Management of a prestyloid parapharyngeal first branchial cleft cyst from puncture to surgical excision and how a routine procedure can turn into an emergency

Abstract

The anatomy of the parapharyngeal space (PPS) is complex and the differential diagnosis of tumours in this area broad. Although primary tumours of the PPS account for only 0.5% of head and neck neoplasms and are benign lesions in 80% of the cases, the surgical management is crucial and needs specific planning and evaluation of CT and/or MRI scans. In literature, there are several ways to surgically deal with PPS tumours and due to location and differentiation, can reach from transparotid, submandibular transcervical and transoral approaches, extending in a mandibulotomy, further radiotherapy. Parapharyngeal cleft cysts are extremely rare and their management can be complex. We describe the presentation, the diagnosis and further management of a 71-year-old woman with a 6 cm first branchial cleft cyst in the PPS from puncture over emergency tracheostomy to elective excision via a combined transcervical/transparotid and transoral approach. We highlight the importance of the differential diagnosis and the and the correct clinical management of this rare entity. BACKGROUND

Full text

1
FiedlerLS. BMJ Case Rep 2021;14:e238727. doi:10.1136/bcr-2020-238727
Management of a prestyloid parapharyngeal first
branchial cleft cyst from puncture to surgical excision
and how a routine procedure can turn into
anemergency
Lukas S Fiedler
Case report
To cite: FiedlerLS. BMJ
Case Rep 2021;14:e238727.
doi:10.1136/bcr-2020-
238727
Otorhinolaryngology and Head
and Neck Surgery, Klinikum
Mutterhaus der Borromäerinnen
gGmbH, Trier, Deutschland,
Germany
Correspondence to
Dr Lukas S Fiedler;
l. fiedler@ gmx. at
Accepted 7 December 2020
© BMJ Publishing Group
Limited 2021. No commercial
re- use. See rights and
permissions. Published by BMJ.
SUMMARY
The anatomy of the parapharyngeal space (PPS) is
complex and the differential diagnosis of tumours in
this area broad. Although primary tumours of the PPS
account for only 0.5% of head and neck neoplasms
and are benign lesions in 80% of the cases, the surgical
management is crucial and needs specific planning and
evaluation of CT and/or MRI scans. In literature, there
are several ways to surgically deal with PPS tumours
and due to location and differentiation, can reach from
transparotid, submandibular transcervical and transoral
approaches, extending in a mandibulotomy, further
radiotherapy. Parapharyngeal cleft cysts are extremely
rare and their management can be complex. We describe
the presentation, the diagnosis and further management
of a 71- year- old woman with a 6 cm first branchial
cleft cyst in the PPS from puncture over emergency
tracheostomy to elective excision via a combined
transcervical/transparotid and transoral approach. We
highlight the importance of the differential diagnosis
and the and the correct clinical management of this rare
entity.
BACKGROUND
Parapharyngeal space (PPS) tumours can be divided
after their location and their entity. The PPS is an
inverted pyramid- like region from the base of the
skull, reaching the cornu of the hyoid bone. The
borders are defined cranially by the temporal bone,
the vertebrae and the prevertebral muscles poste-
riorly, the buccopharyngeal fascia medially and
laterally by the mandible and the medial pterygoid
muscle.1 2 The PPS can be divided into a prestyloid
and retrostyloid or anterolateral and posteromedial
space.2 3 The prestyloid space consists of fat, the
deep lobe of the parotid and small salivary glands.
The retrostyloid compartment is composed of the
internal carotid, the internal jugular vein, the cranial
nerves IX, X, XI and XII, lymph nodes and the
glomus tissue. Most tumours of the PPS are repre-
sented by salivary gland tumours (40%–50%) and
neurogenic tumours (17%–25%). Third, the para-
gangliomas make 10%–15% of all PPS tumours.
