体外膜肺氧合下先天性膈疝转运与外科治疗一例并文献复习

Extracorporeal membrane oxygenation assisted transport and surgery of congenital diaphragmatic hernia： case report and literature review

目的探讨体外膜肺氧合（ECMO）下先天性膈疝治疗及转运中的重、难点。方法回顾性分析1例严重缺氧性呼吸衰竭先天性膈疝，ECMO建立、转运及治疗等临床资料并结合Pubmed数据库截止2015年前部分文献复习讨论当前先天性膈疝ECMO置入的适应证、时机、ECMO模式、新生儿ECMO抗凝难点、转运风险及产前辅助检查的意义。结果患儿，男，39周，3．75kg，产前诊断左膈疝，2015年8月1日18时30分在浙江大学妇产科医院出生，5minApgar7分，之后气促，发绀不断加重，紧急气管插管，状况仍不见明显改善，14h后紧急呼救浙江大学儿童医院ECMO团队，18h后建立VA-ECMO，缺氧立即改善，内环境逐渐稳定20h后实施ECMO转运，2h完成两家医院顺利对接，并完成手术修补，术后继续ECMO辅助，于2015年8月5日17时撤离ECMO，总运行76h。撤离ECMO出现上下肢差异性紫绀，上肢Sp0295％～100％，下肢Sp0285％～90％，血压52／34mmHg，肺动脉压／血压0．7～1．0，降低肺动脉压力，提高体循环收缩压措施，差异紫绀减轻，约2周后，差异紫绀消失，撤离呼吸机后，血气PaC02在75～55mmHg之间，肾脏代偿，HC0330．8～41．7mmol／L，pH7．39-7．40，出生25d血气PaC02下降接近正常水平，44d出院。ECMO间发生右髂静脉血栓，撤离后肝素溶栓成功。整个治疗过程均未发生颅内出血、栓塞性脑梗及伤口出血。随访6个月，生长发育良好，体重增长，呼吸平稳，X线胸片示左肺体积略小，膨胀良好。检索文献共有6篇文献专门就先天性膈疝ECMO置入的适应证、时机进行了相关讨论。结论ECMO辅助可提高先天性膈疝存活率，掌握置入时机及治疗特点是成功的关键。

Objective To explore the essential points in treatment and transferring of neonates with congenital diaphragmatic hernia using extracorporeal membrane oxygenation （ECMO）. Methods Retrospective reviews were conducted for the clinical records of one neonate with severe congenital diaphragmatic hernia. ECMO, neonatal transferal and treatment were analyed with a literature review. Indications for ECMO, optimal timing, anticoagulation difficulties, risks in transferral and value of prenatal diagnosis were systematically summarized. Results This boy was born at 39 weeks of gestation with a birth weight of 3.75 kg at Obstetrical ＆ Gynecological Hospital, Zhejiang University School of Medicine. The prenatal diagnosis was left diaphragmatic hernia. His Apgar score at 5 minute was 7 and apnea occurred immediately after birth. Emergency orotracheal intubation was performed because of worsening cyanosis. However, his conditions failed to improve after intubation. Then venoarterial ECMO was performed at 18 hours post-birth. Anoxia improved immediately after ECMO and hemodynamics became stable. ECMO transfer was implemented after 20 hours. He was trans[erred smoothly to our hospital within 2 hours. Surgical repair was performed and postoperative ECMO support maintained. ECMO was evacuated after a duration of 76 hours. Differential cyanosis of upper and lower extremities appeared after removing ECMO with SpO2 95%-100~~ in upper extremities and SpO2 85%-90% in lower extremities. The level of blood pressure was 52/34 mmHg and the ratio of pulmonary arterial pressure/blood pressure 0. 7-1.0. Efforts were undertaken for reducing pulmonary arterial pressure and increasing systemic systolic blood pressure; differential cyanosis improved and disappeared after 2 weeks. After removing orotracheal intubation, blood gas analysis showed PaCO2 75-55 mmHg, HCO3- 30. 8-41.7 mmol/L and pH 7. 39-7. 40; PaCO2 returned to normal at 25 days post-birth. He was discharged after 44 days. During ECMO, right iliae vein thrombosis occurred and responded well to optimal thrombolysis. No intracranial hemorrhage, embolic cerebral infarction and wound bleeding occurred during treatment. He had normal growth and development after a 6-month follow-up period. Chest radiograph showed smaller left lung with decent inflation. Candusions ECMO support boosts survival in patients with congenital diaphragmatic hernia. And grasping the optimal timing of ECMO and understanding the clinical characteristics of patients are essential.