Respiratory Physiology Part III Respiratory Distress Syndrome, Transient Tachypnea of the Newborn, and Other Causes of R

1. Respiratory Physiology Part IIIRespiratory Distress Syndrome, Transient Tachypnea of the Newborn, and Other Causes of Respiratory Distress Staci Olister, MD Assistant Professor of Pediatrics Division of Neonatology LSU Health Sciences Center New Orleans, LA

2. Respiratory Distress Syndrome (RDS) remains one of the major causes of neonatal morbidity & mortality despite advances in neonatal care greatly improved outcomes can be attributed to introduction of pharmacologic acceleration of pulmonary maturity and development of surfactant replacement incidence of complications in survivors of RDS remains significant, including IVH, PDA, sepsis, pulmonary hemorrhage, BPD Are these disorders sequelae of RDS, its treatment or underlying prematurity?

3. RDS: diagnosis diagnosis made clinically & radiographically consider �respiratory insufficiency of prematurity� in absence of radiographic signs

4. RDS: risk factors low gestational age birth weight (gms) RDS 501-1500 56% 501-750 86% 751-1000 79% 1001-1250 48% 1251-1500 27% maternal diabetes hyperinsulinemia decrease surface-active phospholipid synthesis perinatal asphyxia

5. RDS: pathophysiology impaired or delayed surfactant synthesis followed by series of events that increase severity of disease for several days microscopically: diffuse atelectasis with few widely dilated alveoli; eosinophilic membrane lining airspaces consists of fibrinous matrix of cellular debris derived from injured epithelium

6. RDS: clinical presentation shortly after birth, infant develops tachypnea, grunting, flaring, retractions, cyanosis, apnea, cardiovascular instability progressive worsening of symptoms with peak severity by 2 to 3 days and onset of recovery by 72 hours recovery phase: regeneration of alveolar cells, including type II pneumocytes, with resultant increase in surfactant production

7. RDS: radiograph diffuse reticulogranular pattern, �ground-glass appearance� due to alveolar atelectasis and pulmonary edema air bronchograms typically homogeneous and symmetric cannot be reliably differentiated from neonatal pneumonia

8. RDS: radiograph

9. Antenatal steroids (ANS) 1970s: Liggins noticed lack of RDS & increased survival in preterm animals exposed to steroids. After years of study, most successful method to induce fetal maturation is prenatal corticosteroid administration. Accelerated lung maturation occurs with physiologic stress levels of corticosteroids via receptor mediated induction of specific developmentally regulated proteins. ANS induce structural maturation of the lung which translates into increased lung volume, increased lung compliance and increased response to exogenous surfactant, not an increase in alveolar surfactant pool size.

10. ANS no significant effects of combination therapy with steroids and TRH abnormal neurodevelopmental outcome in long-term follow-up when administered to mother 24-48 hours prior to delivery, decrease in incidence and severity of RDS most effective when administered before 34 weeks and no more than 7 days prior to delivery some reduction in neonatal mortality, RDS and IVH when given < 24 hours before delivery; therefore, always consider ANS administration unless immediate delivery is anticipated decreased fetal growth & poorer developmental outcome have been reported in retrospective clinical studies of repeated courses of ANS

11. ANS reduced incidence of IVH may be due to secondary stabilization of cerebral blood flow or steroid-induced maturation of vascular integrity in germinal matrix proven benefit of combined use of ANS and postnatal surfactant in preterm infants maternal steroids may induce an increase in total leukocyte and immature neutrophil counts in infant

12. RDS: treatment continuous noninvasive monitoring of arterial hemoglobin saturation (pulse oximetry) small changes in saturation at PaO2>60 mmHg �signal extraction technology� may improve accuracy of pulse ox measurements arterial blood gas samples complication of indwelling arterial catheters is aneurysmal dilatation with dissection of abdominal aorta

