eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Pulmonary Hypoplasia: Follow-up

Author: Terry Chin, MD, PhD, Associate Professor of Pediatrics, Pediatric Allergy/Immunology/Pulmonology, Department of Pediatrics, University of California Irvine School of Medicine; Associate Director, Miller Children's Hospital at Long Beach Memorial Medical Center
Coauthor(s): Girija Natarajan, MD, Assistant Professor, Division of Neonatology, Children's Hospital of Michigan & Wayne State University; Ibrahim Abdulhamid, MD, Assistant Professor of Pediatrics, Wayne State University; Director of Pediatric Pulmonary Medicine, Clinical Director of Pediatric Sleep Laboratory, Children's Hospital of Michigan
Contributor Information and Disclosures

Updated: Mar 5, 2009

Follow-up

Further Outpatient Care

  • Because bronchopulmonary dysplasia (BPD) is likely in survivors of pulmonary hypoplasia, infants have an increased risk of fatality and serious morbidity with upper respiratory tract infection (URI) and lower respiratory tract infection (LRI).
  • Respiratory syncytial virus (RSV) prophylaxis should be considered during RSV season in infants younger than 2 years who have required oxygen in the previous 6 months. The dose of palivizumab is 15 mg/kg/mo through the RSV season, which, in most parts of the United States, is from October to March.
  • Patients with pulmonary hypoplasia should be considered as having a chronic pulmonary disease. Therefore, once they are older than 6 months, they should receive the influenza vaccine at the start of every influenza season, which is October or November in the United States. The dose of influenza vaccine is 0.25 mL per dose of the split virus vaccine. Children younger than 8 years should receive 2 doses 2 months apart for the first time. Children older than 8 years require 1 dose.
  • Similarly, these patients are considered high risk and should be administered the pneumococcal vaccine (PCV 7) at a dose of 0.5 mL intramuscularly until they are aged 5 years.

Inpatient & Outpatient Medications

  • Because these patients should be considered as having chronic lung disease, various aerosolized medications such as bronchodilators and corticosteroids should be considered if symptoms suggest reactive airway disease or obstructive airway disease.
  • Persistent pulmonary hypertension can be treated with various vasodilators and endothelin inhibitors.

Complications

  • Reduced exercise tolerance, even in mild cases
  • Scoliosis in adolescent years
  • Recurrent respiratory infections
  • Pneumothorax - Spontaneous or as a result of respiratory support
  • Persistent pulmonary hypertension caused by a reduced pulmonary vascular bed and worsened by hypoxia or a coexisting left-to-right intracardiac shunt
  • BPD in survivors of pulmonary hypoplasia caused by prolonged ventilatory support
  • Chronic pulmonary insufficiency - May persist in diaphragmatic hernia and pulmonary hypoplasia
  • Airway abnormalities, including tracheobronchial compression and tracheomalacia caused by the displaced aorta and enlarged left pulmonary artery

Prognosis

  • Right-sided hypoplasia has a worse prognosis than left-sided hypoplasia, probably because of the loss of the bigger right lung mass and more severe mediastinal shift and great vessel displacement.
  • The oligohydramnios tetrad of pulmonary hypoplasia, positional limb deformities, Potter facies, and intrauterine growth retardation has a very poor prognosis.
  • A minimum lung volume of 45% compared with age-matched control subjects has been shown to be a predictor of survival in neonates with diaphragmatic hernia treated with extracorporeal membrane oxygenation (ECMO). Similarly, a functional residual capacity of 12.3 mL/kg, about one half the normal capacity, has been thought to be a predictor of survival in pulmonary hypoplasia with congenital diaphragmatic hernia (CDH).

Miscellaneous

Medicolegal Pitfalls

  • When clinically recognized, pulmonary hypoplasia has a poor prognosis. It is frequently associated with severe and irreversible lesions, such as renal agenesis and thanatophoric dysplasia. Therefore, families must be fully informed and encouraged to participate in decisions to start aggressive and invasive treatments.
  • Medical care, respiratory support (eg, extracorporeal membrane oxygenation [ECMO]), surgical intervention (eg, fetal surgery), and dialysis should be carefully considered in view of poor outcomes and prognosis.
 
Acknowledgments

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



More on Pulmonary Hypoplasia

Overview: Pulmonary Hypoplasia
Differential Diagnoses & Workup: Pulmonary Hypoplasia
Treatment & Medication: Pulmonary Hypoplasia
Follow-up: Pulmonary Hypoplasia
Multimedia: Pulmonary Hypoplasia
References
[ CLOSE WINDOW ]

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. 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].

  9. 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].

  10. 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].

  11. 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].

  12. 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].

  13. 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].

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

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

  16. 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].

  17. 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].

  18. 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].

  19. 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].

  20. 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].

  21. 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].

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

  23. 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].

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

  25. 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].

  26. 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].

  27. 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].

  28. 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].

  29. 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].

  30. 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].

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

  32. 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].

  33. 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].

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

  35. 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].

  36. 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].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

pulmonary hypoplasia, pulmonary aplasia, bronchopulmonary dysplasia, BPD, treatment, diagnosis, underdevelopment of the lung, hypoplastic lung, carina, congenital diaphragmatic hernia, cystic adenomatoid malformation, CAM, prolonged rupture of membranes, fetal renal dysplasias, lung hypoplasia, oligohydramnios, hydrops fetalis, respiratory distress, apnea, ventilatory support, pneumothorax, arthrogryposis, Potter facies, hypertelorism, epicanthus, retrognathia, depressed nasal bridge, abdominal masses, tracheoesophageal fistula, imperforate anus, communicating bronchopulmonary foregut malformation, pleural effusion, asphyxiating thoracic dystrophy, achondroplasia, thanatophoric dwarfism, osteogenesis imperfecta, thoracic neuroblastoma, hydrothorax, urinary tract obstruction, renal dysplasia, tetralogy of Fallot

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

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

Medical Editor

Susanna A McColley, MD, Director of Cystic Fibrosis Center; Head, Division of Pulmonary Medicine; Associate Professor, Department of Pediatrics, Children's Memorial Medical Center of Chicago, Northwestern University
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 Consulting fee Consulting; Novartis Consulting fee Consulting; Altus Consulting fee Consulting; Axcan Scandi Consulting fee Consulting; Boston Scientific Consulting fee Consulting

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Heidi Connolly, MD, Associate Professor of Pediatrics and Psychiatry, University of Rochester; 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.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
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: Merck Honoraria Speaking and teaching

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.