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A REVIEW OF POSTNATAL MANAGEMENT OF CONGENITAL PULMONARY AIRWAY MALFORMATIONS

Part of: Bespoke shallow archive Weizmann

Published online by Cambridge University Press:  01 August 2009

SANDRA CHUANG ,
ELLA SUGO  and
ADAM JAFFE
SANDRA CHUANG*
Affiliation:
Department of Respiratory Medicine, Sydney Children's Hospital, Randwick, Sydney, Australia.
ELLA SUGO
Affiliation:
Department of Anatomical Pathology, Prince of Wales Hospital, Randwick, Sydney, Australia.
ADAM JAFFE
Affiliation:
School of Women's and Children's Health, University of New South Wales, Sydney, Australia.
*
Sandra Chuang, Respiratory Laboratory, Level 0, Sydney Children's Hospital, High Street, Randwick, NSW, Australia.
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Extract

Congenital cystic adenomatoid malformations of the lung (CCAMs) were originally described by Ch'In and Tang in 1949 as rare lung lesions occurring in premature or stillborn infants with anasarca. In 1977 Stocker et al divided CCAM into three types based on their clinical and pathological features. The nomenclature has since changed to congenital pulmonary airway malformations (CPAMs) to reflect the possible absence of cystic changes and the probable underlying pathogenesis of different types. CPAMs are relatively rare congenital abnormalities with a reported incidence varying from 1 in 10,000 to 1 in 35,000. There is a slight male predominance, but no racial predilection has been noted. This review will outline the current nomenclature and features of different types of CPAMs, highlight possible mechanisms underlying the development of CPAMs, review current antenatal imaging modalities and interventions, address the debate surrounding the postnatal management of CPAMs, and suggest areas for future research.

Type
Research Article
Information
Fetal and Maternal Medicine Review , Volume 20 , Issue 3 , August 2009 , pp. 179 - 204
Copyright
Copyright © Cambridge University Press 2009

