Cardiothoracic Surgery Department, Children's Hospital of Fudan University, Shanghai, China.
Respirology Department, Children's Hospital of Fudan University, Shanghai, China.
Birth Defects Res. 2020 Mar;112(5):418-426. doi: 10.1002/bdr2.1652. Epub 2020 Jan 29.
Few studies have assessed the comorbid diseases in situs inversus totalis (SIT) comprehensively. The aim of this study was to provide insight into the spectrum and prevalence of comorbidities in SIT.
Children ≤18 years of age with SIT were enrolled in this retrospective observational study. Situs status and comorbidities were independently confirmed by two physicians, based on review of radiologic, ultrasonic examination, operative records, and case notes.
A total of 155 children (median age: 1.24 years; range: 1 day-17.8 years) confirmed to have SIT were recruited between January 2008 and December 2018. Associated conditions were diagnosed in 114 children (73.5%). Among them, 25 children (16.1%) had multiple anomalies affecting two or more organ systems. The most commonly associated conditions were congenital heart defects (n = 72, 46.5%) followed by primary ciliary dyskinesia (n = 19, 12.3%), renal disorders (n = 12, 7.7%), biliary atresia (n = 7, 4.5%), skeletal dysplasia (n = 8, 5.2%), and mental retardation (n = 4, 2.6%).
A substantial proportion of children with SIT have comorbidities affecting multiple systems, especially cardiovascular and respiratory abnormalities. Children with SIT warrant careful examination for the presence of congenital and acquired abnormalities.
仅有少数研究全面评估了完全性内脏逆位(SIT)的合并症。本研究旨在深入了解 SIT 合并症的谱和患病率。
本回顾性观察性研究纳入了≤18 岁的 SIT 患儿。根据对影像学、超声检查、手术记录和病历的审查,两名医生独立确认了内脏位置和合并症。
2008 年 1 月至 2018 年 12 月期间共纳入了 155 例确诊为 SIT 的儿童(中位数年龄:1.24 岁;范围:1 天至 17.8 岁)。在这些患儿中,114 例(73.5%)存在相关疾病。其中,25 例(16.1%)患儿存在影响两个或多个器官系统的多种异常。最常见的相关疾病是先天性心脏病(n = 72,46.5%),其次是原发性纤毛运动障碍(n = 19,12.3%)、肾脏疾病(n = 12,7.7%)、胆道闭锁(n = 7,4.5%)、骨骼发育不良(n = 8,5.2%)和智力迟钝(n = 4,2.6%)。
相当一部分 SIT 患儿存在影响多个系统的合并症,尤其是心血管和呼吸系统异常。SIT 患儿需要仔细检查有无先天性和获得性异常。