This study aimed to summarise the clinical features and management strategies concerning auditory canal duplication anomalies in children with congenital first branchial cleft anomalies (CFBCAs), and to provide guidance for precise treatment.

We retrospectively analysed 84 children with CFBCAs who had complete data, diagnosed between December 2018 and February 2024.

All the lesions identified were located around the external auditory canal or near the mandibular angle, manifested as pinhead-sized perforations in 10 cases, painless masses in 18 cases, recurrent swelling and pain with purulent discharge in 52 cases, and otorrhea in 4 cases. Otoscopy examinations revealed external auditory canal swelling in seven children, fistulas within the auditory canal in four children, and a myringa web in three children. Fifty-six children had a preoperative history of infection. Using Work's classification system, Work I and II in 70 (87.5%) and 14 (12.5%) children, respectively. Intraoperatively, 80 (95.2%) children had auditory canal duplication anomalies at the base of the lesion, closely associated with the cartilage of the inferior wall of external auditory canal(EAC), We then classified auditory canal duplication anomalies into three types: Type A (duplication anomalies of epithelial tissue structure between the skin of the EAC and the cartilage of the inferior wall, n = 16 children), Type B (duplication anomalies of the epithelial and/or skin tissue structure, sharing a wall with the cartilage of the inferior wall, n = 40), and Type C (duplication anomalies of the skin and cartilage tissue structure, connected to the cartilage of the inferior wall of EAC, n = 24). Sixty-eight children had lesions superficial to the facial nerve, 12 had lesions deep to the facial nerve, and four had lesions between branches. There were two cases of transient postoperative facial paralysis, three cases of CFBCA recurrence, and two cases of transient auditory canal stenosis.

Auditory canal duplication anomalies are an important feature of first branchial cleft anomalies in children. Precise staging and accurate identification of the base of the lesion facilitate complete removal, thereby increasing the cure rate.

Incomplete obliteration of the branchial apparatus results in the formation of branchial cleft anomalies. First branchial cleft anomalies may persist anywhere in the first branchial arch, from the external auditory canal at the level of the bony cartilaginous junction to the submandibular triangle. The majority of cases present in childhood as an opening in the skin though they may present as cysts or neck masses, mostly mistaken for neck abscesses which leads to inadequate treatment and complications. Here different cases of first branchial cleft anomalies with variable presentation and treatment are illustrated. The need for proper diagnosis and adequate treatment cannot be overemphasized to avoid mismanagement and complications.

To investigate the anatomical relationships between the structures adjacent to the cartilaginous portion of the ear canal in children with Work type I congenital branchial cleft anomalies (CFBCAs) and to develop new classifications and surgical strategies.

Retrospective analysis was performed on 50 children with Work type I CFBCAs admitted between December 2018 and December 2022.

Among the 50 children, total parotidectomy was performed on 49 sides. In 44 cases (88%), the main body of the lesion was closely associated with the cartilage of the inferior ear canal wall. Among these cases, the lesions in 40 cases occurred within the space enclosed by the dorsal inferior wall cartilage, mastoid process, and parotid gland, while in the remaining four cases, the lesions were located between the anterior inferior wall cartilage and parotid gland. Based on the preoperative imaging observations, clinical manifestations, and intraoperative findings, the cases were classified into 6 subtypes (a to f) including 21 cases (42%) of Type Ia (inferior wall of EAC), 7 cases (14%) of Type Ib (bottom wall of EAC), 12 cases (24%) of Type Ic (posterior-inferior wall of EAC), 4 cases (8%) of Type Id (anterior-inferior wall of EAC), 4 cases (8%) of Type Ie (anterior ear wall of EAC), and 2 cases (4%) of Type If (isolated from parotid).

Surgical intervention is the only treatment for first branchial cleft anomalies and a comprehensive understanding of the classifications will help with the precise localisation and excision of the lesions.

Our aim was to explore the clinical value of multimodal imaging examinations in the diagnosis of congenital pyriform fossa fistula in children, so as to provide clues for the early diagnosis and treatment of congenital pyriform fossa fistula.

