# Acquired Jugular Vein Aneurysm

Authors: Erkki Hopsu; Jussi Tarkkanen; Seija I. Vento; Anne Pitkäranta
Journal: International Journal of Otolaryngology
 (2009)

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## Abstract

Venous malformations of the jugular veins are rare findings. Aneurysms and phlebectasias are the lesions most often reported. We report on an adult patient with an abruptly appearing large tumorous mass on the left side of the neck identified as a jugular vein aneurysm. Upon clinical examination with ultrasound, a lateral neck cyst was primarily suspected. Surgery revealed a saccular aneurysm in intimate connection with the internal jugular vein. Histology showed an organized hematoma inside the aneurysmal sac, which had a focally thinned muscular layer. The terminology and the treatment guidelines of venous dilatation lesions are discussed. For phlebectasias, conservative treatment is usually recommended, whereas for saccular aneurysms, surgical resection is the treatment of choice. While an exact classification based on etiology and pathophysiology is not possible, a more uniform taxonomy would clarify the guidelines for different therapeutic modalities for venous dilatation lesions.


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## Body

## 1. Introduction

The most common lesions of venous malformations in the
head and neck region are internal jugular vein aneurysms and phlebectasias.
Although these terms are frequently used as synonyms, they pertain to two
divergent conditions, likely of different etiology and pathophysiology.
Macroscopically, aneurysms are described as saccular [1, 2] and
phlebectasias as fusiform [1, 3] (Figure 1).Schematic drawing of (a) a normal vein, (b) a phlebectasia,
and (c) an
aneurysm (courtesy of Seppo Piirainen, Peijas-Rekola Hospital, Vantaa).
(a)(b)(c)While phlebectasia in the neck region is considered to
be congenital in origin or to arise from a primary congenital weakness within
the muscular layer of the venous wall [4–6], venous aneurysm often
occurs secondary to trauma or in association with diseases involving veins [2].
Fusiform jugular phlebectasia is most often described as manifesting in
childhood [7]. Secondary or acquired venous dilatation lesions are
usually saccular in form, and multiple etiological factors have been suggested
for these thrombosed venous aneurysms, which are typically seen in adults [2, 8].We report on an adult patient with a large tumorous mass on the left
side of the neck identified as a jugular aneurysm.


## 2. Report of a Case

Three days prior to admission to the University Central Hospital  in Helsinki,
a 71-year-old woman noticed a rapidly growing lump on the left side of her neck. Physical
examination revealed a mass underneath the anterior border of the
sternocleidomastoid muscle in the supraomohyoid region of the neck. The tumor
was slightly tender and warm upon palpation, but the patient had no infectious
symptoms. There was no known trauma to this area. Ear, nose, and throat status
was normal. C-reactive protein and blood white cell count were normal. Past
medical history included elevated blood pressure and bronchial asthma with
medication. No previous operations or arterial/venous catheterizations had been
performed.Ultrasound revealed a smooth, rounded cystic
configuration (diameter3.5×1.9 cm) filled with a thick fluid. In differential
diagnosis, either an infected cyst or a necrotized lymph node was suspected.The patient was treated with intravenous
kefuroxime and metronidazole for one day and thereafter with per oral
kefalexine and metronidazole for ten days because of a suspected infected
lateral neck cyst. During a six week follow-up, the mass had not regressed. A
malignant involvement could not be excluded and the surgery was performed.Upon surgery under general
anesthesia, a saccular venous aneurysm was detected. A short, wide-based
connection was present between the venous wall of the jugular vein and the
cyst-like aneurysm, which had a smooth and rounded contour. The pathological
segment of the jugular vein was resected and the free ends of the vessel
ligated (Figure2). The patient recovered
well.Figure 2
The jugular vein is twisted as the aneurysm is pulled up before resection.Pathological examination showed
a mass of3.5×2.0×1.8 cm in diameter with a macroscopically normal venous
wall encapsulating an organizing hematoma inside the lumen
(Figure 3). Fibrotic
tissue and fat were observed outside the venous wall. Histologically, an
organized hematoma was seen inside a venous lumen, which had a focally thinned muscular
layer. No signs of infection or neoplasia were present in the tissue specimen
(Figure 4).Figure 3
Macroscopic view of the removed aneurysm cut in half longitudinally.Figure 4
Microscopic view of the aneurysm with an organized blood clot in
the lumen and a focally thinned muscular wall.