Other entities combined account for 10%–33%
of tumours in this anatomical region. Mentioned
should be abscesses, necrotic lymph nodes, haeman-
giomas, lymphangiomas, aneurysms, angiosar-
comas, rhabdomyomas, chordomas, meningiomas,
osteomas and finally branchiogenic cysts.3–5
During the embryological development, in the
fourth week of gestation, six pairs of arches, clefts
and pouches form the branchial or pharyngeal
apparatus. Every arch consists of a cartilaginous
element, muscular component, a corresponding
branch of the aortic arch and a cranial nerve. These
components later form various structures in the
head and neck and due to non- fusion or subinvo-
lution can result in branchial cleft cysts.6 7 These
defects can present as fistulas, sinuses or cysts.
The branchial cleft cysts are categorised from first
to fourth branchial cleft cysts, depending on their
embryogenic origin.6
First branchial cleft cysts make out about 7%
of all branchial cleft cysts.6 Anomalies in the first
branchial cleft can be classified by Work, into type I
and type II malformations. Work type I malforma-
tions contain ectoderm only and typically present
laterally to the facial nerve. Work type II cysts
contain both ectoderm and mesoderm, are more
common and classically present at the angle of the
mandible.7 8 Overall, the occurrence of a branchio-
genic cyst in the PPS is a very rare entity.
CASE PRESENTATION
A 71- year- old woman presented in our ENT depart-
ment, directly after consulting her ENT in practice
with a chief complaint of a persisting dysphagia for
9 to 10 weeks and acute otalgia and otorrhea on the
left side. She was unfebrile and complained a subjec-
tive progressive hearing loss over the last weeks,
Figure 1 Axial CT image revealing a cystic
formation from left parapharyngeal skull base to the
pterygopalatine fossa in contact with the parotid and the
jaw.
copyright. on January 12, 2021 by Lukas Fiedler. Protected byhttp://casereports.bmj.com/BMJ Case Rep: first published as 10.1136/bcr-2020-238727 on 12 January 2021. Downloaded from

2FiedlerLS. BMJ Case Rep 2021;14:e238727. doi:10.1136/bcr-2020-238727
Case report
left- sided otorrhoea for 2 days, with no vertigo or tinnitus. She
complained about a lockjaw and pain in the left joint of the jaw.
She takes medicines against her hypothyroidism, rheumatism
and high blood pressure and had a history of a colon carcinoma,
which was treated operatively and with adjuvant chemotherapy
two years ago. In her youth, she had a tonsillectomy.
INVESTIGATIONS
The ENT examination showed a trismus about 1.5 cm with a
painful deviation of the jaw to the left and tenderness of palpa-
tion over the left temporomaxillary joint. The examination of
the oral cavity and endoscopic transnasal inspection showed no
malocclusion and a bulging of the left soft palate with a devi-
ated uvula to the right and a protrusion of the nasopharyngeal
wall, the tonsil and the caudal oropharyngeal wall on the left
side. The auditive tube was compressed on the left side and the
isthmus faucium was constrained from the left side near to the
midline. Also, a perforated otitis media with clear secreting on
the left side was shown. The puncture of the protruded soft
palate set on an acute onset of swelling with a rapid progres-
sion of the swelling until a nearly full constraint of the isthmus
faucium. Emergency CT scan showed a cystic lesion 6.4×4×4
cm in diameter reaching from skull base to the pterygopalatine
fossa down to the submandibular gland and had contact with the
parotid and the mandible. The radiological findings showed no
malignancy criteria. Further, secrete retention of the left mastoid
was shown (figures 1–3).
TREATMENT
In our case, first, the diagnosis was thought a peritonsillar abscess
because this entity can present with trismus, swelling of the soft
palate and a deviated uvula, furthermore ipsilateral otalgia. A
peritonsillar abscess is an acute onset deep neck infection mostly
seen in young adults.9 This pathology, as it is a complication of
acute tonsillitis, can be managed in an inpatient setting through
drainage and incision of the abscess and intravenous antibiotic
treatment.
The puncture of the cranial part of the soft palate, which is
routinely done in a peritonsillar abscess, led to a submucosal
leakage and a soft- tissue protrusion with compression of the
isthmus faucium, which resulted in an emergency tracheostomy.
