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CONGENITAL DIAPHRAGMATIC HERNIA [Recovered].pptx

Congenital diaphragmatic hernia (CDH) is a structural birth defect involving abdominal organs protruding into the thorax due to a defect in the diaphragm, with an incidence of 1 in 2000-4000 births. Management includes prenatal diagnosis through ultrasounds, surgical interventions, and postnatal treatments like ECMO; outcomes vary based on the severity of the condition. Challenges include high long-term morbidity rates and complications such as chronic lung disease, developmental delays, and gastroesophageal reflux.

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CONGENITAL DIAPHRAGMATIC HERNIA Dr. Pritesh B. Patel MBBS , MD(PEDIA), FIAPNEONATOLOGIST NOCARE NICU AND CHILDREN HOSPITAL NAVSARI GUJARAT
HISTORY • 1679 – Riverius recorded the first CDH • 1761 – Morgagni described types of CDH • 1905 – Heidenhain repair CDH • 1925 – Hedbolm suggested that CDH leads to pulmonary hypoplasia and early operation improve survival • 1977- 1st ECMO for CDH by german • 1995- EXIT procedure
DEFINATION • Congenital diaphragmatic hernia (CDH) is a structural birth defect characterized by protrusion of abdominal viscera into the thorax through an abnormal opening or defect that is present at birth
INCIDENCE • 1 in 2000- 4000 births • 8 % of all major congenital anomalies • 80-90% posterolateral defects (Bochdalek) • Morgagni 9% • B/L DH extremely rare 1-2%
RECURRENCE • A negative family history -Recurrence rate in first degree relative -around 2% • Positive family history- genetic syndrome, or a chromosomal abnormality -50%
DIAPHRAGM DEVELOPMENT
• Failure of the pleuro-peritoneal canal to close at 8 weeks’ gestation • Hole can be 1-2 cm in size to total absence of hemidiaphragm. • Abdominal organ will herniate to the chest at 10 weeks’ gestation
• The anterior central tendon forms from the septum tranversum • The dorsolateral portions from the pleuroperitoneal membranes • The dorsal crura from the esophageal mesentery • The muscular portions from the thoracic intercostal muscles
TYPES OF CDH • Bochdalek Hernia- MC, 80-90% • Morgagni Hernia-9%
CAUSES • Unknown • as part of a multiple malformation syndrome • chromosomal anomalies including trisomy 18, trisomy 13,21 10-20%
ASSOCIATED MALFORMATION
PATHOPHYSIOLOGY
PRENATAL DIAGNOSIS • Ultrasonography- 40-90% -Viscera herniation (stomach, intestines, liver, kidneys, spleen and gall bladder) -IUGR -Polyhydramnios- 80% (Kinking of Gastro Oeso junction) -Fetal hydrops -LHR(Metkus et al reported ) <1-100% mortlity 1-1.4 – 38 % survival >1.4- 100% survival
• Prenatal MR Imaging -PPLV(percent predicted lung volume) -FLV • Karyotype evaluation
Antenatal poorer prognosis • Hydrops • LHR <1.0 • Diagnosis before 25 weeks gestation • Associated cardiac anomalies • Liver with in thorax
PRENATAL MANAGEMENT Fetal surgical therapy (Antenatal surgical intervention, In utero tracheal occlusion ) -tracheal occlusion -transuterine endoscopy -FETENDO -endolumenal balloon tracheal occlusion(less invasive)
Prenatal interventions for CDH for improving outcomes The Cochrane Library2015 • There is currently insufficient evidence to recommend in-utero intervention for fetuses with CDH as a part of routine clinical practice. • More studies are needed to further examine the effect of in-utero fetal tracheal occlusion on important neonatal outcomes and long-term infant survival and health. • Long-term followup is of particular importance, and should include morbidity and mortality measures.
• Further studies should examine the benefits of an in- utero intervention on subgroups with moderate and severe congenital diaphragmatic hernia. • Indeed, there are three ongoing studies, being conducted by European, North and South American fetal medicine centres, which will contribute to this gap. • Ongoing research and any implementation into clinical practice should include standardisation of the procedure, inclusion criteria and longterm childhood follow-up.
