Indian Journal of Otology

: 2017  |  Volume : 23  |  Issue : 1  |  Page : 59--61

First branchial cleft anomaly presenting as recurrent postauricular cyst

Prakriti Shukla, Uroos Fatima 
 Department of Pathology, Era's Lucknow Medical College and Hospital, Lucknow, Uttar Pradesh, India

Correspondence Address:
Prakriti Shukla
Department of Pathology, Era's Lucknow Medical College and Hospital, Lucknow - 226 003, Uttar Pradesh


First branchial cleft anomalies are a special group of congenital malformations that represent <10% of all branchial cleft anomalies. They may present as cysts, swellings, or fistulas in the periauricular region or in the lateral aspect of the neck. Failure to identify these lesions can lead to delay in the diagnosis, often misdiagnosis, inadequate treatment, and subsequent recurrence. We report a case of 15-year-old girl who presented with recurrent postauricular cyst. Fine-needle aspiration biopsy was performed, and it was diagnosed as infected cystic lesion. On histopathological examination, the diagnosis was confirmed as first branchial cleft cyst.

How to cite this article:
Shukla P, Fatima U. First branchial cleft anomaly presenting as recurrent postauricular cyst.Indian J Otol 2017;23:59-61

How to cite this URL:
Shukla P, Fatima U. First branchial cleft anomaly presenting as recurrent postauricular cyst. Indian J Otol [serial online] 2017 [cited 2022 Jul 2 ];23:59-61
Available from:

Full Text


First branchial cleft anomalies result from incomplete fusion of the cleft present between the first and second branchial arches.[1] These congenital malformations are seen in the anterolateral region of head and neck.[2] They are so uncommon that their occurrence is often misdiagnosed. They account for <10% of all branchial cleft anomalies.[3] Clinical presentation is usually in the first and second decades of life.[4],[5] This developmental anomaly mostly presents as cyst, sinus, or fistula pre- or post-auricularly.[1] First, branchial cleft anomalies usually arise in relation to external auditory canal (EAC), necessitating a thorough otological examination. These cysts are prone to get infected and, therefore, recur following excision.

 Case Report

A 15-year-old girl presented to the ENT outpatient clinic with a recurrent postauricular swelling behind right ear for the last 5 years. The swelling was associated with fever of low grade. There was no history of ear discharge, hearing loss, weight loss or loss of appetite, and trauma. She was on medications for some time and underwent incision and drainage procedure on two occasions, but the swelling recurred within a span of 6 months. On physical examination, a lobulated and cystic swelling was seen behind the right ear measuring 3 cm × 3 cm in size. Overlying skin was normal [Figure 1].{Figure 1}

Fine-needle aspiration biopsy of the swelling was performed which yielded thick, gelatinous, and pale yellow colored material. Smears were prepared and stained with Leishman and hematoxylin and eosin. Microscopically, smears revealed scattered benign squamous cells, clusters of columnar cells, and moderate lymphocytic infiltrate on a myxoid background. These were admixed with fair number of histiocytes and macrophages along with polymorphs and multinucleated giant cells. Clusters of spindle-shaped fibroblasts were also seen [Figure 2]a, [Figure 2]b, [Figure 2]c, [Figure 2]d. A tentative diagnosis of infected cystic lesion(remnant branchial cleft) was given. Surgical excision of the cyst was carried out, and the specimen was sent for histopathological examination.{Figure 2}

Grossly, a grayish-white cystic mass was seen measuring 3 cm × 2.5 cm × 2 cm in size. The outer surface was smooth and lobulated. Cut surface showed multilocular cyst with smooth wall, and the lumen was filled with gelatinous material. Microscopically, cyst lined by stratified squamous epithelium was visualized. The subepithelial zone showed moderate lymphocytic infiltrate [Figure 3]a. Deeper tissue revealed numerous acini lined by mucus secreting epithelium. Both intraluminal and extracellular mucin pools were noted [Figure 3]b.{Figure 3}


The branchial apparatus consists of the branchial arches, pharyngeal pouches, branchial grooves, and branchial membranes. During the 4th week of intrauterine development, branchial apparatus begins to develop when six pairs of branchial arches appear that are separated by five clefts externally and five pouches internally. By the end of the 7th week, the clefts are obliterated and the arches fuse.[1] Branchial arches develop into the musculoskeletal and vascular components of the head and neck. Branchial pouches develop into the middle ear, tonsils, thymus, and parathyroid glands. The first branchial cleft develops into the EAC whereas the second, third, and fourth branchial clefts merge to form the sinus of His (this normally involutes).