13. RDS: treatment thermoregulation positive nitrogen balance as little as 60 kcal/kg/day with 10% as protein limitation of fluid intake may reduce risk of PDA, NEC, IVH cardiovascular management systemic hypotension in early phase of RDS is common antibiotics maintain Hct 35-40% during acute phase

14. Surfactant 1959, Avery & Meade reported deficiency of pulmonary surfactant had key role in pathogenesis of RDS discovery of surfactant led investigators to administer aerosolized phospholipids to infants with RDS with limited success 1980, Fujiwara, developed mixture of natural & synthetic surface-active lipids and administered exogenous surfactant to 10 preterm infants Hallman administered pooled human surfactant obtained from term amniotic fluid in a trial of infants < 30 weeks gestation

15. Surfactant subsequent trials used unmodified alveolar lavage of calf lung (INFASURF) protein-free synthetic phospholipids with alcohol acting as a spreading agent for dipalmitoyl phosphatidylcholine (EXOSURF) Fujiwara preparation, modified extract of bovine lung mince (SURVANTA) 1991, Cochrane & Revak described new synthetic surfactant containing phospholipids & synthetic peptide, sinapultide, that mimics SP-B (KL4, lucinactant, SURFAXIN)

16. Surfactant proteins SP-A: has role in surface activity & regulatory role in innate immunity SP-B: essential in processing surfactant, maintaining alveolar expansion, reducing surface tension SP-C: has role in surfactant metabolism & function SP-D: has regulatory role in innate immunity genes that code for surfactant proteins have been characterized; therefore, recombinant DNA technology will allow production of modified human surfactant proteins in combination with synthetic phospholipids

17. Surfactant use primary mechanism by which surfactant improves PaO2 is enhanced matching of ventilation & perfusion increase in lung volume & stabilization of FRC may be due to regional overdistention of alveoli multitude of studies evaluated various comparisons of prevention & rescue protocols, as well as comparisons between synthetic & natural preparations all regimens appear to decrease incidence of air leaks, improve oxygenation of ventilated preterm infants, decrease mortality

18. Surfactant incidence of BPD, IVH, PDA & sepsis are unaltered in most studies (may reflect enhanced survival of very premature infants) inconsistent reports of higher rates of pulmonary hemorrhage in surfactant-treated infants occurs in 6% in first 72 hours of life improved compliance after surfactant promotes left-to-right shunt through PDA resulting in pulmonary congestion & elevated capillary pressures with intra-alveolar hemorrhagic edema no adverse immunologic consequences of foreign tissue protein administration with use of natural surfactants no adverse effects on physical growth, respiratory symptoms or neurodevelopmental outcome

19. Elective high frequency oscillatory ventilation versus conventional ventilation for acute pulmonary dysfunction in preterm infants. Cochrane Database Syst Rev. 2003;(4):CD000104. to determine whether the elective use of HFOV as compared to CV in preterm infants who are mechanically ventilated for RDS decreases the incidence of CLD, without adverse effects meta-analysis of 11 eligible studies with 3275 infants there is no clear evidence that elective HFOV as compared with CV offers important advantages when used as the initial ventilation strategy to treat preterm babies with acute pulmonary dysfunction no evidence of reduction in death rate small but inconsistent reduction in rate of CLD short term neurological morbidity cause by HFOV, but not statistically significant 2 trials with increased rate of Grade 3 or 4 IVH& PVL with HFOV inadequate information on long term neurodevelopmental outcome

20. A multicenter randomized masked comparison trial of synthetic surfactant versus calf lung surfactant extract in the prevention of neonatal respiratory distress syndrome. Hudak ML. Pediatrics. 1997 Jul;100(1):39-50. to compare efficacy and safety of prophylactic Exosurf vs Infasurf in the prevention of RDS 846 eligible infants < 29 WGA significant reductions in incidence of RDS & associated pulmonary air leaks, severity of early respiratory disease, mortality attributable to RDS suggest that Infasurf is more effective than Exosurf however, Infasurf prophylaxis associated with greater risk of total but not severe IVH (39.0% vs 29.9%)

