THE TERM NEONATE WITH RESPIRATORY DISTRESS

1. THE TERM NEONATE WITH RESPIRATORY DISTRESS N. Ambalavanan MD University of Alabama at Birmingham Department of Pediatrics Division of Neonatology May 2003

2. Overview of talk • The emphasis is on evaluation and diagnosis • Management will depend mainly on the diagnosis

3. EVALUATION OF NEONATES WITH RESPIRATORY DISTRESS History Premature delivery Postmature delivery Fetal distress Meconium-stained fluid Maternal diabetes Oligohydramnios/ polyhydramnios Decreased fetal movements Traumatic delivery Drugs Cesarean section Vaginal bleeding

4. EVALUATION OF NEONATES WITH RESPIRATORY DISTRESS Physical Exam Major signs: cyanosis, tachypnea grunting, retraction, flaring Stridor, wheezes, hoarseness, and other airway findings Cardiovascular assessment Blood pressure Neurologic assessment Abdominal assessment Chest wall assessment Temperature

5. EVALUATION OF NEONATES WITH RESPIRATORY DISTRESS Laboratory Workup Chest radiograph Arterial blood gas Blood glucose Central hematocrit White blood cell and differential If indicated: Blood culture Echocardiogram Other diagnostic imaging

6. Neonate with respiratory distress Abnormal Chest X-Ray ? No Look for abnormalities of: Yes Common Uncommon Diaphrag. Hernia Trach-Esoph fistula Cysts, tumors Hypoplasia Hemorrhage Cong. Lobar Emph. Lymphangiectasia Sequestration AV fistulae Resp Distress Synd Transient Tachypnea Aspiration Syndr. Pneumonia Air leaks Effusion Pulmonary Edema Perfusion, BP, HCT Neuro-muscular Upper or lower airway Diaphragm or Chest wall Cardiac problems Other or Mixed findings Abdomen

7. Neonate with Acute Respiratory Distress Abnormal Yes No Lungs by Chest Radiograph Abnormalities in Common Uncommon Respiratory distress Diaphragmatic hernia Perfusion Neuro - Diaphragm syndrome Tracheoesophageal BP muscular or Chest Transient fistula HCT Findings Wall tachypnea Cysts and tumors Pneumonia Congenital lobar Aspiration syndromes emphysema Pneumothorax and Pulmonary hypoplasia Anemia Asphyxia Chest wall air leaks Accessory or sequestered lobes Polycythemia Intracranial disorders Pulmonary edema Pulmonary Hypotension hemorrhage Diaphragmatic Pleural effusion lymphangiectasia Hypovolemia Neuromuscular disorders Pulmonary hemorrhage Pulmonary disorders arteriovenous fistula Drugs CVS Other Airway Abdominal Findings or Mixed Findings Findings or Echo Findings Upper Airway Persistent fetal Ascites Sepsis Laryngeal circulation Necrotizing Acidosis Lower airway Cyanotic congenital enterocolitis Hypothermia, hear disease Abdominal mass cold stress Obstructive lesions Omphalocele Hyperthemia Gastroschisis Hypoglycemia Congestive heart Methemoglobinemia failure

8. NEONATE WITH ACUTE RESPIRATORY DISTRESS Abnormal lungs by chest radiograph - common Respiratory distress Pneumothorax syndrome and air leaks Transient tachypnea Pulmonary edema Pneumonia Pleural effusion Aspiration syndromes

9. Respiratory Distress Syndrome

10. Meconium Aspiration Syndrome

11. ABNORMAL RADIOGRAPHIC FINDINGS RDS - reticulogranular, ground glass appearance, decreased lung volume, air bronchograms TTN - fluid in fissure, interstitial fluid Pneumonia - infiltrates MAS - variable

12. Hypoplastic lungs

13. Congenital lobar emphysema

14. NEONATE WITH ACUTE RESIRATORY DISTRESS Abnormal lungs by chest radiograph - uncommon Diaphragmatic hernia Accessory or Tracheoesophageal fistula sequestered lobes Cysts and tumors Pulmonary Congenital lobar lymphangiectasia emphysema Pulmonary Pulmonary hypoplasia arteriovenous Pulmonary hemorrhage fistula

15. Meconium Asp. Syndrome with Pneumothorax

16. Small right pneumothorax

17. Pneumopericardium

18. Pneumomediastinum

19. AIR LEAKS • Pneumothorax • Pulmonary interstitial emphysema • Pneumomediastinum • Pneumopericardium • Pneumoperitoneum • Pulmonary venous air embolism • Subcutaneous emphysema • Others

20. Cystic adenomatoid malformation (CAM)

21. THORACIC CYSTS AND TUMORS Teratoma Gastric cyst Cystic hygroma Hemangioma Neurogenic tumor Angiosarcoma neuroblastoma Mediastinal goiter ganglioneuroma Thymoma neurofibroma Mesenchymoma Bronchial or bronchogenic cyst Lipoma Intrapulmonary cyst Cystic adenomatoid malformation

22. Pneumonia

23. ORGANISMS THAT MAY CAUSE PNEUMONIA IN THE NEONATE Bacterial Group B Strep E. coli Klebsiella S. aureus S. epidermidis Listeria Enterobacter H. influenzae S. pneumoniae Pseudomonas Bacteroides Others

24. ORGANISMS THAT MAY CAUSE PNEUMONIA IN THE NEONATE Viral Cytomegalovirus Adenovirus Rhinovirus Respiratory syncytial virus Parainfluenza Enterovirus Rubella Herpes simplex Varicella

25. ORGANISMS THAT MAY CAUSE PNEUMONIA IN THE NEONATE Others Candida (and other fungi) Ureaplasma Mycoplasma Chlamydia Syphilis Pneumocystis carinii Tuberculosis

26. Chylothorax/Hydrothorax

27. PLEURAL EFFUSION • Hydrothorax* - hydrops, tumor, pneumonia, congenital viral infection, congestive heart failure, transient tachypnea, Turner syndrome • Chylothorax* - spontaneous, complication of thoracic surgery • Hemothorax* - traumatic, ruptured vessel, disseminated intravascular coagulation, and other bleeding diatheses * Complications of central line placement may result in any of these .