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References

REFERENCES

1Ch'In, KY, Tang, MY. Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 1949; 48: 221–29.Google ScholarPubMed
2Stocker, JT, Madewell, JE, Drake, RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphologic spectrum. Hum Pathol 1977; 8: 155–71.Google Scholar
3Stocker, J. Congenital and developmental diseases. In: Dail, DH Hammer SP, editors. Pulmonary Pathology. 2nd ed. New York: Springer-Verlag, 1994; 155–90.CrossRefGoogle Scholar
4Stocker, J. Congenital pulmonary airway malformation: a new name and an expanded classification of congenital cystic adenomatoid malformations of the lung. Histopathology 2002; 41 (suppl): 424–31.Google Scholar
5Lakhoo, K. Management of congenital cystic adenomatous malformations of the lung. Arch Dis Child Fetal Neonatal Ed 2009; 94: F73–6.CrossRefGoogle ScholarPubMed
6Duncombe, GJ, Dickinson, JE, Kikiros, CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lung. Am J Obstet Gynecol 2002; 187: 950–54.Google Scholar
7Laberge, JM, Flageole, H, Pugash, D, Khalife, S, Blair, G, Filiatrault, D, et al. Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 2001; 16: 178–86.CrossRefGoogle ScholarPubMed
8Stocker, J. Congenital and developmental diseases. In: Dail, DH Tomashefski JF, Cagle, P, Hammar, SP, Farver, C, Colby, TV, et al. , (eds). Dail and Hammar's Pulmonary Pathology. 3rd ed.New York: Springer-Verlag, 2008; 132–35.Google Scholar
9Priest, JR, Williams, GM, Hill, DA, Dehner, LP, Jaffe, A. Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 2009; 44: 14–30.Google Scholar
10Fitzgerald, D. Congenital cyst adenomatoid malformations: resect some and observe all? Paediatr Respiry Rev 2007; 8: 67–76.CrossRefGoogle Scholar
11Langston, C. New concepts in the pathology of congenital lung malformations. Semin Pediatr Surg 2003; 12: 17–37.CrossRefGoogle ScholarPubMed
12De Felice, C, Di Maggio, G, Messina, M, Toti, P, Bagnoli, F, Bracci, R et al. Congenital cystic adenomatoid malformation of the lung associated with esophageal atresia and tracheoesophageal fistula. Pediatr Surg Int 1999; 15: 260–63.CrossRefGoogle ScholarPubMed
13Krous, HF, Harper, PE, Perlman, M. Congenital cystic adenomatoid malformation in bilateral renal agenesis. Its mitigation of Potter's syndrome. Arch Pathol Lab Med 1980; 104: 368–70.Google ScholarPubMed
14Calvert, JK, Boyd, PA, Chamberlain, PC, Syed, S, Lakhoo, K. Outcome of antenatally suspected congenital cystic adenomatoid malformation of the lung: 10 years' experience 1991–2001. Arch Dis Child Fetal Neonatal Ed 2006; 91: F26–8.CrossRefGoogle ScholarPubMed
15Davenport, M, Warne, SA, Cacciaguerra, S, Patel, S, Greenough, A, Nicolaides, K. Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 2004; 39: 549–56.Google Scholar
16Cass, DL, Crombleholme, TM, Howell, LJ, Stafford, PW, Ruchelli, ED, Adzick, NS. Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 1997; 32: 986–90.CrossRefGoogle ScholarPubMed
17MacKenzie, TC, Guttenberg, ME, Nisenbaum, HL, Johnson, MP, Adzick, NS. A fetal lung lesion consisting of bronchogenic cyst, bronchopulmonary sequestration, and congenital cystic adenomatoid malformation: the missing link? Fetal Diagn Ther 2001; 16: 193–95.CrossRefGoogle ScholarPubMed
18Pumberger, W, Hormann, M, Deutinger, J, Bernaschek, G, Bistricky, E, Horcher, E. Longitudinal observation of antenatally detected congenital lung malformations (CLM): natural history, clinical outcome and long-term follow-up. Eur J Cardiothorac Surg 2003; 24: 703–11.Google Scholar
19Adzick, NS, Harrison, MR, Glick, PL, Golbus, MS, Anderson, RL, Mahony, BS, et al. Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 1985; 20: 483–88.CrossRefGoogle ScholarPubMed
20Calvert, JK, Lakhoo, K. Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. J Pediatr Surg 2007; 42: 411–14.CrossRefGoogle ScholarPubMed
21Laberge, JM, Puligandla, P, Flageole, H. Asymptomatic congenital lung malformations. Semin Pediatr Surg 2005; 14: 16–33.CrossRefGoogle ScholarPubMed
22Adzick, NS, Harrison, MR, Crombleholme, TM, Flake, AW, Howell, LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998; 179: 884–89.Google Scholar
23MacSweeney, F, Papagiannopoulos, K, Goldstraw, P, Sheppard, MN, Corrin, B, Nicholson, AG. An assessment of the expanded classification of congenital cystic adenomatoid malformations and their relationship to malignant transformation. Am J Surg Pathol 2003; 27: 1139–46.CrossRefGoogle ScholarPubMed
