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    Congenital Diaphragmatic Hernia

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    Pothina Sree Hari
    This document summarizes congenital diaphragmatic hernia (CDH), including its embryology, pathophysiology, diagnosis, predictors of mortality, management, and recent advances. CDH occurs when there is a defect in the diaphragm that allows abdominal organs to migrate into the chest cavity, inhibiting proper lung development. Management focuses on controlling ventilation, fluid resuscitation, and in severe cases, extracorporeal membrane oxygenation (ECMO) or surgical repair, though survival rates remain challenging. New strategies including prenatal repair and lung transplantation show promise but require further research.

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    Congenital Diaphragmatic Hernia

    Uploaded by

    Pothina Sree Hari
    51 views40 pages
    This document summarizes congenital diaphragmatic hernia (CDH), including its embryology, pathophysiology, diagnosis, predictors of mortality, management, and recent advances. CDH occurs when there is a defect in the diaphragm that allows abdominal organs to migrate into the chest cavity, inhibiting proper lung development. Management focuses on controlling ventilation, fluid resuscitation, and in severe cases, extracorporeal membrane oxygenation (ECMO) or surgical repair, though survival rates remain challenging. New strategies including prenatal repair and lung transplantation show promise but require further research.

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    CDH101

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    This document summarizes congenital diaphragmatic hernia (CDH), including its embryology, pathophysiology, diagnosis, predictors of mortality, management, and recent advances. CDH occurs when there is a defect in the diaphragm that allows abdominal organs to migrate into the chest cavity, inhibiting proper lung development. Management focuses on controlling ventilation, fluid resuscitation, and in severe cases, extracorporeal membrane oxygenation (ECMO) or surgical repair, though survival rates remain challenging. New strategies including prenatal repair and lung transplantation show promise but require further research.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as PPT, PDF, TXT or read online from Scribd
    Download as ppt, pdf, or txt
    51 views40 pages

    Congenital Diaphragmatic Hernia

    Uploaded by

    Pothina Sree Hari
    This document summarizes congenital diaphragmatic hernia (CDH), including its embryology, pathophysiology, diagnosis, predictors of mortality, management, and recent advances. CDH occurs when there is a defect in the diaphragm that allows abdominal organs to migrate into the chest cavity, inhibiting proper lung development. Management focuses on controlling ventilation, fluid resuscitation, and in severe cases, extracorporeal membrane oxygenation (ECMO) or surgical repair, though survival rates remain challenging. New strategies including prenatal repair and lung transplantation show promise but require further research.

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    CONGENITAL

    DIAPHRAGMATIC HERNIA

    Man Mohan Harjai


    OVERVIEW
    •Background
    •Pathophysiology
    •Management
    •New Advances
    BACKGROUND
    •1597 first described
    •1925 literature review
    •1945 CDH repair
    INCIDENCE
    •1 in 12,00 live births
    •Malrotation always present
    •Congenital anomalies 23%
    EMBRYOLOGY
    •Pleuroperitoneal membrane
    •Midgut rotation
    •Foramen of Bockdalek
    •Foramen of Morgagni
    •Esophageal hiatus
    PULMONARY DEVELOPMENT
    •8-10 weeks bronchial buds
    •16 weeks segmental bronchi
    •24 weeks-5 years alveolar formation
    •Growth of blood vessels
    DIAGNOSIS
    •Decreased chest movement
    •Shift of cardiac impulse
    •Presence of bowel sounds
    •Scaphoid abdomen
    DIAGNOSIS
    •Cyanosis
    •Respiratory distress
    •X-ray findings
    PULMONARY HYPOPLASIA
    •Animal model
    •Decreased parenchymal mass
    •Reduced surface area
    •Reduced pulmonary vasculature
    VASCULAR HYPERTROPHY

    •Thickened pulmonary and arteriolar media


    •Extension muscle to resistance vessels
    •Increased response to hypoxemia and acidosis
    VASCULAR HYPERTROPHY
    •Bonn
    •> 1/3 alveolar wall vessels muscularized
    •> 2X normal medial wall thickness
    POSTNATAL PHYSIOLOGY
    •Underdeveloped parenchyma
    •Underdeveloped vascular bed
    •Hypertrophied resistance vessels
    PREDICTORS OF MORTALITY
    •Collins 1977 "Honeymoon period"
    •Preductal PaO2 > 100 PaCO2 < 50
    •Adequate pulmonary parenchyma
    •90% likelihood of death
    PREDICTORS OF MORTALITY
    •Barlett
    •Integrated FIO2 and pH
    •Newborn Pulmonary Insufficiency Index
    PREDICTORS OF MORTALITY

