Otolaryngology Case Reports 6 (2018) 16–18

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Otolaryngology Case Reports

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An unusual presentation of a branchial cleft cyst in a 70-year-old T

a a b a a a a,∗
J. Howlett , P. Horwich , M.J. Bullock , S.M. Taylor , R. Hart , J. Trites , M.H. Rigby
a
Department of Surgery, Division of Otolaryngology – Head and Neck Surgery, Queen Elizabeth II Health Science Centre and Dalhousie University, Halifax, Nova Scotia,
Canada
b
Department of Pathology, Division of Anatomical Pathology, Queen Elizabeth II Health Science Centre and Dalhousie University, Halifax, Nova Scotia, Canada

A R T I C L E I N F O A B S T R A C T

Keywords: Background: Branchial cleft cysts are common congenital head and neck masses predominantly presenting in a
Branchial cleft pediatric setting. Most branchial cleft cysts do not provide a diagnostic challenge as they have consistent phy-
Parapharyngeal space sical locations and clinical presentation. This paper describes a rare case of branchial cleft cyst isolated in the
parapharyngeal space. To our knowledge, this is the oldest presentation of a parapharyngeal space branchial
cleft cyst in the literature.
Case: A 70-year-old non-smoking gentleman presented with a subacute presentation of dysphagia and odyno-
phagia. Physical examination revealed a right posterior oropharyngeal swelling. On imaging he was found to
have a 4.6cm cystic, right parapharyngeal mass abutting the prevertebral fascia. Fine needle aspirate suggested
benign pathology. Surgical resection was pursued and final pathology was consistent with a branchial cleft cyst.
Discussion: Benign congenital branchial cleft cysts should be considered in the differential diagnosis for para-
pharyngeal masses, regardless of age.

Introduction
Branchial cleft cysts are the second most common pediatric head
and neck masses, with patients most often presenting to medical at-
tention between the ages of 11 and 30 [1]. Other branchial abnorm-
alities including fistulas and sinuses present much earlier in life [1].
Branchial cleft cysts are caused by the failure of involution of normal
embryological structures. There are four embryological branchial clefts
that develop between the third to eighth week of gestation, leading to
four possible types of branchial cleft cysts [2].
Second branchial cleft cysts make up approximately 90% of all
branchial cleft abnormalities. In normal embryological development
the second pharyngeal arch overgrows the second, third and fourth
branchial clefts forming the cervical sinus of His [2–4]. Incomplete
closure of this sinus results in abnormalities occurring anywhere along
its embryological tract. For second brachial cleft abnormalities this
tract extends from the anterior border of the sternocleidomastoid (SCM)
muscle, courses between the internal and external carotid arteries,
passes superficial to cranial nerves (CN) XI and XII and terminates at
the posterior aspect of the tonsillar fossa [5]. Branchial cleft cysts can
be limited to a superficial simple cyst, however frequently present
challenges on excision with optional deep extensions along their re-
spective embryological tracts. Second branchial cleft cysts have been
further classified by Bailey into four types and predominately present as
a mass at the anterior border of the middle third of the SCM (75%), but
can also appear in the lower neck, parotid, posterior triangle or infre-
quently in the parapharyngeal space [2,3,5,6]. Histologically, branchial
cleft cysts are lined with squamous of columnar epithelium and occa-
sionally, metaplasia resulting in respiratory epithelium can be found. In
these cases, the cyst walls often contain lymphoid follicles [7]. Clini-
cally significant symptoms occur when the branchial cleft cyst becomes
infected, often during an upper respiratory tract infection [2].
Case
A 70-year-old healthy male presented to clinic with of a 6-month
history of right sided pharyngeal pain. There was no dysphagia, ody-
nophagia, trismus, fever, or weight loss reported. Past medical history
was only significant for a remote tonsillectomy as a child. On physical
examination, significant right posterior oropharyngeal swelling was
noted with no overlying mucosal ulceration or change. Grade one
tonsils were noted at that time, despite the history of a previous ton-
sillectomy. There was no lymphadenopathy. The pharyngeal swelling
was palpated as fluctuant and non-pulsatile - a fine needle aspirate was
therefore performed yielding 25 cc of serosanguinous fluid. Aspirate
cytology revealed inflammatory cells and cell debris, with no malignant
cells identified. A subsequent computed tomography (CT) examination
reported a 3.5 cm × 2.2 cm x 4.6 cm rim-enhancing right