Afterwards, the CT scan had shown a cystic lesion in the para-
pharyngeal space with no malignancy criteria, the differential
diagnosis changed. Considerable diagnosis in PPS cysts are bran-
chial cleft cysts; further differential diagnosis is lymphadenop-
athy, haemangioma, cystic hygroma, salivary gland tumour, cat
scratch disease, atypical mycobacterial infection and cystic squa-
mous cell carcinoma.7 10
On presentation, the protruded soft palate on the left side
was punctured by needle aspiration; we aspirated about 10 mL
of a brown serous liquid. Directly after this aspiration, swelling
of the soft palate was progressing and shifted over the midline
of the isthmus faucium. We set up an instant CT scan of the
neck to rule out an iatrogenic vascular injury and shifted the
patient to the intensive care unit to secure the airway which
got compromised rapidly. The anaesthesiologists performed
nasal intubation; afterwards, we conducted cartilage- conserving
tracheostomy,11 submandibular cyst incision and a Penrose
drainage insertion. Intravenous antibiotic treatment with 3×3 g
ampicillin/sulbactam was started. The swelling of the soft palate
was persisting on the following day, so we had to perform a tran-
scervical incision of the cyst and an insertion of a parapharyngeal
drainage over a left submandibular approach. Two days after the
presentation, we extubated, and the clinical stabile patient was
transferred to our periphery inpatient. Overall, the regression
of swelling of the soft palate gave little satisfaction, that is why
we had to operate again 5 days after presentation and conducted
a transoral incision of the left oropharynx and insertion of a
new Penrose drainage from intraoral to extracervical. This oper-
ation brought sufficient regression of the swelling, the trismus
improved a little, the otalgia stopped and the airway no longer
was in danger.
Ten days after the presentation, we were able to close the
tracheostomy in local anaesthesia after covering it for 3 days
without any clinical complications. The patient has been
discharged after 14 days of inpatient care with intravenous anti-
biotics over 7 days. The histopathology of the needle aspiration
showed parts of a branchial cyst with no malignancy.
Two months after presentation, the cyst had refilled to the size
and volume of the first presentation; we performed an EMG
neuromonitoring–supported left side total parotidectomy and
resection of the cyst via a combined transcervical/transparotid
and transoral approach without any complications. During the
operation, we first positioned the invasive four- canal nerve
monitoring of the facial nerve, reclined and rotated the head
to the right. The skin incision was made preauricular to retro-
mandibular and submandibular. The parotid was prepared and
first, we performed a lateral parotidectomy and a neurolysis of
the facial nerve. The cyst medial to the mandible was gradually
separated from the bone and the medial parapharyngeal fibrotic
links were dissected. As the cyst had contact with the deep
layer of the parotid, we also had to resect the deep layer of the
gland. Moreover, we set in a mouth gag and divided the fibrotic
tissue through the soft palate. Finally, we dissected the cyst and
removed it in toto with the deep layer of the parotid. The facial
Figure 2 Axial CT scan showing left mastoid secret retention.
Figure 3 Coronal CT scan depicting left big cystic lesion compressing
the airway and reaching the skull base.
copyright. on January 12, 2021 by Lukas Fiedler. Protected byhttp://casereports.bmj.com/BMJ Case Rep: first published as 10.1136/bcr-2020-238727 on 12 January 2021. Downloaded from

3
FiedlerLS. BMJ Case Rep 2021;14:e238727. doi:10.1136/bcr-2020-238727
Case report
nerve was uncompromised and showed no lack of function post-
operatively. The final histopathology showed no malignancy,
further could not differentiate between a branchial cyst and an
intracystic parotid adenoma or an intraductal parotid papilloma.
OUTCOME AND FOLLOW-UP
After surgical excision, the patient was discharged regardless of
any functional impairment with an excellent cosmetic result.