The clinical and imaging data of 55 children with pyriform fossa fistula diagnosed surgically in our hospital from 2015 to 2018 were analyzed retrospectively. All 55 patients underwent a CT scan. Of those patients, contrast enhancement CT was performed in 47 cases, MRI was performed in 2 cases, and barium esophagography was performed in 41 cases.

Among the 55 cases, there were 24 male patients and 31 female patients. The age ranged from 11 months to 13 years old, and the median age was 3.8 years old. The lesions of 49 cases (89.1%) were located on the left side, and the imaging of CT showed soft tissue mass in the anterior cervical region blurred boundary. There was ipsilateral thyroid involvement in 50 cases (90.9%), trachea and/or carotid sheath extension in 43 cases (78.2%), abscess formation in 39 cases (70.9%), and pneumatosis in 25 cases (45.5%). The CT examination of 22 children after treatment showed a linear or tubular low-density shadow in the thyroid gland, gas accumulation in the anterior cervical region or thyroid, and residual contrast medium, partly. A total of 24 cases underwent barium esophagography during the acute phase, and 15 cases (62.5%) showed sinus formation from the pyriform fossa downward or punctate high-density shadow in the anterior cervical region. The 2 cases where MRI was performed showed abscess formation in one side of the neck and thyroid involvement.

Pyriform fossa fistula is most common in the left anterior cervical region, and it is closely related to the thyroid gland. The plain and enhanced-contrast CT scan can be used as the first choice during the infection stage. It helps to understand the location, extent, and structure of the surrounding tissue. The preliminary diagnosis of pyriform sinus fistula was according to the imaging features. It provided an important basis for clinical diagnosis and reduced the pain caused by repeated infection or surgical incision and drainage.

To investigate the clinical features and surgical outcomes of pediatric congenital first branchial cleft anomalies (CFBCAs).

We conducted a retrospective analysis of 100 children who were referred to Shanghai Children's Hospital from March 2014 to March 2022 for the treatment of CFBCAs.

This study included 100 patients (33 males, 67 females) with an average age of 4.0 ± 2.7 years. 64 cases were type I FBCAs and 36 were type II. The main clinical manifestations included having a skin pit or discharge from it (62%), painless masses (5%), mucopurulent otorrhea (8%) and recurrent swelling with pain (90%) in the Pochet's triangle area. 92% had infection histories, 84% had incision and drainage histories, and 18% had surgical histories. 6 cases of tympanic membranous attachment were found by auricular endoscopy. Ultrasonography (US) was 55.6% (30/54) accurate and enhanced CT was 75% (75/100) accurate in diagnosing CFBCAs. We dissected the facial nerve (FN) in 46% cases. Lesions ended in the external auditory canal (EAC) wall in 86 cases. 69 exhibited close relationship with the parotid. The patients were followed up 0.25-8.2 years. 11 had postoperative temporary facial paralysis and all improved within 6 months. 3 had recurrence and they were secondarily successfully retreated. No EAC stenosis were found.

CFBCAs often presented with repeated swelling and purulence in Pochet's triangle. CT, US and auricular endoscopy can assist in diagnosis and planning the surgical strategy. Complete excision in non-infection stage as soon as possible is the first choice for the treatment of CFBCAs.

First branchial cleft cyst (FBCC) is a rare entity of congenital anomalies in the head and neck area. Dealing with FBCC is a clinical challenge as the condition is frequently forgotten in the differential diagnosis of lateral neck swelling. We report a rare case of unilateral type I FBCC in an 11-year-old boy who presented with a painless and slow-growing preauricular mass masquerading as a benign cystic lesion of the parotid. The lesion was completely removed via surgical excision. Histopathology report confirmed the findings of squamous epithelium‑lined cyst wall, which was a characteristic of a branchial cleft cyst. The combination of good clinical acumen, with the help of radiological correlation, along with a strong degree of suspicion for the condition, facilitates the diagnosis of this condition and hence proper management.

