Abstract：
OBJECTIVE: To describe characteristics, treatments and clinical outcomes of patients with trisomy 18 and oesophageal atresia, using a nationwide database in Japan.
METHODS: Descriptive study using a retrospective cohort.
METHODS: A nationwide inpatient database including 90% of hospitals with neonatal intensive care units in Japan.
METHODS: Patients hospitalised within a day after birth for both oesophageal atresia and trisomy 18 between July 2010 and March 2020.
METHODS: Radical surgery for oesophageal atresia.
METHODS: Characteristics, treatment course and outcomes.
RESULTS: Among 271 patients with both oesophageal atresia and trisomy 18, 70 patients underwent radical surgery for oesophageal atresia. Patients who underwent radical surgery were less likely to have severe cardiac anomalies (17% vs 32%; p=0.020), but more likely to undergo cardiac surgery (21% vs 9.5%; p=0.012) than those who did not. The overall in-hospital mortality was lower (54% vs 79%; p<0.001) and the median age at death was higher (210 days vs 39 days; p<0.001) in patients who underwent radical surgery than the others. Postoperative mortality within 30 days after radical surgery was 5.7%. Patients who underwent radical surgery were likely to be discharged to home (50% vs 18%; p<0.001), whereas the age at home discharge (median 314 days vs 216 days; p=0.19) and the requirement for each home treatment did not differ significantly by radical surgery.
CONCLUSIONS: This study provides information that will aid the clinical decision-making process for patients with oesophageal atresia and trisomy 18. Radical surgery may be a safe and feasible treatment option.
METHODS: Descriptive study using a retrospective cohort.
METHODS: A nationwide inpatient database including 90% of hospitals with neonatal intensive care units in Japan.
METHODS: Patients hospitalised within a day after birth for both oesophageal atresia and trisomy 18 between July 2010 and March 2020.
METHODS: Radical surgery for oesophageal atresia.
METHODS: Characteristics, treatment course and outcomes.
RESULTS: Among 271 patients with both oesophageal atresia and trisomy 18, 70 patients underwent radical surgery for oesophageal atresia. Patients who underwent radical surgery were less likely to have severe cardiac anomalies (17% vs 32%; p=0.020), but more likely to undergo cardiac surgery (21% vs 9.5%; p=0.012) than those who did not. The overall in-hospital mortality was lower (54% vs 79%; p<0.001) and the median age at death was higher (210 days vs 39 days; p<0.001) in patients who underwent radical surgery than the others. Postoperative mortality within 30 days after radical surgery was 5.7%. Patients who underwent radical surgery were likely to be discharged to home (50% vs 18%; p<0.001), whereas the age at home discharge (median 314 days vs 216 days; p=0.19) and the requirement for each home treatment did not differ significantly by radical surgery.
CONCLUSIONS: This study provides information that will aid the clinical decision-making process for patients with oesophageal atresia and trisomy 18. Radical surgery may be a safe and feasible treatment option.
摘要：
目的：描述特征，18三体综合征和食管闭锁患者的治疗和临床结果，使用日本的全国数据库。
方法：使用回顾性队列的描述性研究。
方法：全国住院数据库，包括日本90%的新生儿重症监护病房医院。
方法：2010年7月至2020年3月期间，因食管闭锁和18三体综合征而在出生后一天内住院的患者。
方法：食管闭锁的根治性手术。
方法：特征，治疗过程和结果。
结果：在271例食管闭锁和18三体的患者中，有70例患者接受了食管闭锁的根治性手术。接受根治性手术的患者发生严重心脏异常的可能性较小(17%vs32%;p=0.020)。但接受心脏手术的可能性高于未接受心脏手术的患者(21%vs9.5%;p=0.012)。接受根治性手术的患者的总体住院死亡率较低（54％vs79％；p<0.001），中位死亡年龄较高（210天比39天；p<0.001）。根治性手术后30天内的术后死亡率为5.7％。接受根治性手术的患者可能会出院回家（50％vs18％；p<0.001），而家庭出院年龄(中位数314天对216天;p=0.19)和每次家庭治疗的需求在根治性手术中没有显著差异.
结论：本研究提供的信息将有助于食管闭锁和18三体综合征患者的临床决策过程。根治性手术可能是一种安全可行的治疗选择。
方法：使用回顾性队列的描述性研究。
方法：全国住院数据库，包括日本90%的新生儿重症监护病房医院。
方法：2010年7月至2020年3月期间，因食管闭锁和18三体综合征而在出生后一天内住院的患者。
方法：食管闭锁的根治性手术。
方法：特征，治疗过程和结果。
结果：在271例食管闭锁和18三体的患者中，有70例患者接受了食管闭锁的根治性手术。接受根治性手术的患者发生严重心脏异常的可能性较小(17%vs32%;p=0.020)。但接受心脏手术的可能性高于未接受心脏手术的患者(21%vs9.5%;p=0.012)。接受根治性手术的患者的总体住院死亡率较低（54％vs79％；p<0.001），中位死亡年龄较高（210天比39天；p<0.001）。根治性手术后30天内的术后死亡率为5.7％。接受根治性手术的患者可能会出院回家（50％vs18％；p<0.001），而家庭出院年龄(中位数314天对216天;p=0.19)和每次家庭治疗的需求在根治性手术中没有显著差异.
结论：本研究提供的信息将有助于食管闭锁和18三体综合征患者的临床决策过程。根治性手术可能是一种安全可行的治疗选择。
