Department of Otolaryngology, Second Hospital of Hebei Medical University, Shijiazhuang, China.
Medicine (Baltimore). 2024 Jun 7;103(23):e38534. doi: 10.1097/MD.0000000000038534.
Crouzon syndrome is an extremely rare craniofacial dysplasia, which is mainly caused by the early ossification and closure of the coronal suture of the skull. Craniofacial deformities can cause stenosis of the nasal cavity and posterior nasal meatus, resulting in sleep apnea.
A 9-year-old boy with sleep snoring for 6 years, progressive aggravation in the past 1 month and accompanied by apnea during sleep.
This case was diagnosed with Crouzon syndrome complicated with severe obstructive sleep apnea and severe hypoxemia.
After adenoidectomy, he was admitted to the pediatric intensive care unit with ventilator-assisted respiration. During this period, the blood oxygen saturation fluctuated greatly. After trying to extubate, the blood oxygen was difficult to maintain and had to be intubated again. After active treatment, extubation was successful.
The wound of nasopharynx recovered well and the sleep state was significantly improved 3 months postoperation.
It is suggested that the time of ventilator-assisted breathing should be prolonged and the perioperative airway management should be strengthened in order to reduce the risk of postoperative complications.
颅缝早闭症是一种极罕见的颅面骨发育不全症，主要由颅骨冠状缝的早期骨化和闭合引起。颅面畸形可导致鼻腔和后鼻道狭窄，从而导致睡眠呼吸暂停。
一名 9 岁男孩，打鼾 6 年，近 1 个月来逐渐加重，并伴有睡眠呼吸暂停。
本例被诊断为颅缝早闭症合并严重阻塞性睡眠呼吸暂停和严重低氧血症。
行腺样体切除术，转入儿科重症监护病房，呼吸机辅助呼吸。期间血氧饱和度波动较大，尝试拔管后血氧难以维持，再次行气管插管。经积极治疗后，成功拔管。
鼻咽部伤口恢复良好，术后 3 个月睡眠状态明显改善。
建议延长呼吸机辅助呼吸时间，加强围手术期气道管理，以降低术后并发症风险。
