Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Perinatal/Neonatal Case Presentation
  • Published:

Perinatal/Neonatal Case Presentation

Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature

Abstract

Unilateral pulmonary hypoplasia is a rare cause of respiratory distress in the neonate. It is usually secondary to other causes such as diaphragmatic hernia. We present a case of a newborn with primary hypoplasia of the right upper lobe who was later found to also have tracheobronchomalacia. We describe the clinical course through early childhood.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1
Figure 2
Figure 3
Figure 4

Similar content being viewed by others

References

  1. Wigglesworth J, Desai R . Is fetal respiratory function a major determinant of perinatal survival? Lancet 1982;i:264–267.

    Article  Google Scholar 

  2. Ashkenazi S, Perlman M . Pulmonary hypoplasia: lung weight and radial alveolar count as criteria of diagnosis. Arch Dis Child 1979;54:614–618.

    Article  Google Scholar 

  3. Hussein A, Hessel R . Neonatal pulmonary hypoplasia: an autopsy study of 25 cases. Pediatr Pathol 1993;13:475–484.

    Article  Google Scholar 

  4. Moessinger A, Santiago A, Paneth N, Rey H, Blanc W, Driscoll J . Time trends in necroscopy prevalence and birth prevalence of lung hypoplasia. Pediatr Perinat Epidemiol 1989;3:421–431.

    Article  CAS  Google Scholar 

  5. Knox W, Barson A . Pulmonary hypoplasia in a regional perinatal unit. Early Hum Dev 1986;114:33–42.

    Article  Google Scholar 

  6. Page D, Stocker J . Anomalies associated with pulmonary hypoplasia. Am Rev Respir Dis 1982;125:216–221.

    CAS  PubMed  Google Scholar 

  7. Porter H . Pulmonary hypoplasia. Arch Dis Child Fetal Neonatal Ed 1999;81:81F–83F.

    Article  Google Scholar 

  8. Benson J, Olsen M, Fletcher B . A spectrum of bronchopulmonary anomalies associated with tracheoesophageal malformations. Pediatr Radiol 1985;15:377–380.

    Article  CAS  Google Scholar 

  9. Sbokos C, McMillan I . Agenesis of the lung. Br J Dis chest 1977;71:183–197.

    Article  CAS  Google Scholar 

  10. Nowotny T, Ahrens B, Bittigau K, et al. Right-sided pulmonary aplasia: longitudinal lung function studies in two cases and comparison to results from healthy term neonates. Pediatr Pulmonol 1998;26:138–144.

    Article  CAS  Google Scholar 

  11. Schwartz M, Ramachandran P . Congenital malformations of the lung and mediastinum — a quarter century of experience from a single institution. J Pediatr Surg 1997;32:44–47.

    Article  CAS  Google Scholar 

  12. Cremin B, Bass E . Retrosternal density: a sign of pulmonary hypoplasia. Pediat Radiol 1975;3:145–147.

    Article  CAS  Google Scholar 

  13. Becmeur F, Horta P, Donato L, Savage P . Accessory diaphragm — review of 31 cases in the literature. Eur J Pediatr Surg 1995;5:43–47.

    Article  CAS  Google Scholar 

  14. Swischuk L . Respiratory system. Imaging of the Newborn, Infant, and Young Child. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1997. p. 56–62.

  15. Thomas RJ, Lathif HC, Sen S, Zachariah N, Chako J . Varied presentations of unilateral lung hypoplasia and agensis: a report of four cases. Pediatr Surg Int 1998;14:94–95.

    Article  CAS  Google Scholar 

  16. Wu C, Chen M, Shih S, Huang F, Hou S . Case report: agenesis of the right lung diagnoses by three-dimensional reconstruction of helical chest CT. Br J Radiol 1996;69:1052–1054.

    Article  CAS  Google Scholar 

  17. Argent A, Cremin B . Computed tomography in agenesis of the lung in infants. Br J Radiol 1992;65:221–224.

    Article  CAS  Google Scholar 

  18. Nakamura Y, Harada K, Yamamoto I, et al. Human pulmonary hypoplasia: statistical, morphological, morphometric, and biochemical study. Arch Pathol Lab Med 1992;116:635–642.

    CAS  PubMed  Google Scholar 

  19. Felson B . Pulmonary agenesis and related anomalies. Semin Roentgenol 1972;7:17–30.

    Article  CAS  Google Scholar 

  20. Hoffman M, Superina R, Wesson DE . Unilateral pulmonary agenesis with esophageal atresia and distal trachoesophageal fistula: report of two cases. J Pediatr Surg 1989;24:1084–1085.

    Article  CAS  Google Scholar 

  21. Knowles S, Thomas R, Lundenbaum R, Keeling J, Winter R . Pulmonary agenesis as part of the VACTERL sequence. Arch Dis Child 1988;63:723–726.

    Article  CAS  Google Scholar 

  22. Osbourne J, Masel J, McCredie J . Spectrum of skeletal anomalies associated with pulmonary agenesis: possible neural crest injuries. Pediatr Radiol 1989;19:425–432.

    Article  Google Scholar 

  23. Renert W, Berdon W, Baker D, Rose J . Obstructive urologic malformations of the fetus and infant — relation to neonatal pneumomediastinum and penumothorax. Radiology 1972;105:97–105.

    Article  CAS  Google Scholar 

  24. Steadland K, Langham M, Greene M, Bagwell C, Kays D, Talbert J . Unilateral pulmonary agenesis, esophageal atresia, and distal tracheoesophageal fistula. Ann Thorac Surg 1995;59:511–513.

    Article  CAS  Google Scholar 

  25. Brenner V . Unilateral pulmonary hypoplasia/agenesis in the neonate: a case report. Neonat Netw 1987;12:49–57.

    Google Scholar 

  26. Booth J, Berry C . Unilateral pulmonary agenesis. Arch Dis Child 1967;42:361–374.

    Article  CAS  Google Scholar 

  27. Sherer D, Davis J, Woods JR Jr . Pulmonary hypoplasia: a review. Obstet Gynecol Surv 1990;45:792–803.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Abrams, M., Ackerman, V. & Engle, W. Primary Unilateral Pulmonary Hypoplasia: Neonate through Early Childhood — Case Report, Radiographic Diagnosis and Review of the Literature. J Perinatol 24, 667–670 (2004). https://doi.org/10.1038/sj.jp.7211156

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.jp.7211156

This article is cited by

Search

Quick links