Neonatal respiratory distress due to a third or fourth branchial pouch sinus: A systematic review

Objectives: Third and fourth branchial pouch sinuses can be rare causes of respiratory distress in neonates. An overview of this distinct clinical entity is missing in literature. To aid clinicians in recognizing and adequately treating this unique entity, we conducted a systematic review to discuss patient characteristics, diagnostic considerations and treatment strategy. Methods: MEDLINE and EMBASE were searched from inception to December 29th, 2020. Original studies concerning patients with respiratory symptoms as a result of a third or fourth branchial pouch sinus, as confirmed with rigid endoscopy, videofluoroscopy or during surgery. Results: Thirty-nine studies describing 56 patients (66% male, aged 0-30 days) were analyzed. Symptoms included cervical mass (76.8%), stridor (55.4%), dyspnea (35.7%) and cyanosis (17.9%) due to a third (39.3%) or fourth (60.7%) branchial pouch sinus. Intubation was performed before treatment in 31.3%. The piriform sinus opening was identified with rigid endoscopy in 81.1%. Surgery was the treatment of choice in the majority of patients (85.7%), with a success rate of 100% and a complication rate of 10.7%. Endoscopic cauterization was successful in 40% and endoscopic cauterization followed by sclerotherapy was successful 100%, with no complications. Conclusion: Third or fourth branchial pouch sinuses can lead to respiratory distress in neonates. It is important to recognize this distinct clinical picture for adequate diagnosis and treatment. Rigid endoscopy is indicated to demonstrate an opening in the piriform sinus and provides the opportunity to directly perform treatment with endoscopic cauterization. If this is insufficient to relief respiratory symptoms due to a persistent cyst, sclerotherapy or surgical excision should be considered.