DISCUSSION
Our patient presented with trismus and an intraoral mass. Due
to literature, most patients with a PPS lesion present with an
intraoral mass (46%) or a cervical mass (52%).12 The primary
differential diagnosis, which turned out to be wrong, given the
duration of the symptoms which the patient complained of, was
a peritonsillar abscess. Peritonsillar abscesses are acute onset
swelling and pain in the peritonsillar region and the soft palate.9
In our case, histology showed a branchial cyst, not surely
distinguishable from or combined with an intraductal adenoma
of the parotid. The PPS is a complex anatomical region where
different neoplasms can rise. Most tumours in this region are
benign.12–14 In the PPS, tumours are benign in 80%–82%; a
variety of neoplasms can develop in this region. Most frequently,
salivary gland tumours and neurogenic tumours are found in
this anatomical region.15 16 Our cystic tumour was located in the
prestyloid part of the PPS. Due to literature, most salivary gland
tumours are located in the prestyloid space, whereas the neuro-
genic differentiated neoplasms are mostly found in the retrosty-
loid portion of the PPS.17
Depending on location and differentiation including benign
salivary gland tumours, radiotherapy can be a safe and feasible
alternative to surgery.13 18 Most PPS tumours are treated by
surgery and therefore need preoperative planning by CT or
MRI techniques, radiographic techniques are showing high
accuracy.15 19–21 Given our case, a preoperative CT scan was
done, and we refrained from conducting an MRI. In cystic
lesions, preoperative planning is highly accurate on a CT- basis
due to orientation and the preoperative planning of the surgical
approach. Various surgical approaches are described and need
strategic preoperative planning not to compromise cranial
nerves or result in poor cosmetic outcome.22 The surgical
approaches reach from transcervical, transcervical- transparotid,
transcervical- transmandibular, endoscopic- assisted transoral
to transnasal techniques, infratemporal, and further transoral
robotic- assisted.17 23 24 The most frequently used approach is the
transcervical, guaranteeing a high safety and producing good
surgical results.12 24
Given the size and the location of our PPS cyst, we had to
combine a transcervical/transparotid and a transoral approach
which resulted in a good cosmetic and functional outcome.
Contributors I independently wrote this article.
Funding The authors have not declared a specific grant for this research from any
funding agency in the public, commercial or not- for- profit sectors.
Competing interests None declared.
Patient consent for publication Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
ORCID iD
Lukas SFiedler http:// orcid. org/ 0000- 0001- 9319- 8260
REFERENCES
1 Attia A, El- Shafiey M, El- Shazly S, etal. Management of parapharyngeal space tumors
at the National Cancer Institute, Egypt. J Egypt Natl Canc Inst 2004;16:34–42.
2 Bozza F, Vigili MG, Ruscito P, etal. Surgical management of parapharyngeal space
tumours: results of 10- year follow- up. Acta Otorhinolaryngol Ital 2009;29:10–15.
3 Andratschke M, Helmberger R, Mees K. [Differential diagnosis of parapharyngeal
mass]. Laryngorhinootologie 2000;79:174–9.
4 Luna- Ortiz K, Villa- Zepeda O, Carrillo JF, etal. Parapharyngeal space tumor:
submandibular approach without mandibulotomy. J Maxillofac Oral Surg
2018;17:616–24.
5 Rzepakowska A, Osuch- Wójcikiewicz E, Krupa Z, etal. Surgical treatment results of
parapharyngeal space tumors: a report of 22 cases. Otolaryngol Pol 2018;72:9–16.
6 Bagchi A, Hira P, Mittal K, etal. Branchial cleft cysts: a pictorial review. Pol J Radiol
2018;83: :204–9.
7 Coste AH, Lofgren DH, Shermetaro C. Branchial cleft cyst. In: StatPearls. Treasure
Island (FL): StatPearls Publishing StatPearls Publishing LLC, 2020.
8 Aronsohn RS, Batsakis JG, Rice DH, etal. Anomalies of the first branchial cleft. Arch
Otolaryngol 1976;102:737–40.
9 Galioto NJ. Peritonsillar abscess. Am Fam Physician 2017;95:501–6.
10 Patel S, Bhatt AA. Thyroglossal duct pathology and mimics. Insights Imaging
2019;10:12.
