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Sudhir. M1, Ramachandra. J2, V. Satish3, Sridhar. S4
Sudhir M, Ramachandra J2, V Satish, Sridhar S. “Branchial anomalies: a retrospective analysis in a tertiary care
hospital”. Journal of Evolution of Medical and Dental Sciences 2013; Vol2, Issue 32, August 12; Page: 59845992.
ABSTRACT: Branchial anomalies are the second most common congenital head and neck masses.
Although congenital, all age groups are known to be affected. Branchial anomalies are common
congenital pediatric head and neck lesions but are comprised by several diverse anomalies.
Treatment must be tailored depending on which branchial arch is involved and whether a cyst mass
or sinus/fistula tract is present.
KEYWORDS: Branchial anomaly, branchial cleft cyst, branchial fistula, branchial sinus, congenital
neck mass, pediatric neck mass
INTRODUCTION: Branchial anomalies comprise approximately 20% of pediatric congenital head
and neck masses, making them, after thyroglossal duct cysts, the second most common1. Although
the term branchial anomaly encompasses first, second, third and fourth branchial cleft cysts, sinuses
and fistulae, all of which are distinct clinical entities and require separate management, all are
thought to result from a similar embryologic error: incomplete obliteration of the branchial
apparatus during embryogenesis2. The branchial apparatus consists of six grooves (ectoderm),
arches (mesoderm) and pouches (endoderm).
When a pouch or groove fails to obliterate, it may communicate with either skin or mucosa of
the upper airway, forming a sinus. When both a pouch and a groove fail to obliterate, it may form a
communication between the skin and mucosa, which is termed a fistula. When a branchial groove
remnant forms an epithelial-lined space without communication to the skin or mucosa, a cyst is
formed1. Each of the six branchial arches (with the exception of the fifth) develops into specific
structures in the head and neck. A branchial anomaly and its associated tract typically lies inferior to
all the derivatives of its associated arch and superior to all derivatives of the next arch 2. The
malformations can, therefore, be divided into first through fourth branchial anomalies.
PATIENTS AND METHODS: A retrospective analysis was conducted between Jan 2008 to June 2012,
over a period of 54 months in KIMS Bangalore. Total number of 31 cases were analysed.
RESULTS: First and second decade was the most common age group affected. (Chart 1). Eighty nine
percent of patients were males. Right sided lesions were seen in 68% of patients, left in 22% and
bilaterally in 10% of patients. Discharge from the external opening and swelling were the most
common symptoms. (Figures 1, 2 and 3). Surgery was performed in all the patients. Elliptical
incision was used in the majority of cases; step ladder incision was the next most common incision
used. (Figure 4).
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5984
FIRST BRANCHIAL ANOMALY: First branchial clefts comprise 5–25% of all branchial anomalies3, 4.
Their location is variable, extending from the retroauricular and parotid region, cervically from the
area below the mandible and above the hyoid5. Symptoms at presentation typically vary with
location. Parotid and retroauricular lesions classically present as an enlarging mass often after
infection with associated erythema and pain. Occasionally, otorrhea may be seen from the external
auditory canal6, 7. When within the parotid, the course of the tract in relationship to the facial nerve
is variable8. Work9 classified first branchial cleft anomalies on the basis of anatomical and
histological features. Type 1 is of ectodermal origin and is considered a duplication of the
membranous external auditory canal. Typically, they present in young children as a thin walled, soft
mass protruding in the external auditory canal. Type 2 is a duplication of the membranous external
auditory canal and pinna, containing skin (ectoderm) and cartilage (mesoderm). Classically, they
present later in childhood as a cyst, sinus or fistula5.
SECOND BRANCHIAL ANOMALY: Second clefts are the most common, ranging from 40 to 95% of all
branchial anomalies1, 10, 11. Classically, their complete course begins near the anterior border of the
sternocleidomastoid muscle (SCM), tracks superior and lateral to the common carotid artery,
passing medially between the external and internal carotid arteries, passing lateral and superior to
the glossopharyngeal and hypoglossal nerves and penetrating the middle pharyngeal constrictor
muscle to open into the tonsillar fossa10, 12. Sinuses are more frequent than cysts, which are more
frequent than fistulae13, 14. Second branchial cysts classically present as a painless neck mass along
the anterior border of the upper third of the SCM, or after acute enlargement with concomitant
respiratory infection15. Sinuses typically present inferiorly with a draining opening near the base of
the neck where the strap muscles meet the SCM. Unilateral fistulae are found much more commonly
on the right side with rates reported as high as 89%, whereas bilateral anomalies may be associated
with branchio-otorenal syndrome16.
