Pediatric Pulmonary Hypoplasia Medication

  • Author: Terry W Chin, MD, PhD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Mar 5, 2012
 

Medication Summary

Preterm rupture of membranes and an imminent preterm delivery is managed with tocolytics to control contractions and to prevent delivery, as indicated. Maternal steroids to accelerate lung maturity of the fetus are indicated in preterm labor.

The most common tocolytic agents used for the treatment of preterm labor are magnesium sulphate (MgSO4), indomethacin, and nifedipine. In the past, beta-mimetic agents, such as terbutaline or ritodrine, were the agents of choice, but in recent years their use has been significantly curtailed due to maternal and fetal side effects, such as maternal tachycardia, hyperglycemia, and palpitations. The use of these agents can lead to pulmonary edema, myocardial ischemia, and cardiac arrhythmia. The tocolytic agents currently used to treat preterm labor appear to be equally efficacious in delaying delivery for at least 48 hours. Although MgSO4 is associated with more maternal toxicity, indomethacin is associated with more fetal and neonatal toxicity. For more information, see Preterm Labor.

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Glucocorticoids

Class Summary

These agents are used to induce or accelerate lung maturity in a preterm newborn at less than 32 weeks' gestation or when lung immaturity is known by amniotic fluid assay. Long-acting steroids (eg, dexamethasone, betamethasone) are recommended by a National Institutes of Health (NIH) Consensus Conference panel for all pregnancies at 24-34 weeks' gestation at risk of preterm delivery, in patients with preterm rupture of membranes at less than 30-32 weeks' gestation, and in complicated pregnancies with anticipated delivery before 34 weeks' gestation unless the corticosteroid will have an adverse effect on the mother.

Dexamethasone (Decadron)

 

Decreases frequency of respiratory distress syndrome, surfactant therapy, and serious intraventricular hemorrhage. Optimal benefit occurs within 24 h and lasts for 7 d.

Betamethasone (Celestone Soluspan)

 

Decreases frequency of respiratory distress syndrome, surfactant therapy, and serious intraventricular hemorrhage. Optimal benefit occurs within 24 h and lasts for 7 d.

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Surfactants

Class Summary

These agents are administered at birth to newborns to improve lung mechanics and oxygenation when treating airspace disease. Following inhaled administration, surface tension is reduced, and alveoli are stabilized, thus decreasing the work of breathing and increasing lung compliance.

Beractant (Survanta)

 

A semisynthetic bovine lung extract that contains phospholipids, fatty acids, and surfactant-associated proteins B (7 mcg/mL) and C (203 mcg/mL).

Calfactant (Infasurf)

 

A natural calf lung extract that contains phospholipids, fatty acids, and surfactant-associated proteins B (260 mcg/mL) and C (390 mcg/mL).

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Contributor Information and Disclosures
Author

Terry W Chin, MD, PhD  Associate Director, Pediatric Allergy/Immunology/Pulmonology, Miller Children's Hospital, Long Beach Memorial Medical Center; Associate Professor, Department of Pediatrics, University of California, Irvine, School of Medicine

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Girija Natarajan, MD  Assistant Professor, Division of Neonatology, Children's Hospital of Michigan & Wayne State University

Girija Natarajan, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Ibrahim Abdulhamid, MD  Associate Professor of Pediatrics, Wayne State University School of Medicine; Director of Pediatric Pulmonary Medicine, Clinical Director of Pediatric Sleep Laboratory, Children's Hospital of Michigan

Ibrahim Abdulhamid, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Sleep Medicine, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Susanna A McColley, MD  Professor of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Boston Scientific Consulting fee Consulting; Gilead Honoraria Speaking and teaching; Caremark Consulting fee Consulting; Vertex Pharmaceuticals Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Heidi Connolly, MD  Associate Professor of Pediatrics and Psychiatry, University of Rochester School of Medicine and Dentistry; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Mary E Cataletto, MD  Director of Children's Sleep Services, Winthrop Sleep Disorders Center, Mineola, NY; Professor of Clinical Pediatrics, State University of New York at Stony Brook, Stony Brook, NY

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the previous contributions of Yazan Said, MD, to the writing and development of this chapter.

References
  1. Gallot D, Marceau G, Coste K, et al. Congenital diaphragmatic hernia: a retinoid-signaling pathway disruption during lung development?. Birth Defects Res A Clin Mol Teratol. Aug 2005;73(8):523-31. [Medline].

  2. Montedonico S, Sugimoto K, Felle P, Bannigan J, Puri P. Prenatal treatment with retinoic acid promotes pulmonary alveologenesis in the nitrofen model of congenital diaphragmatic hernia. J Pediatr Surg. Mar 2008;43(3):500-7. [Medline].

  3. Sugimoto K, Takayasu H, Nakazawa N, Montedonico S, Puri P. Prenatal treatment with retinoic acid accelerates type 1 alveolar cell proliferation of the hypoplastic lung in the nitrofen model of congenital diaphragmatic hernia. J Pediatr Surg. Feb 2008;43(2):367-72. [Medline].

