? Introduction
? Epidemiology
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? Associated anomalies? Embryology
? Pathology
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? Diagnosis
? Pre-natal diagnosis
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? Clinical presentation? Prenatal care
? Pre-operative care
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? Surgical intervention
? Outcomes
Introduction
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? Congenital diaphragmatic hernia (CDH) is a common malformation
characterized by a defect in the posterolateral diaphragm, the
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foramen of Bochdalek, through which the abdominal viscera migrateinto the chest during fetal life.
Epidemiology
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? Incidence- 1 in 2000 to 5000 per live birth.
? One third of neonates with CDH are stillborn.
? So, the exact prevalence of the disease is underestimated.
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? When still births are counted with live birth, the incidence is morecommon in females.
? Infants with isolated CDH are typically premature, macrosomic male.
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? One third are associated with a major congenital anomaly.? Approximately 80% are left sided.
? Bilateral defects are rare and are associated with other major
anomalies.
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? Although the exact etiology remains unknown, mothers that are thin
or underweight may have an increased risk of bearing an infant with
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CDH.? CDH may be due to the exposure of genetical y predisposed or susceptible
individuals to environmental factors.
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? Exposure to a number of pharmacologic agents and environmental hazards
has been implicated in its development.
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? These include insecticides and drugs, such as phenmetrazine, thalidomide,quinine, cadmium, lead, and nitrofen.
? Retinoid-regulated target genes may be responsible for CDH development.
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(Vitamin A deficiency is seen in patients of CDH)
Associated anomalies
? Approximately 50% of CDH are isolated defects.
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? Others are associated with anomalies of the cardiovascular (27.5%), urogenital
(17.7%), musculoskeletal (15.7%), and central nervous (9.8%) systems (CNS).
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? Anomalies as a consequence of diaphragmatic defect:? lung hypoplasia, intestinal malrotation, some cardiac malformations, and patent ductus
arteriosus (PDA) are considered to be consequences of the diaphragmatic defect.
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? Non-CDH-related defects are estimated to occur in 40?60% of cases and can
involve the cardiovascular, CNS, gastrointestinal, and genitourinary systems.
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Development of diaphragm? The fully developed diaphragm is derived from four distinct components:
1. the anterior central tendon forms from the septum transversum
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2. the dorsolateral portions form from the pleuroperitoneal membranes
3. the dorsal crura evolve from the esophageal mesentery
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4. the muscular portion of the diaphragm develops from the thoracic intercostalmuscle groups.
? The pleuroperitoneal folds grow ventral y and fuse with the septum
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transversum and dorsal mesentery of the esophagus during gestational
week 6.
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? Complete closure of the canal takes place during week 8 of gestation.? Anatomical y, the right side closes before the left.
? Neuro-muscularization of the diaphragm is the last in the development and
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matures the diaphragm.
Development of Diaphragm
Pathology of CDH
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? Failure of closure of pleuro-peritoneal canal
? Most common area is a postero-lateral defect ( Bochdalek )
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? Left side more common? Herniated contents
? Left- left lobe of liver, spleen and bowel
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? Right- Liver and other viscera.
Lung development in CDH
? Both the lungs are affected-Lung hypoplasia.
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? Ipsilateral > Contralateral
? No. of bronchial branches ? greatly reduced
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? Alveolar development severely affected? Increased muscle mass in the conducting airways
Pulmonary vasculature in CDH
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? Both the lungs are affected.
? Reduction in the total no. of branches
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? Significant adventitial and medial wall thickening? No significant changes in pulmonary venous structure
? Increased susceptibility to PPH
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? hypoxia, acidosis, hypothermia, stress
Problems: in CDH
Hypoxia
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Respiratory distress
Metabolic acidosis
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HypercarbiaDiagnosis: Prenatal Diagnosis
? Prenatal USG:
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? Mean gestational age at discovery is 24weeks.
? Presence of polyhydramnios (80% cases of CDH)- due to kinking of the gastro-
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esophageal junction by translocation of the stomach into the thorax withresultant foregut obstruction.
? Presence of stomach in the fetal thorax at the same cross-sectional level of
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heart.