11 Probst G, Dubiel S, Deitmer T. [The cartilage conserving concept of surgical
tracheostomy]. Laryngorhinootologie 2004;83:461–5.
12 Kuet M- L, Kasbekar AV, Masterson L, etal. Management of tumors arising from the
parapharyngeal space: a systematic review of 1,293 cases reported over 25 years.
Laryngoscope 2015;125:1372–81.
13 Strohl MP, El- Sayed IH. Contemporary management of parapharyngeal tumors. Curr
Oncol Rep 2019;21:103.
14 Sun F, Yan Y, Wei D, etal. Surgical management of primary parapharyngeal space
tumors in 103 patients at a single institution. Acta Otolaryngol 2018;138:85–9.
15 López F, Suárez C, Vander Poorten V, etal. Contemporary management of primary
parapharyngeal space tumors. Head Neck 2019;41:522–35.
16 Ulku CH, Uyar Y, Arbag H. Management of parapharyngeal space tumors. Ir Med J
2004;97:140–2.
17 Ijichi K, Murakami S. Surgical treatment of parapharyngeal space tumors: a report of
29 cases. Oncol Lett 2017;14:3249–54.
18 Mendenhall WM, Strojan P, Beitler JJ, etal. Radiotherapy for parapharyngeal space
tumors. Am J Otolaryngol 2019;40:289–91.
19 Chu F, Tagliabue M, Giugliano G, etal. From transmandibular to transoral robotic
approach for parapharyngeal space tumors. Am J Otolaryngol 2017;38:375–9.
20 Wang XM, Li H, Chen DS, etal. [The surgical management of upper parapharyngeal
regions]. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018;32:1804–9.
21 Carrau RL, Myers EN, Johnson JT. Management of tumors arising in the
parapharyngeal space. Laryngoscope 1990;100:583–9.
22 Paderno A, Piazza C, Nicolai P. Recent advances in surgical management
of parapharyngeal space tumors. Curr Opin Otolaryngol Head Neck Surg
2015;23:83–90.
23 Liu Y, Yu H- J, Zhen H- T. Transoral and endoscope- assisted transoral approaches to
resecting benign tumours of the parapharyngeal space located in the medial portion
of the carotid sheaths and extending toward the skull base: our experience. J Laryngol
Otol 2018;132:748–52.
24 Tao L, Shi XL, Li XM, etal. [Retrospective analysis of 188 cases of parapharyngeal
space tumors]. Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2018;32:129–33.
Learning points
►The knowledge how patients are presenting with first
branchial cleft cysts and their differential diagnosis.
►The acute and elective management of patients with a
parapharyngeal space tumour.
►How a routine transoral puncture can turn into an emergency
case and how to handle such a situation.
►The surgical approaches and alternative treatments of
parapharyngeal space tumours.
copyright. on January 12, 2021 by Lukas Fiedler. Protected byhttp://casereports.bmj.com/BMJ Case Rep: first published as 10.1136/bcr-2020-238727 on 12 January 2021. Downloaded from

4FiedlerLS. BMJ Case Rep 2021;14:e238727. doi:10.1136/bcr-2020-238727
Case report
Copyright 2021 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit
https://www.bmj.com/company/products-services/rights-and-licensing/permissions/
BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission.
Become a Fellow of BMJ Case Reports today and you can:
►Submit as many cases as you like
►Enjoy fast sympathetic peer review and rapid publication of accepted articles
►Access all the published articles
►Re-use any of the published material for personal use and teaching without further permission
Customer Service
If you have any further queries about your subscription, please contact our customer services team on +44 (0) 207111 1105 or via email at support@bmj.com.
Visit casereports.bmj.com for more articles like this and to become a Fellow
copyright. on January 12, 2021 by Lukas Fiedler. Protected byhttp://casereports.bmj.com/BMJ Case Rep: first published as 10.1136/bcr-2020-238727 on 12 January 2021. Downloaded from