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

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

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Page 1: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Congenital Diaphragmatic Hernia & Eventration Of

Diaphragm

Dr.V.N.Mahalakshmi

Page 2: 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

Page 3: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Development of diaphragm

Page 4: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology of CDH

Failure of closure of pleuro-peritoneal canal

Most common area is a postero-lateral defect ( Bochdalek )

Left side more common

Page 5: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology of CDH

Diaphragmatic defect Abdominal viscera fill the chest

cavity Abdomen small & poorly developed

Page 6: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology of CDH

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

Page 7: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology of CDH

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

lung

Page 8: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology of CDH

Lung hypoplasia

PPHN Mechanical compression

Respiratory distress

Page 9: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

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

Page 10: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

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

Page 11: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi
Page 12: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi
Page 13: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Persistent fetal circulation

Respiratory failure

Page 14: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Diagnosis

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

Page 15: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Chest X - Ray

Page 16: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Problems Hypoxia

Respiratory distress

Metabolic acidosis

Hypercarbia

Page 17: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi
Page 18: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Treatment

Initial goal Stabilisation of respiration

Treatment of PPHN

Page 19: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Treatment

Oxygenation & mechanical ventilation

Correction of PPHN (NO / Vasodilators )

Correction of metabolic acidosis

Page 20: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Treatment

Surgical repair of the defect

Abdominal approach

Post-op ventilation

Page 21: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi
Page 22: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Newer advances in therapy In utero repair

PLUG therapy

ECMO

Page 23: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Prenatal diagnosis

USG @ 16 weeks Herniated viscera in the chest Mediastinal shift to opposite side Stomach in the chest

Associated anomalies 40%

Page 24: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Hernia of Morgagni

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

Page 25: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Eventration of diaphragm

Page 26: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Pathology

Attenuation of central muscular portion of diaphragm

Phrenic nerve damage Idiopathic

( birth injury )

Page 27: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Clinical presentation

Similar to CDH Respiratory distress @ birth Recurrent respiratory tract

infections in infancy

Page 28: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Chest X - Ray

Elevated thinned out diaphragm Bowel loops in chest Mediastinal shift

Page 29: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Chest X - Ray

Page 30: Congenital Diaphragmatic Hernia & Eventration Of Diaphragm Dr.V.N.Mahalakshmi

Treatment

Surgical repair of the defect

Abdominal approach