THIRD BRANCHIAL ANOMALY: Third clefts represent only 2–8% of all branchial malformations4, 17.
Similar to second anomalies, the tract arises at the skin along the mid to lower third of the anterior
border of the SCM, pierces the platysma with variable passage through the thyroid. It then ascends
along the carotid sheath, passing over the superior laryngeal nerve, deep to the glossopharyngeal
nerve, behind the internal carotid, piercing the thyrohyoid membrane and entering the upper lateral
piriform sinus wall18, 19. In contrast to second branchial sinuses, third clefts are predominately on the
left, with a reported rate of 89%20. The classic presentation is as a recurrent left abscess or acute
suppurative thyroiditis during the first decade of life20, 21.
FOURTH BRANCHIAL ANOMALY: The theoretic course of a fourth branchial anomaly is similar to a
third except it passes under the superior laryngeal nerve but over the recurrent laryngeal and
hypoglossal nerves. It then dips back into the chest to pass around the aortic arch on the left and the
subclavian artery on the right. It then ascends to enter the larynx near the cricothyroid joint or
through the lower horn of the thyroid cartilage, through the inferior constrictor, and into the apex of
the piriform sinus19, 22. The key difference between the third and fourth is the relationship to the
superior and recurrent laryngeal nerves. The third will pass superficial to both the superior and
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5985
recurrent where the fourth will pass deep to the superior but superficial to the recurrent. These
anomalies, as in the third, are predominately on the left side, with Nicoucar et al23 reporting 93.5%
DIAGNOSTIC INVESTIGATIONS: CT is the most commonly used radiograph by practitioners1, 15, 20, 23
and used to diagnose cystic lesions.
ULTRASOUND: Ultrasound is easy, fast and does not require sedation or radiation. Branchial cysts
appear as well defined, smoothly outlined and uniformly anechoic lesions with posterior
enhancement24. Studies of diagnostic accuracy vary. Mitroi et al13 report 100% accuracy in the
diagnosis of 23 branchial cysts and sinuses, thus, obviating the need of CT or MRI. Others report
significantly lower accuracy citing operator dependency as a principal cause25, 26. Ultrasound is
limited in its ability to depict hypopharyngeal lesions when compared with CT or MRI25. Nicoucar et
al20 reported a positive predictive value for ultrasound of only 7% for third branchial
COMPUTED TOMOGRAPHY AND MRI: A review of 87 patients who received a preoperative CT for
a reported branchial anomaly revealed a diagnostic accuracy of 95% for cysts, 81% for sinuses and
50% for fistulae1. Several radiologic features of contrast-enhanced CT are typical of branchial clefts
including fat streaking and hypodensity within the ipsilateral thyroid lobe, and gas bubbles along the
tract or within the cyst27. Unfortunately, the ability of CT to chart the entire course of a sinus tract or
fistula is variable. In first branchial anomalies, high-resolution CT effectively shows the relationship
with the external auditory canal and the middle ear6.
MRI is also accurate in detecting branchial cysts but is variable in its effectiveness in
delineation of the fistula or sinus tract28, 29, 30. In regards to first branchial anomalies, MRI allows
assessment of the extent of the lesion, especially within parotid tissue6. Chan et al.7 reported that the
relationship of the facial nerve and the deep portion of the tract was difficult to establish
preoperatively in first branchial anomalies with both MRI and CT. Nicoucar et al.20, 23 report a
positive predictive value for MRI and CT of 84 and 49% for third branchial anomalies and 63 and
46% for fourth branchial anomalies, respectively.
Fistulography, barium swallow, direct laryngoscopy/pharyngoscopy are other investigative
TREATMENT: Historically, definitive treatment of branchial anomalies has been complete surgical
excision. These lesions do not spontaneously regress and often are subject to recurrent infections.
The timing of surgery is controversial and dependent on patient age and infection history. Some 1
recommend early surgical excision at 1 year of age, ideally performed before infections occur and
distort the surgical planes of dissection. Support to this model is given by the increased rate of
recurrence of previously infected anomalies4, 37. This is in contrast to others’ recommendation of
delaying surgery until age 2–34, 17, when adjacent structures are larger and easier to identify.