24van Koningsbruggen, S, Ahrens, F, Brockmann, M, Michalk, D, Rietschel, E. Congenital cystic adenomatoid malformation type 4. Pediatr Pulmonol 2001; 32: 471–75.CrossRefGoogle ScholarPubMed
25Inselman, LS, Mellins, RB. Growth and development of the lung. J Pediatr 1981; 98: 1–15.CrossRefGoogle ScholarPubMed
26Moore, KL. The Respiratory System. The Developing Human Fourth ed: W. B. Saunders; 1988, 207–17.Google Scholar
27Riedlinger, WF, Vargas, SO, Jennings, RW, Estroff, JA, Barnewolt, CE, Lillehei, CW, et al. Bronchial atresia is common to extralobar sequestration, intralobar sequestration, congenital cystic adenomatoid malformation, and lobar emphysema. Pediatr Dev Pathol 2006; 9: 361–73.CrossRefGoogle ScholarPubMed
28Morotti, RA, Cangiarella, J, Gutierrez, MC, Jagirdar, J, Askin, F, Singh, G, et al. Congenital cystic adenomatoid malformation of the lung (CCAM): evaluation of the cellular components. Hum Pathol 1999; 30: 618–25.CrossRefGoogle ScholarPubMed
29Cass, DL, Quinn, TM, Yang, EY, Liechty, KW, Crombleholme, TM, Flake, AW, et al. Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 1998; 33: 1043–46.CrossRefGoogle ScholarPubMed
30Fromont-Hankard, G, Philippe-Chomette, P, Delezoide, AL, Nessmann, C, Aigrain, Y, Peuchmaur, M. Glial cell-derived neurotrophic factor expression in normal human lung and congenital cystic adenomatoid malformation. Arch Pathol Lab Med 2002; 126: 432–36.Google Scholar
31Volpe, MV, Pham, L, Lessin, M, Ralston, SJ, Bhan, I, Cutz, E, et al. Expression of Hoxb-5 during human lung development and in congenital lung malformations. Birth Defects Res A Clin Mol Teratol 2003; 67: 550–56.CrossRefGoogle ScholarPubMed
32Wagner, AJ, Stumbaugh, A, Tigue, Z, Edmondson, J, Paquet, AC, Farmer, DL, et al. Genetic analysis of congenital cystic adenomatoid malformation reveals a novel pulmonary gene: fatty acid binding protein-7 (brain type). Pediatr Res 2008; 64: 11–6.Google Scholar
33Adzick, NS. Fetal thoracic lesions. Semin Pediatr Surg 1993; 2: 103–108.Google Scholar
34Aurora, P, McHugh, K. Pleural pneumatocoeles mimicking congenital cystic adenomatoid malformation of the lung. A case report. Acta Radiol 1998; 39: 520–22.Google ScholarPubMed
35Kim, WS, Lee, KS, Kim, I, Yeon, KM, Kim, CJ, Chi, JG, et al. Cystic intrapulmonary lymphangioma: HRCT findings. Pediatr Radiol 1995; 25: 206–207.CrossRefGoogle ScholarPubMed
36Rettwitz-Volk, W, Schlosser, R, Ahrens, P, Horlin, A. Congenital unilobar pulmonary lymphangiectasis. Pediatr Pulmonol 1999; 27: 290–92.3.0.CO;2-F>CrossRefGoogle ScholarPubMed
37Illanes, S, Hunger, A, Evans, M, Cusick, E, Soothill, P. Prenatal diagnosis of echogenic lung: evolution and outcome. Ultrasound Obstet Gynecol 2005; 26: 145–49.CrossRefGoogle ScholarPubMed
38Matsuoka, S, Takeuchi, K, Yamanaka, Y, Kaji, Y, Sugimura, K, Maruo, T. Comparison of magnetic resonance imaging and ultrasonography in the prenatal diagnosis of congenital thoracic abnormalities. Fetal Diagn Ther 2003; 18: 447–53.CrossRefGoogle ScholarPubMed
39Shanmugam, G, MacArthur, K, Pollock, JC. Congenital lung malformations–antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg 2005; 27: 45–52.Google Scholar
40Hubbard, AM, Adzick, NS, Crombleholme, TM, Coleman, BG, Howell, LJ, Haselgrove, JC, et al. Congenital chest lesions: diagnosis and characterization with prenatal MR imaging. Radiology 1999; 212: 43–48.CrossRefGoogle ScholarPubMed
41Adzick, NS, Flake, AW, Crombleholme, TM. Management of congenital lung lesions. Semin Pediatr Surg 2003; 12: 10–16.CrossRefGoogle ScholarPubMed
42Bunduki, V, Ruano, R, da Silva, MM, Miguelez, J, Miyadahira, S, Maksoud, JG, et al. Prognostic factors associated with congenital cystic adenomatoid malformation of the lung. Prenat Diagn 2000; 20: 459–64.3.0.CO;2-F>CrossRefGoogle ScholarPubMed
43MacGillivray, TE, Harrison, MR, Goldstein, RB, Adzick, NS. Disappearing fetal lung lesions. J Pediatr Surg 1993; 28: 1321–24.CrossRefGoogle ScholarPubMed
44Usui, N, Kamata, S, Sawai, T, Kamiyama, M, Okuyama, H, Kubota, A, et al. Outcome predictors for infants with cystic lung disease. J Pediatr Surg 2004; 39: 603–606.Google Scholar
45Butterworth, SA, Blair, GK. Postnatal spontaneous resolution of congenital cystic adenomatoid malformations. J Pediatr Surg 2005; 40: 832–34.Google Scholar
46Mann, S, Wilson, RD. Antenatal diagnosis and managemnet of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 2007; 12: 477–81.CrossRefGoogle Scholar
47Morris, LM, Lim, FY, Livingston, JC, Polzin, WJ, Crombleholme, TM. High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg 2009; 44: 60–65.Google Scholar