Objective:To summarize the CT features of congenital branchial cleft anomaly in children, to reduce misdiagnosis and improve surgical strategy. Methods:This study enrolled 323 pediatric patients with congenital first branchial cleft anomaly, congenital second branchial cleft anomaly and congenital piriform sinus fistula confirmed by surgery and post-operative histopathology, who was admitted to Shanghai Children's hospital from August 2014 to January 2021., CT imaging data were retrospectively analyzed. Results:A total of 323 children with congenital branchial cleft deformity were included. There were 145 males and 178 females, aged from 22 days to 15 years. 119（119/323, 36.8%） cases were diagnosed as congenital first branchial cleft anomaly. Among them, 96 cases（96/119, 80.67%） were related to the wall of external auditory canal, and 89 cases（89/119, 74.78%） were related to parotid gland. The positive rate of CT examination was 87.4%（104/119）. 57 cases（57 / 323, 17.6%） had congenital second branchial cleft anomaly. Among them, 46 cases（46/57, 80.7%） were related to submandibular gland. The positive rate of CT examination was 84.2%（48/57）. 147 cases（147/323, 45.5%） had congenital piriform sinus fistula, in which 129 cases（129/147, 87.8%） were related to thyroid. The positive rate of CT was 89.1%（131/147）. Conclusion:The CT findings of congenital first branchial cleft anomaly are characterized by lesions in the inferior and/or posterior wall of ipsilateral external auditory canal and parotid gland. The CT features of congenital second branchial cleft anomaly are that the lesion is located on the ipsilateral submandibular gland （posterior and medial）. The CT features of congenital piriform sinus fistula are that the focus is located on the dorsal side of the upper pole of the lateral lobe of the thyroid gland.

First branchial cleft cysts are rare congenital abnormalities of the lateral neck. These anomalies can be a source of recurrent infection and require surgical excision as definitive treatment. This case report details the diagnosis and treatment of a first branchial cleft sinus with an associated duplicate external auditory canal.

A wide spectrum of disorders involves the parotid glands, in infancy and childhood. Acute or chronic inflammatory/infectious diseases are predominant. The first branchial cleft anomalies are congenital lesions that typically manifest during childhood. Tumor lesions are more likely to be benign, with infantile hemangioma the most common in infancy and pleomorphic adenoma the most frequent in childhood. Malignant tumors are uncommon, with mucoepidermoid carcinoma the least rare. Infiltrative parotid diseases are rare and have some pediatric clinical specificities. These common and uncommon disorders of parotid glands during childhood and their imaging characteristics are reviewed.

Objective:To explore the adjacent anatomic relationship and classification of Work Ⅰ congenital first branchial cleft anomaly（CFBCA） in order to guide clinical practice. Method:The data of 48 cases of Work Ⅰ CFBCA with complete data were analyzed retrospectively. Result:All 48 lesions were completely resected. 48 sides（100.0%） were anatomically preserved facial nerve, partial parotidectomy was performed on 33 sides（68.8%）, superficial lobe+deep lobe partial parotidectomy was performed on 9 sides（18.8%）, and 6 sides（12.5%） was not performed parotidectomy. External auditory canal reconstruction was performed on 32 sides（66.7%）; 43 sides（89.6%） underwent the anatomical preservation of the auricular lobe branch of the great auricular nerve; 9 sides（18.8%） was performed selective cervical lymphadenectomy at the same time. 45 sides of Work Ⅰ CFBCA presented irregular cystic or lobulated structure, the lesion was located below and behind the junction of the external auditory meatus bone and cartilage and distributed along the longitudinal axis of the external auditory canal; 3 sides were isolated in superficial lobe parenchyma of parotid gland. All lesions were located on the superficial surface of the main trunk and branches of the facial nerve. The average value of the shortest vertical distance between the lower edge of the lesion and the outlet of the main trunk of the facial nerve at the stylomastoid foramen was 7.2 （0-13.4 ）mm. Based on preoperative images and intraoperative findings, according to the adjacent relation with external auditory meatus , parotid gland and facial nerve, the classification of Work Ⅰ congenital first branchial cleft anomaly is proposed, which is specifically divided into 4 types: C1（posterior wall of the external auditory meatus） 17 cases（35.4%）, C2（inferior wall of the external auditory meatus） 13 cases（27.1%）, C3 （multi wall of the external auditory meatus） 15 cases（31.3%）, C4（isolated from parotid gland parenchyma） 3 cases（6.3%）. Conclusion:Work Ⅰ CFBCA has a close relationship with the adjacent anatomy of the region, and familiar with the classification of Work Ⅰ congenital first branchial cleft anomaly is helpful for the treatment of type and accurate resection.

