Cystic adenomatoid malformation of fetus

Authors: K. Bihariová 1;  D. Kobidová 1;  T. Bielik 2;  P. Molitoris 1
Authors‘ workplace: Gynekologicko-pôrodnícke oddelenie, NsP Zvolen, a. s., primár MUDr. P. Molitoris 1;  Gynekologicko-pôrodnícka klinika, Fakultná nemocnica s poliklinikou F. D. Roosevelta, Banská Bystrica, prednosta doc. MUDr. T. Bielik, PhD. 2
Published in: Ceska Gynekol 2017; 82(6): 474-477


Presentation of prenataly diagnosed fetus with congenital cystic adenomatoid malformation (CCAM). Summary of clinical and histological findings in fetuses with CCAM, in utero ultrasound diagnosis, prognosis, in utero dispensarisation, timing of delivery and postanatal management.

Case report.

Nemocnica s poliklinikou, Zvolen, a.s., gynekologicko-pôrodnícke oddelenie.

Case report:
In this article we would like to introduce the case report of fetus suffering from CCAM.

Congenital cystic adenomatoid malformation is a rare congenital disorder. The clinical and histological findings can vary. The diagnose can be made in prenatal period due to the availability of prenatal ultrasound examination.

congenital cystic adenomatoid malformation of lung, prenatal ultrasound diagnosis, polyhydramnion


1. Adzick, NS., Harrison, MR., Crombleholme, TM., et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol, 1998, 179, p. 884–889.

2. Adzick, NS., Harrison, MR., Glick, PL., et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg, 1985, 20, p. 483–488.

3. Bunduki, V., Ruano, R., Marques DaSilva, M., et al. Prognostic factors associated with cystic adenomatoid malformation of the lung. Prenat. Diagn, 2000, 20, p. 459–464.

4. Cangiarella, J., Greco, MA., Askin, F., et al. Congenital cystic adenomatoid malformation of the lung: insights into the pathogenesis utilising quantitative analysis of vascular marker CD34 (QBEND-10) and cell proliferation marker MIB-1. Mod Pathol, 1995, 8, p. 913–918.

5. Cass, DL., Cromleholme, TM., Howell, LJ., et al. Cystic lung lesions with systemic arterial blood suply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg, 1997, 32, p. 986–990.

6. Cass, DL., Olutoye, OO., Cassady, CI., et al. Prenatal diagnosis and outcome of fetal lung masses. J Pediatr Surg, 2011, 46, p. 292–298.

7. Sfakianaki, AK., Copel, JA. Congenital cystic lessions of the lung: congenital cystic adenomatoid malformation and bronchopulmonary sequestration. Rev Obstet Gynecol, 2012, 5(2), p. 85–93.

8. Gilbert-Barness, E. The respiratory system. In: Gilbert-Barness, E., ed. Potter´s pathology of the fetus and infant. St. Louis: Mosby-Year book, 1997, p. 712.

9. Gornall, AS., Budd, JL., Draper, ES., et al. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenat Diagn, 2003, 23, p. 997–1002.

10. Granata, C., Gambini, C., Balducci, T., et al. Bronchio­loalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformation. Pediatr Pulmonol, 1998, 25, p. 62–66.

11. Hubbard, AM., Adzick, NS., Crombleholme, TM., et al. Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology, 1999, 212, p. 43–48.

12. Laberge, JM., Flageole, H., Pugash, D., et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther, 2001, 16, p. 178–186.

13. Laje, P., Liechty, KW. Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn, 2008, 28, p. 612–618.

14. Lima, M., Gargano, T., Ruggeri, G., et al. Clinical spectrum and management of congenital pulmonary cystic lesions. Pediatr Med Chir, 2008, 30(2), p. 79–88.

15. Mandell, G. Imaging in congenital cystic adenomatoid malformation.

16. Matsuoka, S., Takeuchi, K., Yamanaka, Y., et al. Comparison of magnetic resonance imaging and ultrasonography in the prenatal diagnosis of congenital thoracic abnormalities. Fetal Diagn Ther, 2003, 18, p. 447–453.

17. Mehta, AA., Viswanathan, N., Vasudevan, AK., et al. Congenital cystic adenomatoid malformation: a tertiary care hospital experience. J Clin Diagn Res, 2016, 10, p. SCO01-SC04.

18. Molinaro, F., Angotti, R., Garzi, A., et al. Prenatal diagnosis, 3D virtual rendering and lung sparing surgery by ligasure device in a baby with „CCAM and intralobar sequestration“. Open Med (Wars), 2016, 11(1), p. 200–203.

19. Murphy, JJ., Blair, GK., Fraser, GC., et al. Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases. J Pediatr Surg, 1992, 27(10), p. 1364–1367.

20. Priest, JR., Williams, GM., Hill, DA., et al. Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol, 2009, 44, p. 14–30.

21. Prima, FAFD., Bellia, A., Inclimona, G., et al. Antenatally diagnosed congenital cystic adenomatoid malformations (CCAM): research review. J Prenat Med, 2012, 6(2), p. 22–30.

22. Sauvat, F., Michel, JL., Benachi, A., et al. Management of asymptomatic neonatal cystic adenomatoid malformation. J Pediatr Surg, 2003, 38(4), p. 548–552.

23. Stanton, M., Njere, I., Ade-Ajayi, N., et al. Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions. J Pediatr Surg, 2009, 44, p. 1027–1033.

24. Stocker, JT. Cystic lung disease in infants and children. Fetal Pediatr Pathol, 2009, 28, p. 155–184.

25. Stocker, JT., Madewell, JE., Drake, RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol, 1977, 8, p. 155–171.

26. Stone, AE. Cystic adenomatoid malformation.

27. Tsao, K., Hawgood, S., Vu, L., et al. Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy. J Pediatr Surg, 2003, 38, 508–510.

28. Witlox, RS., Lopriore, E., Oepkes, D., et al. Neonatal outcome after prenatal interventions for congenital lung lesions. Early Hum Dev, 2011, 87, p. 611–618.

Paediatric gynaecology Gynaecology and obstetrics Reproduction medicine

Article was published in

Czech Gynaecology

Issue 6

2017 Issue 6

Most read in this issue
Forgotten password

Don‘t have an account?  Create new account

Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account