FIRST BRANCHIAL ANOMALIES: The ideal surgical approach is a superficial parotidectomy with
facial nerve dissection and complete excision of the lesion1, 2, due to the potential intimate and
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5986
variable course of the tract with the facial nerve. In D’Souza’s et al8 literature review, of 87 cases of
excision with facial nerve identification, 21% resulted in temporary nerve paralysis and less than
1% with permanent paralysis. This is compared with 29 and 12%, respectively, in cases in which the
facial nerve was not identified. Case reports of less invasive surgical techniques that bypass
parotidectomy have been reported with acceptable results5, 7, 38.
SECOND BRANCHIAL ANOMALIES: The traditional treatment of second branchial anomalies is
surgical excision of the entire lesion. This involves a transverse cervical incision encompassing the
external sinus opening when present.
During cyst excision, careful exploration for an associated sinus tract must be performed15.
During sinus excision, a second ‘step-laddered’ incision may be required for better exposure near
the pharynx10, 21. Fistula excision may be aided with cannulation of the tract with 2–0 or 3–0
monofilament suture or lacrimal probe15. A sinus tract may be injected externally with methylene
blue; however, care must be taken as extravasation into surrounding tissues may make dissection
more difficult39. Exploration of the carotid sheath to the lateral pharyngeal wall may be undertaken
to fully excise any tract that may not have been identified on preoperative imaging1. Approaches
offering less visible scarring have been described. Roh and Yoon11 approach second branchial
anomalies via a retroauricular incision, which allows excellent visualization with ‘invisible’ external
scarring. Endoscopic excision of second cysts via a retroauricular approach may be employed,
eliminating nearly all visible scarring40.
THIRD AND FOURTH BRANCHIAL ANOMALIES: Treatments for third and fourth branchial
anomalies have historically been by complete surgical excision of the entire tract with thyroid
lobectomy when involved [10&, 19, 22, 23]. Madana et al.21 reported no recurrences with 1–3-year
follow-up after excision of 18 lesions with hemithyroidectomy. Pereira et al.19 describe a combined
approach including direct laryngoscopy with catheter insertion into the piriform defect prior to
formal external surgical excision with no complications or recurrences. A literature review by
Nicoucar et al.20, 23 of third and fourth branchial anomalies revealed a recurrence rate of 94 and 89%,
respectively, after incision and drainage alone. The reported recurrence rate after primary excision
alone was 15% for both third and fourth fistulae. When combined with hemithyroidectomy,
recurrence rate remained the same for third fistulae, but dropped to 8% for fourth. The complication
rate for both lesions was significantly higher when performed on children under 8 years of age.
Complete excision is challenging due to their complex anatomical course10, 41. Care must be taken to
preserve the recurrent laryngeal nerve (RLN), which is often intimately associated with the tract and
easily injured22, 27, 42. Endoscopic techniques have shown successful outcomes with possible
reduction of complications including RLN paralysis. These aim to seal the piriform defect using
several modalities including electrocoagulation with diathermy probe43, 44, low-power diode laser45,
chemical cauterization with trichloroacetic acid46, 47 and silver nitrate stick48 and fibrin glue49.
Leboulanger et al.50, in their review of 20 patients with fourth branchial anomalies treated with laser
endoscopic cauterization, report low failure rates (13%) except in neonates (40%). Nicoucar’s et al.
review20,23 reports that the recurrence rate for endoscopic procedures was 18 and 15% for third and
fourth branchial anomalies respectively, which is almost identical to open excision. No complications
were reported.
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5987
CONCLUSION: Branchial anomalies are common congenital pediatric head and neck lesions but are
comprised by several diverse anomalies. Treatment must be tailored depending on which branchial
arch was involved and whether a cyst mass or sinus/fistula tract is present.
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Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5990
FIGURE 1 (Cyst & Fistula):
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5991
1. Sudhir. M
2. Ramachandra. J.
3. V. Satish.
4. Sridhar. S.
1. Associate Professor, Department of General
Surgery, KIMS Hospital and Research
Center, Bangalore.
2. Professor, Department of General Surgery,
KIMS Hospital and Research Center,
3. Professor and H.O.D, Department of
General Surgery, KIMS Hospital and
Research Center, Bangalore.
Assistant Professor, Department of General
Surgery, KIMS Hospital and Research
Center, Bangalore.
Dr. Sudhir. M,
Associate Professor,
Department of General Surgery,
K.R. Road, V.V. Puram,
Bangalore – 560004.
Email – [email protected]
Date of Submission: 27/07/2013.
Date of Peer Review: 03/08/2013.
Date of Acceptance: 05/08/2013.
Date of Publishing: 07/08/2013.
Journal of Evolution of Medical and Dental Sciences/ Volume 2/ Issue 32/ August 12, 2013
Page 5992