Phlebectasia as a cause of intermittent cervical mass

Article Outline

• Summary
• 1. Introduction
• 2. Case report
• 3. Discussion
• References
• Copyright

Summary 

Phlebectasia is an abnormal sacculofusiform dilatation, usually unilateral, which may affect any vein. It is infradiagnosed as it is generally asymptomatic. Sometimes it is diagnosed during the study of an intermittent mass. Phlebectasia of internal jugular vein is a rare disease, which mostly involved only the right side and it usually is a childhood disease. Its treatment is controversial. Nowadays a conservative approach to unilateral or bilateral asymptomatic phlebectasia is recommended. Symptomatic phlebectasia requires surgery. We present a case of a bilateral phlebectasia in a 23-month-old male child.

Keywords: Phlebectasia, Internal jugular vein, Aneurysm, Venoma, Varicocele

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1. Introduction 

Phlebectasia is an abnormal sacculofusiform dilatation (without tortuosity) [1], [2], [3], [4], which may affect any vein [1]. It is randomly detected during examination of patients, most of them pediatric patients [1], [3]. This infradiagnosed entity receives several names such as venous aneurysm, venous pseudoaneurysm, congenital venous cyst, venoma, venous ectasia, aneurysmal varix, essential venous dilatation, and varicocele [1], [2], [6], [7], [8], [9].

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2. Case report 

A 23-month-old male child was admitted to the Emergency Unit due to mild acute laryngitis. During examination a mass was accidentally observed in the region of the right anterior triangle of the neck, which his parents had not noticed before. It was a soft, compressible, painless mass of a bluish colour of 3cm×2cm which was not attached to deep structures. No fremitus or murmurs were detected. The mass appears on performing the Valsava maneuver and disappears at rest (Fig. 1, Fig. 2). When the patient cries intensely we observe that the mass is bilateral, as we detected a similar but smaller mass in the left region. The patient referred a previous history of trauma without clinical consequences when a piece of furniture had accidentally fallen and hit the child on the neck 24h before. The patient had a previous history of recurrent bronchitis, but no other significant data with the exception that both parents suffered from asthma.

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• Fig. 1. 

  A right cervical dilatation of the internal jugular vein.

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• Fig. 2. 

  (a) A left cervical dilatation of the internal jugular vein. It is smaller than right cervical dilatations of the internal jugular vein. (b) The appearance of the neck before Valsalva manoeuvre. (c) The appearance of the neck after Valsalva manoeuvre.

Considering this information and on the suspicion of the cause of the mass, we performed a bilateral cervical echography which revealed a bilateral dilatation of the internal jugular vein, more significant in the right region. The dilatation increased on performing the Valsava maneuver. We decided not to do surgery because the child was asymptomatic. One year later the child continued to be asymptomatic and the neck mass was smaller. A new ultrasound showed a neck mass of 2.6cm×1.6cm.

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3. Discussion 

Phlebectasia of the venous system of the neck was first described by Harris in 1928 [2], [8], [9], [10], [11] and defined by Gerwing in 1952 [2], [8], [9], [10], [11]. Till the 1970s few cases of phlebectasia affecting the jugular and cervical venous systems had been reported, however, in recent years and thanks to advances in surgery and non-invasive diagnostic techniques, the number of cases has increased, although it remains an infradiagnosed entity [1].

This venous anomaly may affect the venous system of the limbs (knee, groin, thigh, and sural nerve) [7], [8], [9], the abdominal wall, the superior mediastinum [7], superior vena cava, portal, splenic veins and more frequently cranial and cervical venous systems [1] (internal, external and anterior jugular vein, jugular bulb, posterior facial vein, superficial cervical communicant veins) [14]. Within the cervical venous system the vein most commonly affected is the internal jugular vein, as is the case of our patient, especially the right vein [1], [2], [3], [4], [5], [6], [7], [9], [10], [11], [12], [15], [17]. The incidence of phlebectasia is higher among male patients and it rarely has a bilateral presentation, hence, the interest of the case here reported [14]. Bowler et al. have reviewed all the cases published so far in the literature and of 28 cases only 1 had a bilateral presentation [7]. Xu et al. have described 29 cases of phlebectasia of internal jugular vein and only 3 of them with a bilateral presentation [3]. Further reviews of cases performed by Al-Dousary et al. and Palieri et al. show similar percentages [9] (Table 1).

