胎儿右侧先天性膈疝合并肝肺融合3例并文献复习

Management of right-sided fetal congenital diaphragmatic hernia with hepatopulmonary fusion: a report of three cases and literature review

目的探讨右侧先天性膈疝(right-sided congenital diaphragmatic hernia,RCDH)合并肝肺融合(hepatopulmonary fusion,HPF)的临床特点和管理。方法回顾性分析2022年6月至12月在广州市妇女儿童医疗中心收治的3例RCDH合并HPF患儿的临床特点,并根据国际膈肌缺损分级标准进行缺损分级。在中国知网、万方数据库、中华医学期刊全文数据库及维普中文期刊以“先天性膈疝”“肝肺融合”检索1997年1月至2023年4月的中文文献报道,在PubMed、Embase及UpToDate数据库以“congenital diaphragmatic hernia”“hepatopulmonary fusion”为关键词检索同期相关英文文献。分析RCDH合并HPF的临床特点、管理及预后等情况。结果(1)本院病例资料:病例1和病例2产前超声发现胎儿右侧膈疝,产前MRI均提示胎儿肝脏疝入右侧胸腔、右肺发育不良、左肺大小正常和无纵隔左移。病例1和病例3出生后的胸部X射线检查可见右肺致密影和纵隔右移。病例2为D型缺损,出生后胸部X射线检查示纵隔稍左移。3例患儿术前影像学检查均高度怀疑HPF。病例1和病例2为肝肺完全分离,并采用补片修补,病例2进行部分肺切除,2例患儿均存活(住院时间分别为22和23 d)。病例3在手术探查后未进行肝肺分离,未采用补片修补,术后死于持续性肺动脉高压。(2)文献复习:共检索到40例CDH合并HPF病例,加上本院3例RCDH合并HPF,共43例,其中27例(62.8%)术前发现纵隔右移或无移位,9例(20.9%)纵隔左移,7例(16.3%)未描述;73.1%(19/26)进行肝肺完全分离的患儿存活,13例进行肝肺部分分离中6例存活,4例未行肝肺分离均死亡。结论对于RCDH患儿需要考虑是否存在HPF可能,影像学提示未伴纵隔左侧移位应高度怀疑HPF。此类病例的外科手术术前评估需按照重大手术(可能需要肝或部分肺切除术)进行管理。

Objective To investigate the clinical features and management of right-sided congenital diaphragmatic hernia(RCDH)with hepatopulmonary fusion(HPF).Methods This retrospective study analyzed the clinical characteristics of three cases of RCDH complicated by HPF that were treated in Guangzhou Women and Children's Medical Center from June to December 2022.Diaphragm defects in the three cases were classified according to the international standard of diaphragm defect classification.Besides,an extensive search of publications was performed including domestic and foreign databases,including CNKI,Wanfang Database,Yiigle,VIP Chinese journals,PubMed,Embase and UpToDate databases from January 1997 to April 2023 using terms including"congenital diaphragmatic hernia"and"hepatopulmonary fusion".Clinical features and prognosis of RCDH complicated by HPF were summarized.Results(1)Cases in the present study:RCDH was found in case 1 and case 2 during routine prenatal ultrasound examination;antenatal fetal MRI showed partial displacement of the hepatocele into the right hemithorax,right lung hypoplasia,a normal-sized left lung and without left shift of the mediastinum in both cases.Postnatal chest radiographs of case 1 and case 3 showed dense shadow in the left lung and mediastinum shifted to the right.Case 2 had a D-type defect and a slight shift of the mediastinum to the left was observed on the postnatal chest radiograph.Preoperative imaging findings indicated highly suspected HPF in the three cases.Case 1 and case 2 had complete separation of liver and lung and underwent diaphragmatic herniorrhaphy with patch.Partial lung resection was performed in case 2.Both case 1 and case 2 survived(length of hospital stay was 22 d and 23 d,respectively).Case 3 did not undergo hepatopulmonary separation or herniorrhaphy after exploratory operation and died of persistent pulmonary hypertension.(2)Literature review:Only 40 cases of CDH with HPF were retrieved from PubMed.Among the 43 cases including the above three cases,27(62.8%)had a right shift or no deviation of the mediastinum before surgery and nine(20.9%)had a left shift of the mediastinum,while the condition of seven patients(16.3%)were not described.There were 26 patients undergoing complete separation of liver and lung and 19(73.1%)of them survived.Thirteen patients underwent partial separation of liver and lung and six of them survived.Four patients died without receiving separation.Conclusions HPF should be considered in patients with RCDH,especially in cases with no left shift in the mediastinum in the imaging.Preoperative evaluation for surgery in such cases needs to be managed as if it were a major operation that may require hepatectomy or partial pneumonectomy.