The Outcome of Expectant Management of Congenital Cystic Adenomatoid Malformations (CCAM) of the Lung

Abstract

Introduction: The appropriate management of asymptomatic congenital cystic adenomatoid malformations of the lung (CCAM) remains controversial. The aim of this study is to determine the outcome of expectant management of these lesions and the sensitivity of antenatal ultrasound diagnosis.

Material and Methods: A retrospective review was undertaken of all cases identified from prospectively collected databases with an antenatal or postnatal diagnosis of CCAM in the Northern region of England between 1985 and 2006 where such lesions underwent resection only when symptomatic.

Results: Thirty-seven cases of confirmed CCAM were identified antenatally or postnatally. Twenty-six (70%) were identified by antenatal ultrasound scans (during a period of near-universal antenatal scanning), of whom 21 (81%) were liveborn. In total, 16 of 29 (55%) liveborn infants with CCAM were symptomatic, and either died within the first postnatal week or underwent resection. Thirteen (45% of livebirths) were managed expectantly and remained asymptomatic. The sensitivity of antenatal ultrasound screening for CCAM increased over the period to 90% in the latter half of the study (p=0.035), although the positive predictive value (66%) did not improve.

Conclusions: Expectant management was a reasonable option for almost half of the babies, but over one third required surgery for CCAM, most becoming symptomatic in infancy. In view of the uncertainty that surrounds decisions regarding expectant or pre-emptive resection in asymptomatic infants, the authors advocate having an open and honest discussion when counselling parents (particularly antenatally) regarding surveillance or expectant management as a reasonable strategy.

Key words

congenital cystic adenomatoid malformation of the lung - asymptomatic - antenatal - management

References

• 1 Adzick AS, Flake AW, Crombleholme TM. Management of congenital lung lesions.  Semin Pediatr Surg. 2003;  12 10-16
• 2 Aziz D, Langer JC, Tuuha SE. et al . Perinatally diagnosed asymptomatic congenital cystic adenomatoid malformation: to resect or not?.  J Pediatr Surg. 2004;  39 ((3)) 329-334
• 3 Butterworth SA, Blair GK. Postnatal spontaneous resolution of congenital cystic adenomatoid malformations.  J Pediatr Surg. 2005;  40 832-834
• 4 Calvert JK, Boyd PA, Chamberlain PC. et al . Outcome of antenatally suspected congenital cystic malformation of the lung: 10 years experience 1991–2001.  Arch Dis Child Fetal Neonatal Ed. 2006;  91 F26-F28
• 5 Davenport M, Warne SA, Cacciaguerra S. et al . Current outcome of antenatally diagnosed cystic lung disease.  J Pediatr Surg. 2004;  39 549-556
• 6 Fitzgerald DA. Congenital cyst adenomatoid malformations: resect some and observe all?.  Paed Resp Rev. 2007;  8 67-76
• 7 Gomall AS, Budd JL, Draper ES. et al . Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population.  Prenatal Diagn. 2003;  23 997-1002
• 8 Hancock BJ, DiLorenzo M, Youssef S. et al . Childhood primary pulmonary neoplasms.  J Pediatr Surg. 1993;  28 1133
• 9 Hsieh CC, Chao AS, Chang YL. et al . Outcome of congenital cystic malformation of the lung after antenatal diagnosis.  Int J Gyn Obst. 2005;  89 99-102
• 10 Jaffé A, Chitty LS. Congenital cystic adenomatoid malformations may not require surgical intervention.  Arch Dis Child Fetal Neonatal Ed. 2006;  91 F464
• 11 Khosa JK, Leong SL, Borzi PA. Congenital cystic adenomatoid malformation of the lung: indications and timing of surgery.  Pediatr Surg Int. 2004;  20 ((7)) 505-508
• 12 Laberge J-M, Flageole H, Pugash D. et al . Outcome of the prenatally diagnosed congenital cystic adenomatoid malformation: a Canadian experience.  Fetal Diagn Ther. 2001;  16 178-186
• 13 Laberge J-M, Puligandla P, Flageole H. Asymptomatic congenital lung malformations.  Semin Pediatr Surg. 2005;  14 16-33
• 14 Langston C. New concepts in the pathology of congenital lung malformations.  Semin Pediatr Surg. 2003;  12 17-37
• 15 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;  18 447-453
• 16 Priest JR, Williams GM, Hill DA. et al . Pulmonary cysts in early childhood and the risk of malignancy.  Pediatr Pulmonol. 2009;  44 14-30
• 17 Richmond S, Atkins J. A population-based study of the prenatal diagnosis of congenital malformation over 16 years.  Br J Obstet Gynaecol. 2005;  112 1349-1357
• 18 Sauvat F, Michel J-L, Benachi A. et al . Management of asymptomatic neonatal cystic adenomatoid malformations.  J Pediatr Surg. 2003;  38 ((4)) 548-552
• 19 Shanmugam G, MacArthur K, Pollock JC. Congenital lung malformations – antenatal and postnatal evaluation and management.  Eur J Cardiothoracic Surg. 2005;  27 ((1)) 45-52
• 20 Stocker JT, Madwell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Classification and morphological spectrum.  Hum Pathol. 1977;  8 156-171
• 21 Stocker JT. Congenital and developmental diseases.. In: Dail OH, Hammar SP (eds) Pulmonary Pathology. New York: Springer; 1994: 155
• 22 Tsai AY, Liechty KW, Hedrick HL. et al . Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions.  J Pediatr Surg. 2008;  43 513-517
• 23 Yan-Sin Lo A, Jones S. Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cystic adenomatoid malformation of the lung.  J Pediatr Surg. 2008;  43 797-799

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