    •O'Rouke
    •Postductal PaO2 >100
    PREDICTORS OF MORTALITY
    •Raphaely and Downs
    •Prognostic test after closure
    •A-a02
    PREDICTORS OF MORTALITY
    •Oxygen Index
    •Based on MAP
    •Postductal O2 > 100
    PREDICTORS OF MORTALITY
    •Inconsistent site of sampling
    •Inconsistent time to repair
    •No ventilatory parameters referenced
    •Integration pulmonary hypertension/hypoplasia
    PREDICTORS OF MORTALITY
    •Bohn
    •Integrated PaCO2 and Ventilatory Index
    •Four survival groups
    •Inconsistent use
    DELAYED SURGICAL REPAIR
    •Original surgical therapy
    •Respiratory/cardiovascular function
    •Langer 1984 advocated delay
    •Preoperative improvement
    DELAYED SURGICAL REPAIR
    •Partial reduction possible
    •Not atelectasis but hypoplasia
    •Repair often decreased thoracic compliance
    MANAGEMENT
    •Controlled ventilation
    •Fluid resuscitation
    •IV access
    •Arterial line
    •Central line
    MANAGEMENT
    •Narcotic-02-relaxant
    •Avoid N20
    •Inhalational agents
    •Abrupt decay
    MANAGEMENT
    •Hypoxemia,hypercarbia,acidosis,hypothermia
    •IVC compression
    •Patch?
    •Prolonged anesthetic state
    •< 50% survival with repair
    RECENT ADVANCES
    •Postnatal management
    •Prenatal repair
    •Perinatal lung transplant
    METHOD OF VENTILATION
    •HFOV
    •Decreased A-a02 gradient
    •Decreased FI02/PaC02
    •Lower airway pressure
    ECMO
    •Barlett 1972
    •Neonatal respiratory failure
    •Flows 100-120 ml/kg/min
    •Preductal Pa02 80-100
    •PaC02 30-45
    ECMO
    •Limit airway pressure
    •Limit oxygen toxicity
    •Eliminate shunting
    •Reverse systemic hypoxemia
    •Reduce pulmonary blood flow
    •Allow gradual expansion of lung
    ECMO

    •Complications
    •Contraindications
    ECMO
    •Stolar
    •Demise of "Honeymoon period"
    •Stimulation of sympathoadrenal axis
    ECMO
    •O'Rouke
    •Weaning to CMV
    •Changed survival high risk CDH
    REPAIR DURING ECMO
    •Hematocrit > 40
    •Platelets 50-100 K
    •Heparin
    •Colloid/crystalloid
    •Ventilation
    •Narcotic infusion
    •Venous return
    RECENT ADVANCES
    •77% mortality for prenatal diagnosis
    •Pulmonary hypoplasia difficult to assess
    •Dismal prognosis
    PRENATAL REPAIR
    •Harrison
    •Fetal lamb model
    •Critical time growth/development
    •Placental bypass
    PRENATAL REPAIR
    •Successful intrauterine repair
    •Criteria for consideration
    •Best conditions 22-30 weeks
    PRENATAL REPAIR
    •Operative management
    •Maternal complications
    •Gortex implant
    LUNG TRANSPLANT
    •Adzick
    •Transplant as potential treatment
    •ECMO as bridge until surgery
    •LLL transplant in neonatal swine
    LONG TERM SURVIVAL
    •10% mental retardation
    •Pulmonary hypoperfusion
    •Pulmonary function testing
    •Pulmonary disability in adulthood?
    LONG TERM SURVIVAL
    •Falconer
    •Pectus/scoliosis
    •V/Q mismatch
    •Overdistention
    •Gastroesophageal dismotility
    CONCLUSION
    •Continued morbidity/mortality
    •New strategies
    •Ongoing research
    •Cost
    •New etiology?

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