Congenital midline cervical cleft: Management of a case series and literature review.

Congenital midline cervical cleft is a rare anomaly classified as a malformation of the branchial arches and represents less than 2% of congenital cervical malformations. Its clinical presentation involves cervical midline deformities: cephalic nodular lesion, linear groove with atrophic surface, and/or caudal sinus. Other midline alterations of variable complexity may also be present. Early treatment allows for avoiding long-term complications. Based on our experience in four clinical cases, a performed literature search on the topic in the last twenty years, and subsequent discussion of the employed surgical approaches, we included 150 reported cases in our review. Correct diagnosis and early treatment with complete removal of the fibrous midline band is paramount to avoid patient complaints until adolescence or adulthood.

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

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. All cases were examined with an enhanced CT scan, and pathological reports were generated. RESULTS: 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. CONCLUSION: 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.

Reworking Classification of First Branchial Cleft Anomalies.

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. Imaging and pathologic slides were re-reviewed in a blinded fashion by experts in those respective areas. Parents were contacted via telephone to obtain extended follow-up. An alternate classification method based on the presence (type II) or absence (type I) of parotid involvement is provided. RESULTS: Only 55% of first BCAs could be successfully classified using Work's method. First BCAs within the parotid were more likely to present with recurrent infections, involve scarred tissue planes and lymphadenopathy, and demonstrate enlarged lymphoid follicles on pathology. The overall recurrence rate was 16%, and recurrence was 5.3 times more likely when external auditory canal cartilage was not resected. Preoperative imaging was useful for predicting the extent of surgery required and the presence of scarred tissue planes. CONCLUSION: First BCAs within the parotid gland involve more difficult and extensive surgical resection and the potential for morbidity related to facial nerve dissection. Appropriately aggressive surgical resection, which may include the resection of involved ear cartilage, is necessary to prevent morbidity related to recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:459-465, 2024.

Age-related outcomes after pediatric branchial cleft cyst excision via NSQIP-P.

OBJECTIVE: Branchial cleft cysts present at varying ages; sometimes excision is delayed because of concern about operating in small children. Our goal was to determine if outcomes and complications differed among pediatric age groups. STUDY DESIGN: Retrospective, cross-sectional. SETTING: American College of Surgeons' National Surgical Quality Improvement Pediatric database. METHODS: Patients who underwent a branchial cleft cyst excision between 2016 and 2020 were identified in the American College of Surgeons National Surgical Quality Improvement Program (ACS NSQIP) Pediatric database. Patients with CPT code 42,815: 'excision branchial cleft cyst, vestige, or fistula, extending beneath subcutaneous tissues and/or into the pharynx' as their primary procedure were included. Variables of interest included patient demographics, comorbidities, pre-operative risk factors and complications. RESULTS: 2267 patients with median age of 3.9 (IQR: 7.4, range: 0.04-17.9) years were included. The most common pre-operative risk factors were 149 (7 %) patients with prematurity, 136 (6 %) with developmental delay, 135 (6 %) with congenital malformation, and 128 (6 %) with open wound or wound infection. 68 (3 %) patients experienced at least one post-operative complication, with 73 post-operative complications documented in total. Surgical site infections were the most common complications with 49 (67.1 %) superficial infections, 11 (15.1 %) deep infections, and 4 (5.5 %) organ/space infections. Surgical site infections were the most common reason for related readmission. Duration of anesthesia and operative time increased significantly as patient age increased (p < .001 for both). There was no significant correlation between age and complication incidence. CONCLUSION: Branchial cleft cyst excision is a relatively safe operation with a low complication rate, even in young pediatric populations.

Pulse granuloma presenting as a lateral neck mass: An unusual presentation of a fourth branchial cleft fistula.

BACKGROUND: The pulse granuloma (PG) is believed to represent a distinctive foreign body reaction to ingested particles of legumes. Its presentation in the neck is entirely unexpected. METHODS: A woman presented with a mass of the lower neck that recurred following incision and drainage. The recurrent mass was found to be associated with an open sinus tract at the apex of the left pyriform sinus. The opening of the sinus tract was closed and the cyst was removed. RESULTS: Histologic examination of the neck mass showed vegetable material with an associated granulomatous reaction known as PG. CONCLUSIONS: The documentation of a PG arising in the neck would seemingly discredit the legume theory, but it only further supports it. Its association with a fourth branchial cleft cyst provides evidence for the existence of the complete fourth branchial cleft fistula with seeding of ingested material through sinus tract opening.

Use of contrast-enhanced ultrasound in preoperative planning before resection of a second branchial cleft fistula.