Table 1. The published cases of phebectasia

Authors	Reference	Year	Age	Sex	Side	Symptom	Diagnosis test	Treatment
Guerrero	[1]	2005	4.5 years	M	R. IY	Neck Mass 7.7 mm	Ultrasound TC	Conservative
Martínez	[2]	2005	7 years	M	R. IY	Neck Mass 1.8 cm	Ultrasound Angio TC Doppler	Conservative
Hu	[3]	2005	1.5–15 years		22 cases: R IY	All patients: neck mass	Ultrasound Doppler	32 surgery
					4 cases: I. IY	Some children complained of slight discomfort or pain during deglutition		4 Conservative
					3 cases: bilateral
					5 cases: R. EY
					2 cases: L. EY
Cheong Woo	[4]	2002	6 years	M	R. IY	2.5cm×3cm	Ultrasound and Doppler or CT	Conservative
			6 years	M	R. IY bilateral IY	2.5cm×3cm
			2 years	M		Derecho 2.5cm×3cm; Izquierdo 1.5cm×2cm
Sander	[5]	1999	14 years	F	R. IY	All patients: neck mass	Ultrasound	Surgery
			12 years	M	R. IY	5° uncomfortable feeling during swallowing	Doppler
			3 years	M	L. IY	7ª y 8°ª dysphonic when reading aloud.
			3 years	Fe	R. IY
			4 years	F	L. IY
			5 years	M	R. IY
			7 years	M	R. IY
			8 years	M	R. IY
Castro	[6]	1999	3–12 years	M	4 cases: L. IY.	Neck mass	Ultrasound Doppler	Conservative
				M	2 cases: R. IY			Cases with thrombosis: surgery
				M
				M
				F
				M
Bowdler	[7]	1986	6 years	M	R. IY	Neck mass, asthma	Venography	Conservative
Walsh	[8]	1993	4 years	M	L.	Neck mass, complete bony defect in the floor in the floor of middle ear. Bilateral otitis media	Arteriography	Conservative
Al-Dousary	[9]	1997	11 years	M	R. IY	Neck mass 3.4cm×4.5cm, voice change	US Doppler Spiral TC	Conservative
Fishman	[10]	2003	2 years	M	R. IY	Neck mass: 3cm×3cm	MRA and Venography	Surgery
					R. EY	neck mass: 2cm×1cm×0.8cm
Kwok	[11]	2002	10 years	M	R. IY	Neck mass 5cm×3cm	Ultrasound
			10 years	M	R. IY	Neck mass	Ultrasound
			8 years	F	R. IY	Neck mass 3cm	Ultrasound, Doppler
Palri	[12]	2002	10 years	F	R	Neck mass 5cm×2cm	Ultrasound	Conservative
			3 years	M	R	Neck mass 3cm×2cm
Lau	[13]	1998	30 years	M	R. EY	Neck mass 5cm×3cm×2cm. Vegetant intravascular hemangioendothelioma	CT	Surgery
Rajendran	[14]	2004	10 years	F	R IY	Neck mass 13mm×20mm	Ultrasound, Doppler	Conservative
Dkk	[15]	2000	10 years	M	R. IY	Neck mass	Ultrasound, Doppler	Conservative
Yoon	[16]	2001	6 weeks	M	R. IY	Neck mass 6cm×8cm. Right-sided lymphatic malformation	CT	Surgery
Harris	[17]	1982	5 month	F	R. IY	Neck mass	Clinical examination	Surgery
Rowe	[7]	1946	38 years	F	R. IY	Neck mass	Clinical examination	Surgery
Gerwig	[7]	1962	12 years	M	R. IY	Neck mass	Clinical examination	Surgery
Pataro	[7]	1961	5 years	M	R. IY	Neck mass	Clinical examination	Surgery
Pataro	[7]	1961	7 years	M	R. IY	Neck mass	Clinical examination	Conservative
Garrow	[7]	1964	13	F	L. IY	Neck mass	Clinical examination	Surgery
Okay	[7]	1970	65 years	M	R. IY	Neck mass	Venography	Conservative
Alonso	[7]	1970	44 years	F	R. IY	Neck mass	Clinical examination	Surgery
Gilbert	[7]	1972	3 years	M	R. IY	Neck mass	Arteriography	Conservative
Gilbert	[7]	1972	2.5 years	F	R. IY	Neck mass	Arteriography	Surgery
Gordon	[7]	1976	6 years	M	R. IY	Neck mass	Venography	Surgery
Gordon	[7]	1976	2 years	F	R. IY	Neck mass	Venography	Surgery
Gordon	[7]	1976	10 years	M	R. IY	Neck mass	Venography	Surgery
La Monte	[7]	1976	6 years	M	R. IY	Neck mass	Clinical examination	Conservative
La Monte	[7]	1976	20 years	F	R. IY	Neck mass	Arteriography, venography	Conservative
Mallik	[7]	1977	7 years	M	R. IY	Neck mass	Clinical examination	Surgery
Passeriello	[7]	1979	5 years	M	R. IY	Neck mass	Venography	Surgery
Passeriello	[7]	1979	6 years	F	R. IY	Neck mass	Arteriography	Surgery
Passeriello	[7]	1979	4 years	M	R. IY	Neck mass	Arteriography Venography	Conservative
Stevens	[7]	1982	10 years	M	L. IY	Neck mass	Venography, ultrasound, xenography	Conservative
Danis	[7]	1982	2 years	F	L. IY	Neck mass	Clinical examination	Surgery
Danis	[7]	1982	9 years	F	L. IY	Neck mass	Clinical examination	Surgery
Danis	[7]	1982	4 years	M	L. IY	Neck mass	Clinical examination	Surgery
Leung	[7]	1983	9 years	M	Bilateral IY	Neck mass	Clinical examination	Surgery
Yashiro	[7]	1984	10 years	M	R. IY	Neck mass	Xenography	Conservative
Yashiro	[7]	1984	5 years	F	R. IY	Neck mass	Venography, CT	Conservative
Som	[7]	1985	68 years	F	R. IY	Neck mass	Venography	Conservative
Yokomori	[9]	1990	6 years	M	R R. IY	Neck mass 3cm×5cm	–	Surgery
Shimizu	[9]	1992	20 years	F	L. IY	Neck mass 3cm	–	Conservative
Kuo	[9]	1992	6 years	F	–	Neck mass	–	Surgery
Balik	[9]	1993	8 years	M	R. IY	Neck mass	–	Surgery
Mickelson	[9]	1995	66 years	M	L. IY	Neck mass 3cm×5cm	–	Surgery
Zukschwerdt	[12]	1929	6 years	F	R. IY	Neck mass	–	Surgery
Malik	[12]	1977	7 years	M	R. IY	Neck mass	–	Surgery
Hughes	[12]	1988	3 years	M	L. IY	Neck mass	–	Conservative
Nwako	[12]	1989	5 years	M	R. IY	Neck mass	–	Surgery
Dhillon	[12]	1991	8 years	M	R.IY	Neck mass	–	Conservative
Balik	[12]	1993	8 years	M	R. IY	Neck mass	–	Surgery
Gendeh	[12]	1994	4 years	M	Bilateral IY	Neck mass	–	Conservative
Inci	[12]	1995	8 years	M	R. IY	Neck mass		Conservative
Hussein	[12]	1996	0.2 years	M	R. IY	Neck mass	–	Conservative

