CPAM, congenital pulmonary airway malformation

Czech version

Authors: K. Tichá ¹; B. Kučerová ²; K. Křížová ²; B. Prosová ³; R. Kelčík ⁴; J. Košata ⁴; J. Janota ¹
Authors‘ workplace: Novorozenecké oddělení s JIRP, Gynekologicko-porodnická klinika 2. LF UK a FN Motol, Praha ¹; Klinika dětské chirurgie 2. LF UK a FN Motol, Praha ²; Klinika zobrazovacích metod 2. LF UK a FN Motol, Praha ³; Gynekologické oddělení s JIRP, Gynekologicko-porodnická klinika 2. LF UK a FN Motol, Praha ⁴
Published in: Čes-slov Neonat 2024; 30 (1): 31-36.
Category: Reviews

Overview

Congenital pulmonary airway malformation is one of the most common congenital lung malformations. Prenatal ultrasound screening plays an important role in its diagnosis. Therefore, in most cases, the congenital developmental defect is already known before birth. The clinical manifestations of the defect are variable and can appear at any age during childhood. The variability of clinical signs depends on the size of the deposit. With the improvement of ultrasound technology even the smallest congenital pulmonary airway malformations, which are completely asymptomatic, are often detected. Magnetic resonance imaging of the fetus is suitable to clarify the ultrasound finding, which will help us in more accurate differential diagnosis. Definitive treatment of congenital pulmonary airway malformation is surgical resection of the lesion. The surgery is planned according to the child's clinical manifestations. If a child is born with respiratory distress or breathing problems appear soon after birth, surgery is indicated immediately. However, in the neonatal period, most of the findings are asymptomatic and surgery can be postponed. In this case the child must be carefully and regularly examined by the general practitioner and the surgeon. Around 4−5 years of age, a CT scan of the lungs is indicated and, according to the findings, an operation is arranged. Surgery is highly recommended for the possible development of frequent respiratory infections or the development of a malignant disease in later life. With careful monitoring and resection of the lesion the overall prognosis of the congenital malformation is favorable. The article is presenting data regarding approaches and treatment of patients from the Department of neonatology and the Department of pediatric surgery, Motol University Hospital.

Keywords:

Surgical resection – congenital pulmonary airway malformation – CPAM – fetal magnetic resonance – prenatal ultrasound screening – respiratory distress

Sources

Draganovičová J, Hyršl M, Jiroušek M, Hanke I, Hornychová H, Kočová E. Kongenitální plicní malformace (CPAM typ I), diagnóza dětského věku u dospělé pacientky. Čes. Radiol. 2019; 73(4): 267−270. Dostupné z: http://www.cesradiol.cz/dwnld/ CesRad_1904_267_270.pdf.
Egloff A, Bulas D, et al. Congenital pulmonary airway malformation prenatal diagnosis and managment. Up to date 2023. Dostupné z: https://www.uptodate.com/contents/con- genital-pulmonary-airway-malformation-prenatal-diagno sis-and-management?csi=1b27a355-9351-49fc-a3d3-23d4dd7 953fe&source=contentShare.
Gleason ChA, Juul SE. Disorders of the pleural cavity. In: Avery‘s diseases of the newborn, 10th ed. Philadelphia: Elsevier 2018: 708−712. ISBN 978-0-323-40135-5.
Gomella TL. Neonatology: Management, procedures, on-call problems, diseases, and drugs, 8th ed. New York: McGraw-Hill Medical 2020: 188−189, 679. ISBN 978-1-259-64481-8.
Hořínová V, Drábová K, Nosková H, Bajčiová V, Šoukalová J, Černá L, Hůrková V, Slabý O, Štěrba J. Syndrom DICER1. Klin. Onkol. 2019; 32(Suppl 2): 2S123−2S127. Dostupné z: https://www. prolekare.cz/casopisy/klinicka-onkologie/2019-supplementum2/ syndrom-dicer1-113315.
Aryal K, et al. Congenital pulmonary airway malformation (CPAM): A case report and review of the literature. Radiol Case Rep 2023; 18(10): 3483−3486. Dostupné z: https://www.sciencedirect. com/science/article/pii/S1930043323004594.
Mehta PA, Sharma G. Congenital pulmonary airway malformation [online]. Treasure Island: StatPearls 2023. Dostupné z: https:// www.ncbi.nlm.nih.gov/books/NBK551664/.
Mihál V, Kopřiva F, Vyhnánek M, Šnajdauf J, Michálková K, Venháčová P. Oslabené dýchání a kašel jako první příznaky kongenitální malformace dýchacích cest. Pediatr. Praxi. 2012; 13(6): 415–417.
Oerman ChM, et al. Cognital Pumonary airway malformation. Up to date 2023. Dostupné z: https://www.uptodate.com/contents/ congenital-pulmonary-airway-malformation?csi=91e7b53f-761 9-4758-a2ae-d074f71403e6&source=contentShare.

Konflikt zájmu: žádný Došlo do redakce: 5. 3. 2024

Adresa pro korespondenci:
MUDr. Katarina Tichá
Novorozenecké oddělení s JIRP
Gynekologicko-porodnická klinika 2. LF UK a FN Motol
V Úvalu 84
150 06 Praha 5
e-mail: katarina.ticha@fnmotol.cz