Abstract
Background
The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients.
Methods
Fourteen patients were reviewed regarding the antenatal ultrasonography findings and postnatal clinical course. They were then classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 2), group B had respiratory symptoms just after birth (n = 6), and group C was asymptomatic at birth (n = 6). The postnatal clinical courses in three groups were reviewed.
Results
In group A, all two patients with hydrops fetalis died just after birth. In group B, six patients had a severe respiratory distress and underwent operation during the neonatal period. In group C, five out of six patients were asymptomatic and received elective operation during the early infant period. In the remaining one patient, the lesion spontaneously disappeared over time after birth. The mean age at the time of operation in group B and group C was 4.5 days and 4.5 months of age, respectively. In almost all patients in group C, we performed an operation within the first 6 months. During this observation period, we did not experience any complications associated with CCAM.
Conclusion
In patients with hydrops fetalis, fetal intervention is thought to be needed. In patients with asymptomatic CCAM, an elective operation during the early infant period is recommended to prevent the risk of complications associated with CCAM before 6 months of age. In addition, we recommend the performance of a partial lung resection using an axillary skin crease incision in order to obtain a good postoperative quality of life.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Laje P, Liechy KW (2008) Postnatal management and outcome of prenatally diagnosed lung lesions. Prenat Diagn 28:612–618
Laberge JM, Flageole H, Pugash D et al (2001) Outcome of the prenatally diagnosed congenital cystic adenomatoid lung malformation: a Canadian experience. Fetal Diagn Ther 16:178–186
Stocker JT, Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung: Classification and morphologic spectrum. Hum Pathol 8:155–171
Mann S, Wilson RD, Bebbington MW et al (2007) Antenatal diagnosis and management of congenital cystic adenomatoid malformation. Semin Fetal Neonatal Med 12:477–481
Taguchi T, Suita S, Yamanouchi T et al (1995) Antenatal diagnosis and surgical management of congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther 10:400–407
Adzick NS, Harrison MR, Crombleholme TM et al (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179:884–889
Wazak P, Claris O, Lapillonne A et al (1999) Cystic adenomatoid malformation of the lung: neonatal management of 21 cases. Pediatr Surg Int 15:326–331
Calvert JK, Lakhoo K (2007) Antenatally suspected congenital cystic adenomatoid malformation of the lung: postnatal investigation and timing of surgery. J Pediatr Surg 42:411–414
Kim YT, Kim JS, Park JD et al (2005) Treatment of congenital cystic adenomatoid malformation—does resection in the early postnatal period increase surgical risk? Eur J Cardiothorac Surg 27:658–661
Khosa JK, Leong SL, Borzi PA (2004) Congenital cystic adenomatoid malformation of the lung: indications and timing of surgery. Pediatr Surg Int 20:505–508
Marshall KW, Blane CE, Teitelbaum DH et al (2000) Congenital cystic adenomatoid malformation: Impact of prenatal diagnosis and changing strategies in the treatment of the asymptomatic patient. AJR Am J Roentgenol 175:1551–1554
Ozcan C, Celik A, Ural Z et al (2001) Primary pulmonary rhabdomyosarcoma arising within cystic adenomatoid malformation: a case report and review of the literature. J Pediatr Surg 36:1062–1065
Ramos SG, Barbosa GH, Tavora FR et al (2007) Bronchioalvelor carcinoma arising in a congenital pulmonary airway malformation in a child: case report with an update of this association. J Pediatr Surg 42:E1–E4
Meshoulan A, Leclair MD, Yvinec M et al (2007) Pleuropulmonary blastoma: a case of early neonatal diagnosis through antenatal scan screening. Gynecol Obstet Fertili 35:437–441
Horak E, Bodner J, Gassner I et al (2003) Congenital cystic lung disease: diagnostic and therapeutic considerations. Clin Pediatr 42:251–261
Lo AY, Jones S (2008) Lack of consensus among Canadian pediatric surgeons regarding the management of congenital cyst adenomatoid malformation of the lung. J Pediatr Surg 43:797–799
Bianchi A, Sowande O, Alizai NK et al (1998) Aesthetics and lateral thoracotomy in the neonate. J Pediatr Surg 33:1798–1800
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Nagata, K., Masumoto, K., Tesiba, R. et al. Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung. Pediatr Surg Int 25, 753–757 (2009). https://doi.org/10.1007/s00383-009-2431-x
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00383-009-2431-x