Division of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
Fetal Diagn Ther. 2022;49(5-6):250-255. doi: 10.1159/000521690. Epub 2022 Apr 29.
Congenital pulmonary airway malformations (CPAMs) complicated by hydrops portend significant morbidity and mortality, with fetal survival estimates less than 10%.
We report successful use of ultrasound-guided radiofrequency ablation at 21-week gestation in a hydropic fetus with CPAM, with subsequent resolution of hydrops. Thirty-two-week MRI noted persistent mediastinal shift, and US at 36 weeks and 5 days noted polyhydramnios. Maternal gestational hypertension prompted delivery at 37 weeks, with a cesarean section performed after a failed trial of labor. The infant required CPAP at 100% and weaned to 21%. Tachypnea persisted, and chest CT on day of life 2 demonstrated multiple large cysts in the right lower lobe with anterior pneumothorax. On day of life 3, she successfully underwent a thoracoscopic right lower lobectomy. Adhesions to the chest wall and rib abnormalities were noted. She was extubated to CPAP at the conclusion of the procedure. She was able to wean to 21% on POD2 and transitioned to oral feeds. Her chest tube was removed with resultant ex vacuo pneumothorax noted. She remained asymptomatic and was discharged home on room air POD11. Pathology confirmed a type 1 CPAM.
In utero radiofrequency ablation may be an adjunct to the management of large CPAM.
先天性肺气道畸形（CPAMs）合并水肿预示着严重的发病率和死亡率，胎儿存活率估计不到 10%。
我们报告了一例在 21 孕周患有 CPAM 合并水肿的胎儿中成功使用超声引导下射频消融的案例，随后水肿得到缓解。32 周 MRI 显示纵隔移位持续存在，36 周 5 天时的 US 显示羊水过多。母体妊娠高血压促使在 37 周进行分娩，在一次试产失败后进行剖宫产。婴儿需要 100%的 CPAP，并在 21%时逐渐减少。呼吸急促持续存在，出生后第 2 天的胸部 CT 显示右下叶有多个大囊肿，并伴有前气胸。出生后第 3 天，她成功地接受了胸腔镜下右下肺叶切除术。注意到胸腔壁粘连和肋骨异常。手术结束时，她被拔管并接受 CPAP 治疗。她在第 2 天成功地从 CPAP 过渡到口服喂养。胸部引流管被移除，出现真空性气胸。她没有症状，并在第 11 天出院，在室内空气下继续接受治疗。病理证实为 1 型 CPAM。
宫内射频消融术可能是治疗大型 CPAM 的辅助手段。
