Cantrell 五联症患儿胸骨缺损的外科治疗.

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. [ABSTRACT FROM AUTHOR]