? Three-dimensional estimation of the fetal lung volume: important prognostic
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indicator.? Lung-to-head ratio has been the most widely used prognostic indicator.
? Fetal MRI
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Fetal ultrasound image at
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the level of the four
chamber
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heart(dotted
arrow). Gastric bubble (solid
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arrow) at the level of the
four-chamber heart suggests
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CDH.Clinical presentation
? Newborns with CDH typically present with respiratory distress.
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? Immediate respiratory distress with associated low Apgar scores to an initial
stable period and a delay in respiratory distress for 24 to 48 hours.
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?? Initial signs associated with respiratory distress include tachypnea, chest wall
retractions, grunting, cyanosis, and pallor.
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? On physical examination:? scaphoid abdomen and an increased chest diameter.
? The point of maximal cardiac impulse is often displaced, suggesting
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mediastinal shift.
? Bowel sounds may be auscultated within the chest cavity with a decrease in
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breath sounds bilaterally.? Chest excursion may be reduced, suggesting a lower tidal volume.
? The diagnosis of CDH is typically confirmed by a chest radiograph
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demonstrating intestinal loops within the thorax.
? The abdominal cavity may have minimal to no gas.
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Right-sided CDH.
? Occasionally, CDH may be completely asymptomatic and is only
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discovered incidentally.
? Older patients who present later in life have a much better prognosis
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due to milder or absent associated complications, such as pulmonaryhypoplasia and hypertension.
Prenatal Care
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? Referral to tertiary care centres where respiratory distress of
neonates can be managed.
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? Prenatal corticosteroids:? To enhance the lung development in the premature infants
? Role in CDH is not determined.
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Pre-operative care? Resuscitation:
? Cardio-respiratory system stabilisation
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? Endotracheal intubation
? Nasogastric tube insertion
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? Ventilation by mask and Ambu bag is contraindicated to avoid distention ofthe stomach and intestines that may be in the thoracic cavity.
? Arterial and venous access through umbilicus
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? Infant to be properly sedated
? Ventilation at low pressures and high rate
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? Pharmacology:
? Drugs to reduce pulmonary hypertension:
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? calcium channel blockers, prostacyclin derivatives, endothelin receptor antagonists, andphosphodiesterase-5 inhibitors such as sildenafil
? Surfactants:
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? There is deficiency of surfactants in CDH.
? May improve respiratory function: doubtful overall benefit
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? Inhalational nitric oxide:? Potent vasodilator
? May reduce pulmonary hypertension
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Surgery
? Timing of surgery:
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? CDH is a medical emergency not a surgical emergency.
? Surgery once the baby is stabilised with minimal respiratory support and least
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pulmonary hypertension.? Surgical steps:
? Ipsilateral abdominal incision
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? Defect is exposed.
? Reduction of the abdominal viscera
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? Closure of the defect : Primary or grafts? Wound closure
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Case -3: (CDH)
Intra-operative pictures showing the diaphragmatic defect and margins of diaphragm
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Case -3: CDH
? Other surgical approaches
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? Thoracic approach
? Minimal access surgery
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? Thoracoscopic approach? Laparoscopic approach
? Post surgery, the baby is kept on mechanical ventilatory supports
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with abdominal decompression with the help of nasogastric tube,rectal washes, and urinary catheterisation.
? Gradually, supports are weaned.
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Outcome
? Survival rates at advanced centres are 60-90%.
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? CDH survivors are at significant risk for chronic neurologic,developmental,
gastrointestinal,
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nutritional,
pulmonary,
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musculoskeletal, and other disorders.? Late deaths have been reported in approximately 10% of initial
survivors, mainly because of the consequences of persistent
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pulmonary hypertension.
? Respiratory risk:
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? Pneumonias? Reactive airway disease
? Cor pulmonale
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? Gastrointestinal:
? Gastroesophageal reflux
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? Nutritional and growth related problems? Malrotation
? Musculoskeletal:
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? Chest wall deformities and scoliosis
? Neurodevelopmental abnormalities.
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? Problems in motor and cognitive skills.