Congenital Diaphragmatic Hernia & Eventration Of

Diaphragm

Dr.V.N.Mahalakshmi

Development of diaphragmTissues 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 CDHFollowing delivery Bowels fill with air Compression of ipsilateral lung Mediastinal shift Compression of contralateral lung mechanical compression of lung

Pathology of CDH Lung hypoplasia

PPHN Mechanical compressionRespiratory 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

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

PathologyAttenuation 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