In the head and neck area, a large proportion of clinically relevant fistulas occur in childhood. The present case describes the use of contrast enhanced ultrasound with intraductal administration of the contrast medium for preoperative visualization of the fistula duct in the case of a second brachial cleft fistula. This provided the surgeon with important additional information, such as the detailed course of the fistula and its relation to the large vessels of the neck. The method can help to improve surgical planning, reduce radiation exposure and to avoid imaging under general anesthesia, especially in children.

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

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. 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). CONCLUSION: 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.

Collaural fistula: Our experience.

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.

[The managements of cervical chondrocutaneous branchial remnants].

Objective:To investigate the embryologic origin and diagnosis and management of cutaneous cartilage remains of gill origin in the neck. Methods:A total of 15 patients with cervical chondrocutaneous branchial remnants treated in Guangdong Provincial People's Hospital from January 2005 to December 2021 were retrospectively analyzed. They had a common feature showing a tumor in the lower third of the front of sternocleidomastoid muscle. The tumor looked like accessory auricle, never appeared pain or other symptoms of infection, and had no skin orifice. All patients underwent ultrasound examination, which showed an anechoic area under subcutaneous tissue of the neck or face. MRI examination in 6 cases showed subcutaneous irregular nodules the location of the lesion. Surgical resection of cervical chondrocutaneous branchial remnants was performed in all cases. Results:Postoperative pathological examination showed elastic cartilage. No complications were noticed. Recurrence was not observed in the cases by following-up of 8 months to 52 months（median: 41 months）. Conclusion:Cervical chondrocutaneous branchial remnants are relatively rare, which may originate from the second branchial arch and may be associated with other congenital malformations. The curative treatment is a complete excision preschool.

Branchial cleft cyst and branchial cleft cyst carcinoma, or cystic lymph node and cystic nodal metastasis?

BACKGROUND: Lateral cervical cysts are usually considered as of branchial cleft origin, despite many studies showing that branchial cysts do not arise from the remnants of the branchial apparatus. In the same way, some authors still consider that a true clinicopathological entity such as 'branchial cleft cyst carcinoma' could exist, at least in theory. Despite insufficient evidence in support of the branchial theory, a number of publications continue to emphasise this concept. METHODS: A literature review of articles in Medline and PubMed databases was carried out to retrieve papers relevant to the topic. RESULTS AND CONCLUSION: The evidence from lateral cervical cyst studies and knowledge about cystic metastasis of Waldeyer's ring could be applicable for both diagnoses. Terms such as 'branchial cleft cyst' and 'branchial cleft cyst carcinoma' are confusing and misleading, and it is questionable as to whether their usage is still tenable.

Congenital first branchial cleft anomalies in children: a study of 100 surgical cases and a review of the literature.

OBJECTIVE: To investigate the clinical features and surgical outcomes of pediatric congenital first branchial cleft anomalies (CFBCAs). METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Isolated First Branchial Cleft Anomalies of the External Auditory Canal.

BACKGROUND Branchial cleft anomalies are congenital aberrations of the first to fourth pharyngeal pouches. First branchial cleft anomalies are classified into 2 subtypes according to anatomical and histological features. Their diagnosis can be difficult and depends on radiological and histological findings. In contrast, the required treatment is surgical removal, owing to the high risk of infection or malignancy. This case report introduces a first branchial cleft anomaly in an older woman with exclusive involvement of the external auditory canal (EAC). CASE REPORT This case report introduces a first branchial cleft anomaly in an 82-year-old woman with exclusive involvement of the EAC. She reported a history of mixed moderate hypoacusis and recurrent otitis media in the last year, without facial nerve involvement. Computed tomography and magnetic resonance imaging were performed to plan surgical treatment, which consisted of canaloplasty and Thiersch grafting. The histopathological examination on operative findings revealed a cystic lesion that was lined by cylindrical epithelium adjacent to the squamous cells, compatible with a diagnosis of first branchial arch malformative residues. CONCLUSIONS This is the unique case of first branchial cleft anomalies reported in an adult patient that exclusively involved the EAC. The onset of the disease was atypical, and surgery with the total removal of the lesion was the only possible treatment. Histopathology results revealed cylindric epithelium not represented in the EAC, compatible with first branchial arch malformative residues. This rare condition is a potential diagnostic option that should be considered in the differential diagnosis of cysts of the EAC.

Horn-shaped Symmetric Nodules on the Earlobes: An Unusual Presentation of First Branchial Cleft Anomaly.