  4. Nogueira-Silva C, Moura RS, Esteves N, Gonzaga S, Correia-Pinto J. Intrinsic catch-up growth of hypoplastic fetal lungs is mediated by interleukin-6. Pediatr Pulmonol. Jul 2008;43(7):680-9. [Medline].

  5. Gonzaga S, Henriques-Coelho T, Davey M, et al. Cystic adenomatoid malformations are induced by localized FGF10 overexpression in fetal rat lung. Am J Respir Cell Mol Biol. Sep 2008;39(3):346-55. [Medline].

  6. Logan JW, Rice HE, Goldberg RN, Cotten CM. Congenital diaphragmatic hernia: a systematic review and summary of best-evidence practice strategies. J Perinatol. Sep 2007;27(9):535-49. [Medline].

  7. Bush A, Hogg J, Chitty LS. Cystic lung lesions - prenatal diagnosis and management. Prenat Diagn. Jul 2008;28(7):604-11. [Medline].

  8. Hsu JS, Lee YS, Lin CH, Li FY, Jeng MJ, Soong WJ, et al. Primary congenital pulmonary hypoplasia of a neonate. J Chin Med Assoc. Feb 2012;75(2):87-90. [Medline].

  9. Berger RM, Beghetti M, Humpl T, Raskob GE, Ivy DD, Jing ZC, et al. Clinical features of paediatric pulmonary hypertension: a registry study. Lancet. Feb 11 2012;379(9815):537-46. [Medline].

  10. Morokuma S, Anami A, Tsukimori K, Fukushima K, Wake N. Abnormal fetal movements, micrognathia and pulmonary hypoplasia: a case report. Abnormal fetal movements. BMC Pregnancy Childbirth. Aug 17 2010;10:46. [Medline]. [Full Text].

  11. Baguma-Nibasheka M, Kablar B. Pulmonary hypoplasia in the connective tissue growth factor (Ctgf) null mouse. Dev Dyn. Feb 2008;237(2):485-93. [Medline].

  12. Deprest J, Jani J, Van Schoubroeck D, et al. Current consequences of prenatal diagnosis of congenital diaphragmatic hernia. J Pediatr Surg. Feb 2006;41(2):423-30. [Medline].

  13. Zaupa P, Kleinlein B, Hollwarth ME. Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia?. Pediatr Surg Int. Jul 2007;23(7):711-3. [Medline].

  14. Deprest JA, Flemmer AW, Gratacos E, Nicolaides K. Antenatal prediction of lung volume and in-utero treatment by fetal endoscopic tracheal occlusion in severe isolated congenital diaphragmatic hernia. Semin Fetal Neonatal Med. Feb 2009;14(1):8-13. [Medline].

  15. Gerards FA, Twisk JW, Fetter WP, Wijnaendts LC, van Vugt JM. Predicting pulmonary hypoplasia with 2- or 3-dimensional ultrasonography in complicated pregnancies. Am J Obstet Gynecol. Jan 2008;198(1):140.e1-6. [Medline].

  16. Tsukimori K, Masumoto K, Morokuma S, et al. The lung-to-thorax transverse area ratio at term and near term correlates with survival in isolated congenital diaphragmatic hernia. J Ultrasound Med. May 2008;27(5):707-13. [Medline].

  17. Obenauer S, Maestre LA. Fetal MRI of lung hypoplasia: imaging findings. Clin Imaging. Jan-Feb 2008;32(1):48-50. [Medline].

  18. Taylor GA, Atalabi OM, Estroff JA. Imaging of congenital diaphragmatic hernias. Pediatr Radiol. Jan 2009;39(1):1-16. [Medline].

  19. Matsushita M, Ishii K, Tamura M, et al. Perinatal magnetic resonance fetal lung volumetry and fetal lung-to-liver signal intensity ratio for predicting short outcome in isolated congenital diaphragmatic hernia and cystic adenomatoid malformation of the lung. J Obstet Gynaecol Res. Apr 2008;34(2):162-7. [Medline].

  20. Okuyama H, Kubota A, Kawahara H, Oue T, Kitayama Y, Yagi M. Correlation between lung scintigraphy and long-term outcome in survivors of congenital diaphragmatic hernia. Pediatr Pulmonol. Sep 2006;41(9):882-6. [Medline].

  21. Lin AE, Pober BR, Adatia I. Congenital diaphragmatic hernia and associated cardiovascular malformations: type, frequency, and impact on management. Am J Med Genet C Semin Med Genet. May 15 2007;145C(2):201-16. [Medline].

  22. Koumbourlis AC, Wung JT, Stolar CJ. Lung function in infants after repair of congenital diaphragmatic hernia. J Pediatr Surg. Oct 2006;41(10):1716-21. [Medline].

  23. Morikawa N, Kuroda T, Honna T, et al. The impact of strict infection control on survival rate of prenatally diagnosed isolated congenital diaphragmatic hernia. Pediatr Surg Int. Oct 2008;24(10):1105-9. [Medline].

  24. Peetsold MG, Heij HA, Kneepkens CM, Nagelkerke AF, Huisman J, Gemke RJ. The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity. Pediatr Surg Int. Jan 2009;25(1):1-17. [Medline].