21. Natural surfactant extract versus synthetic surfactant for neonatal respiratory distress syndrome. Cochrane Database Syst Rev. 2001;(2):CD000144. to compare effects of synthetic to natural surfactant preparations meta-analysis of 11 trials both natural and synthetic extracts are effective in treatment of RDS greater early improvement in requirement for ventilator support, fewer pneumothoraces, fewer deaths, increase in IVH (not Grade 3 or 4) associated with natural surfactant

22. Prophylactic versus selective use of surfactant in preventing morbidity and mortality in preterm infants. Cochrane Database Syst Rev.2001;(2)CD000510. to compare effect of prophylactic surfactant administration to surfactant treatment of established RDS meta-analysis of 8 studies prophylactic treatment resulted in decreased incidence of pneumothoraces, PIE, BPD and death; infants < 30 weeks significant reduction in mortality and BPD unclear criteria to judge �at risk� infants

23. Early surfactant administration with brief ventilation vs selective surfactant and continued mechanical ventilation for preterm infants with or at risk for respiratory distress syndrome. Cochrane Database Syst Rev.2004;(3):CD003063. to compare early surfactant administration with brief ventilation (< 1 hour) followed by extubation vs later selective surfactant administration & continued mechanical ventilation 4 randomized controlled trials full meta-analysis not available early surfactant with extubation to NCPAP is associated with reduced need for mechanical ventilation, increased use of exogenous surfactant

24. Surfactant administration by transient intubation in infants 29-35 weeks� gestation with respiratory distress syndrome decreases the likelihood of later mechanical ventilation: a randomized controlled trial. Reininger A. J Perinatol. 2005 Nov;25(11):703-8. in infants with early RDS, compare surfactant with extubation to NCPAP vs NCPAP alone transient intubation for surfactant administration reduces later mechanical ventilation overall surfactant use, duration of oxygen therapy, length of stay and BPD were unaffected

25. Comparison of Infasurf (calfactant) and Survanta (beractant) in the prevention and treatment of respiratory distress syndrome.Bloom TB. Pediatrics 2005;116:392-399. previous smaller comparison demonstrated that Infasurf led to more rapid decrease in oxygen & mean airway pressure compared to Survanta; however, sample size too small to reach any conclusion in terms of lung injury, survival or CLD 2 prospective, randomized trials prophylaxis of infants born between 23 weeks 0 days & 29 weeks 6 days treatment of infants with clinical evidence of RDS primary endpoint: survival to 36 weeks without use of supplemental oxygen

26. Comparison of Infasurf (calfactant) and Survanta (beractant) in the prevention and treatment of respiratory distress syndrome.Bloom TB. Pediatrics 2005;116:392-399. both trials halted after 32 month recruitment period based on poor enrollment Mar 2001- Nov 2003 interest in CPAP changes in clinical practice including reduction in postnatal steroid use & introduction of NO would make comparability difficult data not to be interpreted as treatments under investigation were equivalent

27. A multicenter, randomized, masked, comparison trial of lucinactant, colfosceril palmitate, and beractant for the prevention of respiratory distress syndrome among very preterm infants. Moya FR. Pediatrics. 2005 Apr;115(4):1018-29. 1294 infants 600-1250 grams, < 32 weeks were randomly assigned to receive Exosurf, Surfaxin or Survanta within 20-30 minutes after birth lucinactant is more effective than colfosceril significantly reduced incidence of RDS at 24 hours & BPD at 36 weeks lucinactant significantly reduced RDS-related mortality at 14 days compared with both lucinactant is effective therapeutic option for preterm infants at risk for RDS

