Abstract

BackgroundFirst branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies. We report an even more rare and unique case of a branchial cleft cyst with features of both first and second arch derivatives.Case presentationA 6-year-old boy presented to us with a left conductive hearing loss associated with pre-tympanic keratin debris and an ipsilateral painful cervical mass. He had a past medical history of left ear surgery for presumed cholesteatoma 2 years prior and left neck abscess drainage 6 months prior. CT and MRI revealed a lesion originating in the external auditory canal and extending cervically through a bony canal located medial to the facial nerve and terminating as a parapharyngeal cyst. The complete removal was accomplished in one surgical stage consisting of three distinct steps: robotic assisted transoral resection of the pharyngeal cyst, an endaural approach and a parotidectomy approach.ConclusionWe believe that our detailed description of this rare first branchial cleft cyst with pharyngeal extension, possibly a hybrid case between a first and second branchial cyst, can serve as a valuable tool to Otolaryngologists – Head and Neck Surgeons who come across a similar unusual presentations.

Highlights

  • First branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies

  • Final pathology of the tissue excised from both the ear and parapharyngeal regions was consistent with the diagnosis of a first branchial cleft cyst, revealing cystic spaces filled with keratin debris and lined by stratified squamous epithelium containing pilosebaceous units (Fig. 3a and b)

  • Histopathological analysis of our case is in favor of a first branchial cleft cyst given the absence of both respiratory epithelium and associated lymphoid tissue, two common findings in second branchial cleft cysts [7]

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Summary

Conclusion

We hereby describe an unusual presentation of a first branchial cleft cyst with a pharyngeal extension, possibly a hybrid first and second branchial cyst. Otolaryngologists should keep this possibility in mind when evaluating a child with both otologic and neck symptoms. Thorough pre-operative radiologic workup should aim at establishing the presence of a connecting fistulous tract. Operative management is greatly aided by combining traditional and robotic approaches

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