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Background Fourth branchial apparatus anomalies, are rare clinical entities, and present as complex cysts, sinuses and fistulae in the neck that can be difficult to manage. Methods This is a retrospective review of a series of consecutive patients with fourth branchial apparatus anomalies treated at Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, from Apr 2014 to Nov 2019. Results Ten patients with fourth branchial apparatus anomalies were identified, including 8 patients with fourth branchial fistula, and 2 patients with fourth branchial pouch sinus. There were 6 female patients and 4 male patients. Their age was from 6 years old to 39 years old (average age 20.4 years old, median age was 21 years old). All 8 fistulae were on the left side, while 2 pouch sinuses were both on the right side. Pre-operative examination with fiberoptic laryngoscope, barium swallow X-ray, CT or MRI identified internal orifice at pyriform fossa apex in 8 (80%) patients. All patients underwent challenging surgical resection by the senior author. Intra-operative direct laryngoscope confirmed or identified internal orifice in 9 (90%) patients. The tracts were all followed to the vicinity of inferior cornu of the thyroid cartilage and the cricothyroid space. Complete resection of cervical lesions and their attachment to hypopharynx were achieved in 9 cases. No complication occurred. One recurrence was detected, in the only patient whose internal orifice could not be located pre- or intra-operatively, and the hypopharyngeal attachment could not be removed. Conclusions Direct laryngoscopy under general anesthesia is a reliable method of diagnosis for the fourth branchial apparatus anomalies. Complete surgical removal of fourth branchial apparatus anomalies, including their hypopharyngeal attachment, is the treatment of choice, and the key to prevent recurrence.

Recurrent neck abscess is a typical feature of branchial anomaly. Open surgical excision has been the primary treatment modality for past decades however several alternative treatment modalities such as endoscopic electrocauterization has recently gained popularity and acceptance universally. This series aims to introduce endoscopic assessment as the first line diagnostic and therapeutic management for branchial fistula. 5 patients underwent examination under general anesthesia via direct laryngoscopy, endoscopic assessment and cauterization in our centre from 2016 to 2019. They were then followed up at our clinic to assess disease progression. Comparison between open neck surgery and endoscopic cauterization of internal sinus tract opening as the primary treatment has reported similar recurrence rate. Proposal of endoscopic assessment as the first line investigation and diagnostic tool with the aim of therapeutic cauterization at the same setting. MRI may be needed in scenarios such as failed endoscopic treatment or when open surgery is required.

We report the clinical presentation and CT and MR imaging findings in a child with an unusual first branchial apparatus fistula that extends from the external auditory canal to the oropharynx.

Branchial fistulas are uncommon in the clinical setting. The coexistence of first and second branchial fistulas has not been previously reported. We herein describe a 12-year-old girl who presented with a 2-year history of repeated swelling and purulence behind the right earlobe and neck. According to the patient’s physical and auxiliary examination findings, she was diagnosed with coexisting first and second branchial fistulas, both of which were completely removed by surgery. No clinical signs of fistula recurrence were present at the patient’s 20-month postoperative follow-up. Ipsilateral coexisting first and second branchial fistulas are very rare; thus, a false-positive diagnosis can easily occur if the doctor does not carefully perform specialized physical examinations. Surgery is an effective method for treating this condition. Adequate preoperative imaging preparation is imperative to ensure the most effective course of treatment. The purpose of this article is to improve clinicians’ awareness of this disease, thereby effectively reducing the rates of missed diagnosis and recurrence.

We report a case of complete branchial fistula in a 13-year-old boy which could be evaluated by a contrast study. The tract was completely excised and the patient had an uneventful recovery. Branchial fistulae are formed due to the persistence of the embryonic second branchial cleft. Complete fistulae with the internal opening in the region of the tonsillar fossa are not common and should be properly evaluated before going to surgical exploration.TAJ 2011; 24(2): 145-147

Type 2 branchial cleft anomalies are the most common cause of lateral neck swelling. We report two cases of type 2 branchial cleft anomalies. The first case is branchial cleft cyst and the second one is branchial fistula. Both cases were managed surgically. The post operative outcomes were uneventful. Second branchial cleft anomalies are the most common branchial anomalies. Branchial cysts are more common than sinuses and branchial fistulae are extremely rare. There is no gender predilection. The location, clinical symptoms and imaging findings aid in the diagnosis of this condition. Surgical excision is the mainstay of treatment.Keywords: branchial cleft; branchial cyst; branchial fistula.