## 3. Comment

No established classification of
venous malformations can be found in vascular pathology handbooks, and the
terms used for venous dilatation lesions vary. Two of these terms—aneurysm
and phlebectasia—can be differentiated based on macroscopic
clinicopathological findings of the lesions [1].
The pathogenesis of
venous aneurysms and phlebectasias remains, however, obscure, with scattered
pathological findings and conflicting theories of pathophysiology [5, 7].One of the first descriptions of an internal jugular
phlebectasia by Gerwig Jr. [3] defines phlebectasia as a solitary fusiform
dilation of a vein. Abbott and Leigh [9], in categorizing aneurysmal
venous diseases, made a distinction between congenital primary fusiform and
saccular lesions, suggesting that a saccular lesion can be classified as a true venous aneurysm.
Eifert et al. [1] also made a distinction between the terms phlebectasia and
aneurysm.Opinions on the etiology of aneurysms and phlebectasias vary. Jugular
fusiform phlebectasia is thought to be a childhood disease [4, 7, 10–12].
In these phlebectasias,
patent blood flow is typically present [11–13] and the size of the
internal jugular phlebectasia can even become less evident as the child reaches
puberty [10]. In localized phlebectasias, the tendency for thrombus
formation is not
higher than in normal veins [1, 6].
Saccular aneurysms, by contrast,
have a predilection for thrombosis [2, 8].
The etiopathology of thrombosed aneurysms
appears to differ from that of childhood jugular vein phlebectasias. The macroscopic
appearance of these lesions (fusiform versus saccular) seems to correlate with
the age of the patient. However, saccular lesions do occasionally present in
children [14] and fusiform jugular lesions, phlebectasias, have been
reported in adults [13], especially in the anterior or external jugular
veins [15].Pathological changes of vein walls vary
histologically [2, 5, 8]. In phlebectasias, the muscular layer is usually
thinned. In saccular aneurysms, which tend to thrombose, degenerative
histological changes are seen [2, 8].
Based on histological findings,
aneurysm is considered a more apt term than phlebectasia when all layers of the
vein wall are present [16].Inconsistency in the terms used is reflected in the
nonuniform recommendations for treatment strategies of venous dilation lesions.
The outcome of venous dilatation lesions is dependent on their anatomical
location [5, 17].
Thus, treatment options, besides being affected by the
form of the lesion, also depend on its location. Lesions in the head and neck
region, including the internal jugular vein, tend to have a benign natural
history, with no serious complications being reported [5, 18]. In rare
instances, an embolism or rupture of a venous aneurysm may occur in other
locations, resulting in a surgical emergency or death, especially in the case
of deep aneurysms of the abdominal region and the lower extremities [5, 17, 18]. Although no reports of life-threatening complications for jugular vein
aneurysms exist, a surgical resection is the treatment of choice for saccular
aneurysms in the jugular vein [8, 14, 16]. For jugular phlebectasias, by
contrast, conservative follow-up is preferred [4, 6, 7, 10–13, 15], with
excision only if the lesion is symptomatic, enlarging, or disfiguring [6, 7, 17].Jugular venous aneurysms may be
clinically confused with branchial cysts, laryngoceles, hemangiomas, and
lymphangiomas, or other cystic lesions, such as carcinoma arising in primary
lateral neck cyst or metastatic squamous cell carcinoma. Ultrasonography can
assist in making the correct diagnosis but was not useful in this case. Doppler
sonography, computed tomography, or angiographies are helpful investigations to
increase accuracy in diagnosis of a
cervical mass [19]. Gadolinium-enhanced magnetic resonance angiography imaging
is a reliable method for neck vein imaging [20].The etiopathology of the aneurysm here remains totally open, its
manifestation appeared to be spontaneous and acquired, and histological
examination yielded no special pathological clues of its etiology.


## 4. Conclusions

The rare presentation and inconsistent terminology of
venous dilatation lesions can cause confusion in choosing an optimal treatment.
The treatment options depend on the location and the form of the lesion. For
jugular phlebectasias, conservative treatment is usually recommended, whereas
for saccular aneurysms, which often present with thrombosis, surgical resection
is the treatment of choice. Although an exact taxonomy based on etiology or
pathophysiology is not yet possible, a more uniform nomenclature would clarify
the guidelines for different therapeutic modalities for venous dilatation
lesions.



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*Source: 535617-2009-02-25.xml*