Pediatric Radiology

, Volume 35, Issue 1, pp 79–84 | Cite as

Congenital cystic adenomatoid malformation: is there a difference between the antenatally and postnatally diagnosed cases?

  • Mohamed I. Tawil
  • David W. PillingEmail author
Original Article


Background: The majority of congenital cystic adenomatoid malformation (CCAM) lesions are diagnosed antenatally. A few cases however may not be recognised antenatally and present in infancy or later childhood with chest symptoms, including chest infection. Objective: To review the clinical and radiological spectrum of CCAM, comparing the antenatally with the postnatally diagnosed cases. Materials and methods: Fifteen cases of antenatally and/or postnatally diagnosed and histopathologically proven CCAM were retrospectively identified over a period of 4 years. Clinical notes, chest radiograph and chest CT were reviewed in all cases. Results: Nine patients were diagnosed antenatally and six postnatally. All antenatally diagnosed patients were asymptomatic at birth, six remained asymptomatic until they had elective surgery and the remaining three developed symptoms before the age of 2 years. In the postnatally diagnosed group, one patient was symptomatic at birth and one patient presented at 16 years; the remaining four presented before the age of 2 years. Depending on the type of lesion, we recognised five radiographic patterns of CCAM. CCAM lesions were classified as CT Stocker type I in seven cases, type II in seven cases and type III in one case. Conclusions: No significant difference was found between the two groups. Recognition of these lesions antenatally would benefit patients by avoiding delay in making the diagnosis, which can lead to serious complications. CT was successful in accurately diagnosing and grading CCAM lesions.


Lung Congenital Cyst Congenital cystic adenomatoid malformation Radiography CT Children Fetus 


  1. 1.
    Stocker JT, Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung: classification and morphological spectrum. Hum Pathol 8:155–171PubMedGoogle Scholar
  2. 2.
    Rosado-de-christenson ML, Stocker JT (1991) Congenital cystic adenomatoid malformation. Radiographics 11:865–886PubMedGoogle Scholar
  3. 3.
    Miller RK, Sieber WK, Yunis EJ (1980) Congenital adenomatoid malformation of the lung: a report of 17 cases and review of the literature. Pathol Annu 15:387–402PubMedGoogle Scholar
  4. 4.
    Wolf SA, Hertzler JH, Philippart AI (1980) Cystic adenomatoid dysplasia of the lung. J Pediatr Surg 15:925–930PubMedGoogle Scholar
  5. 5.
    Miller JA, Corteville JE, Langer JC (1996) Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome. J Pediatr Surg 31:805–808CrossRefPubMedGoogle Scholar
  6. 6.
    Barnes NA, Pilling DW (2003) Bronchopulmonary foregut malformations: embryology, radiology and quandary. Eur Radiol 13:2659–2673CrossRefPubMedGoogle Scholar
  7. 7.
    Leeuwen KV, Tietelbaum DH, Hirschl RB, et al (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indication and natural history. J Pediatr Surg 34:794–799CrossRefPubMedGoogle Scholar
  8. 8.
    Takeda S, Miyoshi S, Inoue M, et al (1999) Clinical spectrum of congenital cystic disease of the lung in children. Eur J Cardiothorac Surg 15:11–17CrossRefPubMedGoogle Scholar
  9. 9.
    Kim WS, Lee KS, Kim IO, et al (1997) Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. AJR 168:47–53PubMedGoogle Scholar
  10. 10.
    Patz EF Jr, Muller NL, Swensen SJ, et al (1995) Congenital cystic adenomatoid malformation in adults: CT findings. J Comput Assist Tomogr 19:361–364PubMedGoogle Scholar
  11. 11.
    Bentur L, Canny G, Thorner P, et al (1991) Spontaneous pneumothorax in cystic adenomatoid malformation. Unusual clinical and histologic features. Chest 99:1292–1293PubMedGoogle Scholar
  12. 12.
    Lacy DE, Shaw NJ, Pilling DW, et al (1999) Outcome of congenital lung abnormalities detected antenatally. Acta Paediatr 88:454–458CrossRefPubMedGoogle Scholar
  13. 13.
    Barret J, Chitayat D, Sermer M, et al (1995) The prognostic factors in the prenatal diagnosis of the echogenic fetal lung. Prenat Diagn 15:849–853PubMedGoogle Scholar
  14. 14.
    Adzick NS, Harrison MR, Glick PL, et al (1985) Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 20:483–488PubMedGoogle Scholar
  15. 15.
    Donnelly LF, Frush DP (1998) Localized radiolucent chest lesions in neonates: causes and differentiation. AJR 172:1651–1658Google Scholar
  16. 16.
    Winters WD, Effmann EL, Nghiem HV, et al (1997) Disappearing fetal lung masses: importance of postnatal imaging studies. Pediatr Radiol 27:535–539CrossRefPubMedGoogle Scholar
  17. 17.
    MacGillivray TE, Harrison MR, Goldstein RB, et al (1993) Disappearing fetal lung lesions. J Pediatr Surg 28:1321–1324PubMedGoogle Scholar
  18. 18.
    Bromley B, Parad R, Estroff JA, et al (1995) Fetal lung masses: prenatal course and outcome. J Ultrasound Med 14:927–936PubMedGoogle Scholar
  19. 19.
    Wexler HA, Dapena MV (1978) Congenital cystic adenomatoid malformation. A report of three unusual cases. Radiology 126:737–741PubMedGoogle Scholar
  20. 20.
    Schwartz MZ, Ramachandran P (1997) Congenital malformations of the lung and mediastinum—a quarter century of experience from a single institution. J Pediatr Surg 32:44–47CrossRefPubMedGoogle Scholar
  21. 21.
    Mata JM, Caceres J, Lucaya J, et al (1990) CT of congenital malformations of the lung. Radiographics 10:651–674PubMedGoogle Scholar
  22. 22.
    Mata JM, Castellote A (2002) Pulmonary malformations beyond the neonatal period. In: Lucaya J, Strife J (eds) Paediatric chest imaging. Springer, Berlin Heidelberg New York, pp 93–111Google Scholar
  23. 23.
    Hubbard AM, Adzick NS, Crombleholme TM, et al (1999) Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology 212:43–48PubMedGoogle Scholar
  24. 24.
    Matsuoka S, Takeuchi K, Yamanaka Y, et al (2003) Comparison of magnetic resonance imaging and ultrasonography in the prenatal diagnosis of congenital thoracic abnormalities. Fetal Diagn Ther 18:447–453CrossRefPubMedGoogle Scholar
  25. 25.
    Breysem L, Bosmans H, Dymarkowski S, et al (2003) The value of fast MR imaging as an adjunct to ultrasound in prenatal diagnosis. Eur Radiol 13:1538–1548CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  1. 1.Department of RadiologyRoyal Liverpool Children’s Hospital Alder HeyWest DerbyUK

Personalised recommendations