Objective To evaluate the efficacy of CO 2 laser cauterization with suspension microlaryngoscopy as a definitive surgical treatment for pediatric Congenital Pyriform Sinus Fistula (CPSF). Material and methods This is a cohort retrospective study. Thyroid function and cervical ultrasonography examinations were performed before operation. Enhanced magnetic resonance imaging (MRI) was performed on patients with a repeated infection (≥2 times) and/or if they had a prior open surgery. Patients were divided into two groups: the Results CO 2 laser cauterizations with suspension microlaryngoscopy were performed for 104 CPSF patients. No complications occurred. Three patients had a recurrence in the follow-up. The number of surgical cauterization operations was fewer than 3 in 85.1% of the patients. There was no significant difference in the number of cauterizations among the different age groups or between the initial treatment and retreatment groups ( P >0.05). Conclusion CO 2 laser cauterization with suspension microlaryngoscopy is a safe, effective, and minimally invasive approach to CPSF with optimal patient outcomes. Type of study Treatment Study. Level of evidence Level III.

Branchial arch anomalies are one of the most common congenital anomalies and are usually unilateral in nature. The bilateral occurrence of more than one anomaly is not only rare, but its presence along with hearing loss results in branchiootic syndrome, which has important clinical and genetic implications. Branchiootic syndrome is a part of the spectrum of branchiootorenal syndrome, which is a rare autosomal dominant condition with incomplete penetrance characterized by the malformations of the external, middle, and inner ear, hearing loss, branchial fistulae, and renal abnormalities. We shall be discussing two such rare cases in this article.

Background Neonatal and infantile fistulas are diverse and include many types. Conventional contrast images are used to delineate the fistulous tracts. The aim of this study is to illustrate the conventional radiological spectrum of neonatal and infantile fistula with surgical correlation. Methods Neonates and infants with suspected fistulas were included in this study. The patients may be presented by cutaneous fistula with discharge, profuse oral secretion and cyanosis on feeding, or imperforate anus. After full clinical data were taken, plain X-ray, contrast imaging, and abdominal sonography were done to the patients. Treatment was tailored according to the case. Results Our study included 34 patients (25 boys and 9 girls): one case of branchial fistula; 2 cases with thyroglossal fistula; 10 cases of tracheo-esophageal, esophago-cutaneous, and esophago-pleural fistulas; 3 cases with vitello-intestinal fistula; one case with urachal fistula; 12 cases with recto-urinary, recto-vaginal, and recto-perineal fistula; 4 cases with peri-anal fistulas; and finally one case with iatrogenic urethra-cutaneous fistula. Radiological findings were well correlated with the surgical data. Conclusion Many types of neonatal and infantile fistulas were shown in this series including tracheo-esophageal, recto-urinary, and other neonatal fistulas. Radiological and surgical data were well matched.

Branchio-oto-renal (BOR) syndrome is an autosomal dominant disorder characterized by the coexistence of branchial cysts or fistulae, external ear malformation with pre-auricular pits or tags, hearing impairment and renal malformations. However, the presence of the main features varies in affected families. Here, we present a 16-year-old boy admitted to the Department of Nephrology at the Pediatric Clinic, University Clinical Center of Kosovo, Pristina, Republic of Kosovo because of severe renal insufficiency diagnosed 6 years ago, which progressed to end-stage renal failure. Clinical examination on readmission showed a pale, lethargic and edematous child, with auricular deformity, pre-auricular tags and pits as well as bilateral branchial fistulae. Laboratory tests revealed high blood urea nitrogen (BUN) 15.96 mmol/L and serum creatinine 633.0 µmol/L; low glomerular filtration rate (GFR) 12 mL/min./ 1.73 m2 and massive proteinuria 4+. Abdominal ultrasound showed bilateral kidney hypoplasia. A novel mutation of the EYA1 gene was confirmed. Daily hemodialysis is continuing until renal transplantation is done. This case is presented to increase awareness among general practitioners to consider BOR syndrome or other renal abnormalities in patients with branchial fistula and/ or external ear anomalies or similar findings in other family members.

