest that IJP occurs in approximately 14% (9/66) of Menkes disease patients and appears to be clinically benign with no specific medical or surgical actionability. We surveyed the medical literature for prior reports of IJP in pediatric subjects and identified 85 individuals and reviewed the distribution of this abnormality by gender, sidedness, and underlying etiology. Taken together, Menkes disease accounts for 16% (15/94) of all reported IJP individuals. Neck masses from IJP represent underappreciated abnormalities in Menkes disease.

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Internal Jugular Phlebectasia: A Case Report and Literature Review

Tamami¹

2015

JOENTR

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Jugular phlebectasia: A cause of neck swelling—Case report

Cited by 3 publications

References 8 publications

A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia

A Case of Vernet Syndrome Associated With Internal Jugular Phlebectasia

Neck masses due to internal jugular vein phlebectasia: Frequency in Menkes disease and literature review of 85 pediatric subjects

Internal Jugular Phlebectasia: A Case Report and Literature Review

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