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  1. 1. Congenital diaphragmatic hernia DR RANJITH KUMAR CS KKCTH
  2. 2. History • 1679 – Riverius recorded the first CDH • 1761 – Morgagni desribed tycpes of CDH • 1905 – Heidenhain repair CDH • 1925 – Hedbolm suggested that CDH led to pulmonary hypoplasia and early operation improve survival • 1946 – Gross correct CDH < 24 hours of age • 1980-1990 – delayed correction become wide
  3. 3. Incidence • 1 : 2000-5000 live birth • 8 % of all major congenital anomalies
  4. 4. • the diaphragm is derived from the following structures: (a) the septum transversum, which forms the central tendon of the diaphragm (b) the twopleuroperitoneal membranes (c) muscular components from the lateral and dorsal body walls (d) the mesentery of the esophagus, in which the crura of the diaphragm develop
  5. 5.
  6. 6. • is most frequently caused by failure of one or both of the pleuroperitoneal membranes to close the pericardioperitoneal canals • Occasionally a small part of the muscular fibers of the diaphragm fails to develop- parasternal hernia
  7. 7. • Most infants with congenital diaphragmatic hernia (CDH) are mature • two thirds are male, • 90% the hernia is left-sided
  8. 8. • The diaphragm develops anteriorly as a septum between the heart and liver • progresses backward to close last at the left Bochdalek foramen around 8 to 10 weeks of gestation
  9. 9. • The bowel migrates from the yolk sac at about 10 weeks • if it arrives in the abdominal cavity before the foramen has closed, a hernia into the left hemithorax may result
  10. 10. • Lung compression from an early age is associated with pulmonary hypoplasia, most severe on the ipsilateral side but also present on the contralateral side if there is mediastinal shift to the right.
  11. 11. • There is a marked reduction in the number of bronchial generations, with a less marked reduction in the number of alveoli per acinus.
  12. 12. • The number of arterial generations is reduced proportionately to the reduction in airway numbers • and there is a modest increase in the medial muscle of pulmonary arterioles, together with abnormal peripheral extension of muscle into arterioles at the acinar level
  13. 13. • The lungs are immature even at full-term gestation, appearing arrested in the saccular phase of development • The terminal saccules have fewer septa, the interstitium is thicker, and there are fewer capillaries • Granular pneumocytes are more numerous, but they contain more glycogen and fewer surfactant lamellar bodies.
  14. 14. • The saccular lining cells are more cuboidal, and thin membranous pneumocytes are less developed
  15. 15. Causes • The cause of CDH is largely unknown • CDH can occur as part of a multiple malformation syndrome in up to 40% of infants (cardiovascular, genitourinary, and gastrointestinal malformations) • Karyotype abnormalities have been reported in 4% of infants with CDH, and CDH may be found in a variety of chromosomal anomalies including trisomy 13, trisomy 18, and tetrasomy 12p mosaicism
  16. 16. Pathogenesis • Lung Hypoplasia and Arterial Muscle Hyperplasia • Lung Immaturity and Surfactant Insufficiency
  17. 17. Associated Anomalies • 39% had associated anomalies and 61% were isolated • About 2/3rd of the anomalies were cardiac, including hypoplastic left heart syndrome, atrial septal defect, ventricular septal defect, coarctation of the aorta, and Ebstein anomaly • Other anomalies in this series included esophageal atresia, trisomy 18, hydronephrosis, hydrocephalus, and omphalocele.
  18. 18. Prenatal Diagnosis • ultrasonography diagnosis (as early as the second trimester) Mediastinal shunt Viscera herniation (stomach, intestines, liver*, kidneys, spleen and gall bladder) Abnormal position of certain viscera inside the abdomen Stomach visualization out of its usual position Intrauterine growth retardation* Polyhydramnios* Fetal hydrops* * bad prognosis
  19. 19. Fetal diafragmatic hernia: Ultrasound diagnosis
  20. 20. Prenatal MR Imaging - single-shot turbo spin-echo (HASTE)- of congenital diaphragmatic hernia
  21. 21. Prenatal MR Imaging of congenital diaphragmatic hernia
  22. 22. Pulmonary hypoplasia
  23. 23. Anatomopathology show of CDH
  24. 24. Prenatal Counseling multidisciplinary team • patient's obstetrician • perinatologist • geneticist • surgeon • social worker
  25. 25. Prenatal management • Glucocorticoids • Thyrotropin-releasing hormone • Fetal surgical therapy (Antenatal surgical intervention, In utero tracheal occlusion )
  26. 26. Delivery Room Management • affected infants should be delivered in a center that has experienced personnel and available therapies. • the team in the delivery room consist of personnel experienced in the immediate resuscitation and stabilization of critically ill neonates • affected patients in any respiratory distress require positive pressure ventilation in the delivery room. • To prevent distension of the gastrointestinal tract and further compression of the pulmonary parenchyma, a double-lumen nasogastric or orogastric tube of large caliber is placed to act as a vent. • Early intubation is preferable to bag-mask ventilation or continuous positive airway pressure via mask or nasal prongs
  27. 27. Postnanal Diagnosis • Respiratory distress • Scaphoid abdomen • Auscultation of the lungs reveals poor air entry • Shift of the heart to the side opposite
  28. 28. Associated Anomalies • 39% had associated anomalies • 61% were isolated • About 2/3rd of the anomalies were cardiac, including hypoplastic left heart syndrome, atrial septal defect, ventricular septal defect, coarctation of the aorta, and Ebstein anomaly • malformation syndrome involving the brain, heart, kidneys, bowel, or skeleton.
