Clinical Outcome for Congenital Diaphragmatic Hernia at the Age of 1 Year in the Era of Fetal Intervention

Neonatology ◽  
2017 ◽  
Vol 112 (4) ◽  
pp. 365-371 ◽  
Author(s):  
Eva Van Ginderdeuren ◽  
Karel Allegaert ◽  
Herbert Decaluwe ◽  
Jan Deprest ◽  
Anne Debeer ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Fetal Intervention

Clinical outcome at 1 year of age for congenital diaphragmatic hernia in the era of fetal intervention

2017 ◽  
Author(s):  
Marijke Proesmans ◽  
Eva Van Ginderdeuren ◽  
Karel Allegaert ◽  
Herbert Decaluwe ◽  
Jan Deprest ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Fetal Intervention

Outcomes for Prenatally Diagnosed Right Congenital Diaphragmatic Hernia

2015 ◽  
Vol 47 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Corey W. Iqbal ◽  
S. Christopher Derderian ◽  
Leslie Lusk ◽  
Amaya Basta ◽  
Roy A. Filly ◽  
...  
Keyword(s):  
Survival Rate ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Retrospective Review ◽  
Prognostic Indicators ◽  
Tertiary Referral ◽  
Fetal Intervention ◽  
Tertiary Referral Center ◽  
Referral Center ◽  
Overall Mortality

Background: Right congenital diaphragmatic hernia (CDH) occurs less frequently than left CDH. Therefore, prognostic indicators for right CDH are not as well studied as for left CDH. Methods: A retrospective review from a single, tertiary referral center (from 1994 until July 2013) of patients with unilateral right CDH was conducted. Prenatal characteristics were evaluated and correlated with survival to discharge and need for extracorporeal membranous oxygen (ECMO). Results: In total, 34 patients were identified. There were 12 postnatal deaths and 2 fetal demises (6%), representing an overall mortality of 41%. Six patients required ECMO. Nine patients underwent fetal intervention and were analyzed separately. For patients not undergoing fetal intervention, the survival rate was 52% and a higher mean (±SD) lung-to-head ratio (LHR) was associated with survival (1.1 ± 0.4 vs. 0.8 ± 0.2, p = 0.03). There were no deaths or need for ECMO in any patient with an LHR ≥1.0. Of the 9 patients who underwent fetal intervention, survival was 78% and only 1 patient required ECMO. Fetal intervention was primarily tracheal occlusion (n = 8). Conclusions: An LHR <1.0 is associated with worse survival for right CDH and may also reflect the need for ECMO.

Lung Pathology in Patients with Congenital Diaphragmatic Hernia Treated with Fetal Surgical Intervention, Including Tracheal Occlusion

2003 ◽  
Vol 6 (6) ◽  
pp. 536-546 ◽  
Author(s):  
Amy E. Heerema ◽  
Joseph T. Rabban ◽  
Roman M. Sydorak ◽  
Micheal R. Harrison ◽  
Kirk D. Jones
Keyword(s):  
Body Weight ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Pulmonary Hypoplasia ◽  
Weight Ratio ◽  
Lung Pathology ◽  
Body Weight Ratio ◽  
Fetal Intervention ◽  
Tracheal Occlusion ◽  
Significant Difference

