Partridge Emily A, Peranteau William H, Herkert Lisa, Rendon Norma, Smith Haylee, Rintoul Natalie E, Flake Alan W, Adzick N Scott, Hedrick Holly L
The Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA.
The Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA.
J Pediatr Surg. 2016 Jun;51(6):900-2. doi: 10.1016/j.jpedsurg.2016.02.049. Epub 2016 Mar 3.
Right-sided congenital diaphragmatic hernia (R-CDH) occurs in up to 25% of all CDH cases, but has been less widely studied compared to left-sided defects. We sought to compare characteristics and outcomes of left- versus right-sided defects in a large cohort of CDH patients who underwent standardized treatment and follow-up at a single institution.
We retrospectively reviewed charts of all CDH patients in our pulmonary hypoplasia program from January 2002 through December 2014. Categorical variables were analyzed by Fisher's exact test and continuous variables by Mann-Whitney t-test (p≤0.05).
A total of 330 CDH patients were treated more than the 12-year study period, with 274 (83%) left-sided and 56 (17%) right-sided cases identified. Specific pulmonary morbidities were associated with R-CDH, with increased duration of nitric oxide therapy, increased requirement for tracheostomy, increased requirement for supplemental oxygen at the time of NICU discharge, and increased chronic pulmonary hypertension with requirement for long-term Sildenafil therapy.
In this series, R-CDH was not associated with increased mortality, but was associated with increased requirement for pulmonary vasodilatory therapy and requirement for tracheostomy. The high incidence of pulmonary complications indicates increased severity of pulmonary hypoplasia in R-CDH, supporting a role for delivery in tertiary centers with expertise in CDH management.
右侧先天性膈疝(R - CDH)占所有先天性膈疝病例的25%,但与左侧缺陷相比,其研究较少。我们试图比较在单一机构接受标准化治疗和随访的一大群先天性膈疝患者中,左侧与右侧缺陷的特征和结果。
我们回顾性分析了2002年1月至2014年12月在我们肺发育不全项目中所有先天性膈疝患者的病历。分类变量采用Fisher精确检验分析,连续变量采用Mann - Whitney t检验(p≤0.05)。
在超过12年的研究期间,共治疗了330例先天性膈疝患者,其中274例(83%)为左侧病例,56例(17%)为右侧病例。特定的肺部疾病与右侧先天性膈疝相关,包括一氧化氮治疗时间延长、气管切开需求增加、新生儿重症监护病房出院时补充氧气需求增加以及慢性肺动脉高压增加且需要长期使用西地那非治疗。
在本系列研究中,右侧先天性膈疝与死亡率增加无关,但与肺血管扩张治疗需求增加和气管切开需求增加有关。肺部并发症的高发生率表明右侧先天性膈疝中肺发育不全的严重程度增加,这支持在具有先天性膈疝管理专业知识的三级中心进行分娩的作用。