28. A multicenter, randomized, controlled trial of lucinactant versus poractant alfa among very premature infants at high risk for respiratory distress syndrome. Sinha SK. Pediatrics. 2005 Apr;11(4):1030-8. 252 infants born between 24-28 weeks, birth weights 600-1250 grams were randomly assigned to receive either surfactant within 30 minutes of life similar in terms of efficacy and safety

29. Lucinactant: a novel synthetic surfactant for the treatment of respiratory distress syndrome. Donn SM. Expert Opin Investig Drugs. 2005 Mar;14(3):329-34. being investigated for other indications including meconium aspiration, treatment of BPD, acute RDS and asthma novel aerosol formulation administered with NCPAP under development for treatment of respiratory insufficiency non-animal origin makes it an attractive alternative to present animal-derived surfactants by eliminating risks of infection and immunogenicity

30. Comparison of clinical efficacy of Newfacten versus Surfacten for the treatment of respiratory distress syndrome in the newborn infants. Choi CW. J Korean Med Sci. 2005 Aug;20(4);591-7. Newfacten: bovine lung-derived, semi-synthetic surfactant; less expensive, domestic Surfacten: Japanese modified bovine surfactant 492 infants with RDS, randomized comparable in clinical efficacy in treatment of RDS

31. Prophylaxis of neonatal respiratory distress syndrome by intra-amniotic administration of pulmonary surfactant. Zhang JP. Chin Med J. 2004 Jan;17(1):120-4. to evaluate the efficacy and safety of intra-amniotic administration of pulmonary surfactant 15 women due for preterm delivery received intra-amniotic injection of pulmonary surfactant and delivered within several hours significant reduction in proportion of NRDS and time in hospital

32. Surfactant use for neonatal lung injury: beyond respiratory distress syndrome. Finer NN. Paediatr Respi Rev. 2004;5 Suppl A:S289-97. evidence for surfactant use in disease processes other than RDS meconium aspiration, group B strep pneumonia/sepsis, pulmonary hemorrhage, CDH, near-term infants with severe respiratory distress

33. Transient Tachypnea of the Newborn (TTN) delayed resorption of fetal lung fluid risk factors: elective C/S prolonged labor with failure to progress prolonged administration of hypotonic fluids vaginal compression? lung function tests showed increased liquid volume and decreased gaseous volume in infants not exposed to vaginal compression C/S infants with same FRC but increased respiratory rate consistent with higher pulmonary water content

34. TTN: clinical presentation term or near term infants with respiratory distress in first few hours of life (grunting, flaring, retractions, mild cyanosis) self-limited, lasts 12-72 hours no risk of residual lung dysfunction distinguish from cerebral hyperventilation: tachypneic, term infant with respiratory alkalosis due to irritation of respiratory drive center

35. TTN: radiograph prominent perihilar markings represent engorged periarterial lymphatics that clear alveolar fluid patchy infiltrates

36. TTN: radiograph

37. Transient tachypnea of the newborn. An analysis of neonatal and obstetric risk factors. Rawlings JS. Am J Dis Chil. 1984 Sep;138(9):869-71. clinical data of 200 infants higher incidence of male sex, macrosomia, longer labor intervals, failure to progress in infants with TTN excessive maternal sedation, perinatal asphyxia and elective c/s delivery without preceding labor were not seen more frequently when TTN developed

38. Cesarean section, gestational age, and transient tachypnea of the newborn: timing is key. Riskin A. Am J Perinatol. 2005 Oct;22(7):377-82. to identify risk factors and characterize infants with TTN 67 newborns with TTN, > 35 weeks lower GA, C/S delivery higher in TTN infants scheduling elective C/S at GA >38 weeks may decrease frequency of TTN

39. Furosemide for transient tachypnea of the newborn. Cochrane Database Syst Rev. 2002;(1):CD003064. to determine whether furosemide reduces duration of oxygen therapy and respiratory symptoms and shortens hospital stay in term infants with TTN one trial was methodologically sound; 50 infants with TTN given oral furosemide 2mg/kg, then 1 mg/kg 12 hours later no difference in tachypnea, severity of symptoms or hospital stay question remains whether iv furosemide or maternal administration prior to delivery might shorten duration of illness

