Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Development of diaphragm Tissues of origin Septum transversum Esophageal mesentry Mesoderm from body wall Lumbar somites

Development of diaphragm

Pathology of CDH Failure of closure of pleuro-peritoneal canal Most common area is a postero-lateral defect ( Bochdalek ) Left side more common

Pathology of CDH Diaphragmatic defect Abdominal viscera fill the chest cavity Abdomen small & poorly developed

Pathology of CDH Both lungs hypoplastic More so on the ipsilateral side Pulmonary vessels hypoplastic PPHN

Pathology of CDH Following delivery Bowels fill with air Compression of ipsilateral lung Mediastinal shift Compression of contralateral lung mechanical compression of lung

Pathology of CDH Respiratory distress Lung hypoplasia PPHN Mechanical compression Respiratory distress

Lung development in CDH No. of bronchial branches – greatly reduced Alveolar development severely affected Increased muscle mass in the conducting airways Seen in contra lateral lung too

Pulmonary vasculature in CDH Reduction in the total no. of branches Both in ipsilateral and contra lateral lungs Significant adventitial and medial wall thickening Increased susceptibility to PPH hypoxia, acidosis, hypothermia, stress

Persistent fetal circulation pulmonary artery resistance pressures vascular flow Right to left shunting Hypoxia & Progressive desaturation Respiratory failure

Diagnosis CXR diagnostic Absence of diaphragm Scaphoid abdomen Bowel loops in chest Mediastinal shift

Chest X - Ray

Problems Hypoxia Respiratory distress Metabolic acidosis Hypercarbia

Treatment Initial goal Stabilisation of respiration Treatment of PPHN

Treatment Oxygenation & mechanical ventilation Correction of PPHN (NO / Vasodilators ) Correction of metabolic acidosis

Treatment Surgical repair of the defect Abdominal approach Post-op ventilation

Newer advances in therapy In utero repair PLUG therapy ECMO

Prenatal diagnosis USG @ 16 weeks Herniated viscera in the chest Mediastinal shift to opposite side Stomach in the chest Associated anomalies 40%

Hernia of Morgagni Antero-medial defect Para-esophageal Lucencies in mediastinum Respiratory distress Surgical correction

Eventration of diaphragm

Pathology Attenuation of central muscular portion of diaphragm Phrenic nerve damage Idiopathic ( birth injury )

Clinical presentation Similar to CDH Respiratory distress @ birth Recurrent respiratory tract infections in infancy

Chest X - Ray Elevated thinned out diaphragm Bowel loops in chest Mediastinal shift

Chest X - Ray

Treatment Surgical repair of the defect Abdominal approach