First branchial anomaly

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...

INTRODUCTION AND IMPORTANCE: The first branchial cleft (FBC) is an extremely rare occurrence, manifesting as cysts, sinuses or fistula. Among all branchial anomalies, FBC represents 8-10 % of branchial clefts. It accounts for about 17 % of all cervical masses in childhood. FBC can be caused by inadequate removal or closure of the ectoderm. The location of the fistula can be adequately determined by magnetic resonance imaging (MRI). CASE SERIES PRESENTATION: We report three cases from the first branchial cleft fistula (FBCF) in our ENT Department with a good response to surgical treatment...

PURPOSE: Branchial cleft anomalies (BCAs) are common pediatric head and neck lesions; however, only 1-4% involve the first branchial cleft. The rare occurrence of first BCAs, their presentation at a young age, and the possible facial nerve involvement make diagnosis and treatment challenging. METHODS: A retrospective chart review was conducted for children diagnosed with their first BCA between 2000 and 2020. Data on demographics, presenting symptoms, physical findings, imaging features, previous surgery, and treatment outcomes were collected and analyzed...

Objective: This study aims to summarize the data and treatment of 35 children with Work type II congenital first branchial cleft anomalies (CFBCAs) to provide significant insights into the correlation between these anomalies and the facial nerve. Methods: A total of 35 children diagnosed with Work type II CFBCAs who received treatment at the Department of Otolaryngology-Head and Neck Surgery at Shenzhen Children's Hospital from August 2017 to March 2023 were analyzed retrospectively. Pearson chi-square tests and Fisher's exact tests were used to examine the relationship between clinical characteristics and the location of the lesion, which included the superficial and deep surfaces as well as the area between the branches of the facial nerve...

OBJECTIVE: To investigate the clinical significance of the inferior wall cartilage of the auditory meatus in surgical treatment of congenital first branchial cleft anomalies (CFBCAs) in children. METHODS: Twenty children diagnosed with CFBCAs who underwent surgery between December 2018 and June 2022 at our hospital were retrospectively analyzed and classified according to their Work lesion type. The guiding significance of the inferior wall cartilage in the surgical treatment of CFBCAs was summarized by investigating the adjacent relationships of the surgical lesions with the external auditory canal and facial nerve...

The Goldenhar syndrome also known as oculo-auriculo-vertebral dysplasia is one of the rare congenital defects that usually involves the impaired development of structures derived from first and second branchial arches such as ears, eyes, mandible, palate and various other structures of the face along with spinal abnormalities. The severity of Goldenhar syndrome anomalies can range from minor to severe, and patients with modest facial asymmetry to those with a highly evident facial abnormality. The most typical characteristics of this condition are dental ailments and impaired development of the mandible, maxilla, zygomatic, orbital, lips, tongue, and palate...

Branchial cysts are a congenital anomaly in humans and other animal species. In this study, twenty commercially bred slaughtered pigs ranging from 120 to 150 days of age, sourced from different farms and lots, were found to have cysts in the oropharyngeal region at meat inspection despite the absence of clinical signs. Two cysts were selected for histopathological examination. The first cyst was surrounded by fibrous connective tissue and lined by a simple single cell layer of epithelium. The second cyst comprised a squamous pseudostratified to simple stratified epithelium, accompanied by a mild inflammatory infiltrate...

Preauricular sinus is a common congenital external ear anomaly. It occurs due to the incomplete fusion of hillocks of His of the first and second branchial arches. Tuberculosis (TB) is endemic in Malaysia, which imposes a major public health problem. It is caused by Mycobacterium tuberculosis , which causes chronic, recurrent diseases and poor healing of a wound. Pulmonary TB is the most common form of infection, some manifesting as extrapulmonary TB. We share our experience in managing a series of three patients with recurrent tuberculous preauricular sinus abscesses in different age groups...

OBJECTIVE: The ultrasonic diagnosis of cervical and facial cystic masses, as well as cases of missed diagnosis and misdiagnosis, was examined, to improve the diagnosis of branchial cleft anomalies. METHODS: A retrospective analysis was conducted on 17 patients with branchial cleft cyst anomalies, including 11 males and 6 females, aged 12-53 years, with an average age of 33 ± 2 years, were unilateral single. All patients who underwent an ultrasound examination and image storage for retrospective analysis, and both longitudinal and transverse sections were scanned to observe the shape, size, boundary, peripheral relationship, and blood flow signal of the masses...

