DOI:10.2214/AJR.04.1332
AJR 2005; 185:1355-1357
© American Roentgen Ray Society
Unusual Anterior Neck Mass Visible Only During Valsalva's Maneuver in a Child
Ka Tak Wong1,
Dennis L. Y. Lee2,
Monica S. M. Chan1,
Raymond K. Y. Tsang2,
Edmund H. Y. Yuen1 and
Anil T. Ahuja1
1 Department of Diagnostic Radiology & Organ Imaging, The Chinese University
of Hong Kong, Prince of Wales Hospital, 30-32 Ngan Shing St., Shatin, Hong
Kong, SAR.
2 Department of Surgery, Division of Otorhinolaryngology, The Chinese University
of Hong Kong, Shatin, Hong Kong, SAR.
Received August 25, 2004;
accepted after revision October 15, 2004.
Address correspondence to K. T. Wong
(wongkatakjeffrey{at}hotmail.com).
Introduction
A child with an anterior neck mass is a common clinical presentation. To
the best of our knowledge, there are only a few conditions in which the neck
mass is apparent or aggravated only during Valsalva's maneuver. We present an
unusual case of a superior herniation of the normal mediastinal thymus into
the lower anterior neck in a child during Valsalva's maneuver. The role of
imaging in the diagnosis and common differential diagnoses are discussed.
Case Report
An 8-year-old boy presented with a lower anterior midline neck mass that
had been present for 4 years that appeared only during shouting or straining.
He had no dysphagia, hoarseness of voice, or shortness of breath. Physical
examination revealed a tense swelling in the midline position of the lower
anterior neck that became apparent only during Valsalva's maneuver (Figs.
1A and
1B). The lower border of the
mass was not palpable. The mass was not compressible and did not move when
swallowing; tongue tug was negative. The overlying skin was intact with no
discoloration or ulceration. No other neck mass or cervical lymphadenopathy
was detected. Flexible laryngoscopy did not show any abnormalities in the
larynx or hypopharynx. The preliminary differential diagnoses included
laryngocele and jugular phlebectasia.

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Fig. 1A 8-year-old boy with anterior neck mass that appears only
during Valsalva's maneuver. Clinical photographs show mass in lower anterior
neck appears during Valsalva's maneuver (A) and disappears during
normal breathing (B).
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Fig. 1B 8-year-old boy with anterior neck mass that appears only
during Valsalva's maneuver. Clinical photographs show mass in lower anterior
neck appears during Valsalva's maneuver (A) and disappears during
normal breathing (B).
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High-resolution sonography of the neck with the child performing Valsalva's
maneuver showed a well-circumscribed hypoechoic mass with lobulated outlines,
multiple small echogenic foci, and linear echogenic lines in the midline of
the lower neck anterior to the cervical trachea
(Fig. 1C). The mass descended
back into the upper mediastinum after completion of Valsalva's maneuver.

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Fig. 1C 8-year-old boy with anterior neck mass that appears only
during Valsalva's maneuver. Transverse gray-scale sonogram of lower anterior
neck during Valsalva's maneuver shows well-defined hypoechoic mass (solid
arrows) with lobulated margins and multiple echogenic foci and lines
anterior to cervical trachea (arrowheads). Note its close
relationship with trachea and right common carotid arteries (open
arrow), which are neither compressed nor displaced.
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Because the inferior portion of the mass could not be well delineated on
sonography, MRI was performed; MRI showed a triangular-shaped suprasternal
mass continuous inferiorly with the normal thymus in the anterior superior
mediastinum (Fig. 1D). The neck
mass had a signal intensity similar to that of the normal thymus. There was no
compression on adjacent structures in the anterior mediastinum and lower
anterior neck. The mass herniated superiorly into the lower neck with bulging
contours during Valsalva's maneuver (Fig.
1E). No focal mass was detected within the thymus.

