Work Type II First Branchial Cleft Cyst with External Auditory Canal Duplication
Sandy Mong, BS1, Anthony C Nichols, MD2 and Daniel G Deschler MD2
1Harvard
Medical School, Boston MA. 2Dept. of Otology and Laryngology, Massachusetts Eye and Ear Infirmary, Boston MA.
Abstract
A 25 year-old male presented with a tender, fixed 3-cm right parotid mass persisting over 2 years with recent
interval enlargement. CT scan demonstrated a cystic mass that did not invade adjacent soft tissue or bone.
Fine needle aspiration (FNA) yielded cyst fluid. Total parotidectomy with facial nerve dissection for a presumed
parotid tumor demonstrated a mass tracking deep to the facial nerve, coursing medially and superiorly to
terminate adjacent to the cartilaginous external auditory canal (EAC). Pathology revealed an epithelial-lined
cyst with hair matrix surrounded by elastic cartilage in focal areas consistent with a Work type II first branchial
cleft cyst with EAC duplication.
Table 1. Classification of First Branchial Cleft Abnormalities
continued
Arnot
Presents in
Location
Derived from
Type I
Young Adults
Preauricular cyst in parotid
Cell rests buried during
closure of ventral portion of
Žrst cleft
Type II
Children
Continuous track to EAC in anterior
triangle
Incomplete closure of cleft
Table 2. Demographics and Preoperative History 3
This case supports the literature in demonstrating that 1. misdiagnosis occurs commonly and frequently
necessitates revision; 2. full facial nerve dissection reduces incidence of its injury; and 3. increased familiarity
with its clinical symptoms, inclusion on the differential for parotid masses, preoperative imaging, and
identification of a tract in the specimen can reduce recurrence rates. Work type II first branchial cleft anomalies
must be included in the differential diagnosis for head and neck masses above the level of the hyoid bone.
Preoperative imaging, more so than FNA, can confirm the diagnosis. Improved diagnosis can ensure the
appropriate surgical approach, and decrease the risk of post-operative complications.
Mal-development of First Branchial Cleft Leads to External
Auditory Canal Malformations 2
Failure of …
leads to…
4 wks
Medial Elongation
Aplasia
6 wks
Obliteration of
ventral cleft
Duplication anomalies
Cyst, sinus, fistula
Gestational
Age
Medial growth of
1st cleft
Formation of ventral
& dorsal limbs of cleft
Sex ratio
Age
Sidedness
2:1 female predominance
0-2nd decade
Fistulas: left (64%);
Sinuses, cysts: no side
Cysts reported 2x as common as Sinuses; fistulas combined*
Cysts; sinuses;
fistulas
Work I vs II
Work II > 2X or greater incidence
Rare
microtia, EAC atresia, cholesteatoma of mastoid and middle ear
associations?
PRESENTATION Swellings: ~1/3 Cervical, ~1/3 parotid, ~1/3 periauricular
Draining fistulas or sinuses
Chronic Infection
Asymptomatic attachment to tympanic membrane
Delay to
Up to 3.5-4 years
diagnosis
Due to likely misdiagnosis and prior inappropriate treatment
I&D or surgery
Ranges up to 50+%
before correct
dx
Preoperative
CT scan most common
imaging
(Barium swallow with fistulography, Ultrasound, MRI)
Discussion
Table 3. Complications of Operative Repair
Case
series
N
Triglia,
1998
39
DÕSo
uza,
2002
158
(pooled)
Shroeder,
2007
MartinezPero, 2006
Solares,
2003
Results
Recanalization
Atresia or stenosis
Birth
Figure A. Preoperative head neck CT
demonstrates a fluid filled cyst without
invasion of adjacent structures
Figure B. Intra-operative image demonstrates
an elevated facial nerve dissected away from a
parotid mass, that contains skin, cartilage and hair
Table 1. Classification of First Branchial Cleft Abnormalities2
Work
Duplication
Histology
Presentation
Tract
Type I
Membranous EAC
Skin WITHOUT adnexal
structures or cartilage
Cystic mass
Parallel to EAC
Medial to concha
Ends lateral to tympanic
annulus.
Type II
Membranous and cartilaginous EAC
Skin with adnexal structures such as
hair, cartilage
Cyst, sinus or fistula
Inferior portion opens below the
angle of the mandible; extend
upwards superŽcial to, deep to or
through branches of CNVII, ending
in or around the external auditory
meatus
Position relative Superior; associated w/ inferior medial or lateral; associated with
to CNVII
divison of nerve
main trunk
Derived from
Ectoderm of first arch only
Mesoderm of first or second arch,
ectoderm of first cleft
Present as
Preauricular mass, abscess
mass at the angle of the
mandible
Diagrammed
n/a
Up to
50%
Position of
tract over
CNVII
39% superficial
50% deep;
11% between
branches
41-76%
superficial
Up to 37%
deep
8% between
n/a
Infection
CNVII paresis with
dissection
Recurrence
n/a
15% total;
12% temporary, 3%
permanent
13%
6% if facial
nerve
identified;
8% if not
identified
n/a
22% total;
6%
1y
n/a
n/a
10
1y
>50%
39%
0
(10/18) superficial;
44% deep; 11%
between
Not
20% superficial 10%
specifi 70% deep
ed
10% between
22%
Not noted
0
1y
21% temporary, 1%
permanent
10%
6%
11% temporary
0% permanent
Not
specified
0
0
0
0
Conclusions
12 wks
28 wks
Prior
surger
y
44%
13
(out of
97)
18
Jakubikov, 9
2005
Dorsal cleft becomes
the EAC
Epithelial plug
Obliterates the EAC
Follow
up
(years)
3.7 y
Figure C. Post-operative images demonstrate full and symmetric
facial nerve function and a well healed modified-Blair incision
1. First cleft abnormalities may not neatly fit into the Work/Arnot classification system,
and can instead also be conceptualized as a failure of cleft elongation, ventral cleft
obliteration, or recanalization during development as proposed by Blevins.
2. The differential diagnosis for neck swelling, particularly in the presence of openings or
masses on ear, neck, or tympanic membrane should not only include abscess,
atheroma, benign parotid tumors, 2nd branchial cleft abnormality aka lateral cervical
cyst, preauriculuar cysts/sinuses, but also 1st branchial cleft abnormalities. Failure to
consider it on the differential may lead to misdiagnosis.
3. Preoperative Diagnosis rests on a combined knowledge of regional embryogenesis,
knowledge of possible presentations and appropriate preoperative imaging. CT may
be useful in confirming clinical diagnosis of a parotid mass and demonstrating the soft
tissue extension of the tract for surgical planning. A fistulogram may be helpful if a
sinus is present.
4. Complications are due to inadequate excision with chronic infection and recurrence.
5. If the lesion is a sinus or cyst, the tract is most likely superficial to the CNVI. If a fistula,
it is more likely to be deep to or between branches of nerve.
6. Complications such as recurrence and facial nerve injury can be reduced by correct
diagnosis, tract identification on the specimen, absence of preoperative infection, and
full and complete dissection of the facial nerve.
References
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