Objectives/Hypothesis We present a new method of optimizing the results of surgery for branchial cleft anomalies based on the intraoperative injection of fibrin glue combined with methylene blue dye. Study Design Retrospective single-center cohort study. Methods The method was applied in 17 patients suffering from branchial anomalies. Six (35.29%) had a preauricular lesion; three (17.65%) had lesions derived from the first arch/pouch/groove (type I), four (23.53%) had lesions derived from the first (type II), one (5.88%) had lesions derived from the second, one (5.88%) had lesions derived from the third, and two (11.76%) had lesions derived from the fourth. The median and mean age at surgery were 10 and 10.6 years, respectively. All patients were followed by periodic clinical and ultrasonographic examination. Results The combination of fibrin glue with methylene blue facilitated the correct assessment of the extension of the lesions and their intraoperative manipulation. After a mean follow-up of 47.8 months, all patients were free of disease. Conclusions Intraoperative injection of branchial fistulae and cysts by a mixture of fibrin glue and methylene blue is an effective, easy, and safe tool to track lesions and achieve radical resection. The technique requires a definitive validation on a large cohort with adequate stratification of patients. Level of Evidence 4 Laryngoscope, 2015

Fourth branchial pouch anomalies represent one of the rarest types of all branchial apparatus anomalies. They appear in the first lifedecade in a form of recurrent left-sided neck masses which demands surgical treatment. Accidental finding, appearance later in life and spontaneous resolving are really rare. We present a case of a 43-year- old man with accidental finding of asymptomatic fourth pouch cyst and fistula following follicular thyroid cancer surgery. The day after the surgery, suction bottle was filled with little white crumbs and the wound started to suppurate. Barium swallow revealed the presence of a fistulous canal that arose from the left pyriform sinus. Meanwhile, the pathologist confirmed the presence of a lateral neck cyst within this thyroid lobe. The patient was operated on but fistulous canal was not visualized. In the meantime, wound discharge ceased spontaneously. At 1-year follow up, the patient was still well and free from any symptoms. These anomalies may manifest not only in childhood but may stay asymptomatic for a long time. It seems that the fistula can resolve spontaneously and that conservative approach is an alternative to multiple surgical procedures.

Branchial arch anomalies are the most common congenital neck masses. The second branchial arch anomalies followed by first arch anomalies are seen commonly in the descending order. They originate from remnants of branchial arches and clefts. They may present as cysts, sinus tracts, fistulae or cartilaginous remnants. They are mostly located in the lateral aspect of the neck anterior to the sternocleidomastoid, anterior to the hyoid bone, preauricular region or at the angle of the mandible. A complete fistula communicating with a branchial arch cyst is a very rare congenital anomaly of the branchial apparatus. These patients are generally asymptomatic but may present with mucoid discharge from the tract. Here we present a case of branchial fistula associated with a branchial cyst in a 11 year old child.

IntroductionComplete branchial fistulae are rare anomalies of the branchial cleft apparatus. Incomplete fistulae occur more commonly and may be completely asymptomatic. They are often neglected as they do not lead to recurrent infection, while complete branchial fistula have persistent symptom of discharge. The branchial fistula very rarely has two openings. Usually, a thin membrane covering the internal opening is present even if both ends are patent. Demonstration of a complete branchial fistula on imaging studies is also very uncommon. This is a study of five cases of complete second branchial fistulae, which were diagnosed and treated surgically in a tertiary care teaching hospital over a period of 3 years. The case study also make special emphasis on preoperative imaging for the confirmation of diagnosis and for appropriate surgical approach for complete excision of fistulous tract in order to minimize the recurrence as the revision second-look surgery in neck region is usually difficult.How to cite this articleBist SS, Purohit K, Agarwal V, Bharti B, Monga U. Complete Second Branchial Fistula: Diagnostic Imaging and Surgical Aspects. Int J Otorhinolaryngol Clin 2016;8(1):6-10.

