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Congenital Diaphragmatic hernia - a review
#MMPMID28331629
Chandrasekharan PK
; Rawat M
; Madappa R
; Rothstein DH
; Lakshminrusimha S
Matern Health Neonatol Perinatol
2017[]; 3
(?): 6
PMID28331629
show ga
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in
the diaphragm leading to protrusion of abdominal contents into the thoracic
cavity interfering with normal development of the lungs. The defect may range
from a small aperture in the posterior muscle rim to complete absence of
diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and
immaturity associated with persistent pulmonary hypertension of newborn (PPHN)
and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and
position of the liver by ultrasound are used to diagnose and predict outcomes.
Delivery of infants with CDH is recommended close to term gestation. Immediate
management at birth includes bowel decompression, avoidance of mask ventilation
and endotracheal tube placement if required. The main focus of management
includes gentle ventilation, hemodynamic monitoring and treatment of pulmonary
hypertension followed by surgery. Although inhaled nitric oxide is not approved
by FDA for the treatment of PPHN induced by CDH, it is commonly used.
Extracorporeal membrane oxygenation (ECMO) is typically considered after failure
of conventional medical management for infants???34 weeks' gestation or with
weight >2 kg with CDH and no associated major lethal anomalies. Multiple factors
such as prematurity, associated abnormalities, severity of PPHN, type of repair
and need for ECMO can affect the survival of an infant with CDH. With advances in
the management of CDH, the overall survival has improved and has been reported to
be 70-90% in non-ECMO infants and up to 50% in infants who undergo ECMO.
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