DOI QR코드

DOI QR Code

Evaluation of Newborn Infants with Prenatally Diagnosed Congenital Pulmonary Airway Malformation: A Single-Center Experience

  • Lim, Joohee (Division of Neonatology, Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Han, Jung Ho (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Shin, Jeong Eun (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Eun, Ho Sun (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Lee, Soon Min (Division of Neonatology, Department of Pediatrics, Gangnam Severance Hospital, Yonsei University College of Medicine) ;
  • Park, Min Soo (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Namgung, Ran (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine) ;
  • Park, Kook In (Division of Neonatology, Department of Pediatrics, Severance Children's Hospital, Yonsei University College of Medicine)
  • 투고 : 2019.04.16
  • 심사 : 2019.07.02
  • 발행 : 2019.08.31

초록

Purpose: Congenital pulmonary airway malformation (CPAM)-a rare developmental anomaly-affects the lower respiratory tract in newborns. By comparing the reliability of diagnostic tools and identifying predictive factors for symptoms, we provide comprehensive clinical data for the proper management of CPAM. Methods: We reviewed the medical records of 66 patients with prenatally diagnosed CPAM delivered at Severance Children's Hospital between January 2005 and July 2017. Results: We enrolled 33 boys and 33 girls. Their mean gestational age and birth weight were 38.8 weeks and 3,050 g, respectively. Prenatal ultrasonography and postnatal radiography, lung ultrasonography, and chest computed tomography (CT) showed inconsistent findings. Chest CT showed superior sensitivity (100%) and positive predictive value (90%). Among the 66 patients, 59 had postnatally confirmed CPAM, three had pulmonary sequestration, one had cystic teratoma, and one had a normal lung. Of the 59 patients with CPAM, 21 (35%; mean age, 23.4 months) underwent surgery, including 15 who underwent video-assisted thoracoscopy. Twenty-five and 12 patients exhibited respiratory symptoms at birth and during infancy, respectively. Apgar scores and mediastinal shift on radiography were significantly associated with respiratory symptoms at birth. However, none of the factors could predict respiratory symptoms during infancy. Conclusion: Radiography or ultrasonography combined with chest CT can confirm an unclear or inconsistent lesion. Apgar scores and mediastinal shift on radiography can predict respiratory symptoms at birth. However, symptoms during infancy are not associated with prenatal and postnatal factors. Chest CT combined with periodic symptom monitoring is important for diagnosing and managing patients with prenatally diagnosed CPAM and to guide appropriate timing of surgery.