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Fig. 1D 8-year-old boy with anterior neck mass that appears only
during Valsalva's maneuver. Midline sagittal T2-weighted MR image (TR/TE,
1,000/65; number of signals averaged, 2) during normal breathing shows
suprasternal mass (solid arrow) continuous with normal thymus in
anterior superior mediastinum (open arrow). Note its triangular
configuration at its superior margin, identical signal intensity to thymus,
and lack of mass effect on underlying large vessels.
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Fig. 1E 8-year-old boy with anterior neck mass that appears only
during Valsalva's maneuver. Midline sagittal T2-weighted MR image (1,000/65;
number of signals averaged, 2) obtained during Valsalva's maneuver shows there
is superior herniation of thymus into lower anterior neck (arrow),
causing focal bulging of contour in anterior neck.
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The overall imaging appearances were of a normal thymus with unusual
superior herniation into the lower neck during Valsalva's maneuver.
Discussion
The thymus develops from the third pharyngeal pouch together with the
inferior parathyroid glands at the fifth week of gestation. The developing
encapsulated thymic primordia maintains its close relation with the parietal
pericardium and descends with the pericardium during 7-8 weeks of gestation to
assume its characteristic location in the superior anterior mediastinum
anterior to the great vessels
[1]. Mandell et al.
[2] showed intermittent
superior movement of the normal thymus from the superior mediastinum into the
cervical region during periods of forced exhalation that caused posterior
buckling of the cervicothoracic trachea in infants. However, the phenomenon is
subclinical and does not cause any tracheal luminal compromise. This case is
unusual because the child is older and the degree of superior herniation of
the mediastinal thymus is dramatic, causing the clinical symptom of a palpable
neck mass. The proposed mechanism postulated is that the connective tissue
limiting the normal movement of the thymus is exceptionally loose, which
causes the thymus to move into the neck during increased intrathoracic
pressure produced by Valsalva's maneuver.
The diagnosis of superior herniation of normal thymus in the neck in this
child was made radiologically by virtue of its shape, location, continuity
with the normal mediastinal thymus, absence of compression on adjacent
structures on MRI, and characteristic sonographic appearances. In children,
normal thymic parenchyma has multiple echogenic foci and linear structures on
high-resolution sonography [3,
4]. The appearance is
presumably related to the presence of connective tissue septa or blood vessels
within the septa [5].
The differential diagnosis of midline anterior neck mass in children
includes thyroglossal duct cyst, dermoid-epidermoid cyst, cystic hygroma,
thyroid mass, and ectopic thymic mass
[6]. The fact that the mass in
this child occurred only during Valsalva's maneuver virtually rules out all
those possibilities.
Laryngocele and phlebectasia are the only two conditions that may produce
similar clinical presentation and can be easily differentiated from one
another on imaging. Laryngocele, an abnormal dilatation of laryngeal saccule,
can occur in children but is more common in adult men in the fifth decade. CT
is the preferred imaging technique for evaluating laryngocele, which appears
as a gas-filled dilated laryngeal saccule extending to the paraglottic region
(internal laryngocele). It may protrude through the thyrohyoid membrane to the
lateral neck (external laryngocele)
[7] and, at that time, be
visualized even on sonography. Laryngocele may be filled with air, fluid, pus,
or a combination [8].
Phlebectasia is dilatation of an isolated vein, and the internal jugular
vein is the most commonly affected. It may present with cervical swelling that
expands with increased intrathoracic pressure
[9]. On rare occasions, the
anterior jugular vein can be affected
[10]. Clinically it would be
located in a more lateral position (more commonly on the right), which could
help to differentiate it from a laryngocele. Sonography supplemented by
Doppler examination accurately identifies the dilated internal jugular vein on
Valsalva's maneuver with venous blood flow
[9,
10].
In summary, symptomatic superior herniation of the normal mediastinal
thymus into the lower anterior neck during Valsalva's maneuver is a rare
condition. High-resolution sonography and MRI confirm the diagnosis by showing
its continuity with the normal mediastinal thymus and the typical sonographic
echo pattern of normal thymic tissue. Otorhinolaryngologists and radiologists
should be aware of this unusual clinical entity to prevent unnecessary biopsy
or surgery.
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