Indian Journal of Otology

: 2016  |  Volume : 22  |  Issue : 4  |  Page : 291--293

Second type of first branchial cleft anomaly

Hitesh Verma1, Arjun Dass2, Surinder K Singhal2, Nitin Gupta2,  
1 Department of Otorhinolaryngology and Head and Neck Surgery, All India Institute of Medical Sciences, New Delhi, India
2 Department of Otorhinolaryngology and Head and Neck Surgery, Government Medical College and Hospital, Chandigarh, India

Correspondence Address:
Hitesh Verma
Department of Otorhinolaryngology and Head and Neck Surgery, 4th Floor, Teaching Block, All India Institute of Medical Sciences, New Delhi - 110 029


First branchial cleft fistula is a rare congenital malformation of the head and neck with an incidence of <8% of all branchial cleft defects. The patient presenting with discharging sinus in the neck with discharging ear should be investigated for a possible branchial cleft anomaly. Rarity and diverse presentation often lead to misdiagnosis and inadequate treatment. Recurrent infection and scarring make intraoperative identification of facial nerve made very difficult. We report a case of a 5-year-old boy with a first branchial cleft fistula, in which discharge was observed through the fistulous opening on the right side of the face and along the ear canal.

How to cite this article:
Verma H, Dass A, Singhal SK, Gupta N. Second type of first branchial cleft anomaly.Indian J Otol 2016;22:291-293

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Verma H, Dass A, Singhal SK, Gupta N. Second type of first branchial cleft anomaly. Indian J Otol [serial online] 2016 [cited 2021 Sep 22 ];22:291-293
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In the 4 th week of human embryological development, six branchial arch appear as bar in the wall of pharynx which will form the future lower face and neck. [1] In between the branchial arches, the branchial pouches form internally, and the branchial groove develop (cleft) laterally. The external ear canal develops form dorsal part of the first branchial groove. The ventral part of first branchial groove normally disappears but may persist as a preauricular sinus, or a collaural fistula, and it accounts for <8% of all branchial cleft abnormalities. [2] A colloaural fistula is the least common of the first branchial cleft anomalies. The principle of management includes early diagnosis, controlling the infection status, and complete excision of tract with preservation of facial nerve function.

 Case Report

A 5-year-old male child presented with a history of recurrent discharge from opening at the right side of the face and right ear since birth. There was no history of any swelling, redness, and any prior surgery. Local examination revealed a fistulous opening on right side of face 1 cm above the angle of mandible [Figure 1]. The external auditory canal (EAC) was normal on examination no opening seen. The magnetic resonance fistulogram revealed an oblique tract of about 3 cm in size, was extending from skin of neck anteriorly to rounded focal part in posterior aspect of superficial lobe of right parotid gland which lied deep to bony part of external auditory meatus (EAM) posteriorly without any branching [Figure 2]a and b. This tract showed increase intensity on T2-weighted images. The computed tomography (CT) scan showed tract running obliquely running tract passing through the tissue of superficial lobe of right parotid gland [Figure 3]. Consider these clinical and radiological findings; diagnosis of collaural fistula was made. The patient was planned for excision of tract with conservative superficial parotidectomy under general anesthesia. The modified parotidectomy incision was made on the right side; flaps elevated, and landmark for facial nerve explored. The facial nerve identified and the fistulous tract was present lateral to nerve [Figure 4]. The tract was removed with part of skin around the opening on face and EAM, piece of cartilage of EAM and surrounding tissue of superficial lobe of parotid. The part of tract close to EAM was cartilaginous. The negative suction drain was put, and the wound was closed in layers. The facial nerve function was normal in all branches. The patient is under regular follow-up and symptom-free.{Figure 1}{Figure 2}{Figure 3}{Figure 4}


The six branchial arches are the embryologic precursors of the ear and the muscles, blood vessels, bones, cartilage, and mucosal lining of the face, neck, and pharynx. By the 7 th week of development, the arches fuse and the clefts are obliterated. Collaural fistula is the remnant of embryonic development and results from failure of ventral part of the first branchial cleft to involute. [3] It is epithelial lined tract, and it runs from the bony cartilaginous junction of EAM down into the neck where it opens at a point between the angle of mandible and the sternocliedomastoid. The common sites of external opening of fistulas and sinuses are the EAC (40%), upper neck (32.5%), concha (20%), and posterior auricular area (7.5%) whereas in our case external opening was present just above angle of mandible on the right side. The course of fistulous tracts relative to the facial nerve can be superficial (41%), deep (37%), or between the branches (22%). [4],[5] The close relationship of first branchial cleft anomalies to the facial nerve results in frequent complications from surgical treatment. The risk of facial nerve injury increases in patients who have had multiple infections or surgical procedures.

Classification of first branchial cleft anomalies is on the basis of tissue type lining the tract: Type I contains ectoderm, whereas Type II has both ectoderm and mesoderm. [6],[7] On the basis of location of external opening: Type I is adjacent to the EAC, whereas Type II is may pass through the parotid gland in a variable relation to the facial nerve and the fistulaous tract may end either in the cartilaginous EAC or extend to the face or upper neck. At the angle of the mandible. According to this classification, either type is regarded as duplication of either the membranous (Type I) or both the membranous and cartilaginous (Type II) portions of the EAC. In our case, duplication of cartilaginous portion was present.

First branchial cleft anomalies usually come to the clinician's attention because of an external opening or recurrent infections as seen in our case. [5] Diagnosis of collaural fistula is achieved mainly through careful physical examination of both the neck and the EAC first branchial cleft anomalies are seen approximately twice as often in female (69%) as in male (31%). The external opening of fistulas occurs more frequently on the left side (64%); sinuses whereas in our case it was on right side. [8] The study by Triglia et al. showed fistula opening in EAM in 44% of the patients and asymptomatic membranous attachment between the floor of the EAC and the tympanic membrane was found in 10% of the cases. [9]

The high variability in the course of these lesions and the necessity of complete excision during treatment makes radiographic visualization crucial to directing treatment. Radiological examinations can be helpful diagnostically, to determine the typical anatomical localization, the extent, the size, and the relationship of the lesions to surrounding structures. Fistulas and sinuses are not always obvious on a CT scan, and a fistulogram can be useful. [10] The adverse side effect from the administration of contrast media varies from minor physiological changes to some serious life-threatening situation. [11] The high-resolution-CT imaging shows its exact relationship with the EAC and the middle ear. [12] The magnetic resonance imaging allows assessment of the extent of the anomaly, especially in the parotid area and branching pattern. Despite this, there are few reports of radiographic analyses of first branchial cleft anomalies.

Surgical excision of the entire fistulous tract with resection of a small amount of skin and cartilage within the EAM is the treatments of choice. [13] To avoid damage to the facial nerve a conservative superficial parotidectomy with full exposure of the facial nerve [5] is required. The incidence of temporary facial nerve palsy was 21% in the patients in whom the nerve was identified and 29% in the patients in whom the facial nerve was not identified. The incidence of permanent facial paralysis was 1% in the patients in whom the nerve was identified and 12% in the patients in whom the nerve was not identified. [14]

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