DELIVERY ROOM MANAGEMENT • Spontaneous NVD preferable • delivered in a center that has experienced personnel and available therapies. • immediate resuscitation and stabilization • prevent distension of the GI tract and further compression of the pulmonary parenchyma • Early intubation is preferable to BMV or CPAP via mask or nasal prongs
POST NATAL DIAGNOSIS • Respiratory distress • Scaphoid abdomen • Auscultation of the lungs reveals poor air entry • Shift of the heart to the side opposite • Asymmetrical distended chest • LATE PRESENTATION(mild RDS, feeding problem)- 10-20%
• LAB STUDIES • Arterial blood gas • Chromosome studies • IMAGING STUDIES • Cardiac ultrasonography- rule out CHD • Renal ultrasonography- rule out anomalies • Cranial ultrasonography- rule out IVH, anomalies
large arrow -nasogastric tube in the left hemithorax. small arrow -left angulation of the UV Catheter
DIFFERENTIAL DIAGNOSIS • Eventration of Diaphragm(differentiated by fluoroscopy) • Anterior Diaphragm Hernia of Morgagni(radiolucent shadow overlying heart) • Congenital Esophageal Hiatal Hernia • Congenital Cystic Disease of Lung(normal gas+ NGT in abdomen) • Primary Agenesis of Lung
• Small number of babies-minimal RDS, respond well to ventilator • Large number are born in extremis, never resuscitable, die with in few hours • Middle group (honeymoon period), vasoconstriction of abnormally reactive pulmonary vascular bed
POSTNATAL MANAGEMENT • CDH is a physiologic emergency, not a surgical emergency • Severe cases – EXIT procedure with immediate institution of ECMO • Intubation – all cases • Large IFT with continues suction
• Mechanical ventilation- -acceptable post ductal saturation(PaO2 > 60mmHg, PaCo2 < 40mmHg), avoid iatrogenic injury, severe hyper carbia -100 % FiO2 and low PIP(<25 cm H2O with 5 cm of PEEP) should be used • Modes of ventilator – contraversial, HFV
• Nitric Oxide- to control PPHN(responsiveness of CDH disappointing) • Surfactant – controversial(demonstrate no benefit) • ECMO(70% survival in selected high risk newborn)
• Surgery -timely operation often be performed after 100hours with minimal oxygen and airway pressure requirement - small defect- permanent suture and Teflon pladgets -large defect is repaired with polytetrafluoroethylene membrane
Late versus early surgical correction for CDH in newborn infants The Cochrane Library 2000 • There is no clear evidence which favors delayed (when stabilized) as compared with immediate (within 24 hours of birth) timing of surgical repair of congenital diaphragmatic hernia, but a substantial advantage to either one cannot be ruled out. • A large, multicenter randomized trial would be needed to answer this question.
• Left- abdominal(subcostal)/thoracic approach • Right- thoracic(7-9th posterolateral rib) approach • B/L –transverse upper abdominal incision • LUNG TRANSPLANTATION
Systemic review of ECMO in infants with CDH, UK Evidence based research • Early mortality was signifacantly lower with ECMO compare to CMV • Late mortality was similar
• Pulmonary care- severely affected infants have chronic lung disease. These infants may require prolonged therapy with supplemental oxygen and diuretics, an approach similar to that for bronchopulmonary dysplasia • Neurologic evaluation(40%)- for CNS injury ,SNHL • Feeding- : Incidence of significant gastroesophageal reflux(45-85%) is very high • Growth: Failure to thrive • Developmental follow-up - for CNS insult and sensorineural hearing loss
COMPLICATION • Bleeding • Pleural effusion • Incisiona hernia • Recurrence 5-15%(patch repair-50%) • GERD – 50-90% • Intestinal obstruction • Chest wall deformities(pectus carinatum-30%, scoliosis-20%) • Chronic lung disease
PROGNOSIS Pulmonary hypertension is the primary determinant of mortality -very good prognosis in absence of other anomalies • Pulmonary recovery: When all resources, including ECMO, are provided, survival rates range from 40-69%. • Long-term morbidity: Significant long-term morbidity, including CLD, growth failure, GERD, and neurodevelopmental delay.
HERNIAS THROUGH THE CENTRAL TENDON OF THE DIAPHRAGM • Right side -mushroom-like projection of liver can be seen -misinterpreted a diaphragmatic tumor -Distinguishing can be done by creating a pneumoperitoneum - Air appears around the liver protrusion -Operation is not necessary
• Left side -stomach may herniate, -a air-containing cyst can be seen on the top of the diaphragm. -The operation is done through an abdominal approach
MORGAGNI HERNIA • anterior retrosternal diaphragmatic hernia. • Lack of fusion or muscularization of pleuroperitoneal membrane anteriorly -1/3 patients have no symptom -Cardiorespiratory symptoms are less common than GI symptoms. -Chest radiograph reveals pericardiophrenic density that is solid or contain air. -Contrast study of colon -Surgical repair
CONGENITAL DIAPHRAGMATIC HERNIA [Recovered].pptx