40. Maternal asthma and transient tachypnea of the newborn. Demissie K. Pediatrics. 1998 Jul;102(1pt1):84-90. to examine relationship between TTN & asthma complicating pregnancy 11,445 mother-infant dyads; 2289 coded with maternal asthma maternal asthma is a risk factor for TTN especially among male, term infants mechanism for this association is undetermined

41. Extrapulmonary causes of respiratory distress Neuromuscular brain: asphyxia, drugs, hemorrhage, infection nerves: phrenic nerve injury Myasthenia gravis Muscular dystrophies Hematologic anemia polycythemia Acid-base metabolic acidosis Mechanical-restrictive Airway obstruction Rib cage thoracic dystrophies generalized bone disease Diaphragm phrenic nerve injury congenital eventration CDH pleural effusion abdominal distention Cardiovascular CHD PPHN

42. Congenital diaphragmatic hernia (CDH) unilateral diaphragmatic herniation with displacement of abdominal contents into thoracic cavity failure of closure of pleuroperitoneal canal during 8th week of life occurs ~1/2200 live births 80% involve left hemi-diaphragm, foramen of Bochdalek right-sided hernias have been associated with group B strep pneumonia ipsilateral lung with decreased airway & alveolar numbers, small pulmonary artery contralateral lung with decreased alveolar number, increased muscularization of peripheral arteries??fixed & variable pulmonary hypertension

43. CDH: clinical presentation varying degrees of respiratory distress, cyanosis, scaphoid abdomen, mediastinal shift 16-22% with other congenital anomalies

44. CDH: radiograph

45. CDH: radiograph

46. CDH after repair, reduced number of bronchi & alveoli, persistently increased muscularity of pulmonary blood vessels for months pulmonary hypoperfusion may persist for years chronic pulmonary insufficiency may develop late complications: recurrence of hernia, small bowel obstruction, GER, thoracolumbar scoliosis survival: infants with symptoms within 8 hours of life who reach a treatment center have 40-60% survival rate

47. Evidence-based management of infants with congenital diaphragamatic hernia. Moya FR. Semin Perinatol. 2005 Apr;29(2):112-7. limited evidence available suggests better outcomes are observed by delivering infants with CDH at experienced centers, by delaying surgical repair until hemodynamic and respiratory stability is achieved, and by the judicious use of non-aggressive mechanical ventilation and permissive hypercapnea only 9 randomized trials over past 15 years enrolling 250 infants use of ANS in pregnancies beyond 34 weeks to �mature� the lungs cannot be recommended at this time no clear evidence for delayed (when stabilized) surgical repair as compared with immediate (within 24 hours) because lack of clinical evidence, administration of surfactant not recommended optimal mode of ventilation not clear; strategies to limit lung distention are associated with higher survival usefulness of perfluocarbon-induced lung growth remains unclear need to establish networks of centers that manage enough infants with CDH to conduct appropriately sized trials to answer critical questions about management and long-term outcomes

48. The role of extracorporeal membrane oxygenation in the management of infants with congenital diaphragmatic hernia. Khan AM. Semin Perinatol 2005 Apr;29(2):118-22. indication, type and timing of ECMO in relation to surgery for CDH continues to evolve �failure to respond to medical therapy� most infants with CDH can be treated with VV ECMO pre-operative stabilization rather than postoperative rescue difficult to prove benefit provided by ECMO support some literature shows adverse outcomes among infants with CDH treated with ECMO

