First branchial cleft anomalies are uncommon paediatric congenital lesions that may present as persistent pre-auricular or post-auricular sinuses and can involve the parotid region and facial nerve. We present a single-centre 10-year experience describing presentation, imaging, management and outcomes. Retrospective review of a prospectively collected local dataset of paediatric patients with first branchial arch cleft cysts/tracts who underwent evaluation and surgery at a tertiary paediatric centre. Data elements included age at presentation, presenting symptoms, imaging modality, surgical treatment, postoperative complications, facial nerve dysfunction, fistula course, recurrence and follow-up. Eleven patients were included (mean age 5.55 years, median 4 years). The commonest presentation was a persistent sinus with purulent discharge (9/11). Laterality was predominantly left-sided (8/11). MRI was the most used imaging modality (8/11). Postoperative wound infection occurred in 3/11 patients (27%); marginal mandibular branch weakness was recorded in 2/11 (both documented as transient/resolved). One patient had documented recurrence. The fistula/tract most commonly tracked from level II to the ear canal (9/11). In this paediatric series, first branchial arch cleft anomalies most commonly presented as persistent left-sided sinuses with purulent discharge. MRI was commonly used for preoperative assessment. Surgical excision was associated with wound infection in a minority and transient marginal mandibular weakness in several cases. Larger multi-centre series with systematic prospective follow-up are needed.

To discuss the clinical manifestations and difficulties in the diagnosis and treatment of Type Ⅱ first branchial cleft anomaly with parotid fistula，to summarise experience，to reduce misdiagnosis and missed diagnosis，and to select surgical methods accurately. A comprehensive literature review was performed, and the management of one patient with this rare combination was retrospectively analyzed. Particular attention was paid to presenting symptoms, ancillary imaging findings, and surgical pearls. Complete pre-operative evaluation, accurate anatomical delineation, and individualized surgical planning based on clinical and radiological features are essential for successful treatment and avoidance of recurrence. 摘要: 探讨Ⅱ型第1鳃裂畸形合并腮腺瘘临床表现及诊治难点，总结经验，减少误诊及漏诊，精准选择手术方式。阅读文献并对1例Ⅱ型第1鳃裂畸形合并腮腺瘘患者的诊疗过程进行总结，分析该患者的临床表现、辅助检查结果及手术关键点。Ⅱ型第1鳃裂畸形合并腮腺瘘较为罕见，术前完善检查、明确诊断，正确根据患者症状、体征及术前影像学检查选择适合的手术方式非常重要。.

We present an unusual type I congenital first branchial cleft anomaly (CFBCA) characterized by a distinct sinus tract in the superior wall of the cartilaginous external auditory canal (EAC), classified as the sinus variant of type I congenital first branchial cleft anomalies (CFBCAs). This study aims to characterize its distinctive clinical features and present management approaches for this rare entity. Medical records of patients undergoing excision for sinus variant of type I CFBCAs between January 2007 and September 2023 (a 16-y period) were retrospectively analyzed. Clinical characteristics, therapeutic interventions, histopathologic diagnoses, and long-term outcomes were systematically catalogued. The sinus variant of type I CFBCAs is a rare manifestation of CFBCAs, accounting for 11 of 188 cases (5.9%) in our series. Cardinal clinical features included recurrent periauricular inflammation (9/11 cases, 81.8%), with all cases demonstrating a characteristic concealed sinus orifice at the superior cartilaginous EAC (11/11 cases, 100%). All patients (11/11 cases, 100%) were initially misdiagnosed as lymphadenitis, epidermal cysts, or localized infections. The concealed sinus orifices at the superior cartilaginous EAC were frequently overlooked, with definitive diagnosis established by visual inspection in only 3 patients (3/11 cases, 27.3%). In the remaining 8 patients (8/11 cases, 72.7%), occult sinus orifices were identified through combined palpation and visual inspection. All lesions originated exclusively from the superior cartilaginous EAC (11/11 cases, 100%) and maintained a relative simple relationship with the facial nerve trunk (11/11 cases, 100%). Complete surgical excision of the sinus tract along with any adherent periauricular cysts was determined to be the optimal treatment approach. Clinicians should maintain a high index of suspicion for the sinus variant of type I CFBCAs when evaluating pediatric patients with recurrent preauricular inflammation. The identification of a characteristic sinus orifice at the superior cartilaginous EAC during focused examination is diagnostic. Level 4.

Background: Congenital cheek sinuses are exceedingly rare craniofacial anomalies, typically present at birth and often misdiagnosed due to their resemblance to dermal pits or dimples. Only a few cases have been reported in the literature. This case is notable for its histopathological confirmation as a type II first branchial cleft anomaly containing an accessory tragus, highlighting the embryologic and clinical overlap between congenital cheek sinuses and branchial cleft anomalies. Case presentation: We report a three-year-old boy presenting with a congenital dimple along the right nasolabial fold. The lesion was present since birth and occasionally discharged clear fluid but had no history of infection or inflammation. Imaging demonstrated a subcutaneous tract without communication to the oral cavity or parotid gland. Under general anesthesia, surgical excision was performed, and the sinus tract was dissected in continuity to a cartilaginous remnant. Histopathologic analysis confirmed the presence of an accessory tragus consistent with a type II first branchial cleft anomaly. The patient's postoperative recovery was uneventful, and at three months' follow-up, there was no recurrence and an excellent cosmetic outcome. Conclusions: This case emphasizes the importance of considering branchial cleft anomalies in the differential diagnosis of congenital cheek sinuses. Although these lesions may appear benign and superficial, histopathological confirmation is critical for accurate classification. Complete surgical excision remains the definitive treatment, ensuring both diagnostic clarity and favorable aesthetic results. Increased awareness of such rare anomalies can improve diagnostic accuracy and surgical planning, particularly with respect to their potential proximity to critical facial structures.

Herein described is an eight-year-old female patient presenting with bilateral external ear canal duplication and a history of recurrent postauricular abscesses, otorrhea, and moderate left-sided conductive hearing loss. Previous incision and drainage of the postauricular abscesses yielded little long-term success. Medical history was otherwise unremarkable. Physical examination revealed a cystic mass in the right ear and postauricular fibrosis, likely a result of previous surgical interventions. An audiogram revealed asymmetric hearing function, with mild-to-moderate conductive hearing loss in the left ear contrasting normal hearing in the right ear, while tympanometry confirmed an intact right tympanic membrane but was precluded on the left due to purulent otorrhea. A head CT scan revealed postauricular soft tissue swelling and well-pneumatized mastoid air cells. Both ears were explored intraoperatively via postauricular incisions. Surgical excision included removal of a cyst, cartilaginous remnants, and fibrotic tissue. The patient was followed up to assess for postsurgical complications. The surgical site healed uneventfully; no new symptoms or recurrences of past symptoms of otorrhea, cyst formation, or abscesses had developed within the twelve weeks post-surgery. Although external ear canal duplications are rare, it is imperative that early, accurate diagnoses be made to ensure optimal patient outcomes.