IntroductionBranchial arch fistula which results from a second arch anomaly is rarely seen in practice. The patients usually seek medical advice for persistent discharge from an opening in the neck or mucopurulent discharge from the opening of an infected fistulous tract. The treatment of choice for such a fistula is excision. Materials and MethodsA total of seven patients were operated for branchial fistula between 2010 and 2012. Among them, three children could not undergo a fistulogram due to allergy to the dye used and hence they were selected for our modified procedure. Surgical ProcedureA 3-0 or 4-0 polypropylene (Prolene™) thread was inserted into the fistulous tract. Both the ends of the tract were secured with the thread and the sinus tract was dissected out under gentle traction. ResultsThe patients who were allergic to dye could not have preoperative fistulograms and per-operative injection of dye was also avoided. The blue colour of the polypropylene thread inserted in the fistula tract helped in tracing and excision of the sinus tract. No major complications occurred during or after fistulectomy and the post-operative recovery was uneventful. No recurrence was seen in the scheduled follow-ups. ConclusionSurgical excision of branchial fistulas in children with allergy to dyes can be challenging. Insertion of a polypropylene thread into the fistula tract makes its subsequent dissection easy with minimal disruption of adjacent structures.

Sialoliths greater than 10 mm in size can be called as those of an unusual size. Only 7.65 % of the sialoliths are >15 mm. Cutaneous fistulas due to submandibular sialoliths are even rarer. A case of a submandibular gland sialolith of an unusual size presenting with a cervical fistula mimicking a branchial fistula has been reported. Intra-operatively, the fistulous tract was found to extend upto the supraclavicular space. Complete excision of the gland along with fistulous tract was performed. Histopathology revealed a sialolith with acute and chronic sialedinitis. The lesion also mimicked a Kuttnor's tumour however a final diagnosis of the same could not be made due to inconclusive Ig G value and absence of plasmacytic infiltration.

Patient years male A year old boy presented with the complaints of watery discharge from an opening in the right hand side of neck on and off since birth There was no history of trauma or operation nbsp

Branchial cleft fistulae are rare congenital abnormalities that arise from the abnormal persistence of branchial apparatus remnants. Fourth branchial cleft fistulae pass deep to second arch structures and over third arch structures, in a direction extending from the anterior border of sternocleidomastoid (SCM) muscle to the upper pole of the ipsilateral pyriform fossa. Because of this anatomical route, these long tubular structures are intimately associated with major neuro-vascular structures in the neck. We present the clinical presentation and surgical management of a fourth branchial cleft fistula, in a 23-year-old male. Preoperative CECT fistulogram of the neck clearly demonstrated the fistula. Patient was managed using combined stepladder transcervical and intra

Congenital anomalies of the branchial arches are well known with fistulas presenting in the head and neck region. A proper diagnosis of this uncommon entity is mandatory for a successful surgical repair in the first attempt itself. In this case report, we profile a patient who was subjected to multiple surgeries only to result in subsequent breakdowns and resurfacing of symptoms. An innovative attempt was made with the use of endoscopes and minimally invasive surgical methods to repair this recurrent fistula and followed up with a repeat fistulogram which showed a complete closure of the tract without any co-morbidity.

Branchial cleft anomalies comprise approximately 30% of congenital neck mass and present as cyst, sinus or fistula. They occur due to disturbance in the maturation of the branchial apparatus during fetal development. They are congenital lesions usually present in childhood, but they are usually diagnosed in later childhood or early adulthood because of enlargement or infection. Branchial cleft fistulae are usually diagnosed earlier than cysts. Correct diagnosis leads to proper management. Complete surgical excision is the treatment of choice. Second branchial cleft and pouch anomalies are commonest amongst all branchial cleft lesions, but complete second branchial cleft anomalies with external and internal opening is rare. Branchial anomalies with family history are also very rare. Here we present a case report of complete branchial fistula with family history which was managed by complete excision of fistula through transcervical and transoral approaches. 3 members of one family in 2 generations presented with branchial apparatus anomalies (father and his two children, elder son and younger daughter). All of them were having branchial fistula on right side of neck since birth.