[1]吕成杰,王鹏,马东,等.产儿合作一体化管理模式诊治巨型脐膨出的临床研究[J].临床小儿外科杂志,2024,(12):1133-1137.[doi:10.3760/cma.j.cn101785-202409081-005]
　Lyu Chengjie,Wang Peng,Ma Dong,et al.Childbirth cooperation integrated diagnosis and treatment of giant omphalocele[J].Journal of Clinical Pediatric Surgery,2024,(12):1133-1137.[doi:10.3760/cma.j.cn101785-202409081-005]
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产儿合作一体化管理模式诊治巨型脐膨出的临床研究

《临床小儿外科杂志》[ISSN:1671-6353/CN:43-1380/R] 卷: 期数: 2024年12 页码: 1133-1137 栏目: 专题·出生缺陷的产前产后一体化诊治 出版日期: 2024-12-01
Title:
Childbirth cooperation integrated diagnosis and treatment of giant omphalocele
作者:
吕成杰1;  王鹏1;  马东1;  赖登明1;  陈正2;  应力阳3;  谭征4;  王利权5;  钭金法1
1. 浙江大学医学院附属儿童医院新生儿外科, 杭州 310003;
2. 浙江大学医学院附属儿童医院新生儿重症监护病房, 杭州 310003;
3. 浙江大学医学院附属儿童医院心脏外科, 杭州 310003;
4. 浙江大学医学院附属儿童医院胸外科, 杭州 310003;
5. 浙江大学医学院附属第二医院产前诊断中心、产科, 杭州 310003
Author(s):
Lyu Chengjie1;  Wang Peng1;  Ma Dong1;  Lai Dengming1;  Chen Zheng2;  Ying Liyang3;  Tan Zhen4;  Wang Liquan5;  Tou Jinfa1
1. Department of Neonatal Surgery,Second Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China;
2. Neonatal Intensive Care Unit,Second Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China;
3. Department of Cardiac Surgery,Second Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China;
4. Department of Thoracic Surgery,Affiliated Children’s Hospital,Second Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China;
5. Department of Obstetrics,Center for Prenatal Diagnosis,Second Affiliated Hospital,Zhejiang University School of Medicine,Hangzhou 310003,China
关键词:
疝; 脐; 学科间信息交流; 妊娠结局; 治疗结果; 疾病管理
Keywords:
Hernia; Umbilical; Interdisciplinary Communication; Pregnancy Outcome; Treatment Outcome; Disease Management
DOI:
10.3760/cma.j.cn101785-202409081-005
摘要:
目的 探索产儿合作一体化管理模式在巨型脐膨出诊治中的应用价值。方法 回顾性分析2020年1月至2023年12浙江大学医学院附属儿童医院和浙江大学医学院附属第二医院采取产儿合作一体化管理模式诊治的巨型脐膨出患儿临床资料,包括产前、产时、产后诊疗全过程临床资料;随访9~36个月,观察指标包括腹壁切口愈合、喂养以及生长发育情况。结果 共76例巨型脐膨出患儿接受产儿合作一体化管理模式诊治,均经产前超声检查明确诊断,产前染色体和基因筛查无异常。其中产前咨询建议终止妊娠3例(1例泄殖腔畸形,1例脊柱畸形,1例体蒂综合征);家属选择终止妊娠2例,均为巨型脐膨出伴复杂心脏畸形。71例继续妊娠至出生的患儿均于产房包扎囊膜,其中2例囊膜破裂(1例于产前、1例于产时);入院时低血糖1例,低体温1例;合并肠旋转不良68例,梅克尔憩室2例,房间隔缺损6例,室间隔缺损4例,动脉导管未闭8例,食管闭锁2例,膈疝1例,支气管肺发育不良8例,肺动脉高压10例;予保守治疗7例,一期修补术35例,二期修补术27例,分期修补术2例,联合手术3例(1例膈疝,2例食管闭锁)。并发新生儿坏死性小肠结肠炎1例,肺炎、肺不张5例,切口感染3例,肠梗阻5例(1例保守治疗期间肝脏尾状叶压迫肠管导致肠梗阻,1例保守治疗期间肠粘连导致肠梗阻,3例手术治疗后出现粘连性肠梗阻)。66例治疗后出院存活,5例住院期间死亡(1例肺动脉高压,1例心功能不全,2例重症肺炎,1例腹腔间隙综合征)。66例出院存活患儿随访9~36个月,于居家喂养中意外呕吐致窒息死亡1例,胃食管反流致重症肺炎死亡1例,肺动脉高压转外院治疗过程中死亡2例;其余62例腹壁愈合良好,生长发育正常。结论 产儿合作一体化管理模式可以从产前、产时、产后全流程管理巨型脐膨出患儿,是巨型脐膨出的一种有效疾病管理模式。
Abstract:
Objective To explore the application of childbirth cooperative management model of giant omphalocele (GO). Methods From January 2020 to December 2023,prenatal,prenatal and postpartum treatment data were retrospectively reviewed for 76 GO neonates.During a follow-up period of (9-36) month,healing of abdominal wall incision,feeding and growth were recorded. Results Prenatal ultrasonography offered a definite diagnosis and prenatal chromosome and genetic screening showed no abnormalities.As recommended by antepartum multi-disciplinary treatment (MDT),termination was performed for cloaca malformation (n=1),spinal malformation (n=1) and pedicle syndrome (n=1).And pregnancy was terminated due to complex heart malformation (n=2) and 71 cases continued pregnancy.In the whole group,capsule was wrapped in delivery room.There were capsule rupture (n=2,antenatal and labor each),hypoglycemia on admission (n=1) and hypothermia (n=1).The whole group included intestinal malrotation (n=68),Meckel’s diverticulum (n=2),atrial septal defects (n=6),ventricular septal defects (n=4),patent ductus arteriosus (n=8),esophageal atresia (n=2),diaphragmatic hernia (n=1),bronchopulmonary dysplasia (n=8) and pulmonary hypertension (n=10).The interventions included conservative measures (n=7),primary repair (n=35),second stage repair (n=27),staged repair (n=2) and combined surgery (n=3,diaphragmatic hernia,n=1; esophageal atresia,n=2).There were NEC (n=1),pneumonia atelectasis (n=5),incision infection (n=3) and intestinal obstruction (n=5) (hepatic caudate lobe compression during conservative period,n=1; intestinal adhesion during conservative treatment,n=1; adhesive intestinal obstruction post-operation,n=3).During treatment period,66 patients were discharged and 5 died in hospital (pulmonary hypertension,n=1; cardiac insufficiency,n=1; severe pneumonia,n=2; abdominal space syndrome,n=1).During follow-ups,death was due to accidental vomiting & asphyxia (n=1) and severe pneumonia from gastroesophageal reflux (n=1).Two cases of pulmonary hypertension were transferred to external hospital for treatment and the remainders recovered well. Conclusions The above integration mode may provide early interventions and effective managements of neonatal GO.

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备注/Memo

收稿日期:2024-9-30。
基金项目:浙江省自然科学基金(LY22H040006)
通讯作者:钭金法,Email:toujinfa@zju.edu.cn

更新日期/Last Update: 2024-12-28