Cantrell五联症患儿胸骨缺损的外科治疗

Surgical treatments for sternal defects in children with pentalogy of Cantrell

目的总结Cantrell五联症(pantelogy of Cantrell)患儿胸骨缺损的修补方法及临床疗效,以优化其外科治疗策略。方法回顾性分析2009年1月至2022年12月于上海交通大学医学院附属上海儿童医学中心接受外科治疗的11例Cantrell五联症患儿临床资料,患儿一期手术时年龄为181 d(49,343)d,手术时体重为(7.9±4.9)kg。11例均存在胸骨下段缺损及先天性心脏结构畸形,其中完全型Cantrell五联症7例,不完全型Cantrell五联症4例;均接受一期心脏回纳及胸骨重建手术。根据胸骨修复方式分为直接缝合组(n=6)和非直接缝合组(n=5);非直接缝合组中,采取GORE-TEX?补片修补3例,人造胸骨1例,钛网修复1例。根据患儿一期心脏回纳术前影像学资料,测量胸廓前壁正中膈肌水平处的胸廓横径、胸骨缺损横径,测量残留胸骨最下端至胸廓前壁正中膈肌水平的距离作为胸骨缺损纵径;计算胸骨缺损指数(sternal defect index,SDI),SDI=胸骨缺损横径×2/(胸骨缺损纵径+胸廓横径×0.2)。结果直接缝合组患儿一期手术时SDI值小于非直接缝合组,差异有统计学意义(P=0.003);两组手术年龄、体重、完全型Cantrell五联症占比及复杂心脏畸形占比差异均无统计学意义(P>0.05)。11例Cantrell五联症患儿中,2例于心脏手术后因心力衰竭死亡,其余9例随访6个月至10年,除1例尚未接受心脏根治手术外,其余患儿心脏功能良好,胸廓外观满意,生活质量良好。结论Cantrell五联症患儿的预后主要取决于心脏异位与心内畸形的严重程度。SDI作为一个创新指标,对手术策略的选择具有一定参考意义。对于1岁以内、SDI<1的患儿,可首先尝试直接缝合胸骨,如无法直接缝合,可使用人造材料重建胸骨。

Objective To optimize the surgical strategies for sternal defect in children with pentalogy of Cantrell(PC)through comparing two types of sternal repair and outcomes.Methods From January 2009 to December 2022,clinical data were retrospectively reviewed for 11 PC children.They underwent stage I surgeries at a median age of 181 days(interquartile range:49 to 343 days)and with an average weight of(7.9±4.9)kg.There were substernal segment defects and congenital heart structural deformities,including complete PC(n=7)and incomplete PC(n=4).Stage I cardiac retraction and sternal reconstruction were performed.According to the mode of repairing sternum,they were assigned into two groups of direct closure(n=6)and non-direct closure(GORE-TEX?patch repair,n=3;artificial sternum,n=1;titanium mesh,n=1).According to the imaging data before stage I cardiac retraction operation,transverse dimension of the thorax on the level of diaphragm at the middle of anterior thoracic wall,the transverse dimension of sternal defect on the same level were measured,and the distance from the lowest margin of residual sternum to the above-mentioned level was measured as longitudinal dimension of sternal defect.We proposed a sternal defect index(SDI)and calculated,that is,the transverse dimension of sternal defect×2/(longitudinal dimension of sternal defect+transverse dimension of thorax×0.2).Results In the whole cohort,2(2/11)died from cardiac failure post-operation.Nine survivors(9/11)were followed up for 6 to 120 months.One(1/9)child did not undergo complete cardiac surgery while the remainders(8/9)obtained excellent cardiac function,satisfactory thoracic appearance and decent quality-of-life.SDI value of direct closure group was lower than that of non-direct closure group with significant statistic difference(P=0.003);No significant inter-group differences existed in average age,body weight,proportion of complete PC or complex cardiac deformities(P>0.05).Conclusion The prognosis of PC is dependent largely upon the severity of cardiac heterotopia and intracardiac malformation.Different techniques of sternal reconstruction may be guided by SDI.It is an innovative tool for evaluating the severity of sternal defect.For children aged under 1 year with SDI<1,direct sternal closure is recommended;if direct closure is not available,artificial material repair is a practical alternative.