A review of congenital diaphragmatic hernia

Marlow, Jay; Thomas, Joseph

doi:10.1002/j.2205-0140.2013.tb00092.x

Cited by 13 publications

(7 citation statements)

References 37 publications

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“…Advancements in neonatal respiratory care, such as inhaled nitric oxide, have not resulted in an increase in survival rates in subsets of patients with CDH 13 , 14 . The inability of these therapies to improve outcomes suggests that a fixed component of the defective pulmonary vasculature is not responsive to postnatal pulmonary vasodilator therapy 15 , 16 and that directed prenatal therapy targeting the developing pulmonary vasculature may improve outcomes in CDH.…”

Section: Introductionmentioning

confidence: 99%

Intra-amniotic Sildenafil Treatment Modulates Vascular Smooth Muscle Cell Phenotype in the Nitrofen Model of Congenital Diaphragmatic Hernia

Okolo

Zhang

Rhodes

et al. 2018

Sci Rep

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The etiology of pulmonary vascular abnormalities in CDH is incompletely understood. Studies have demonstrated improvement in pulmonary vasculature with prenatal therapy in animal models. We hypothesize that prenatal sildenafil may attenuate defective pulmonary vascular development via modulation of vSMC phenotype from undifferentiated, proliferative phenotype to differentiated, contractile phenotype. We utilized the nitrofen model of CDH to examine the effect of IA sildenafil on pulmonary vSMC phenotype during lung development. Timed-pregnant CD-1 mice were gavage fed 25 mg nitrofen or olive oil (control) at E8.5 of gestation. Single IA injections of Sildenafil (Revatio; 10 µL of 4 mg/4 ml solution) or dextrose control were performed at E12.5. Mice were sacrificed on various gestational days for embryonic lung harvest. Markers of vSMC development of undifferentiated and differentiated phenotypes were analyzed by immunostaining and western blot. Across all time points in gestation, nitrofen-treated embryonic lungs demonstrated increased vSMC expression of NOTCH3, Hes-5, PDGFR-β, desmin and α-SMA and decreased expression of calponin and SMMHC, compared to oil controls. IA dextrose treatment had no effect on expression levels. However, IA Sildenafil treatment resulted in down-regulation of NOTCH3, Hes-5, PDGFR-β, desmin and α-SMA and upregulation of calponin and SMMHC, comparable to oil controls. In the nitrofen model, vSMC express markers consistent with more undifferentiated proliferative phenotype, resulting in hypermuscularization of intrapulmonary arterioles in CDH. A single dose of IA Sildenafil treatment early in gestation, results in sustained normalization of vSMC phenotype. Pharmacologic modulation of the vSMC phenotype at key gestational points may have therapeutic potential.

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Section: Introductionmentioning

confidence: 99%

Intra-amniotic Sildenafil Treatment Modulates Vascular Smooth Muscle Cell Phenotype in the Nitrofen Model of Congenital Diaphragmatic Hernia

Okolo

Zhang

Rhodes

et al. 2018

Sci Rep

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“…This combination of unilateral agenesis of the diaphragm with bilateral renal agenesis has never been reported. Apart from all the above findings the gall bladder was absent too [5].…”

Section: Discussionmentioning

confidence: 86%

Unilateral Agenesis of Diaphragm with Bilateral Renal Agenesis: An Anomaly Diagnosed Prenatally During a NT Scan (11–14 Weeks)

Kumar

2022

Journal of Fetal Medicine

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Unilateral agenesis of the diaphragm with bilateral renal agenesis is an extremely rare lethal developmental anomaly. A detailed evaluation of fetal organs during nuchal translucency scan is required to diagnose this type of developmental anomaly. Detecting lethal developmental abnormality at an early gestational period (11–14 weeks) will be invaluable in facilitating the decision to terminate the pregnancy. Termination in the first trimester reduces psychological stress to the parents. We present a rare case of combined developmental anomaly which has never been reported in the literature.

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“…This in turn, interferes with normal lung development. The severity of these adverse effects vary, depending on the gestational age when the viscera herniate [4].…”

Section: Discussionmentioning

confidence: 99%

Role of Predictive Mortality Calculator in Antenatal Assessment of Congenital Diaphragmatic Hernia

Ishak

Hassan

Jamil

et al. 2021

Journal of Fetal Medicine

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Congenital diaphragmatic hernia (CDH) is one of the most common major congenital anomalies. In utero visceral organ herniation into the thoracic cavity can result in lung hypoplasia and pulmonary hypertension may ensue. Post-natal mortality rates in isolated CDH remain high reaching up to 80% in severe cases. Several prenatal predictors of morbidity and mortality have been proposed. Reliable predictive markers can aid clinicians in providing effective family counselling, prediction of survival, and propose therapeutic options. Decreased total fetal lung volume (TFLV) via magnetic resonance imaging (MRI) has been reported to be significantly associated with mortality. We report on 2 cases of CDH, with fetal MRI performed at third trimester, focusing on the TFLV and observed-to-expected TFLV. We would like to highlight the importance of predictive mortality calculator which provides statistical data for healthcare providers in counselling families and aids in riskstratification.

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A review of congenital diaphragmatic hernia

Cited by 13 publications

References 37 publications

Intra-amniotic Sildenafil Treatment Modulates Vascular Smooth Muscle Cell Phenotype in the Nitrofen Model of Congenital Diaphragmatic Hernia

Intra-amniotic Sildenafil Treatment Modulates Vascular Smooth Muscle Cell Phenotype in the Nitrofen Model of Congenital Diaphragmatic Hernia

Unilateral Agenesis of Diaphragm with Bilateral Renal Agenesis: An Anomaly Diagnosed Prenatally During a NT Scan (11–14 Weeks)

Role of Predictive Mortality Calculator in Antenatal Assessment of Congenital Diaphragmatic Hernia

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