Fourth Branchial Anomalies: Diagnosis, Treatment, and Long-Term Outcome

Introduction: Fourth branchial anomalies, the rarest among anomalies of the branchial apparatus, often present diagnostic and therapeutic challenges. We evaluated the clinical presentation and radiographic features, the treatment and the long-term outcome of patients in this setting.Patients and Methods: Of 12 patients treated in the University Hospitals Leuven from 2004 until 2020, 12 variables were collected: date of birth, gender, age of onset of the symptoms, age at final diagnosis, presentation, laterality, previous procedures, diagnostic tools, treatment (open neck surgery, endoscopic laser excision, or combination), complications, recurrence, and period of follow-up. Descriptive statistics were calculated and results were compared to the existing literature.Results: The most common clinical manifestations were recurrent neck infections with and without abcedation. Definitive diagnosis using direct laryngoscopy, visualizing the internal sinus opening, was possible in all patients. A CT study revealed the typical features of fourth branchial anomalies in seven patients out of nine, an ultrasound study in five out of nine patients. All patients underwent open neck surgery. If this was insufficient, secondary endoscopic laser resection of the ostium at the apex of the piriform sinus was performed (n = 4). In eight patients a thyroid lobectomy was needed for safe complete resection. Postoperative complications were minimal and at long-term, none of the patients showed further recurrence. Average time of follow-up was 8.6 years.Conclusions: Direct laryngoscopy and CT are the most accurate diagnostic tools. Our recommended treatment schedule consists of complete excision of the sinus tract by open neck surgery as the primary treatment because this ensures the best results. In case of recurrence afterwards, endoscopic laser resection of the pharyngeal ostium solved the problem.

First Branchial Cleft Anomalies: Rare Work Type I and Type II Entities

First branchial cleft anomalies (FBCAs) are the most infrequent malformations that occur during the development of the branchial apparatus, appearing in less than 8% of all branchial anomalies. Traditionally, they are classified into Work type I and II, depending on their origin. We present a pair of rare FBCAs: a case of a preauricular Work type I cyst with twin fistulae coursing toward the parotid gland and a Work type II cyst of significant dimensions.

Bilobed tongue base associated with multiple branchial anomalies

A bilobed tongue base was identified in an infant with multiple other head, neck and cardiac congenital anomalies. This anatomical variation of the posterior tongue is rare, with only two other cases identified in the literature. We report a case of a 5-month-old boy with a bilobed posterior tongue incidentally identified during workup for cardiac surgery.

Second Branchial Cleft Cyst: A Case Report

Objective: Branchial anomalies are congenital pathologies that are seen in the lateral region of the neck and are generally benign. The branchial clefts develop in the 2nd-7th weeks of fetal life as embryonal development. The branchial anomalies are caused by non-disappearance, abnormal development and, incomplete emerger of the branchial clefts and pockets during embryonal development. The branchial anomalies are generally seen as the cyst. The most common cyst was the second branchial cleft cyst with 95%. Their diameter is usually a few centimeters. Case: A 37-year old male patient was admitted to the hospital because of a swelling on the left side of his neck for four years. Physical examination revealed a mobile cystic mass in level 2 at the upper left jugular region of the neck. The cystic mass and the surrounding lymph nodes were excised and sent to the pathology laboratory. A cystic mass, approximately 5x4x3.5 cm in size, containing cystic areas was observed macroscopically. Microscopically, the cyst was lined with squamous epithelium and contained large lymphocyte groups in the subepithelial area. The case was reported as the branchial cleft cyst. Conclusion: Branchial cleft cysts should be excised before reaching large sizes, as they may cause pain and pressure on the surrounding tissue. In addition, it should be kept in mind that malignancy may develop from branchial cleft cysts, although rare.