BACKROUND: Branchial cleft anomalies (BCA) can occur as sinuses, fistulas or cysts. They arise from the first, second, third or fourth pharyngeal cleft due to non-fusion or subinvolution. Mostly, located in Robbin's neck-level II, BCA clinically present as a painless compressible swelling, cutaneous draining sinus, or fistula. AIMS: Surgical treatment is the gold standard to prevent recurrence in BCA, though the necessity of ipsilateral tonsillectomy is discussed and was being examined within this work...

Ear tags or accessory auricles are branchial cleft remnants that clinically appear as asymptomatic nodules or papules in the preauricular region. They occur in various syndromes affecting the first and branchial arches during embryogenesis. The presence of an ear tag can have a psychological impact on one's life due to its unesthetic appearance, thereby affecting their quality of life. Talon cusp usually occurs in the maxillary central or lateral incisor. A fissured tongue or cerebriform tongue is characterized by the presence of horizontal or vertical grooves, usually affecting the dorsum of the tongue...

Congenital anomalies of the external auditory canal (EAC) are classically divided into congenital aural atresia (CAA) and congenital aural stenosis (CAS). CAA can present as an isolated anomaly, unilateral or bilateral, or in the setting of a craniofacial syndrome. Hearing testing (ABR with air and bone conduction thresholds for both ears) early in the perinatal period is important to document hearing thresholds. Hearing status thus informs parent counseling on options for hearing habilitation: Bone conducting technology is a must for children with bilateral CAA to support normal speech and language development...

First branchial cleft anomalies (FBCAs) are rare congenital malformations that require complete surgical removal. A stenosis of the external auditory canal (EAC) may be the consequence of the disease and its treatment. The aim of this study is to present the details and results of an innovative surgical technique using part of the abnormality for reconstruction purposes. This study covered 28 surgically treated children with FCBA between 2014 and 2021. The analysis included the clinical manifestation form of the abnormality, presence of the EAC deformity, histopathological results, complications, and distant results...

PURPOSE: 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. METHODS: Retrospective analysis was performed on 50 children with Work type I CFBCAs admitted between December 2018 and December 2022. RESULTS: Among the 50 children, total parotidectomy was performed on 49 sides...

Commissural or lateral facial cleft (macrosomia), classified at Tessier number 7 craniofacial clefts, is a rare congenital anomaly usually associated with deformities of other structures developed from the first and second branchial arches. It affects the esthetics and functional aspect of the oral cavity. Bilateral transverse cleft occurring alone is uncommon and it's with tracheoesophageal fistula (TEF) has not been reported to the best of our knowledge. We report a case of esophageal atresia (EA) and TEF with macrosomia...

INTRODUCTION & IMPORTANCE: Branchial fistulas and cysts are uncommon anomalies of embryonic development that involve soft tissues of the neck. According to Bailey-Proctor classification, second BCCs are classified into four types: Type-I cysts are situated along the anterior border of the sternocleidomastoid muscle beneath the superficial cervical fascia. Type-II ones are the most common and lie just laterally to great vessels beneath enveloping fascia of the neck. Type-III ones pass between internal and external carotid arteries...

The external ear develops from an organized convergence of ventrally migrating neural crest cells into the first and second branchial arches. Defects in external ear position are often symptomatic of complex syndromes such as Apert, Treacher-Collins, and Crouzon Syndrome. The low set ears (Lse) spontaneous mouse mutant is characterized by the dominant inheritance of a ventrally shifted external ear position and an abnormal external auditory meatus (EAM). We identified the causative mutation as a 148 Kb tandem duplication on Chromosome 7, which includes the entire coding sequences of Fgf3 and Fgf4...

Branchial cleft anomalies are congenital, arising from the first to the fourth pharyngeal clefts. The most common is a second arch anomaly. As it is congenital, it presents at birth though may become symptomatic later. The spectrum of anomalies includes sinus, cyst, or fistula formation or a combination of these. Here we present a case series based on first cleft anomalies. The principles of management include early diagnosis, excision of any fistulous tract, and prevention of injury to the facial nerve.

OBJECTIVES: To evaluate the clinical features of first branchial cleft anomalies (BCAs) and their relationship to pre-operative imaging, pathologic data, and post-operative surveillance outcomes. Additional aims were to assess the validity of the Work classification and describe features of recurrent cysts. METHODS: Records for 56 children (34 females, 22 males; age at surgery of 5.6 ± 4.4 years) collected over a 12-year period (2009-2021) were reviewed...

The pharyngeal arches form the cornerstone of the complex anatomy of the face and neck. These embryologic structures are the foundation of face and neck development, and anomalous growth can result in craniofacial abnormalities. Surgeons who manage head and neck pathology and pathoanatomy will invariably encounter conditions associated with aberrant pharyngeal arch anatomy, and a thorough understanding of the normal and pathological development of these important structures is paramount to accurate diagnosis and treatment...