50E / 213 – ELECTIVE SURGERY FOR CONGENITAL CYSTIC ADENOMATOID MALFORMATION
AT ONE YEAR OF AGE IS SAFE AND WITHOUT RESPIRATORY SEQUELAE.
Kongstad T1, Buchvald F1, Tabor A2 Peersen BL3, Brenøe J4, Nielsen KG1.
1Pediatric Pulmonary Service, JMC
2Clinic of Fetal Medicine and Ultrasonography, JMC
3Department of Pathology, DIA
4Department of Thorax Surgery, Hjertecentret.
All: Copenhagen University Hospital, Rigshospitalet, Denmark.
Background:
Congenital Cystic Adenomatoid Malformation (CCAM) is the most common cystic lung lesion in
children, accounting for approximately 95% of casesi and detection of these malformations in utero by
ultrasonography is increasing.
Different positions to treatment exist, from early surgery ii to a more expectant approach.iii
In Denmark elective surgery after one year of life has been the main standard procedure for the last
decade.
Purpose:
The aim of the study was to review the cases of CCAM from Copenhagen University Hospital from
1995 to 2008.
Methods:
From the National Patient Registry information on personal identification numbers (PIN) of infants
diagnosed with CCAM in the period 01.01.1995 to 31.12.2008 was retrieved. These PINs were crosschecked with databases from the departments of pediatric pulmonology, pathology, radiology and
antenatal ultrasonography.
Results:
Twenty-nine children were diagnosed with CCAM. Sixteen cases (55.2%) where discovered at
antenatal ultrasonography, and almost all cases (94%) within the last 7 years.
Although 6 children had neonatal distress, only 1 infant had severe symptoms demanding immediate
radical surgery. The remaining children improved and elective surgery was planned.
Twenty-one children (72.4 %) have undergone surgery and complications were seen in 14.3 %
(bronchopleural fistula demanding additional surgery, prolonged pneumothorax and atelectasis).
Three children are scheduled for surgery in near future, while in 3 children (10.3 %) CT examinations
showed spontaneous regression.
Three children were discontinued due to normal standard x-ray examinations and no symptoms: Two
of these children later presented (age 2 and 7 years) with recurrent lung infections, CT-scans revealed
the CCAM still present, and surgery was performed. One child was treated with drainage due to
pneumothorax at the age of 14 years and discontinued after 2 years without relapse.
Median (range) age at surgery was 1.3 years (1 day to 14.8 years) and median (range) duration of
admission was 4 days (3-38).
Most
children were asymptomatic in the postoperative period (months to years). Two children have mild
respiratory symptoms (wheeze and recurrent infections).
Conclusion:
Elective surgery in infants with CCAM at one year of age is safe and without respiratory sequelae. A
few CCAM regress spontaneously and if untreated, CCAM might be responsible for respiratory
symptoms demanding surgery later in life.
i
Cloutier MM, Schaeffer DA Hight D: Congenital cystic adenomatoid malformation. Chest 103:761764, 1993
ii Jean-Martin Laberge, Pramod Puligandla, Hélène Flageole. Asymptomatic congenital lung
malformations. Seminars in Pediatric Surgery (2005) 14, 16-33
iii Ryo Sueyoshi, Tadaharu Okazaki, Naoto Urushihara et al. Managing prenatally diagnosed
asymptomatic congenital cystic adenomatoid malformation. Pediatr Surg Int (2008) 24:1111-1115