OBJECTIVE: To discuss the embryology, classification, clinical experience with, and management of first branchial cleft anomalies. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with a first branchial cleft anomaly. INTERVENTION: Surgery or revision surgery. MAIN OUTCOME MEASURES: Classifications according to Work, Olsen, Chilla; previous diagnostic and therapeutic pitfalls; outcome of intervention (including facial nerve function). RESULTS: Between 1984 and 1999, first branchial cleft anomalies were diagnosed in 18 patients. Surgical treatment was the treatment of choice. The authors' approach in Work type I and type 2 lesions is described, and surgical aspects of revision surgery are discussed. The importance of early establishment of the relationship of the anomaly to the facial nerve is stressed. In 8 patients, previous surgical attempts had been undertaken without establishment of the diagnosis first. After intervention, the outcome was favorable. CONCLUSIONS: First branchial cleft anomalies occur sporadically in ordinary clinical practice. They may go unrecognized or may be mistaken for tumors or other inflammatory lesions of in the periauricular region. However, the distinct clinical features, which can be derived from embryologic development, usually lead to the correct diagnosis. This avoids both treatment delay and eventual failure.
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