∗
Corresponding author. Queen Elizabeth II Health Sciences Center, 3rd Floor Dickson Building, VG Site, 5820 University Avenue, Halifax, Nova Scotia B3H 2Y9, Canada.
E-mail address: mhrigby@dal.ca (M.H. Rigby).

https://doi.org/10.1016/j.xocr.2017.11.005
Received 13 September 2017; Received in revised form 17 October 2017; Accepted 25 November 2017
Available online 26 November 2017
2468-5488/ © 2017 Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/).
J. Howlett et al.
Fig. 1. Cystic right sided parapharyngeal mass displacing great vessels laterally, abutting
prevertebral fascia.
parapharyngeal space mass (Fig. 1). The patient returned to clinic two
weeks later with mild pharyngeal pain, however with a normal or-
opharyngeal examination, and was started on a two-week course of
antibiotics which resolved his symptoms.
Two months later on routine follow-up, fullness was noted in the
right posterior oropharynx once again, posterior to the anterior pillar
extending laterally and inferiorly. This enlargement was now ap-
proaching the midline, however once again there was no overlying
mucosal change. The remainder of the head and neck examination was
within normal limits. Incision and drainage was attempted and 2 cc of
serosanguinous fluid was aspirated. Aspirate cytology was once again
benign. At this time, it was perceived that this mass was most likely a
residual peritonsillar or parapharyngeal abscess, or a malignancy. A
repeat CT examination showed a marginally decreased right para-
pharyngeal mass measuring 2.4 cm × 1.6 cm. Following a discussion
with regards to the benefits of a definitive diagnosis, consent was signed
in clinic for a diagnostic right revision tonsillectomy and examination
under anesthesia.
Intraoperatively, an encapsulated cyst traveling deep into the
parapharyngeal space was encountered, tightly adhered to the super-
ficial residual right tonsillar tissue. The posterior plane of the capsule
rested on, but was superficial to the prevertebral fascia. The capsule
extended laterally into the parapharyngeal fat. Careful transoral dis-
section and excision of the cyst capsule resulted in a significant right
pharyngeal defect, which was left to heal by secondary intention.
Pathological examination of the surgical specimen revealed a cyst with
a thick, inflamed fibrous wall, lined by pseudostratified, ciliated (re-
spiratory type) epithelium. Parts of the epithelium were denuded and
were replaced by polypoid granulation tissue. Foreign material, con-
sistent with plant origin (presumed food material), were found within
the cyst (Fig. 2). There was a possibility that the lesion was a mucous
17
Otolaryngology Case Reports 6 (2018) 16–18
Fig. 2. High power image of cyst wall showing pseudostratified, ciliated epithelium on
right, with adjacent chronic inflammation. Fragment of retractile plant material (food
debris) is seen on left (H&E 100×).
retention cyst, however there was no mucin accumulation within the
cyst, and the lining was pseudostratified ciliated. The lining of a mu-
cous retention cyst is typically squamous or columnar without cilia, and
is often flattened. The findings were therefore consistent with a bran-
chial cleft origin.
Discussion
The parapharyngeal space is a deep potential space best described as
an inverted pyramid shape, with the base situated at the petrous part of
the temporal bone and extending inferiorly to the lesser cornu of the
hyoid [8]. Tumors of this space are rare, making up 0.5% of head and
neck tumors [6]. These lesions are predominantly benign (70–80%)
[4,9]. The parapharyngeal space can be divided into two parts, pre-
styloid and post-styloid. The division between these parts is being the
fascia between the styloid process and the tensor veli palatine muscle,
termed the aponeurosis of Zuckerkandl and Testut [8]. The anatomical
contents of the parapharyngeal space can be found in Table 1. Lesions
within the parapharyngeal space often enlarge painlessly, usually to-
wards the oropharynx, and rarely have neurologic involvement [3].
Generally, pre-styloid tumors are salivary tumors displacing the carotid
sheath laterally and posteriorly [6]. Post-styloid masses are more likely
to be neurogenic in nature such as schwannomas or vascular neoplasms
