Yan Wenbo,Wang Jun,Pan Weihua,et al.Clinical characteristics and efficacy comparison of congenital diaphragmatic eventration patients with left/right side lesion[J].Journal of Clinical Pediatric Surgery,2019,18(12):1057-1060,1066.[doi:10.3969/j.issn.1671-6353.2019.12.014]
左右侧别先天性膈膨升的临床特征及预后比较研究
- Title:
- Clinical characteristics and efficacy comparison of congenital diaphragmatic eventration patients with left/right side lesion
- Keywords:
- Thoracoscopy; Diaphragmatic Eventration; Prognosis; Comparative Study; Child
- 分类号:
- R726;R565
- 摘要:
- 目的 先天性膈膨升（congenital diaphragmatic eventration，CDE）是一种膈肌发育缺陷的疾病，可影响呼吸功能及生长发育。本文旨在比较CDE左右侧病变的临床特征及预后。方法 回顾性分析2007年1月至2017年6月收治的先天性膈膨升43例，男童31例，女童12例，平均年龄12个月（2~72个月）。右侧35例，左侧8例。除1例经腹开放手术，1例经胸开放手术，2例经腹腔镜手术外，其余39例均采用胸腔镜微创手术。同时对左右侧别CDE的基本资料、临床表现、合伴畸形、胸部X线平片膈肌膨升的高度、治疗效果进行比较。随访时间平均为4.75年。结果 不同侧别CDE患儿的性别、年龄、出生体重、胎龄及分娩方式等基本资料无统计学差异（P>0.05）。合伴畸形中以先天性心脏病为主9/43（20.93%），左侧合并先天性心脏病的比例为62.5%（5/8），明显高于右侧11.43%（4/35），差异有统计学意义（P<0.01）。88%（38/43）CDE均有明显的呼吸道症状表现，左右侧比较差异无统计学意义（P=0.18）。但是胸部平片显示膨升膈肌高度左侧比右侧高，差异有统计学意义（t=3.18，P<0.01）。有17例存在反复呼吸道感染病史，其感染时间的长短与膈膨升高度比较，9例符合，8例不符，故目前尚不足以证实膈膨升高度与临床症状的严重程度呈正相关。所有患儿平均住院（13.30±5.05）d后痊愈出院，出院随访发现除1例右侧CDE复发外，其他患儿临床症状均缓解。结论 先天性膈膨升是一种横膈发育缺陷疾病，以右侧多见，左右侧病变比较基本上无多大差别；左侧膈膨升合伴先天性心脏病多于右侧；左侧膈膨升横膈抬高更明显。左右侧临床症状及预后情况基本一致。
- Abstract:
- Objective Congenital diaphragmatic eventration (CDE) due to diaphragmatic dysplasia may impair respiratory functions and arrest normal growth.The goal of this study was to compare the clinical characteristics and prognoses of CDE patients with left versus right-sided lesions.Methods Retrospective reviews were conducted for 43 CDE children from January 2007 to June 2017.There were 31 boys and 12 girls with an average age of 12 (2-72) months.And the involved side was right (n=35) and left (n=8).The procedures included thoracoscopic mini-invasive surgery (n=39),open transabdominal surgery (n=1),open transthoracic surgery (n=1) and laparoscopy (n=2).Basic profiles,clinical manifestations,associated malformations,height of diaphragm on chest plain film and surgical efficacy were compared. Results During an average follow-up period of 4.75(1-11) years,no significant difference existed in gender,age,birth weight,gestational age or delivery mode between left/right siders.Congenital heart diseases accounted for 20.93% (9/43) of CDE-associated malformations.And 5/8 cases were diagnosed with left-side CDE and 4/35 cases with right-side CDE.A significant difference existed between the incidence of congenital heart disease associated with left-side (62.5%) and right-side (11.43%) CDE.88% of CDE children developed respiratory symptoms and no significant difference between left/right-side CDE (P=0.18).And height of elevated diaphragm was higher at left side than at right side on chest plain film (t=3.18,P<0.01).Among 17 cases of recurrent respiratory infections,9 cases with long-duration infection had a high level of CDE.Therefore it was insufficient to confirm that height of CDE was positively correlated with severity of clinical manifestations.The average length of hospitalization stay was (13.3±5.05) days.All patients had clinical remission except for one recurrence of right-side CDE.Conclusion CDE occurs more frequently right side.Few differences exist between left/right-side CDE except that congenital heart disease is more often associated with left-side CDE and the height of elevated diaphragm is higher at left side than right side on chest plain film.However,no significant difference exists clinical symptoms or prognosis.
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备注/Memo
收稿日期:2018-10-15。
基金项目:上海交通大学医工交叉项目（编号：YG2017MS76）
通讯作者:王俊,Email:wangjun@xinhuamed.com.cn;施诚仁,Email:Shicr629@163.com