First branchial cleft anomaly (BCA) is a rare congenital malformation of the head and neck, <10% of all BCA. it occurs between the first and second branchial arches. The diagnosis of first BCA is often delayed because of its atypical shape and rarity. A 30-year-old male presented to our clinic with bilateral earlobes nodules, he first noticed ~20 years prior. The lesions had gradually increased in size, and he stated no other specific symptoms. Bilaterally, a 1-cm-sized, sharp, horn-shaped, dark-colored, keratinized mass was palpated hardly, and no infectious signs were observed. Bilateral resection was performed, and the lesions did not form a tract in any other direction. Histopathology confirmed the lesions were first BCA composed of a cystic cavity lined by stratified squamous epithelium. Surgeons should suspect first BCA in patients with nonspecific lesions of the earlobe, and our case will help with future diagnoses.

[The role of triangular space of ear canal-parotid-mastoid in the surgery for first branchial cleft anomalies].

Objective:To explore the role of triangular space of ear canal-parotid-mastoid in the operation of the first branchial cleft deformity. Methods:The clinical features and intraoperative characteristics of 25 cases with first branchial cleft anomalies who underwent surgery from September 2011 to September 2019 were analyzed, and the role of the triangular space of ear canal-parotid-mastoid in the surgery was explored. Results:Following dissecting and lesions removel of the triangular space of ear canal-parotid-mastoid, all the lesions were resected completely. Eighteen cases had fistula in the floor wall of ear canal, seven cases had duplicated of external auditory canal in the inferior of the floor wall. The recurrent cases were all attributable to the residual lesions in the triangular space. There was no recurrence, salivary leakage or stenosis of external canal. One case suffered from HB2 level facial paralysis. Conclusion:Surgery is the optimal treatment for first branchial cleft anomalies. Following the active dissection of the ear canal-parotid gland-mastoid space and depending on the microscopic operation, the deep lesions would be exposed clearly and the facial nerve could be marked and protected. Cleaning this triangle space can lead to completely lesion removal, avoid facial paralysis, salivation and recurrence.

Management of Nasopharyngeal Cyst of Second Branchial Cleft Origin: Case Series and Systematic Review.

Branchial cyst of the second pouch is the most common lesion of the nasopharyngeal lateral wall, generally localized between the pharyngeal wall and internal carotid artery. Cases consistent with such lesion, were collected. Symptomatic patients were treated with endoscopic trans-nasal marsupialisation, asymptomatic cases were followed-up. Among the 10 patients included, 4 were symptomatic and accordingly treated. In the literature, 36 cases were found, all of which were treated, most commonly with a total excision. Considering the benign nature of branchial nasopharyngeal cyst, its treatment should be tailored to each patient: endoscopic marsupialization in symptomatic lesion, follow-up in asymptomatic one. Laryngoscope, 132:1904-1908, 2022.

Suture Ligation of Pyriform Sinus Fistulas Following Cauterization to Treat Branchial Cleft Anomalies.

OBJECTIVES: Obliteration with cauterization of the internal opening of pyriform sinus fistulas, with or without marsupialization, has been a mainstay for preventing recurrence. However, this procedure predisposes patients to recurrence caused by the reopening of the cauterized orifice. We applied suture ligation to secure the closure of the internal orifice following cauterization and evaluated treatment. METHODS: A total of 42 patients were diagnosed with third or fourth branchial cleft anomaly with internal pyriform sinus fistula and treated either with cauterization or with cauterization and suture ligation, between January 2008 and December 2020. The medical records were reviewed to assess demographic characteristics, clinical presentations, diagnoses, surgical treatment, and outcomes. Treatment flow characteristics for intractable patients were analyzed. RESULTS: The median age of onset was 9 years (range, 0-57 years). Neck swelling (n = 32, 76.2%) was commonly encountered symptom, and a history of neck infection was found in 27 patients (64.3%). After initial treatment, 11 cases (56.2%) recurred. Younger age (≤9 years) and thyroid involvement were associated with recurrence (P = .034 and P = .046, respectively). Cauterization with suture ligation presented lower recurrence rate (19.2%) than cauterization only (37.5%, P = .037). Cauterization with suture ligation for recurrent cases (n = 11) showed 81.8% (9/11) of cure rate. Intractable fistulas (n = 2) from both groups were also salvaged by suture ligation. CONCLUSIONS: Suture ligation with cauterization for an internal orifice of branchial anomaly showed lower recurrence rate than cauterization only. This method was beneficial for refractory cases.

First branchial arch cyst in an elderly patient: diagnostic dilemma and subsequent management.