Congenital cholesteatoma (CC) presents as a white pearl-like lesion behind a normal tympanic membrane (TM), without a history of otorrhea, infection, perforation or previous otologic surgery. Several recent studies provided new data improving this pathology characterization. The aim of this paper is to expand the knowledge about CC and to provide new insights on its pathogenesis.

The study consisted of two main research parts: (1) systematic review and meta-analysis; (2) medical literature review englobing anatomy, histology, embryology and congenital pathology of the ear.

The search strategy identified a total of 636 papers. Seventy retrospective studies were included. A total of 1497 cases were studied and the mean age was 6.58 years, with a male-female ratio of 3:1, 34% were asymptomatic, 26% had hearing loss and 2% had facial dysfunction/paralysis. The overall estimate for antero-superior quadrant involvement was 0.70 [95% confident interval (CI) 0.64-0.76], in the postero-superior quadrant was 0.60 (95% CI 0.52-0.69), in the antero-inferior quadrant was 0.32 (95% CI 0.23-0.41), in the postero-inferior quadrant was 0.38 (95% CI 0.29-0.47), in the attic was 0.53 (95% CI 0.43-0.63) and in the mastoid was 0.33 (95% CI 0.26-0.41). More advanced Potsic stages were present in older patients. The most likely inclusion place seems to be between the pars flaccida and the upper quadrants of the pars tensa.

During the last decades, a substantial improvement in CC diagnosis and management had been achieved. The presented mechanism seems to explain most of middle ear CC.

First branchial cleft anomalies (FBCAs) are rare and often misdiagnosed, which can delay proper management and increase surgical risks. Complete excision often requires parotidectomy with facial nerve dissection. The literature reports that younger patients more often have lesions deep to the nerve with higher rates of nerve injury. We hypothesized that the rate of nerve injury and complications in children with FBCAs was not different in those ≤2 years of age compared to those >2 years of age.

Retrospective review of pediatric patients who underwent resection of histopathologically confirmed FBCAs between 2007 and 2017 at a tertiary care, pediatric hospital. Presenting symptoms, lesion classification, prior procedures, imaging techniques, extent of surgery performed, facial nerve position, and complications were reviewed and compared between patients ≤2 years of age and >2 years of age at time of surgery.

43 cases of FBCAs were included in the study: 12 in the younger group and 31 in the older group. There was no difference between groups regarding the presenting symptoms, gender breakdown, lesion classification, prior procedures performed, or extent of surgery. Lesions were more commonly deep to or running between branches of the facial nerve in the younger group (33.3% vs 9.7%, p = .0496). Rates of postoperative complications and facial nerve weakness were comparable between the younger and older groups (8.3% vs 25.8%, p = .206; 25.0% vs 16.1%, p = .503). In combining the age groups, FBCAs located deep to the facial nerve had increased risk of nerve weakness postoperatively (RR 7.2) and those with a history of prior incision and drainage or resection had increased risk of postoperative complications (RR 2.36). Imaging was obtained on all subjects with accuracy rates of 80-100%.

Presenting characteristics of FBCAs in patients ≤2 years of age and >2 years of age are comparable, but lesions in younger subjects had a greater likelihood of being deep to or coursing between branches of the facial nerve. However, the rates of facial nerve injury and postoperative complications are comparable in younger and older children, owing likely to accurate preoperative imaging and appropriate surgical planning.

Neck masses are a common clinical concern both in adults and children. The differential diagnosis is quite broad, from congenital lesions to malignant tumors. Based on the different pathologic diagnosis, the treatments are varied. To the best of our knowledge, limited reports, which specifically discuss the incidence and distribution of neck diseases in early childhood, have been published. Because of the great population in China, a thorough review of the previous clinical data for pediatric neck masses might be potentially valuable for oral and maxillofacial surgeons, which might provide a deeper understanding and better the accuracy of our clinical diagnosis and management. In this article, a 10-year retrospective review was completed; the whole cases were diagnosed and treated in the same major clinical institute in east China, which might represent as a typical cohort for the patient population and serve as a reference for future clinical management.