First branchial cleft anomalies arise due to incomplete closure of the ectodermal portion of the first branchial cleft.[1] Whether the defect is a fistula, sinus, or cyst depends on the degree of the closure. The most common head and neck manifestation is swelling in the periauricular (24%), parotid (35%), or cervical region (35%).[6] In practice, it is cumbersome to diagnose first branchial cleft anomalies without surgical exploration.

In 1972, Work [5] proposed a histological classification of these lesions. He classified first branchial cleft anomalies into two groups. Type I anomalies present as a cystic mass and are purely ectodermal, which contain squamous epithelium but no cutaneous adnexal structures, or cartilage while Type II anomalies present as a cyst, sinus, or fistula and are of ectodermal and mesodermal origin containing squamous epithelium with cutaneous adnexal structures or cartilage.[5] Our patient presented with a cystic swelling and on microscopy only ectodermal component was observed. Thus, it was categorized as Type I branchial cleft anomaly.

First, branchial cleft anomalies are very rare, with Type II cysts more common than Type I. Most presentations occur before the age of 10 years and must be considered in the setting of recurrent parotid infections.[4],[5] They are approximately twice as common in woman than in men as in our case.[7] Fistulas occur more frequently on left side, but sinuses show no side preference.[7] These are rarely found in association with other facial malformations.[8] Yalçin et al.[9] described a case of first branchial cleft lesion that was associated with cholesteatoma of mastoid and middle ear, microtia, and aural atresia. Sometimes, in the long course, it can turn into squamous cell carcinoma. However, in the present case, there was no such association observed. Differential diagnosis includes dermoid cyst, cystic hygroma, nodular fasciitis, and metastatic squamous cell carcinoma.

Computed tomography with contrast is a useful tool to identify the tract close to the EAC and its extension into the middle ear. A fistulogram is useful in case of a sinus or fistula without the signs of inflammation or cyst formation.[10]

The treatment of choice is surgical exploration and complete surgical excision. Prompt diagnosis and treatment are essential to avoid recurrent infections and development of secondary fistulous tracts.[11]


Branchial cleft cysts are benign lesions; however, superinfection, mass effect, and surgical complications account for morbidity. The lining of these cysts and fistulous tract is usually squamous epithelium, but ciliated columnar epithelium is also common, along with abundant lymphoid tissue. They may also contain serous, mucinous, or seromucinous fluids. Squamous cell carcinoma is an extremely rare occurrence in these cysts. These anomalies are usually misdiagnosed and inappropriately treated. Thus, first, branchial cleft anomalies should always be considered in the setting of lesions arising near parotid gland or high in the lateral aspect of neck.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1Benson MT, Dalen K, Mancuso AA, Kerr HH, Cacciarelli AA, Mafee MF. Congenital anomalies of the branchial apparatus: Embryology and pathologic anatomy. Radiographics 1992;12:943-60.
2Chavan S, Deshmukh R, Karande P, Ingale Y. Branchial cleft cyst: A case report and review of literature. J Oral Maxillofac Pathol 2014;18:150.
3Ford GR, Balakrishnan A, Evans JN, Bailey CM. Branchial cleft and pouch anomalies. J Laryngol Otol 1992;106:137-43.
4Arnot RS. Defects of the first branchial cleft. S Afr J Surg 1971;9:93-8.
5Work WP. Newer concepts of first branchial cleft defects. Laryngoscope 1972;82:1581-93.
6Ku WY, Wang KJ, Jou YL, Chang YH, Chou CS. Type II first branchial cleft anomaly – A case report. Tzu Chi Med J 2005;17:357-60.
7D'Souza AR, Uppal HS, De R, Zeitoun H. Updating concepts of first branchial cleft defects: A literature review. Int J Pediatr Otorhinolaryngol 2002;62:103-9.
8Triglia JM, Nicollas R, Ducroz V, Koltai PJ, Garabedian EN. First branchial cleft anomalies: A study of 39 cases and a review of the literature. Arch Otolaryngol Head Neck Surg 1998;124:291-5.
9Yalçin S, Karlidag T, Kaygusuz I, Demirbag E. First branchial cleft sinus presenting with cholesteatoma and external auditory canal atresia. Int J Pediatr Otorhinolaryngol 2003;67:811-4.
10Goudakos JK, Blioskas S, Psillas G, Vital V, Markou K. Duplication of the external auditory canal: Two cases and a review of the literature. Case Rep Otolaryngol 2012;2012:924571.
11Chan KC, Chao WC, Wu CM. Surgical management of first branchial cleft anomaly presenting as infected retro-auricular mass using a microscopic dissection technique. Am J Otolaryngol 2012;33:20-5.