The branchial system plays a significant role in the embryological development of the many internal and external human body structures. Failure of normal development of these systems may result in branchial system anomalies. Anomalies of the first branchial cleft are rare and account for 1-8% of all branchial anomalies. They have an incidence of 1 per 1 million births, most of which are diagnosed in early childhood. We present an unusual case of a first branchial arch cyst in an elderly gentleman: a 65-year-old man who presented with a persistent swelling in the left pre-auricular region with no associated sinus, fistulae or lymphadenopathy and with an intact facial nerve. Investigations including fine needle aspiration, ultrasound and magnetic resonance imaging led to the diagnosis of a lesion of salivary origin and an extracapsular dissection was undertaken. The histological appearance on excision was, however, in keeping with a first arch branchial cyst. In conclusion, the nonspecific clinical and radiological presentation of first branchial arch anomalies may lead to difficulty and often delay in the diagnosis of these lesions, particularly in elderly patients as it is more often associated with childhood and adolescence. A high level of suspicion is mandatory to prevent inappropriate management in the form of incision and drainage, which further increases the risk of recurrence and facial nerve injury at the time of formal excision due to scarring.

Computed Tomography Findings of Complete Branchial Cleft Fistula.

Branchial cleft anomalies are embryonic remnants of the branchial arches and are described as the second most common congenital neck mass. Depending on their extent, these anomalies are classified as a cyst, sinus, or fistula with branchial cysts being the most common. Branchial cysts deriving from the second branchial arch are by far the most common, accounting for approximately 95% of all cases. Complete second branch arch fistulas with both an internal and external opening are a rare variant of this anomaly, and even less have been well-documented on computed tomography (CT) imaging in the literature. We present here a case of a 20-year-old female with CT findings consistent with a complete second branchial arch fistula extending from the tonsillar fossa to the external lateral neck.

Analysis of the Clinical Features and Surgical Outcomes of First Branchial Cleft Anomalies.

OBJECTIVES: To analyze the clinical features and otologic manifestations of first branchial cleft anomalies (FBCAs) and the disparity between Work's classification, and to explore the relationships between postoperative facial paralysis and features of FBCAs. STUDY DESIGN: Retrospective clinical study. METHODS: A retrospective analysis of 109 patients with FBCAs was conducted, including clinical characteristics and otologic features. Pearson chi-square tests and Fisher's exact tests were used to compare disparity between Work's classification, and the impact factors of postoperative facial paralysis among 86 patients who were explored in follow-up. RESULTS: Patients with FBCAs presented with otologic symptoms, including cysts or fistula in the external auditory canal (EAC) and periauricular (43.2%), microtia (3.7%), EAC web (1.8%), otitis media (1.8%), and otorrhea (4.6%). Eighty-five cases (78.0%) were type I FBCAs and 24 (22.0%) were type II. Compared to type I FBCAs, type II (58.3%) was more likely to be located deep to the facial nerve (FN) and to have superficial parotidectomy on them (79.2%). This difference was statistically significant (P < .001). FBCAs deep to the FN had a higher incidence of postoperative facial paralysis (P < .05). CONCLUSION: The majority of patients (55.0%) had otologic symptoms. The FBCAs of Work type II was commonly deep to the FN and superficial parotidectomy was frequently performed. Postoperative facial paralysis was associated with FBCAs located deep to the FN, but not with Work's type. LEVEL OF EVIDENCE: 4 Laryngoscope, 132:1008-1014, 2022.

Patho-Anatomic Spectrum of Branchial Cleft Anomalies: Proposal of Novel Classification System.

PURPOSE: Histogenesis, nomenclature, and classification of branchial cleft anomalies (BCAs) have been subjects of controversy for decades. The purpose of this study was to investigate the accuracy of current developmental theories (congenital, lymph node, and hybrid branchial inclusion theories) in defining the anatomic and histopathological characteristics of BCAs. METHODS: Ninety consecutive patients with BCAs who underwent surgical excision were enrolled in this 2-center retrospective cohort study. RESULTS: The present study included 90 patients: 46 (51.11%) women and 44 (48.89%) men (P > .05). The mean age at presentation was 31.89±17.31 years. Altogether, 92 BCAs were identified within the study population including 49 (53.26%) on the left side and 43 (46.74%) on the right side (P > .05). The BCAs included 79 (85.87%) branchial cleft cysts, 11 (11.96%) branchial cleft sinuses, and 2 (2.17%) branchial cleft fistulae. Three (3.26%) BCAs were distributed in the head regions, 88 (95.65%) in the neck regions, and 1 (1.09%) in the thoracic cavity. Following surgery, lymphoepithelial tissue was detected in the histopathological examination in 83 (90.22%) BCAs. The hybrid branchial inclusion theory exhibited significantly higher accuracy in defining patho-anatomic characteristics of BCAs than the branchial apparatus, precervical sinus, thymopharyngeal, and inclusion theories (90.22, 9.78, 2.17, 0.00, and 0.00%; respectively) (P < .05). CONCLUSION: The novel branchial node (BN) classification system based on the hybrid branchial inclusion theory appears to be superior to other classification systems in determining the patho-anatomy of BCAs.