Collaural fistula (Work Type II first branchial cleft anomaly) with prolonged morbidity: A case report

Collaural fistula is a very rare Work Type II first branchial cleft anomaly in which there is a complete fistulous tract between external auditory canal and the neck. Misdiagnosis and mismanagement can lead to prolonged morbidity and complications due to repeated infections. We present a case of an 18-year-old lady with a recurrent discharging sinus on her neck for 4 years. She has been treated with repeated incision and drainage and multiple antibiotics in the past. Otoscopic examination revealed an opening on the floor of the left external auditory canal. A diagnosis of an infected collaural fistula was made. Complete excision of the fistulous tract was done after treatment of the active infection. On follow-up, there was no further recurrence at 1 year. Sound knowledge of embryology of branchial anomalies with good history and examination is important to make correct and early diagnosis to prevent morbidity.

Combination Surgical Procedure for Fourth Branchial Anomalies: Operative Technique and Outcomes

Objective: Branchial apparatus anomalies of the fourth cleft are the rarest subtype of anomaly and occasionally present with suppurative thyroiditis or thyroid abscess due to their relationship with the thyroid gland. Surgical approaches vary and some surgeons favor cauterization of associated pyriform sinus tracts alone versus complete surgical excision. Currently, the literature is scarce and there is limited data on surgical outcomes and procedural steps. Here we describe a combination surgical technique for fourth branchial anomalies including: (1) surgical excision of the cyst and any external pit, (2) hemithyroidectomy, and (3) direct laryngoscopy with cauterization of pyriform apex tract, if present. Methods: A retrospective review was performed on all patients who underwent surgical excision of fourth branchial apparatus lesions (including fistulae, cysts, and sinus tracts) at an urban pediatric university hospital from 2000 to 2019. Data regarding demographics, medical history, surgical methods, complications, and surgical cure rates were collected. Results: A total of 16 patients (9 female, 7 male) underwent a combination surgical procedure for fourth branchial apparatus lesions. Success rate after primary surgery was 94%. One patient had residual disease requiring re-operation. Two patients had post-operative complications: 1 transient vocal fold paresis and 1 seroma, both managed conservatively. A consensus surgical algorithm was created based on operative steps present in the majority of cases. Conclusion: A combination approach to fourth branchial apparatus lesions—including endoscopic cauterization, external excision, and hemithyroidectomy—is safe and provides a high rate of primary cure. Although less invasive options exist, remnants of the branchial lesion, especially in the thyroid, may remain and cause recurrent issues. Therefore, we advocate for complete surgical excision of this rare developmental anomaly, especially when obvious thyroid involvement exists.

Contemporary Management of Benign Parapharyngeal Lesions Using Minimally Invasive Techniques: Case Discussion and Review of the Literature

Branchial anomalies can present in the parapharyngeal space, creating unique challenges in management. Historically, this approach warranted an open approach, including transcervical, transparotid with total parotidectomy and retromandibular dissection, or transmandibular dissection with mandibulotomy. However, the advent of minimally invasive transoral techniques and laser resection have allowed for successful resection of masses in this anatomical region without an external approach. We illustrate these advancements with the case of a 30-year-old man with globus sensation and throat discomfort, found to have a mass of the right posterolateral oropharynx causing severe airway obstruction. Imaging showed a parapharyngeal mass with extension to the carotid sheath and retropharyngeal space, which was successfully resected with potassium-titanyl-phosphate (KTP) laser using a minimally invasive transoral approach with no major complications. Transoral excision offers decreased morbidity and a cosmetically favorable outcome compared to transcervical excision. KTP laser may be safely used for transoral excision of a benign parapharyngeal mass.

Bilateral branchial arch anomaly: a rare case report

Anomalies of branchial arches are uncommon anomalies of embryonic development and may present as cysts, sinus tracts, fistulae or cartilaginous remnants. They represent the embryological precursors of face, neck and pharynx and are the second most common congenital lesions of head and neck in children. Although pharyngeal apparatus (branchial) anomalies are frequently seen, bilateral cases are rare (only 2% to 3%). Our patient was a 3 months old child with a swelling on right side of neck and discharging sinus from left side since birth. Surgical excision of the right branchial cyst with its complete tract was done along with excision of the left branchial fistulous tract upto the tonsillar fossa. Post-operative course was uneventful and patient was discharged the next day.