49. The role of extracorporeal membrane oxygenation in the management of infants with congenital diaphragmatic hernia. Khan AM. Semin Perinatol 2005 Apr;29(2):118-22. West, in press: significant pulmonary, gastrointestinal, cardiac and neurological morbidity in infants with CDH Stolar,1996: ECMO-treated infants without CDH, 89% cognitively normal vs 60% with CDH McGahern,1997: 75% survival of ECMO-treated infants with CDH, 67% with neurologic compromise although worsened neurologic outcomes may be associated with severity of illness, independent ECMO factors could not be ruled out UK Collaborative ECMO Trial Group, 2004: 73 ECMO treated infants with CDH, 1991-2000 46 weaned off ECMO 42 survived to hospital discharge 27 survived > 1 year 7 were problem-free Steven, 2002:duration of ECMO & number of complications have increased progressively Need to redefine role of ECMO in CDH: better selection criteria

50. Birth weight and McGoon Index predict mortality in newborn infants with congenital diaphragmatic hernia. Casaccia G. J Pediatr Surg. 2006 Jan;41(1):25-8. modified McGoon index calculated by ratio between diameters of pulmonary arteries and descending aorta has been used to determine mortality of CDH low birth weight and low MGI has highest prediction of mortality

51. Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Jani J. Ultrasound Obstet Gynecol. 2006 Jan;27(1):18-22. significant predictors of survival were presence of intrathoracic herniation of liver and fetal lung area to head circumference postnatal mortality is substantially higher if there is intrathoracic herniation of liver measurement of LHR at 22-28 weeks gestation is useful in prediction of subsequent survival

52. Current consequences of prenatal diagnosis of congenital diaphragmatic hernia. Deprest J. J Pediatr Surg. 2006 Feb;41(2):423-30. fetuses with isolated left-sided CDH, liver herniation, and lung-to-head ratio < 1 have a poor prognosis percutaneous FETO (fetal endoluminal tracheal occlusion) is minimally invasive and may improve outcome in these selected cases balloon insertion at 26-28 weeks procedure carries risk for PPROM patency of airway restored in either prenatal or perinatal period

53. Improvement of pulmonary hypoplasia associated with congenital diaphragmatic hernia in utero by CFTR gene therapy. Larson JE. Am J Physiol Lung Cell Mol Physiol. 2006 Feb 10 CDH may be ideal candidate for in utero gene therapy as disrupted fetal lung growth plays a significant role in disease outcome in utero over expression of CFTR (cystic fibrosis transmembrane conductance regulator) during fetal development results in lung epithelial proliferation and differentiation induced CDH in rats & found that in utero gene transfer efficiency depends on the stage of lung development

54. Neonatal thorascopic repair of congenital diaphragmatic hernia: selection criteria for successful outcome. Yang EY. J Pediatr Surg. 2005 Sep;40(9):1369-75. neonatal thorascopic CDH repair is safe in selected patients who have good preoperative pulmonary function and anatomy amenable to primary diaphragmatic repair stomach in abdomen, minimal ventilator support with PIP in low 20s, no evidence of pulmonary hypertension

55. Diaphragmatic Paralysis most commonly presents on right following birth trauma, especially if LGA or shoulder dystocia due to stretched nerve roots of C3-5 75% with associated brachial plexus injury or Horner�s syndrome may have fracture of clavicle or humerus

56. Diaphragmatic Paralysis radiograph: elevated hemi-diaphragm fluoroscopy: paradoxical motion of hemi-diaphragm recovery depends on degree of injury avulsion being permanent eventration secondary to muscle atrophy treatment: supportive, with expected resolution surgical plication complications: pneumonia

57. Incidence and treatment of diaphragmatic paralysis after cardiac surgery in children. Joho-Arreola AL. Eur J Cardiothorac Surg. 2005 Jan;27(1):53-7. phrenic nerve injury due to thoracic surgery is the most common cause of DP in children incidence 5.4% spontaneous recovery from postsurgical DP is rare respiratory insufficiency requires diaphragmatic plication in most infants with DP transthoracic plication is an effective treatment

