Shi Xu-Cong, Weng Jian-Bin, Yu Jin, Ma Xiao-Hui, Pu Yi-Qing, Ying Li-Yang, Yu Jian-Gen
Department of Cardiac Surgery, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Department of Neurosurgery, The Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Front Cardiovasc Med. 2022 Jan 26;8:790303. doi: 10.3389/fcvm.2021.790303. eCollection 2021.
Berry syndrome is a challenging disease for surgeons to make early diagnosis and successful surgical correction in the neonatal period. Here, we summarized the clinical features of three neonates with berry syndrome in our center to optimize the therapeutic effect in the future.
From January 2014 to December 2019, three neonates with berry syndrome underwent one-stage surgical repair in our center. We mainly used two different surgical techniques to repair it, and collected clinical data retrospectively from hospitalization history, outpatient records, and telephone follow-up.
The age at operation was 28, 8, and 8 days and the body weight was 3.65, 3.86, and 3.0 kg, respectively. The morphology of the interrupted aortic arch (IAA) was type A in two patients and type B in one patient. The aortopulmonary window (APW) morphology was type IIa, III, and IIb, respectively. The phenotype of the IAA type B combined with APW type III in our second patient was reported for the first time so far. All patients survived and were followed up to date. The second patient using intra-aortic baffle experienced twice reoperation for right pulmonary artery (RPA) stenosis. All patients grew well so far.
Once diagnosed in the neonatal period, berry syndrome can be safely corrected by one-stage surgical repair in experienced cardiac centers. Considering the variability of the location where the RPA arises from the posterior wall of the aorta, it is difficult to find the best surgical method for each patient.
对于外科医生来说，贝里综合征是一种在新生儿期难以早期诊断和成功进行手术矫正的疾病。在此，我们总结了本中心3例贝里综合征新生儿的临床特征，以便未来优化治疗效果。
2014年1月至2019年12月，3例贝里综合征新生儿在本中心接受了一期手术修复。我们主要采用两种不同的手术技术进行修复，并从住院病史、门诊记录和电话随访中回顾性收集临床资料。
手术年龄分别为28天、8天和8天，体重分别为3.65kg、3.86kg和3.0kg。2例患者中断性主动脉弓（IAA）形态为A型，1例为B型。主肺动脉窗（APW）形态分别为IIa型、III型和IIb型。迄今为止，首次报道了我们第二例患者中IAA B型合并APW III型的表型。所有患者均存活并随访至今。第二例使用主动脉内挡板的患者因右肺动脉（RPA）狭窄接受了两次再次手术。到目前为止，所有患者生长良好。
一旦在新生儿期确诊，在经验丰富的心脏中心，贝里综合征可以通过一期手术修复安全矫正。考虑到RPA起源于主动脉后壁位置的变异性，很难为每个患者找到最佳手术方法。