Fetal intervention for congenital diaphragmatic hernia was developed to lessen the high morbidity and mortality of pulmonary hypoplasia. Lung pathology and morphometry in patients treated with fetal intervention have not been described. We report clinical and autopsy findings, as well as basic lung morphometry in 16 cases of congenital diaphragmatic hernia with fetal intervention (12 cases tracheal occlusion; 4 cases hernia repair), and 19 cases of congenital diaphragmatic hernia without fetal intervention. All patients who underwent fetal intervention were born premature. Lung enlargement with increased lung-to-body weight ratio was observed with fetal tracheal occlusion, accompanied by lower than normal radial alveolar counts and increased alveolar size. Patients treated with tracheal occlusion also had early alveolar development (at 29.8, 30.6, and 30.9 wk postconceptual age) as well as mucous fluid pooling in airways and alveoli. All cases showed severe alveolar septal widening, more extensive in patients without fetal intervention. When grouped by postconceptual age, no statistically significant difference was found between patients with and without fetal intervention with respect to lung-to-body weight ratio, radial alveolar count, mean alveolar length, and relative arteriolar media thickness. Lung enlargement has been observed with fetal tracheal occlusion sonographically; our studies suggest that this is due in part to emphysema and mucous fluid pooling. The lung remains abnormal with low radial alveolar counts and increased alveolar size. Tracheal occlusion did not prevent development of lung pathology associated with pulmonary hypoplasia.

scholarly journals Congenital Diaphragmatic Hernia: Review of Current Concept in Surgical Management

ISRN Surgery ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-8 ◽  
Author(s):  
Emeka B. Kesieme ◽  
Chinenye N. Kesieme
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Surgical Management ◽  
Diaphragmatic Hernia ◽  
Minimal Invasive Surgery ◽  
Pulmonary Hypoplasia ◽  
Open Repair ◽  
Minimal Invasive ◽  
Fetal Intervention ◽  
Prosthetic Devices ◽  
Diaphragmatic Hernias

Congenital diaphragmatic hernias (CDHs) occur mainly in two locations: the foramen of Morgagni and the more common type involving the foramen of Bochdalek. Hiatal hernia and paraesophageal hernia have also been described as other forms of CDH. Pulmonary hypertension and pulmonary hypoplasia have been recognized as the two most important factors in the pathophysiology of congenital diaphragmatic hernia. Advances in surgical management include delayed surgical approach that enables preoperative stabilization, introduction of fetal intervention due to improved prenatal diagnosis, the introduction of minimal invasive surgery, in addition to the standard open repair, and the use of improved prosthetic devices for closure.

Fetal Intervention for Congenital Diaphragmatic Hernia: The European Experience

2005 ◽  
Vol 29 (2) ◽  
pp. 94-103 ◽  
Author(s):  
Jan Deprest ◽  
Jacques Jani ◽  
Eduardo Gratacos ◽  
Hilde Vandecruys ◽  
Gunnar Naulaers ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Fetal Intervention ◽  
European Experience

scholarly journals Genetics of diaphragmatic hernia

2021 ◽  
Author(s):  
Yannick Schreiner ◽  
Thomas Schaible ◽  
Neysan Rafat
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Outcome ◽  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Congenital Malformations ◽  
Lung Hypoplasia ◽  
Complex Phenotype ◽  
Life Threatening ◽  
Major Congenital Malformations ◽  
Very High

AbstractCongenital diaphragmatic hernia (CDH) is a life-threatening malformation characterised by failure of diaphragmatic development with lung hypoplasia and persistent pulmonary hypertension of the newborn (PPHN). The incidence is 1:2000 corresponding to 8% of all major congenital malformations. Morbidity and mortality in affected newborns are very high and at present, there is no precise prenatal or early postnatal prognostication parameter to predict clinical outcome in CDH patients. Most cases occur sporadically, however, genetic causes have long been discussed to explain a proportion of cases. These range from aneuploidy to complex chromosomal aberrations and specific mutations often causing a complex phenotype exhibiting multiple malformations along with CDH. This review summarises the genetic variations which have been observed in syndromic and isolated cases of congenital diaphragmatic hernia.

Impact of Fetal Intervention on Postnatal Management of Congenital Diaphragmatic Hernia

2007 ◽  
Vol 17 (6) ◽  
pp. 404-407 ◽  
Author(s):  
L. Saura ◽  
M. Castañón ◽  
J. Prat ◽  
A. Albert ◽  
F. Caceres ◽  
...  
Keyword(s):  
Congenital Diaphragmatic Hernia ◽  
Diaphragmatic Hernia ◽  
Fetal Intervention ◽  
Postnatal Management
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