58. Cervical Spine Transection injury to spinal cord occurs predominantly with breech deliveries or shoulder dystocia complete paralysis below level of injury, usually C7 infants present depressed, with absent respiratory excursions, flaccid, immobile with absent DTRs may have some withdrawal responses mediated by spinal reflex distal to site of injury Diagnosis: MRI

59. Airway Obstruction Nose choanal atresia drugs: reserpine infection: syphilis iatrogenic: tubes, tape Mouth/jaw Pierre-Robin enlarged tongue cysts: thyroglossal, gingival Larynx laryngeal web/cleft cord paralysis laryngospasm laryngomalacia vocal cord paralysis Trachea/bronchi tracheomalacia tracheal stenosis, cyst broncho-malacia/stenosis lobar emphysema Extrinsic goiter vascular rings hemangioma cystic hygroma T-E fistula mediastinal masses

60. Vascular Rings occur early in development of aortic arch & great vessels due to abnormal or incomplete regression of branchial arches 1-6 males = females double aortic arch symptoms due to compression of trachea & esophagus close association with 22q11 deletions MRI/CT defines anatomy treat immediately if symptomatic

61. Vascular Rings

62. Choanal Atresia 1/5000-7000 live births 2/3 unilateral female: male 2:1 right:left 2:1 90% osseous; 10% membranous most cases occur sporadically

63. Choanal Atresia 20-50% associated with other anomalies Apert, Treacher-Collins, Crouzon, Down CHARGE: abnormal embryogenesis of neuroectodermal cell lines 50-70% with heart defects, commonly conotruncal/aortic arch malformations clinical overlap with DiGeorge syndrome cyanosis relieved with crying as predominant nasal breathers for 4-6 weeks diagnosis: CT treatment: transendonasal perforation of atretic membrane or bone

64. Congenital Pulmonary Lymphangiectasia rare entity of respiratory distress & pleural effusion which may progress to fatal outcome despite intensive support disordered growth of lymphatic ducts & obstruction of lymph flow in one or both lungs resulting in cystic lymphatic spaces may be coexistent lymphedema, congenital cardiac malformations males: females 2:1 associated with Noonan, Turner-Ullrich,& Down syndromes radiograph: hyperinflation, diffuse granularity with prominent interstitial markings

65. Congenital Cystic Adenomatoid Malformation (CCAM) caused by failure of pulmonary mesenchyme to progress to normal bronchoalveolar development resulting in cystic replacement of pulmonary parenchyma usually presents in infancy with respiratory distress conservative therapy if asymptomatic if do not resolve, resection to prevent infection and malignant transformation

66. Congenital Cystic Adenomatoid Malformation (CCAM)

67. Congenital Lobar Emphysema disturbed growth in airway or alveolar number partial intraluminal bronchial obstruction & distal hyperinflation may result from lack of bronchial cartilage overdistention of one or more lobes most commonly left upper lobe symptoms commonly begin at 1-2 months radiograph: hyperinflated lobe, mediastinal shift, compression of adjacent lung increased incidence of congenital heart disease conservative management if possible

68. Congenital Lobar Emphysema

69. Chylothorax/Hydrothorax related to traumatic injury of thoracic duct at delivery or congenital malformation of lymphatic system typically unilateral radiograph: large pleural effusion diagnosis: thoracentesis reveals clear fluid rich in lymphocytes which changes to opalescent fluid with high protein & fat content after enteral feeds usually recover after single or multiple thoracenteses, 4-6 weeks chemical pleurodesis has been reported if continuous drainage of chyle severe malnutrition lymphopenia & infections if feeding enterally, use formula high in medium chain triglycerides

70. Octreotide in the treatment of congenital chylothorax. Rasiah SV. J Paediatr Child Health. 2004 Sep-Oct;40(9-10):585-8. cases unresponsive to conservative management usually require surgery parenteral octreotide, somatostatin analogue, may reduce lymphatic drainage by its direct action on splanchnic flow