48Wilson, RD, Baxter, JK, Johnson, MP, King, M, Kasperski, S, Crombleholme, TM, et al. Thoracoamniotic shunts: fetal treatment of pleural effusions and congenital cystic adenomatoid malformations. Fetal Diagn Ther 2004; 19: 413–20.Google Scholar
49Belcher, E, Lawson, MH, Nicholson, AG, Davison, A, Goldstraw, P. Congenital cystic adenomatoid malformation presenting as in-flight systemic air embolisation. Eur Respir J 2007; 30: 801–804.Google Scholar
50Sauvat, F, Michel, JL, Benachi, A, Emond, S, Revillon, Y. Management of asymptomatic neonatal cystic adenomatoid malformations. J Pediatr Surg 2003; 38: 548–52.Google Scholar
51Brenner, D, Elliston, C, Hall, E, Berdon, W. Estimated risks of radiation-induced fatal cancer from pediatric CT. Am J Roentgenol 2001; 176: 289–96.Google Scholar
52Chodick, G, Ronckers, CM, Shalev, V, Ron, E. Excess lifetime cancer mortality risk attributable to radiation exposure from computed tomography examinations in children. Isr Med Assoc J 2007; 9: 584–87.Google Scholar
53Donnelly, LF, Emery, KH, Brody, AS, Laor, T, Gylys-Morin, VM, Anton, CG, et al. Minimizing radiation dose for pediatric body applications of single-detector helical CT: strategies at a large Children's Hospital. Am J Roentgenol 2001; 176: 303–306.CrossRefGoogle Scholar
54Lucaya, J, Piqueras, J, Garcia-Pena, P, Enriquez, G, Garcia-Macias, M, Sotil, J. Low-dose high-resolution CT of the chest in children and young adults: dose, cooperation, artifact incidence, and image quality. Am J Roentgenol 2000; 175: 985–92.CrossRefGoogle Scholar
55Aziz, 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; 39: 329–34.CrossRefGoogle ScholarPubMed
56Lejeune, C, Deschildre, A, Thumerelle, C, Cremer, R, Jaillart, S, Gosselin, B, et al. Pneumothorax revealing cystic adenomatoid malformation of the lung in a 13 year old child. Arch Pediatr 1999; 6: 863–66.CrossRefGoogle Scholar
57Chetcuti, PA, Crabbe, DC. CAM lungs: the conservative approach. Arch Dis Child Fetal Neonatal Ed 2006; 91: F463–64.Google Scholar
58Benjamin, DR, Cahill, JL. Bronchioloalveolar carcinoma of the lung and congenital cystic adenomatoid malformation. Am J Clin Pathol. 1991; 95: 889–92.CrossRefGoogle ScholarPubMed
59Dosios, T, Stinios, J, Nicolaides, P, Spyrakos, S, Androulakakis, E, Constantopoulos, A. Pleuropulmonary blastoma in childhood. A malignant degeneration of pulmonary cysts. Pediatr Surg Int 2004; 20: 863–65.CrossRefGoogle ScholarPubMed
60Papagiannopoulos, KA, Sheppard, M, Bush, AP, Goldstraw, P. Pleuropulmonary blastoma: is prophylactic resection of congenital lung cysts effective? Ann Thorac Surg 2001; 72: 604–605.CrossRefGoogle ScholarPubMed
61Hill, DA, Dehner, LP. A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4 [Letter]. Am J Surg Pathol 2004; 28: 554–55.CrossRefGoogle ScholarPubMed
62MacSweeney, F, Papagiannopoulos, K, Goldstraw, P, Sheppard, M, Corrin, B, Nicholson, AG. A cautionary note about congenital cystic adenomatoid malformation (CCAM) type 4 [Reply]. Am J Surg Pathol 2004; 28: 555.CrossRefGoogle Scholar
63Priest, JR, McDermott, MB, Bhatia, S, Watterson, J, Manivel, JC, Dehner, LP. Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 1997; 80: 147–61.Google Scholar
64Hill, DA, Ivanovich, J, Priest, JR, Gurnett, CA, Dehner, LP, Desruisseau, D, et al. DICER1 Mutations in familial pleuropulmonary blastoma.Science 2009; E Pub 25 June.CrossRefGoogle Scholar
65Khosa, JK, Leong, SL, Borzi, PA. Congenital cystic adenomatoid malformation of the lung: indications and timing of surgery. Pediatr Surg Int 2004; 20: 505–508.Google Scholar
66Kim, YT, Kim, JS, Park, JD, Kang, CH, Sung, SW, Kim, JH. Treatment of congenital cystic adenomatoid malformation-does resection in the early postnatal period increase surgical risk? Eur J Cardiothorac Surg 2005; 27: 658–61.CrossRefGoogle ScholarPubMed
67Marshall, KW, Blane, CE, Teitelbaum, DH, van Leeuwen, K. Congenital cystic adenomatoid malformation: impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. Am J Roentgenol 2000; 175: 1551–554.CrossRefGoogle ScholarPubMed
68Jaffe, A, Chitty, LS. Congenital cystic adenomatoid malformations may not require surgical intervention. Arch Dis Child Fetal Neonatal Ed 2006; 91: F464.CrossRefGoogle Scholar
69Jaffe, A. Orphan lung diseases in childhood: still unadopted? Thorax 2005; 60: 892–94.CrossRefGoogle ScholarPubMed
70Laverty, A, Jaffe, A, Cunningham, S. Establishment of a web-based registry for rare (orphan) pediatric lung diseases in the United Kingdom: the BPOLD registry. Pediatr Pulmonol 2008; 43: 451–56.Google Scholar