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Introduction: Branchial fistulas and cysts are congenital abnormalities that generally occur on the lateral aspect of the neck and predominantly arise as remnants of a branchial cleft. We report herein a case of a patient with a residual first branchial cleft with a branchial fistula on the midline neck and a branchial cyst in the sublingual space. Case presentation: A 4-month-old boy presented to our institute with a history of white secretion drain from the midline neck beginning 1 week prior to presentation. A cutaneous fistula was identified on the midline of the neck. Radiography of the fistula following injection of contrast agent revealed that it was connected to the cyst near the left jaw. Excision of the cyst and fistula was performed, and histopathological examination confirmed a branchial cyst with a branchial fistula. Conclusion: Our experience with the present case suggests that branchial cysts should be differentiated from diseases that form cutaneous fistulas on the midline neck with cystic lesions in the sublingual space. It is important to perform preoperative imaging and complete resection without any remnants to prevent recurrence.

Branchio-oto-renal syndrome (BOR), also known as Melnick-Fraser Syndrome, is an autosomal dominant genetic disorder resulting from a mutation of the EYA-1 gene and characterized by branchial cysts or fistulas, ear anomalies, hearing loss, agenesis or hypoplasia of the kidneys, and chronic renal disease. We describe a unique surgical case of a branchial cleft fistula excision and concurrent ipsilateral cochlear implantation in a pediatric patient with BOR. A 2-year-old female presented with a right draining second branchial cleft fistula and severe bilateral profound sensorineural hearing loss due to bilateral cochlear dysplasia and cochlear nerve hypoplasia. She had previously undergone a left cochlear implant and was diagnosed with BOR. Her parents were interested in a sequential right cochlear implant as she was demonstrating excellent function with her left implant. A computed tomography fistulogram with an injection of contrast dye into the external orifice of the draining structure revealed a fistula connecting the small pit in the right lateral neck to the area of the tonsillar fossa in the oropharynx suggesting the presence of a second branchial cleft anomaly. She underwent simultaneous tonsillectomy, right branchial fistula excision, and right cochlear implant surgery under one exposure to general anesthesia. This is a unique case where special consideration must be taken with the order of procedures, consolidation or separation of the surgeries, and the use of monopolar cautery.

Branchial arch anomalies are one of the most common congenital anomalies and are usually unilateral in nature. The bilateral occurrence of more than one anomaly is not only rare, but its presence along with hearing loss results in branchiootic syndrome, which has important clinical and genetic implications. Branchiootic syndrome is a part of the spectrum of branchiootorenal syndrome, which is a rare autosomal dominant condition with incomplete penetrance characterized by the malformations of the external, middle, and inner ear, hearing loss, branchial fistulae, and renal abnormalities. We shall be discussing two such rare cases in this article.

Although 2nd Branchial arch fistulae (from incomplete closure of Cervical sinus of His) are well known, 1st arch fistulae are much rarer (

Branchial fistula is a congenital anomaly resulting from failure of closure of one of the four branchial clefts. It is usually a clinical diagnosis, but dermoscopy helps in highlighting its features, thereby avoiding the need of imaging studies and other invasive diagnostic procedures. Here, we report a case of 3-year-old female patient who presented with asymptomatic tiny papule on the neck since early childhood. Dermoscopy was done to describe the features of branchial fistula and cleft.