28. ABNORMALITIES IN PERFUSION, BLOOD PRESSURE, AND HEMATOCRIT • Anemia • Polycythemia • Hypotension • Hypovolemia

29. Paralyzed right diaphragm from phrenic N. palsy

30. ABNORMALITIES WITH NEUROMUSCULARFINDINGS • Asphyxia • Intracranial hemorrhage • Neuromuscular disorders • Drugs

31. NEUROMUSCULAR DISORDERS • Myopathies • Myasthenia gravis • Werdnig-Hoffman disease • Spinal cord disorder • Poliomyelitis • Others

32. Left diaphragmatic hernia

33. ABNORMALITIES IN DIAPHRAGM OR CHEST WALL • Diaphragmatic disorders • Chest wall disorders

34. DIAPHRAGMATIC DISORDERS • Diaphragmatic hernia • Congenital eventration of the diaphragm • Diaphragm paralysis • Anterior diaphragmatic defect • Neuromuscular disorders • Accessory diaphragm • Agenesis of the diaphragm

35. CHEST WALL DISORDERS • Achondrogenesis • Chondroectodermal dysplasia • Osteogenesis imperfecta • Spondylocostal dysplasia • Others • Failure of sternal fusion • Benign and malignant tumors • Asphyxiating thoracic dystrophy • Achondroplasia • Thanatophoric dwarfism

36. ABNORMAL AIRWAY • Upper airway • Laryngeal • Lower airway

37. AIRWAY DISORDERS Upper Airway Choanal atresia/stenosis Cystic hygroma Trauma Thyroglossal duct cyst Hemangioma Micrognathia Teratoma Macroglossia Encephalocele Benign and malignant tumors

38. AIRWAY DISORDERS Laryngeal Laryngomalacia Cystic lesion Vocal cord paralysis Benign and malignant Hemangioma tumors Atresia, stenosis, or web Acquired lesion Congenital laryngeal stridor

39. AIRWAY DISORDERS Lower Airway Tracheomalacia Bronchial atresia Tracheoesophageal Abnormal branching fistula Cystic lesion Hemangioma Benign and malignant Vascular ring tumors Congenital tracheal Acquired lesion stenosis

40. Dextrocardia with complex congenital heart disease

41. Total Anomalous Pulmonary Venous Return with Obstruction

42. Transposition of Great Arteries (TGA)

43. ABNORMALITIES IN CARDIOVASCULAR FINDINGS OR ECHOCARDIOGRAM • Persistent fetal circulation • Cyanotic congenital heart disease • Obstructive lesions • Congestive heart failure

44. ABNORMALITIES IN ABDOMINAL FINDINGS • Ascites • Necrotizing enterocolitis • Abnormal mass • Omphalocele • Gastroschisis

45. OTHER ABNORMALITIES OR MIXED FINDINGS • Sepsis • Acidosis • Hypothermia, cold stress • Hyperthermia • Hypoglycemia • Methemoglobinemia

46. Neonate with respiratory distress Abnormal Chest X-Ray ? No Look for abnormalities of: Yes Common Uncommon Diaphrag. Hernia Trach-Esoph fistula Cysts, tumors Hypoplasia Hemorrhage Cong. Lobar Emph. Lymphangiectasia Sequestration AV fistulae Resp Distress Synd Transient Tachypnea Aspiration Syndromes Pneumonia Air leaks Effusion Pulmonary Edema Perfusion, BP, HCT Neuro-muscular Upper or lower airway Diaphragm or Chest wall Cardiac problems Other or Mixed findings Abdomen

47. Management • Management depends on diagnosis • Maintenance of adequate gas exchange • Maintain Airway • Oxygenation and Carbon dioxide elimination • Adequate pH • Adequate cardiac output and perfusion • Surgery • Pneumothorax/Cysts/Diaphragmatic hernia • Supportive therapy • Thermoregulation, Nutrition, Fluid and Electrolyte balance, etc • Antimicrobial therapy

48. Maintenance of gas exchange • Respiratory management depends on diagnosis • Usual sequence: No support  O2 hood  CPAP  Mechanical ventilation (conventional) High Frequency ventilation  add Nitric Oxide  ECMO • Surfactant may be useful in term infants with respiratory failure (Lotze et al. J Pediatr 132:40, 1998; Greenough. Eur J Pediatr 159:635, 2000)

49. Indications for mechanical ventilation • Clinical:Absolute: Apnea (intractable), gasping, cyanosis not responsive to O2 by hood Relative: Severe tachypnea / retractions • Laboratory (while on CPAP or FiO2 > 0.7): pH < 7.25 with PCO2> 60 mm Hg (or) PO2< 45- 50 and / or SpO2 < 85 % • Other: Surgical procedures, compromised airway

50. Term infants with PPHN Confirm diagnosis of PPHN Correct underlying abnormalities (hypothermia, acidosis, hypocalcemia, hypoglycemia, polycythemia) Conservative mechanical ventilation Trial of hyperventilation If low PO2, trial of rescue therapies MetabolicHFVSurfactantVasodilatorsECMO Alkalosis ? NO, PGD2, PGI2, Tolazoline, Adenosine