Branchial Cleft Anomalies Research Articles

Analysis of Clinical Diagnosis and Treatment Modes for Congenital Branchial Cleft Anomalies.

The objective of this study was to investigate and assess the clinical data of 123 patients diagnosed with congenital branchial cleft anomalies (CBCAs), to summarize pivotal aspects concerning their clinical diagnosis and treatment process. The authors conducted a retrospective analysis of 123 patients who underwent surgical intervention for CBCAs at our institution between August 2005 and September 2021. The clinical demographic characteristics of the patients, primary symptoms, treatment chronology, preoperative diagnostic assessments, surgical strategies, occurrences of postoperative complications, and rates of recurrence were subjected to statistical analysis. Among the enrolled patients, there were 43 cases (34.9%) of congenital first branchial cleft anomalies (CFBCA), 76 cases (61.8%) of congenital second branchial cleft anomalies (CSBCA), and 4 cases (3.3%) of congenital third branchial cleft anomalies (CTBCA), with no cases of congenital fourth branchial anomalies (CFBA). Notably, among all cases, 43 anomalies were situated in the upper one-third of the sternocleidomastoid muscle, while 80 anomalies were located in the lower one-third. Different surgical approaches were selected for patients based on the specific type of anomaly presented. Following surgery, there was recurrence in 14 cases, with factors such as patient age, clinical categorization, lesion type, and history of preoperative infection and surgical intervention identified as primary risk factors for it. CBCAs represent comparatively uncommon disorders affecting the head and cervical regions in clinical practice. Diagnostic modes such as ultrasonography and lipiodol contrast radiography can be used for accurate diagnosis, with surgical intervention serving as the primary therapeutic method.

Comprehensive management and classification of first branchial cleft anomalies: An International Pediatric Otolaryngology Group (IPOG) consensus statement

ObjectiveFirst branchial cleft anomalies are rare congenital head and neck lesions. Literature pertaining to classification, work up and surgical treatment of these lesions is limited and, in some instances, contradictory. The goal of this work is to provide refinement of the classification system of these lesions and to provide guidance for clinicians to aid in the comprehensive management of children with first branchial cleft anomalies. Materials and methodsDelphi method survey of expert opinion under the direction of the International Pediatric Otolaryngology Group (IPOG) was conducted to generate recommendations for the definition and management of first branchial cleft anomalies. The recommendations are the result of expert consensus and critical review of the literature. ResultsConsensus recommendations include evaluation and diagnostic considerations for children with first branchial cleft anomalies as well as recommendations for surgical management. The current Work classification system was reviewed, and modifications were made to it to provide a more cogent categorization of these lesions. ConclusionThe mission of the International Pediatric Otolaryngology Group (IPOG) is to develop expertise-based recommendations based on review of the literature for the management of pediatric otolaryngologic disorders. These consensus recommendations are aimed at improving care of children presenting with first branchial cleft anomalies. Here we present a revised classification system based on parotid gland involvement, with a focus on avoiding stratification based on germ layer, in addition to guidelines for management.

Clinical features and management strategies concerning auditory canal duplication anomalies in children with congenital first branchial cleft anomalies.

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.

Case Report: A case of metastasis to branchial cleft cyst from papillary thyroid cancer

Branchial cleft cysts are frequently encountered congenital anomalies, arising from the first to fourth pharyngeal clefts and second branchial cleft anomalies are the most common. These anomalies, even though present from birth, become symptomatic only later in life. Majority of them are benign in nature. However, these cysts can get secondarily infected or can harbour secondary metastases and sometimes even primary malignancy in very rare cases. Here we discuss a case of a middle-aged female presenting with a gradually increasing branchial cleft cyst with incidental thyroid lesion on ultrasonography, later proven to be papillary thyroid carcinoma with metastatic spread to the brachial cleft cyst.

A Novel Classification System and Surgical Strategies of First Branchial Cleft Anomalies.

To define a novel classification of first branchial cleft anomalies (FBCAs) based on the relationship between lesions and the facial nerve in terms of radiographic imaging findings and to introduce the corresponding surgical managements. The clinical data were retrospectively reviewed. Novel classification was proposed according to the head-neck MRI findings and surgical records. FBCAs limited in the cartilaginous segment of external auditory canal (EAC) or superficial parotid gland capsule were classified as type A. Lesions close to the FN and(or) involved into the parotid gland with no scar formation and no previous parotidectomy were classified as type B. FBCAs adhered to the FN and(or) invaded the parotid gland with scar formation due to previous surgery were classified as type C. Appropriate surgery approaches was also described, which was correlated with classification. Fifty-one patients were included, and one patient suffered from bilateral lesions. Thirty-one, twelve, and nine lesions were classified as type A, type B, and type C FBCAs, respectively. One type A patient (1.92%) suffered from recurrence during follow-up. Temporary facial palsy (House-Brackmann II) was identified in 2 type C patients (3.85%) after surgery and recovered to normal within 2 months. One type B patient (1.92%) suffered from facial paralysis due to the FN injury and underwent facial nerve graft simultaneously. No patients with type C complained of hearing loss postoperatively. This novel classification clearly illustrates definitely relationship between lesion and the facial nerve and appropriate surgical strategies were proposed based on the novel classification. 4 Laryngoscope, 134:4246-4251, 2024.