According to Palieri et al., phlebectasia more commonly affects the right region due to anatomic reasons such as the smaller length of the right brachiocephalic truncus in comparison to the left one, the higher position of the right jugular vein bulb in contrast to that of the left one or the fact that the right brachicephalic truncus has valves in 99% of the cases. All this facilitates the transmission of intrathoracic pressure more easily to the right jugular vein than to the left one [12], which favours the formation of non tortuous sacculiform dilatations.

Phlebectasia has been reported in a wide age interval, ranging from 5 months to 68 years [7], [8], being more frequent among pediatric patients [1], [2], [6] especially below 13 years of age [8].

Phlebectasia of internal jugular vein usually presents as a long, sometimes bluish mass, non-pulsatile and painless which appears in the supraclavicular region, in the anterior margin of the sternocleidomastoid muscle. It is detected on performing maneuvers which increase intrathoracic pressure (crying, screaming, coughing, and Valsava maneuver) and on exerting pressure on the inferior region of the ectasia [2], but it disappears at rest [6], [14]. This entity is very often asymptomatic [1], [2], [4], [6], as in the case here reported, although some signs may occur such as pulsatile acuphens (when it is tangentially located with respect to the auricular pavilion) [1], [2], [7], buzzing provoked by turbulences in the dilated area [8], [11], dysphonia or aphonia attributable to recurrent compression of the laryngeal nerve [12], discomfort when swallowing, coughing, doing physical activity [1], [9], pain in the shoulder, sudden difficulty to use the right hand [1], [7], difficulty to speak aloud or pain in the tongue [5].

As regards the etiology of this entity, most of the cases are considered as idiopathic [3], [8], [10], [12]. Palieri et al. suggest as a possible cause an intermittent but prolonged increase of the pressure in the affected venous system. In this sense, Garrow et al. detected an elevated baseline pressure in the internal jugular vein [2], as well as an increment of pressure on coughing or on performing the Valsava maneuver [7]. Among the predisposing factors for phlebectasia, we must mention intracranial compression [2], venous obstruction in the lower region of the neck or mediastinum [1], compression of the right internal jugular vein between the pulmonary dome and the clavicle [1], [7], the sternum and the innominate artery in patients with pectum excavatum and the increased muscular tone of the anterior scalene [7]. When it is accompanied by lymphatic malformations, it could be caused by the compression of such malformation, although there are also reports of phlebectasias more medial to the malformation, so it has also been proposed to be caused by an alteration of the embryologic development [16]. Other possible causes so far described are mechanical ventilation with prolonged positive pressure [2], [7], internal jugular vein duplication [1], [7] or venous wall defects [3], [7]. Three hypotheses have been put forward in this respect: the first one claims a congenital alteration and is supported by the fact that phlebectasia is more common among children [6]; the second hypothesis claims that phlebectasia is associated with endophlebohypertrophy or endophlebosclerosis of the affected nervous system, due to the increase of venous flow; according to the third hypothesis, the development of sacculations is due to post-traumatic inflammations.

