目的:探讨新生儿先天性舌根囊肿的临床特征。方法:回顾性分析2013年6月至2019年12月西安市儿童医院新生儿重症监护病房（neonatal intensive care unit, NICU）收治的35例先天性舌根囊肿新生儿的一般情况、临床表现、辅助检查、治疗及预后等资料。采用描述性统计分析。结果:（1）35例先天性舌根囊肿患儿中位发病日龄为12.5（0~28） d，中位入院日龄为15（0~28） d。常见临床表现：28例（80.0%）喉鸣，26例（74.3%）吸气性呼吸困难、哭闹及吃奶时加重，23例（65.7%）吐奶、呛奶。（2）35例中，15例（42.9%）患儿入NICU时因呼吸衰竭需紧急气管插管，其中5例气管插管时直接喉镜检查时考虑舌根部肿物，10例气管插管时直接喉镜检查考虑有咽喉部占位，不能明确位置及性质，进一步行床旁电子喉镜检查考虑舌根囊肿；7例（20.0%）因有喉喘鸣伴肺炎迁延不愈行纤维支气管镜时考虑舌根囊肿；余13例均行电子喉镜检查考虑为舌根囊肿。所有患儿均行颈部超声检查仅5例发现舌根部肿物。32例行颈部CT检查，仅2例（6.3%）未发现病变。3例行头颅MRI检查均发现有舌根部囊肿。（3）34例全身麻醉下由耳鼻喉头颈外科医师行支撑喉镜下舌根囊肿等离子融切术；另1例舌根囊肿大，气管插管困难，在直接喉镜下进行第1次穿刺抽液术，生命体征平稳后再进行手术治疗。手术治疗后定期随访，仅1例（2.9%）复发。结论:新生儿先天性舌根囊肿症状出现较早，临床表现缺乏特异性。对于有喉鸣、吸气性呼吸困难的患儿，应及时行电子喉镜/纤维支气管镜检查，与颈部CT或MRI检查相结合，尽早明确诊断，及时手术解除梗阻。“,”Objective:To investigate the clinical characteristics of neonatal congenital tongue base cyst.Methods:This retrospective study involved 35 neonates with congenital tongue base cyst diagnosed in the neonatal intensive care unit (NICU) of Xi'an Children's Hospital from June 2013 to December 2019. General information, clinical manifestations, supplementary results, treatment and prognosis of these babies were described.Results:(1) The median age at the onset of the disease was 12.5 (0~28) d and the median age at admission was 15 (0~28) d for these babies. The main clinical manifestations were laryngeal stridor (28/35, 80.0%), inspiratory dyspnea and crying, especially when feeding (26/35, 74.3%) and choking and spitting with feeding (23/35, 65.7%). (2) Among the 35 cases, 15 (42.9%) required emergency endotracheal intubation due to significant dyspnea when were admitted to the NICU and five out of them were considered for having tongue base mass under laryngoscopy, while the other 10 cases underwent bedside electronic laryngoscopy after endotracheal intubation, in which space-occupying lesions were found. Tongue base cyst was considered in seven cases with laryngeal stridor complicated by protracted pneumonia using fiberoptic bronchoscopy. The other 13 cases were examined by electronic laryngoscope and considered as tongue base cyst. Thirty-five cases underwent cervical ultrasound and only five of them were considered as tongue base tumor. Thirty-two cases underwent cervical CT scan and only two of them were normal. Three cases were found to have tongue base cyst by cranial MRI. (3) Thirty-four cases were treated by radiofrequency ablation assisted with self-retaining microlaryngoscope and general anesthesia, while the other one firstly received puncture and drainage under direct laryngoscope due to the difficult intubation because of the huge tongue base cyst and then underwent surgery when stable. Only one case (2.9%) relapsed after surgical treatment during regular follow-up.Conclusions:Neonatal congenital tongue base cyst has an early onset and atypical clinical manifestations. Electronic laryngoscopy/fiberoptic bronchoscopy combined with neck CT or MRI examination should be performed promptly in patients with laryngeal stridor and inspiratory dyspnea to facilitate the accurate diagnosis and timely surgery is required for.