A Case of Auricle Deformity With 2nd Branchial Cleft Anomaly in Patient With Branchio-Otic Syndrome

Branchio-oto-renal (BOR) Syndrome is a rare autosomal dominant genetic disorder characterized by features such as branchial arch anomalies, hearing loss, preauricular fistula, auricular malformations, and renal abnormalities. Also, BOR syndrome without renal anomalies is referred to as branchio-otic syndrome. In this report, we present a case of a 50-year-old female patient with bilateral type 2 branchial arch anomalies, both preauricular fistula, bilateral inner ear malformations, bilateral sensorineural hearing loss, and right auricular malformation. The patient underwent surgical treatment. This report is the first documentation in Korea of auricular malformation in BOR syndrome, where the patient’s family exhibited a typical autosomal dominant inheritance pattern.

Management of a recurrent third branchial arch fistula using combined endoscopic and open approach

Branchial cleft anomalies are rare, congenital lesions, that occur due to the maldevelopment of the branchial apparatus, during fetal maturation. Anomalies of the third and fourth arch contribute to a very small proportion of these. Most cases are detected in childhood, either as a cyst, sinus or fistula in the neck. Some of the common treatment approaches to third branchial cleft fistulas include endoscopic cauterization, open cervical fistulectomy and, in cases of recurrence, selective neck dissection. Here, we present a case of recurrent branchial fistula of the third arch, wherein we used a combined cervical and intraoral approach, conserving the thyroid cartilage, to achieve a complete excision of the fistula tract.

Management of first branchial anomalies in children: 20 years of experience.

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. 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. The cohort included 17 patients with a median age of 5 years at presentation. Seven (41%) had undergone previous surgical intervention before definitive surgery. Eight were classified as Work Type II anomalies, and nine as Work Type I. Sixteen patients (94%) underwent definitive surgical excision at a median age of 6.9. A parotid approach was used in 10 (62%), with dissection of the mass from the facial nerve, and a retro-auricular or end-aural approach was used in 6 (38%). Complete excision was achieved in 14/16 patients (88%). Three patients had transient facial nerve paresis postoperatively. Recurrence was noted in 3/16 patients (18%). Enhancement in imaging was positively correlated with post-operative complications (R = 0.463, P = 0.018). First, BCA poses a diagnostic and surgical challenge; thus, definitive surgical treatment is often delayed. The surgical approach should be tailored to the type of anomaly (Work type I or II) and possible facial nerve involvement. Risk factors for post-operative complications are a history of recurrent infections and previous surgical interventions. The presence of contrast enhancement in preoperative imaging should alert surgeons to perioperative challenges and the risk of post-operative complications.

Appropriate imaging in the management of first branchial cleft anomalies

Background: First branchial cleft anomalies (FBCA) are rare clinical entities of the head and neck.The low incidence and varied presentation often result in misdiagnosis and inappropriate treatment.Correct diagnosis is essential for proper management, while an incorrect diagnosis will often lead toinadequate treatment. A good understanding of the regional anatomy and embryology can lead to anearly diagnosis and thereby effective management of FBCA. Purpose: To present how to diagnose andmanage FBCA. Case report: A case of a 6-year-old female who had an FBCA with a history of swellingand recurrent discharge from the fistula in the infra-auricular area. Complete excision of the tract wasperformed without facial nerve complication. Clinical question: What is the appropriate imaging fordiagnosis FBCA? Review method: Scoping review was done to identify the scientific evidence aboutimaging for diagnosis FBCA. Systemic searching in 4 databases (PubMed, Embase, Proquest, and Webof Science) using keywords “first branchial cleft”, “anomaly”, and “imaging”. Result: Three article wasfound relevant with the topic of imaging for diagnosis FBCA. Conclusion: Proper diagnosis of FBCAcan lead to proper management and good results. Imaging can provide an anatomical picture of eachbranchial arch anomaly, which can be very helpful in preoperative planning to determine a definitivesurgical approach. Early management of FBCA can reduce the recurrence rate significantly.Keywords: first branchial cleft, anomaly, children, imaging

