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Cystic adenomatous lung malformation of type II in the newborn, problems of early diagnosis

https://doi.org/10.52420/2071-5943-2022-21-1-77-84

Abstract

Introduction. Cystic adenomatous malformation (CAM) is a congenital lung malformation resulting from abnormal morphogenesis of lung branching at different stages and in different parts of the tracheobronchial tree, with formation of cysts. The incidence of CAM is one case per 8,300-35,000 live births. The modern classification distinguishes four types of CAM. The aim of the work was to analyze the features of the course of the disease and radiological signs that can help the doctor to suspect the presence of congenital lung malformations in   the newborn in the early stages of  manifestation,  justify  additional diagnostic  methods, and timely  choose an appropriate treatment tactic. Material and methods. The article presents the clinical observation of a newborn infant with type II CAM and concomitant disease «early neonatal sepsis». Medical documents are used, where the results of clinical examination, instrumental (X-ray, ultrasound, CT) and laboratory methods  of investigation are presented. Data of histological examination of biomaterial obtained intraoperatively are presented. Results. An analysis of the clinical and radiological dynamics is given. Based on the examination, congenital malformations were detected, and surgical treatment was performed. The child was discharged from the hospital on the 35th day of life in a satisfactory condition. A detailed analysis of diagnostic limitations at each stage of the child's life was conducted. Discussion. Diagnosis of CAM in the early stages was difficult not only because of the nature of the malformation, but also because of the clinic of sepsis, which was the leading one in the first days of life. The morphology of the malformation leads to problems with its prenatal diagnosis and the lack of a clear radiological and ultrasound picture in the postnatal period. Conclusion. In any case of recurrent air leak syndrome, differential diagnosis of congenital pulmonary malformations and the use of CT or MRI for accurate imaging seems reasonable.

About the Author

E. V. Shestak
Ekaterinburg Clinical Perinatal Center; Ural State Medical University
Russian Federation

Evgenii V. Shestak – physician anesthesiologist-resuscitator, assistant of the department.

Ekaterinburg.



References

1. Wilson RD, Hedrick HL, Liechty KW, Flake AW, Johnson MP, Bebbington M, Adzick NS. Cystic adenomatoid malformation of the lung: review of genetics, prenatal diagnosis, and in utero treatment. Am J Med Genet A. 2006;140(2):151–5.

2. Baird R, Puligandla PS, Laberge JM. Congenital lung malformations: informing best practice. Semin Pediatr Surg. 2014 Oct; 23(5):270-7. doi: 10.1053/j.sempedsurg.2014.09.007. Epub 2014 Sep 4. PMID: 25459011.

3. Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg. 2008 Feb;17(1):2-8. doi: 10.1053/j.sempedsurg.2007.10.002. PMID: 18158136.

4. Laberge JM, Flageole H, Pugash D, Khalife S, Blair G, Filiatrault D, Russo P, Lees G, Wilson RD. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther. 2001 May-Jun;16(3):178-86. doi: 10.1159/000053905. PMID: 11316935.

5. Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A. Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol. 2009 Jan;44(1):14-30. doi: 10.1002/ppul.20917. PMID: 19061226.

6. Usui N, Kamata S, Sawai T, Kamiyama M, Okuyama H, Kubota A, Okada A. Outcome predictors for infants with cystic lung disease. J Pediatr Surg. 2004 Apr;39(4):603-6. doi: 10.1016/j.jpedsurg.2003.12.001. PMID: 15065037.

7. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998 Oct;179(4):884-9. doi: 10.1016/s0002-9378(98)70183-8. PMID: 9790364.

8. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol. 1977 Mar;8(2):155-71. doi: 10.1016/s0046-8177(77)80078-6. PMID: 856714.

9. Stocker JT. Congenital pulmonary airway malformation—anewname for and an expanded classification of congenital cysticadenomatoid malformation of the lung. Histopathology 2002;41:424–431.

10. van Koningsbruggen S, Ahrens F, Brockmann M, Michalk D, Rietschel E. Congenital cystic adenomatoid malformation type 4. Pediatr Pulmonol. 2001 Dec;32(6):471-5. doi: 10.1002/ppul.1160. PMID: 11747251.

11. Под ред. Ю.Ф. Исакова. Неонатальная хирургия. 1-е изд. М: « Династия» 2011, 311-332

12. Ruchonnet-Metrailler I, Leroy-Terquem E, Stirnemann J, Cros P, Ducoin H, Hadchouel A, Khen-Dunlop N, Labbé A, Labouret G, Lebras MN, Lezmi G, Madhi F, Salomon LJ, Thouvenin G, Thumerelle C, Delacourt C. Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations. Pediatrics. 2014 May;133(5):e1285-91. doi: 10.1542/peds.2013-2986. PMID: 24777224.

13. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998 Oct;179(4):884-9. doi: 10.1016/s0002-9378(98)70183-8. PMID: 9790364.

14. Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, Callen PW, Hirsch JH, Luthy DA, Filly RA, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg. 1985 Oct;20(5):483-8. doi: 10.1016/s0022-3468(85)80470-x. PMID: 3903097.

15. Taguchi T, Suita S, Yamanouchi T, Nagano M, Satoh S, Koyanagi T, Nakano H. Antenatal diagnosis and surgical management of congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther. 1995 Nov-Dec;10(6):400-7. doi: 10.1159/000264265. PMID: 8579779.

16. Baird R, Puligandla PS, Laberge JM. Congenital lung malformations: informing best practice. Semin Pediatr Surg. 2014 Oct;23(5):270-7. doi: 10.1053/j.sempedsurg.2014.09.007. Epub 2014 Sep 4. PMID: 25459011.

17. Crombleholme TM, Coleman B, Hedrick H, Liechty K, Howell L, Flake AW, Johnson M, Adzick NS. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg. 2002 Mar;37(3):331-8. doi: 10.1053/jpsu.2002.30832. PMID: 11877643.

18. Parikh DH, Rasiah SV. Congenital lung lesions: Postnatal management and outcome. Semin Pediatr Surg. 2015 Aug;24(4):160-7. doi: 10.1053/j.sempedsurg.2015.01.013. Epub 2015 Feb 3. PMID: 26051048.

19. Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y. Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg. 2003 Apr;38(4):548-52. doi: 10.1053/jpsu.2003.50119. PMID: 12677563.

20. Aziz D, Langer JC, Tuuha SE, Ryan G, Ein SH, Kim PC. Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not? J Pediatr Surg. 2004 Mar;39(3):329-34; discussion 329-34. doi: 10.1016/j.jpedsurg.2003.11.021. PMID: 15017547.

21. Priest JR, Watterson J, Strong L, Huff V, Woods WG, Byrd RL, Friend SH, Newsham I, Amylon MD, Pappo A, Mahoney DH, Langston C, Heyn R, Kohut G, Freyer DR, Bostrom B, Richardson MS, Barredo J, Dehner LP. Pleuropulmonary blastoma: a marker for familial disease. J Pediatr 1996;128:220–224

22. Sauvat F, Michel JL, Benachi A, Emond S, Revillon Y. Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg. 2003 Apr;38(4):548-52. doi: 10.1053/jpsu.2003.50119. PMID: 12677563.

23. Quercia M, Panza R, Calderoni G, Di Mauro A, Laforgia N. Lung Ultrasound: A New Tool in the Management of Congenital Lung Malformation. Am J Perinatol. 2019 Jul;36(S 02):S99-S105. doi: 10.1055/s-0039-1692131. Epub 2019 Jun 25. PMID: 31238368.

24. Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BK, Han MC. Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. AJR Am J Roentgenol. 1997 Jan;168(1):47-53. doi: 10.2214/ajr.168.1.8976918. PMID: 8976918.

25. Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BK, Han MC. Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. AJR Am J Roentgenol. 1997 Jan;168(1):47-53. doi: 10.2214/ajr.168.1.8976918. PMID: 8976918.

26. Singh R, Davenport M. The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 2015 Aug;24(4):187-95. doi: 10.1053/j.sempedsurg.2015.02.003. Epub 2015 Feb 27. PMID: 26051052.

27. Stanton M. The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 2015 Aug;24(4):183-6. doi: 10.1053/j.sempedsurg.2015.01.014. Epub 2015 Feb 3. PMID: 26051051.

28. Baird R, Puligandla PS, Laberge JM. Congenital lung malformations: informing best practice. Semin Pediatr Surg. 2014 Oct;23(5):270-7. doi: 10.1053/j.sempedsurg.2014.09.007. Epub 2014 Sep 4. PMID: 25459011.

29. Jelin EB, O'Hare EM, Jancelewicz T, Nasr I, Boss E, Rhee DS. Optimal timing for elective resection of asymptomatic congenital pulmonary airway malformations. J Pediatr Surg. 2018 May;53(5):1001-1005. doi: 10.1016/j.jpedsurg.2018.02.032. Epub 2018 Feb 10. PMID: 29514740.

30. Muller CO, Berrebi D, Kheniche A, Bonnard A. Is radical lobectomy required in congenital cystic adenomatoid malformation? J Pediatr Surg. 2012 Apr;47(4):642-5. doi: 10.1016/j.jpedsurg.2011.08.002. PMID: 22498375.


Review

For citations:


Shestak EV. Cystic adenomatous lung malformation of type II in the newborn, problems of early diagnosis. Ural Medical Journal. 2022;21(1):77-84. (In Russ.) https://doi.org/10.52420/2071-5943-2022-21-1-77-84

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