키워드

참고문헌

  1. Kulwa E, Tharakan T, Baxi L. Congenital cystic adenomatoid malformation in the fetus: a hypothesis of its development. Fetal Diagn Ther 2005;20:472-4. https://doi.org/10.1159/000086836
  2. Laje P, Liechty KW. Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 2008;28:612-8. https://doi.org/10.1002/pd.1966
  3. Suh YE, Kim HK, Choi YS, Lee BS, Kim KS, Won HS, et al. Neonatal characteristic of congenital cystic adenomatoid malformation of the lung requiring early operation and preoperative intervention. Neonatal Med 2013;20:81-9. https://doi.org/10.5385/nm.2013.20.1.81
  4. Stanton M. The argument for a non-operative approach to asymptomatic lung lesions. Semin Pediatr Surg 2015;24:183-6. https://doi.org/10.1053/j.sempedsurg.2015.01.014
  5. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol 1977;8:155-71. https://doi.org/10.1016/S0046-8177(77)80078-6
  6. Xia B, Yu G, Liu C, Hong C, Tang J. Surgical treatment of congenital cystic adenomatoid malformation: a retrospective study of single tertiary center experience. J Matern Fetal Neonatal Med 2017;30:416-9. https://doi.org/10.1080/14767058.2016.1174988
  7. Leblanc C, Baron M, Desselas E, Phan MH, Rybak A, Thouvenin G, et al. Congenital pulmonary airway malformations: state-of-the-art review for pediatrician's use. Eur J Pediatr 2017;176:1559-71. https://doi.org/10.1007/s00431-017-3032-7
  8. Turan O, Hirfanoglu IM, Beken S, Biri A, Efeturk T, Atalay Y. Prenatally detected congenital cystic adenomatoid malformation and postnatally diagnosed trisomy 13: case report and review of the literature. Turk J Pediatr 2011;53:337-41.
  9. Kitaichi M, Yousem S. Symposium 24: non-neoplastic lung disease. Histopathology 2002;41(Suppl 2):424-58. https://doi.org/10.1046/j.1365-2559.41.s2.58.x
  10. Thakkar HS, Durell J, Chakraborty S, Tingle BL, Choi A, Fowler DJ, et al. Antenatally detected congenital pulmonary airway malformations: the Oxford experience. Eur J Pediatr Surg 2017; 27:324-9. https://doi.org/10.1055/s-0036-1593379
  11. Shanmugam G, MacArthur K, Pollock JC. Congenital lung malformations: antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 2005;27:45-52. https://doi.org/10.1016/j.ejcts.2004.10.015
  12. Hardee S, Tuzovic L, Silva CT, Cowles RA, Copel J, Morotti RA. Congenital cystic lung lesions: evolution from in-utero detection to pathology diagnosis. A multidisciplinary approach. Pediatr Dev Pathol 2017;20:403-10. https://doi.org/10.1177/1093526617698604
  13. Pacharn P, Kline-Fath B, Calvo-Garcia M, Linam LE, Rubio EI, Salisbury S, et al. Congenital lung lesions: prenatal MRI and postnatal findings. Pediatr Radiol 2013;43:1136-43. https://doi.org/10.1007/s00247-013-2668-3
  14. Ruchonnet-Metrailler I, Leroy-Terquem E, Stirnemann J, Cros P, Ducoin H, Hadchouel A, et al. Neonatal outcomes of prenatally diagnosed congenital pulmonary malformations. Pediatrics 2014;133:e1285-91. https://doi.org/10.1542/peds.2013-2986
  15. Gallardo AM, Alvarez de la Rosa RM, De Luis EJF, Mendoza RL, Padilla PAI, Troyano LJ. Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung. Rev Chil Pediatr 2018;89:224-230. https://doi.org/10.4067/S0370-41062018000200224
  16. Shamas AG, Bohara K. Congenital cystic adenomatoid malformation of the lung (CCAM), a retrospective clinical audit and literature review in a tertiary centre in Scotland over a period of 14 years. J Obstet Gynaecol 2017;37:19-24. https://doi.org/10.1080/01443615.2016.1196480
  17. Crombleholme TM, Coleman B, Hedrick H, Liechty K, Howell L, Flake AW, et al. Cystic adenomatoid malformation volume ratio predicts outcome in prenatally diagnosed cystic adenomatoid malformation of the lung. J Pediatr Surg 2002;37:331-8. https://doi.org/10.1053/jpsu.2002.30832
  18. Shirota C, Tainaka T, Nakane T, Tanaka Y, Hinoki A, Sumida W, et al. Usefulness of fetal magnetic resonance imaging for postnatal management of congenital lung cysts: prediction of probability for emergency surgery. BMC Pediatr 2018;18:105. https://doi.org/10.1186/s12887-018-1085-z
  19. Kantor N, Wayne C, Nasr A. Symptom development in originally asymptomatic CPAM diagnosed prenatally: a systematic review. Pediatr Surg Int 2018;34:613-20. https://doi.org/10.1007/s00383-018-4264-y
  20. Morini F, Zani A, Conforti A, van Heurn E, Eaton S, Puri P, et al. Erratum to: current management of congenital pulmonary airway malformations: a "European Pediatric Surgeons' Association" Survey. Eur J Pediatr Surg 2018;28:e1.
  21. Derderian SC, Coleman AM, Jeanty C, Lim FY, Shaaban AM, Farrell JA, et al. Favorable outcomes in high-risk congenital pulmonary airway malformations treated with multiple courses of maternal betamethasone. J Pediatr Surg 2015;50:515-8. https://doi.org/10.1016/j.jpedsurg.2014.08.019
  22. Kapralik J, Wayne C, Chan E, Nasr A. Surgical versus conservative management of congenital pulmonary airway malformation in children: a systematic review and meta-analysis. J Pediatr Surg 2016;51:508-12. https://doi.org/10.1016/j.jpedsurg.2015.11.022
  23. Polites SF, Habermann EB, Zarroug AE, Thomsen KM, Potter DD. Thoracoscopic vs open resection of congenital cystic lung disease: utilization and outcomes in 1120 children in the United States. J Pediatr Surg 2016;51:1101-5. https://doi.org/10.1016/j.jpedsurg.2015.12.004
  24. Rothenberg SS, Middlesworth W, Kadennhe-Chiweshe A, Aspelund G, Kuenzler K, Cowles R, et al. Two decades of experience with thoracoscopic lobectomy in infants and children: standardizing techniques for advanced thoracoscopic surgery. J Laparoendosc Adv Surg Tech A 2015;25:423-8. https://doi.org/10.1089/lap.2014.0350
  25. Ghosh M, Islam N, Ghosh A, Chaudhuri PM, Saha K, Chatterjee U. Pleuropulmonary blastoma developing in a case of misinterpreted congenital pulmonary airway malformation: a case report. Fetal Pediatr Pathol 2018;37:377-86. https://doi.org/10.1080/15513815.2018.1520943
  26. Delacourt C, Hadchouel A, Khen Dunlop N. Shall all congenital cystic lung malformations be removed? the case in favour. Paediatr Respir Rev 2013;14:169-70. https://doi.org/10.1016/j.prrv.2013.06.003

피인용 문헌

  1. Clinical characteristics of congenital pulmonary airway malformation of the lungs: A single-center study vol.9, pp.1, 2019, https://doi.org/10.4168/aard.2021.9.1.21