As regards the histological findings associated with phlebectasia, Bowler et al. consider that in most cases such findings are normal: we only observe dilatation and thinning of the wall. On other occasions, we observe loss of the elastic layer and hypertrophy of connective tissue together with focal thinning of the intima [7]. However, Palieri et al. claim that in most cases we find some type of alteration such as loss of elastic tissue and hypertrophy of connective tissue, thinning of venous wall, fibrosis and decrease or lack of muscular layer [12]. Wash et al. consider that histological findings are normal most of the times with some significant exception where we observe loss of connective tissue, hypertrophy of the intima together with growth of connective tissue and prominent flat muscle and decrease or lack of muscular tissue [8].

In relation to the diagnosis, the majority of cases are diagnosed by chance when they visit the doctor to consult other pathology, usually an upper respiratory infection. The most present-day authors claim that it is possible to make a diagnosis on the basis of an exhaustive clinical history and physical examination [3], confirming the diagnostic suspicion by means of Doppler or non-Doppler echography [1], [2], [6], [8], [12]. According to Al-Dousary et al. echography is allowed for the morphological diagnosis in real time and they consider it the diagnostic technique of choice; it should always be performed bilaterally and accompanied by Doppler in order to observe the flow. Echography also makes it possible to rule out the existence of thrombi [3]. In the case of suspicion of intracranial or intrathoracic phlebectasia, a CT with contrast or a gadolinium enhanced MNR are indicated [9], however, the majority of authors do not realize CT to reject a possible intracranial or thoracic extension.

The differential diagnosis must be performed with masses which appear on performing the Valsava maneuver [1], especially the laryngocele, due to its frequency [3], external laryngeal diverticle, pharyngocele, cervical pulmonary hernia (neumocele), superior mediastinal cyst or tumor [3], [12] or venolymphatic malformation. The presence of pulse and the characteristics of the surface must be examined, as the lack of the first discards an arterial mass and a flat surface rules out the possibility of hemangioma. The normal evolution of phlebectasia diagnosed in a child consists in a growth of size for some years till puberty when it starts to diminish as a result of the thickening of venous walls [1]. The complications are rare, so the patients would have a normal activity and they need any special health care. Some have been reported such as Horner syndrome caused by the compression of post-ganglional sympathetic fibers, venous thrombosis [2] which usually presents as an infected lymphatic node [5], congestive heart failure, pharyngitis or lymphadenitis [9]. Also, a case of massive hemorrhage has been described as a result of misdiagnosis following tonsillectomy [18].

The management of phlebectasia poses some controversy, and the frequency of surgery varies much, depending on authors, symptoms and complications [1]. A conservative approach to asymptomatic or mildly symptomatic phlebectasia is highly recommended in recent years, due to the benign and self-limited nature of this entity [1], [2]. In cases of asymptomatic bilateral phlebectasia the most adequate approach seems to be a conservative one as well and we must operate in case of symptoms or complications [5]. Symptomatic phlebectasia [3], [5] requires surgery, although an adequate contralateral blood flow must always be confirmed previously. Symptomatic bilateral phlebectasia must never be operated simultaneously due to the risk of massive cerebral edema. Surgery is clearly indicated whenever we find such complications as thrombosis [2], [6]. Esthetic reasons can also justify the need for surgery [2], [3], [6] or whenever the mass grows [3]. The most common surgical technique is the resection of the aneurysm. Other options include tangential aneurysmectomy with venorraphy [2], [3], draping with omohyoideus muscle [2] or with dracon [3], which would be more indicated in cases of bulb affectation or in cases of bilateral phlebectasia. There is no published case with pre-operative chemical or mechanical embolization or “coiling”. Hu et al. report possible complications of surgery such as post-surgical recurrence, which may resolve spontaneously and cerebral edema with pontine infarction. Nevertheless, complications of surgery are usually rare, being necessary to consider each case individually.

We can conclude that this infradiagnosed entity has usually a benign evolution without symptoms and makes it possible to adopt a conservative approach which involves the follow-up of the patient and provides some peace to parents.

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