congenital diaphragmatic hernia & eventration of diaphragm dr.v.n.mahalakshmi

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

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

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