in nature [6]. Pre-operative assessment to determine if a lesion is pre or
post-styloid is essential for generating a differential diagnosis and also
for surgical planning [2,6,10].
The differential diagnosis of a cystic lesion in the parapharyngeal
space is broad. This is due to the numerous anatomic structures con-
tained within and surrounding the parapharyngeal space leading to
lesions of various possible origin. Salivary gland neoplasms (40–50%)
are the most often observed in the prestyloid space, with the majority
being pleomorphic adenomas and less often, adenoid cystic carcinomas
Table 1
Contents of the parapharyngeal space.
Pre-styloid Post-styloid
Maxillary artery Common carotid artery
Pharyngeal venous plexus Internal jugular vein
Inferior alveolar nerve Vagus nerve
Lingual nerve Cranial nerves IX, XII
Fat Sympathetic chain
Deep lobe of parotid Carotid bodies
Lymph nodes
J. Howlett et al.
[2,6,8]. These originate from minor salivary glands of the lateral
pharyngeal wall or ectopic salivary glands from within the para-
pharyngeal space [2]. The second most common cystic tumor of the
parapharyngeal space are neurogenic tumors (17–25%), arising mainly
in the post styloid space. Schwannomas with cystic degeneration are the
most frequently described lesion [2,6]. Paragangliomas (glomus jugu-
lare, glomus vagale and carotid body tumors) have also been docu-
mented in the literature [4]. Necrotic metastatic lymph nodes, lym-
phangioma and lymphomas make up 10–15% of lesions of the
parapharyngeal space and can present with a variable cystic component
[6,8]. Rare diagnoses can include cystic lymphatic metastasis of squa-
mous cell carcinoma, papillary thyroid carcinoma, choroid plexus tu-
mors or branchial cleft anomalies [11]. Other cystic masses that should
be included in a comprehensive differential diagnosis include mucous
retention cysts and chronic indolent infections such as tuberculosis [8].
Isolated parapharyngeal presentations of branchial cleft cysts rare
with only 25 cases reported in the literature [12]. These cysts expand
predictably in the path of least resistance in the soft tissue plane re-
sulting in protrusion into the oropharynx [3]. Because of this, lesions
are often difficult to diagnose clinically and require complete surgical
removal for identification. Not surprisingly, parapharyngeal branchial
cleft cysts are frequently misdiagnosed as peritonsillar abscesses. This is
a common scenario with the temporary resolution of symptoms oc-
curring after aspiration of the cystic contents. A clue suggesting a
branchial cleft is the extent of the mass on imaging and the slowly
enlarging cystic nature. The age of a patient should not exclude the
diagnosis of a branchial cleft cyst, as was the case with our case pre-
sentation. Although congenital, these lesions can present later in life
than what is classically described. In these cases of advanced age (with
or without risk factors), a fine needle aspirate biopsy is an acceptable
first investigation, but benign cytology does not completely rule out a
neoplastic process.
Conclusion
This case presents an abnormal presentation of a second branchial
cleft cyst in a patient much older than the routinely encountered. To the
best of our knowledge, this is the oldest presentation of an isolated
parapharyngeal branchial cleft cyst. The ability to maintain a broad
differential when investigating a cystic parapharyngeal space lesion is
important, regardless of the patient age. A comprehensive under-
standing of the complex anatomy and embryology is essential to
18
Otolaryngology Case Reports 6 (2018) 16–18
generate an extensive differential diagnosis. Additionally, proper initial
investigations and imaging are essential to establish surgical planning
and to aid with the diagnosis. Ultimately, surgical resection of this type
of cystic parapharyngeal mass for permanent pathology and histological
evalution is required for a definitive diagnosis. Branchial cleft anoma-
lies are a pathology that should be included in the differential diagnosis
of a cystic neck mass in adults.
Acknowledgements
None.
Appendix A. Supplementary data
Supplementary data related to this article can be found at http://dx.
doi.org/10.1016/j.xocr.2017.11.005.
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