  29. 29. The Cardiac Function Problem • assess these patients with echocardiography • Technically difficult because of the malposition of the heart • cardiac malposition is not corrected by surgical repair of the CDH • left ventricular mass is reduced in CDH and that this may adversely affect the outcome • the hernia presses on the left atriumincreases the vascular pressureleft-to-right shuntingreduces the venous return to the left atrium
  30. 30. Lab Studies • Arterial blood gas – Obtain frequent arterial blood gas (ABG) measurements to assess for pH, PaCO2, and PaO2. – Note the sampling site because persistent pulmonary hypertension (PPHN) with right-to- left ductal shunting often complicates CDH. The PaO2 may be higher from a preductal (right-hand) sampling site. • Chromosome studies – Obtain chromosome studies because of the frequent association with chromosomal anomalies. – In rare cases, chromosomal disorders that can only be diagnosed by skin biopsy may be present. If dysmorphic features are observed on examination, a consultation with a geneticist is often helpful. • Serum electrolytes: Monitor serum electrolytes, ionized calcium, and glucose levels initially and frequently. Maintenance of reference range glucose levels and calcium homeostasis is particularly important
  31. 31. Imaging Studies • Cardiac ultrasonography – Perform ultrasonographic studies to rule out congenital heart diseases. – Because the incidence of associated cardiac anomalies is high (up to 25%), cardiac ultrasonography is needed to evaluate for associated cardiac anomalies. • Renal ultrasonography: A renal ultrasonographic examination may be needed to rule out genitourinary anomalies. • Cranial ultrasonography – Perform cranial sonography if the infant is being considered for extracorporeal support. – Ultrasonographic examination should focus on evaluation of intraventricular bleeding and peripheral areas of hemorrhage or infarct or intracranial anomalies.
  32. 32. Other Tests Pulse oximetry – Continuous pulse oximetry is valuable in the diagnosis and management of PPHN. – Place oximeter probes at preductal (right-hand) and postductal (either foot) sites to assess for a right-to-left shunt at the level of the ductus arteriosus
  33. 33. Postnanal Diagnosis left-sided CDH • Radiograph in a male neonate shows the tip (large arrow) of the nasogastric tube positioned in the left hemithorax. Note the marked apex leftward angulation of the umbilical venous catheter (small arrow).
  34. 34. Right congenital diaphragmatic hernia • Radiograph in a male neonate shows that the nasogastric tube (arrow) deviates to the left of the thoracic vertebral bodies as it passes through the inferior portion of the thorax
  35. 35. Procedures • Intubation and mechanical ventilation – Endotracheal intubation and mechanical ventilation are required for all infants with severe CDH who present in the first hours of life. – Avoid bag-and-mask ventilation in the delivery room because the stomach and intestines become distended with air and further compromise pulmonary function. – Avoid high peak inspiratory pressures and overdistension. Consider high-frequency ventilation if high peak inspiratory pressures are required.
  36. 36. Postnatal management • Mechanical ventilation • Nitric Oxide • Surfactant • ECMO • surgery
  37. 37. PROGNOSIS depends on the degree of pulmonary hypoplasia with its associated reduction in alveolar and vascular surface area.
  38. 38. Prognosis • Pulmonary recovery: When all resources, including ECMO, are provided, survival rates range from 40- 69%. • Long-term morbidity: Significant long-term morbidity, including chronic lung disease, growth failure, gastroesophageal reflux, and neurodevelopmental delay, may occur in survivors.
  39. 39. Prediction of Outcome • those needing treatment in the first 6 hours of life,the mortality rate was 44%, and in those needing treatment after 6 hours, the mortality rate was close to zero • These data imply that if the lungs are good enough to bridge the first 6 hours of life,the results of treatment are likely to be good
  40. 40. • the prognosis is best assessed by the arterial PCO2 and the intensity of conventional mechanical ventilation required, as a reflection of the degree of pulmonary hypoplasia • modified ventilation index= PIP X VR • a value under 1250 with a PCO2 of less than 40 mm Hg indicates mild or modest degree of lung hypoplasia
  41. 41. • the ratio of the left and right pulmonary artery dimensions • left artery was smaller than the right artery, severe pulmonary hypoplasia and severe pulmonary hypertension were more likely to be presen
  42. 42. • ratio of the dimensions for the combined left and right pulmonary arteries to the descending aorta • value of less than 1.3 predicted bad prognosis
  43. 43. Initial Treatment • should receive betamethasone prophylaxis for surfactant insufficiency, even at term gestation. • prenatal diagnoseda doublelumen orogastric tube • The infant should not be ventilated by bag and mask • immediately intubated and ventilated using a low peak pressure (less than 30 cm H2O) if possible and ventilator rates of 20 to 80 breaths/minute. • minimize lung injury • Paralysis benefit that swallowing air is abolished+pneumothorax • Exogenous surfactant • Hyperventilation and Alkalization Therapy
  44. 44. Surgery • After stabilization • no improvement in compliance after surgery, and most patients had major reductions in compliance, suggesting that surgery had made matters worse • due mostly to tension in the chest wall generated by the repaired diaphragm, and to tension generated by the abdominal wall after closure of the muscle layers, rather than to changes in the lung itself. • Thoracostomy tube to protect against the catastrophic development of tension pneumothorax
  45. 45. POSTOPERATIVE CARE • course is complicated by the development of pulmonary hypertension
  46. 46.