РЕДКИЙ СЛУЧАЙ АНОМАЛИИ РАЗВИТИЯ НАРУЖНОГО УХА: УДВОЕНИЕ НАРУЖНОГО СЛУХОВОГО ПРОХОДА

Congenital malformations of the hearing organ occur with a frequency of 1–2 per 500 (mild anomalies) to 1–2 per 10000 (severe anomalies) newborns. Up to 15% of them are heritable and up to 10% are bilateral. Anomalies of the first branchial cleft from which the structures of the external ear predominantly develop, are quite rare malformations (less than 10% of all gill anomalies), but can present a significant problem of differential diagnosis. Most commonly, these anomalies manifest clinically as periauricular cysts, fistulous or reduplication of the external auditory canal. There are both isolated congenital anomalies of any of the parts of the hearing organ and syndromal ones. This article describes our own observation of a congenital anomaly of the external auditory canal in a child (a type of reduplication of the external auditory canal) and secondary cholesteatoma on this background. This case is interesting because the patient had multiple congenital anomalies: branchial cysts on both sides for which she had been operated on earlier and reduplication of the external auditory canal, a much rarer pathology of the first branchial cleft. At the same time, the difference in time when these anomalies manifested and were detected was almost 10 years.

Collaural Fistula on the Left-side of the Face: A Case Report

Collaural fistula, also known as cervico-aural fistula, is a rare anomaly that accounts for less than 8% of all branchial cleft anomalies. These fistulas result from incomplete closure of the first branchial cleft during embryonic development. A 21-yearold female exhibited depression on the left-side of her face since childhood, accompanied by a fistula. She had a history of intermittent discharge and a tuft of hair emerging from it. Additionally, the patient reported left ear discharge and an opening in the floor of the External Auditory Canal (EAC). Diagnostic imaging, including X-ray fistulogram, ultrasound, and Magnetic Resonance Imaging (MRI) neck fistulogram, played a crucial role in confirming the diagnosis of a collaural fistula. Surgical intervention involved excision of the fistulous tract and reconstruction of the EAC floor. The patient received antibiotic therapy, resulting in successful postoperative outcomes. The present case highlights the importance of recognising and appropriately managing collaural fistulas. When these fistulas are discovered early, along with a solid grasp of regional embryology and anatomy, there is a chance for better treatment outcomes.

Doxycycline sclerotherapy in paediatric branchial cleft sinus

Branchial cleft anomalies are the second most common head and neck lesion in children after thyroglossal cyst. They are congenital lesions resulting from improper involution of the branchial apparatus. The most common treatment of choice is surgical excision, which gives a good outcome. Nevertheless, there has been recent literature exploring sclerosant therapy as a viable alternative to nonsurgical treatment. We present the case of a seven-year-old girl with first branchial cleft cyst who presented with recurrent serous discharge persisting for four years and was successfully treated with sclerotherapy injection with doxycycline.

Treatment of Work Type II Congenital First Branchial Cleft Anomalies: A Summary of 35 Cases.

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. Results: All 35 children underwent open incision and complete resection of fistulae. During the surgery, the lesions were found to be in the superficial facial nerve in 12 (34.3%) cases, between branches in 5 (14.3%) cases, and in the deep facial nerve in 18 (51.4%) cases. In those patients, lesions in females, with a lower edge of the lesion located below the angle of the mandible and the presence of a tympanic membranous attachment, are more likely to be located deep to the facial nerve or between its branches. The difference is statistically significant (P = .007, .032, .015). Conclusion: The treatment principle of Work type II CFBCAs consists of achieving a quiescent stage of inflammation, followed by a complete resection of the lesion on the premise of preserving facial nerve function. Certain clinical features of this disease can predict the relationship between the lesion and the facial nerve. The lesions in females, with a lower edge of the lesion located below the angle of the mandible, non-cystic type of Olsen, and the presence of tympanic membranous attachment, tend to be located deep to the facial nerve or between its branches.

Significance of the auditory meatus inferior wall cartilage in the surgical treatment of congenital first branchial cleft anomalies in children

ObjectiveTo 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.MethodsTwenty children diagnosed with CFBCAs...

Perplexing First Branchialcleft Anomalies-A Case Series with Review of Literature.

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.

Ultrasound diagnosis and treatment of branchial cleft cyst and preoperative management.