  25. Fitzgerald DA. Congenital cyst adenomatoid malformations: resect some and observe all?. Paediatr Respir Rev. Mar 2007;8(1):67-76. [Medline].

  26. Nicolai T. Management of the upper airway and congenital cystic lung diseases in neonates. Semin Fetal Neonatal Med. Feb 2009;14(1):56-60. [Medline].

  27. Wilson RD. In utero therapy for fetal thoracic abnormalities. Prenat Diagn. Jul 2008;28(7):619-25. [Medline].

  28. Gucciardo L, Deprest J, Done' E, et al. Prediction of outcome in isolated congenital diaphragmatic hernia and its consequences for fetal therapy. Best Pract Res Clin Obstet Gynaecol. Feb 2008;22(1):123-38. [Medline].

  29. Askenazi SS, Perlman M. Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis. Arch Dis Child. Aug 1979;54(8):614-8. [Medline]. [Full Text].

  30. Cock ML, Albuquerque CA, Joyce BJ, Hooper SB, Harding R. Effects of intrauterine growth restriction on lung liquid dynamics and lung development in fetal sheep. Am J Obstet Gynecol. Jan 2001;184(2):209-16. [Medline].

  31. Kasprian G, Balassy C, Brugger PC, Prayer D. MRI of normal and pathological fetal lung development. Eur J Radiol. Feb 2006;57(2):261-70. [Medline].

  32. Keller RL, Hawgood S, Neuhaus JM, et al. Infant pulmonary function in a randomized trial of fetal tracheal occlusion for severe congenital diaphragmatic hernia. Pediatr Res. Nov 2004;56(5):818-25. [Medline]. [Full Text].

  33. Szabo I, Vizer M, Halvax L. Amnioinfusion to prevent pulmonary hypoplasia after premature rupture of membranes. Am J Obstet Gynecol. Jun 2001;184(7):1583-4. [Medline].

  34. Taylor GA, Atalabi OM, Estroff JA. Imaging of congenital diaphragmatic hernias. Pediatr Radiol. Jan 2009;39(1):1-16. [Medline].

  35. Thibeault DW, Haney B. Lung volume, pulmonary vasculature, and factors affecting survival in congenital diaphragmatic hernia. Pediatrics. Feb 1998;101(2):289-95. [Medline]. [Full Text].

  36. Warburton D, Olver BE. Coordination of genetic, epigenetic, and environmental factors in lung development, injury, and repair. Chest. Jun 1997;111(6 Suppl):119S-122S. [Medline].

  37. Wedegärtner U, Schroder HJ, Adam G. Fetal MRI in experimental tracheal occlusion. Eur J Radiol. Feb 2006;57(2):271-7. [Medline].

  38. Winn HN, Chen M, Amon E, Leet TL, Shumway JB, Mostello D. Neonatal pulmonary hypoplasia and perinatal mortality in patients with midtrimester rupture of amniotic membranes--a critical analysis. Am J Obstet Gynecol. Jun 2000;182(6):1638-44. [Medline].

  39. Yoshimura S, Masuzaki H, Gotoh H, Fukuda H, Ishimaru T. Ultrasonographic prediction of lethal pulmonary hypoplasia: comparison of eight different ultrasonographic parameters. Am J Obstet Gynecol. Aug 1996;175(2):477-83. [Medline].

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Chest radiograph of a newborn with primary pulmonary hypoplasia of the right lung showing shift of the mediastinum to the right hemithorax.
CT scan of the same patient (a newborn with primary pulmonary hypoplasia of the right lung) showing absence of the right lung. Note branching of the left lower lobe bronchus (horizontal arrow) and absence of airways in the right side (vertical arrow).
A posteroanterior radiograph of a 3-month-old infant with primary pulmonary hypoplasia of the right lung.
Lateral view of the same patient (a 3-month-old infant with primary pulmonary hypoplasia of the right lung) showing one dome of the diaphragm.
Bronchogram of the same patient (a 3-month-old infant with primary pulmonary hypoplasia of the right lung) showing absence of the airways in the right side and presence of the left main bronchus and its branches.
A chest radiograph of a 14-year-old child with primary pulmonary hypoplasia of the right side causing secondary scoliosis.
A chest radiograph of a newborn with achondroplasia and small chest causing hypoplasia of both lungs.
A chest radiograph of a newborn with diaphragmatic hernia in the right hemithorax shortly after birth.
CT scan of the same child (a newborn with diaphragmatic hernia in the right hemithorax shortly after birth) showing the presence of abdominal contents in the right hemithorax. Note the presence of the left lower bronchus and its main branches (horizontal arrow) and absence of the right lower lobe bronchus. The liver in the right hemithorax is indicated by the upper arrow.
A chest radiograph of a 10-month-old child after repair of a right diaphragmatic hernia showing loss of lung volume in the right hemithorax.
MRI of the same patient (a 10-month-old child after repair of a right diaphragmatic hernia) showing loss of right lung volume and smaller right pulmonary artery than the left pulmonary artery (arrow).
 
 
 
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