Dahl Mar Janna, Lavizzari Anna, Davis Jonathan W, Noble Peter B, Dellacà Raffaele, Pillow J Jane
School of Human Sciences, University of Western Australia, Perth, Western Australia, Australia.
NICU, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Anat Rec (Hoboken). 2025 Apr;308(4):1066-1081. doi: 10.1002/ar.25059. Epub 2022 Sep 5.
The extent of lung hypoplasia impacts the survival and severity of morbidities associated with congenital diaphragmatic hernia (CDH). The alveoli of CDH infants and in experimental models of CDH have thickened septa with fewer type II pneumocytes and capillaries. Fetal treatments of CDH-risk preterm birth. Therefore, treatments must aim to balance the need for increased gas exchange surface area with the restoration of pulmonary epithelial type II cells and the long-term respiratory and neurodevelopmental consequences of prematurity. Achievement of sufficient lung development in utero for successful postnatal transition requires adequate intra-thoracic space for lung growth, maintenance of sufficient volume and appropriate composition of fetal lung fluid, regular fetal breathing movements, appropriate gas exchange area, and ample surfactant production. The review aims to examine the rationale for current and future therapeutic strategies to improve postnatal outcomes of infants with CDH.
肺发育不全的程度会影响先天性膈疝(CDH)相关疾病的生存率和严重程度。CDH婴儿以及CDH实验模型中的肺泡间隔增厚,II型肺细胞和毛细血管数量减少。胎儿期治疗可降低CDH导致早产的风险。因此,治疗必须在增加气体交换表面积的需求与恢复肺上皮II型细胞以及早产带来的长期呼吸和神经发育后果之间寻求平衡。为了在子宫内实现足够的肺发育以成功实现出生后过渡,需要有足够的胸腔空间用于肺生长、维持足够的胎儿肺液体积和适当成分、规律的胎儿呼吸运动、合适的气体交换面积以及充足的表面活性物质产生。本综述旨在探讨当前和未来治疗策略的理论依据,以改善CDH婴儿的出生后结局。