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. A retrospective analysis was conducted on 17 patients with branchial cleft cyst anomalies, including 11 males and 6 females, aged 12-53years, with an average age of 33 ± 2years, 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. All cases were examined with an enhanced CT scan, and pathological reports were generated. Among the 17 cases of branchial cleft anomalies, 15 cases were branchial cleft cysts, while one case involved fistula formation and one case involved sinus tract formation. Based on the type of branchial cleft, the first, second, and third cysts were classified in 4, 12, and 1 case, respectively. The sensitivity rate and specificity of ultrasonic diagnosis were 14/17 (82.4%) and 4/6 (66.7%), respectively. Ultrasonic characteristic analysis for the masses can be found in simple cystic masses or hypoechoic masses, most of them are of a regular shape and have a distinct boundary, and almost no blood flow signal. All patients who were misdiagnosed exhibited blood flow signals, including 1 patient with an abundant blood flow signal, 1 patient suspected of having ectopic thyroid with an abnormal function due to the rat-tail sign, 2 patients misdiagnosed as local inflammatory focus, and 1 patient misdiagnosed with tuberculous lymphadenitis. Ultrasound has a detection rate of up to 100% for cervical and facial masses, providing a fundamental determination of lesion characteristics and specific guidance for preoperative diagnosis. If the blood flow signals can be identified and carefully considered their peripheral relationship, the diagnostic rate can be improved.

Clinical Pearls of Branchial Cleft Cyst Management in an Adult

INTRODUCTION: Second branchial cleft anomalies most commonly present as cysts followed by sinuses and fistulae. They have been classified into four different sub-types by Bailey. Type II is most common type where the branchial cleft cyst (BCC) lies anterior to the sternocleidomastoid muscle, posterior to the submandibular gland, adjacent and lateral to the carotid sheath. In this article, a case of type II second branchial cleft anomaly is presented. CASE REPORT: This article aims to portray how to evaluate a patient with second branchial cleft cyst focussing, focusing on how its diagnosed and its appropriate management. A young woman who had chief complaint of swelling of left side of the neck visited our outpatient department. She underwent complete excision of the lesion. There was no recurrence at 1year follow-up visit. DISCUSSION: Most branchial anomalies arise from the second branchial apparatus. Most second BCCs are located in the submandibular space. Patients with BCCs are usually older children or young adults. MR imaging provide the surgeon adequate preoperative information. Treatment for these lesions is complete surgical excision.

New classification and surgical strategy for work type I congenital first branchial cleft anomalies in children.

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.

First Brachial Cleft Anomalies in Children: An Innovative Surgical Technique Preventing External Auditory Canal Stenosis.

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. On the basis of Work's classification system, 15 pediatric patients with type II FBCA and 13 children with type I FBCA were included in the further study. One child with type II FBCA and two with type I FBCA had a normal EAC. The preoperative appearance of the EAC was classified into three main types, each potentially accompanied by a skin ostium of the sinus/fistula in the EAC. Reconstruction with our technique was performed in 14 children (1 with type I FBCA and 13 with type II FBCA). Wound healing was uncomplicated in all cases. No recurrences were observed. This innovative surgical technique of the subtotal resection of FBCAs with simultaneous reconstruction is safe and prevents postoperative EAC stenosis. Despite the deliberate use of part of the abnormality wall for reconstructive purposes, it remained free of recurrences.

The Outcome of Treatment in Second Branchial Cleft Anomalies: A Case Series.

Background Branchial-cleft anomalies are second only to thyroglossal duct anomalies among congenital malformations of the neck, and second branchial-cleft anomalies are the most common. These include branchial cysts, branchial sinuses, and branchial fistulas. Clinical symptoms include neck swelling and a discharging sinus or fistula opening. In a small number of cases, they can lead to major complications like abscesses or malignant changes. Surgical resection is the treatment of choice. Various approaches to resection and sclerotherapy have been tried. In this study, we present our treatment outcome with branchial cleft anomalies at a ruraltertiary medical care hospital. Objectives To document the various presentations, clinical features, and outcomes of treatment with second branchial cleft anomalies. Methods This retrospective observational study included 16 patients operated on for second branchial-cleft anomalies. A detailed medical history was elicited, and an accurate clinical examination was done. A contrast-enhanced computed tomography (CECT) scan was done in all cases. A few cases required a fistulogram. The cysts, sinuses, or fistulas were resected en bloc by a single neck crease incision. Primary closure was done in all cases. A recurrence or pharyngocutaneous fistula required axial flap reconstruction. The complications and recurrences were documented. Result There were six children and 10 adults in our study. Seven cysts, five sinuses, and four fistulas were present, of which four were iatrogenic. In seven patients, imaging could not show the entire tract. There were four fistulas from the oropharynx to a cutaneous opening in the neck. A complete resection was done for all. Two pharyngocutaneous fistulas were treated with a pectoralis major myocutaneous (PMMC) flap. Three patients had wound dehiscence postoperatively. None of the patients had neurological or vascular injuries. Conclusion Second branchial cleft anomalies can be completely excised by a single neck crease incision. Meticulous surgery results in a low recurrence or complication rate.Following complete excision, in type IVanomalies, a purse-string suture at the pharyngeal opening ensures good closure and no recurrences.