■在1954年首次描述了Jeune综合征（JS），常被称为窒息性胸廓营养不良,是一种以短肋骨为特征的先天性肌肉骨骼疾病，狭窄的胸部,和四肢小。在这项研究中,我们分析并介绍了我们对有症状的JS患者的胸骨渐进性内部牵引（PIDS）装置的初步经验。此外,我们回顾了关于治疗先天性JS儿童的现有外科技术的当代英文文献。
■对2017年至2023年在我们的三级中心接受症状性JS治疗的儿科患者（<18岁）进行了回顾性分析。
■我们介绍了两名JS患者，他们使用内部胸骨牵引器接受了手术，苏黎世II微型苏黎世模块化牵引器，放置在胸骨的主体之间分开的两半。
■我们在有症状的JS患者中获得了关于这种微创装置用于PIDS的安全性和有效性的有希望的结果。需要对长期结果进行进一步的研究来验证这些发现。
UNASSIGNED: Described for the first time in 1954, Jeune syndrome (JS), often called asphyxiating thoracic dystrophy, is a congenital musculoskeletal disease characterized by short ribs, a narrow thorax, and small limbs. In this study, we analyzed and presented our preliminary experience with a device for progressive internal distraction of the sternum (PIDS) in patients with symptomatic JS. In addition, we reviewed the contemporary English literature on existing surgical techniques for treating children with congenital JS.
UNASSIGNED: A retrospective analysis of pediatric patients (<18 years old) treated for symptomatic JS at our tertiary center between 2017 and 2023 was performed.
UNASSIGNED: We presented two patients with JS who underwent surgery using an internal sternal distractor, a Zurich II Micro Zurich Modular Distractor, placed at the corpus of the sternum among the divided halves.
UNASSIGNED: We obtained promising results regarding the safety and effectiveness of this less-invasive device for PIDS in patients with symptomatic JS. Further studies on long-term outcomes are needed to validate these findings.
PDF(Pubmed)