Congenital cystic adenomatoid malformations of the lung: a retrospective study of diagnosis, treatment strategy and postoperative morbidity in surgically treated patients

Abstract

Objectives The purpose of this study was to evaluate diagnosis, clinical signs and strategy in Congenital Cystic Adenomatoid Malformations (CCAM). Methods In this retrospective study, patients with thoracic surgery for CCAM lesions at the University Hospitals of Leuven from July 1993 to July 2016 were searched. Data on diagnosis, prenatal ultrasound findings, clinical signs, lesion site, CCAM type, associated anomalies, imaging, surgical approach and postoperative morbidity were reviewed. The Fisher’s exact and Mann–Whitney tests were used as appropriate. Results A total of 55 patients were identified with CCAM. In 65% (n = 36/55), CCAM was detected on prenatal ultrasound. Prenatal symptoms due to hydrops or mass-effect were present in 22% (n = 8/36), of which eventually 6 had need for prenatal intervention (Thoracoamniotic shunting or Intrauterine Puncture). Surgery was performed elective in 40% patients (n = 22/55), while others developed clinical signs with need for semi-urgent surgery, with clinical signs of pulmonary infection and respiratory distress being the most common. Most patients had a single lobectomy via mini-thoracotomy. Postoperative complications and length of stay were observed to be significantly higher in CCAM with preoperative clinical signs. Conclusions Surgery in asymptomatic CCAM patients can be performed safely with few postoperative complications and can be planned at young age in patients with high risk of developing clinical signs later in life.

Experience of video-assisted thoracic surgery treatment of congenital pulmonary airway malformation in infants less than 3 months of age

“…An imaging examination should be performed during the follow-up after birth for those with prenatally identified lesions by ultrasound, and it is necessary to comprehensively consider the radiation dose for the child and the family’s assessment of the risk and choose the operation according to the change of the condition. Verhalleman indicated that in his university hospital, if possible, the first 3 months of life are preserved for lung growth and general development ( 4 ). Elective surgery in patients with a high risk for clinical symptoms is recommended before 12 months of age, although no specific guidelines exist ( 5 - 8 ).…”

“…The prevalence of CPAM accounts for about 1/35,000–1/7,200 of the live fetuses, and this rate is gradually increasing ( 2 ). For asymptomatic children with CPAM after birth, the appropriate age for operation is above 3 months old ( 3 , 4 ). Notably, a few children were born with serious respiratory and circulatory disorders due to the compression of the heart and lungs and mediastinal deviation.…”

Congenital pulmonary airways malformation: state of the art review

“…Maternal steroids, thoraco-amniotic shunts, fetal mass resection, and ex utero intrapartum treatment (EXIT) procedures play a significant therapeutic role in congenital lung lesions with hydrops, resulting in lower risk of prematurity, less ventilator requirement, and a better outcome, compared to open or minimally invasive fetal surgery (18)(19)(20)(21)(22).…”

“…In macrocystic lesions, thoracocentesis or thoraco-amniotic shunting are useful for both diagnosis and treatment of hydrops. The indications for open fetal surgery are hydrops and signs of evolving fetal heart failure (20).…”

“…Surgery in asymptomatic patients can be performed safely with few post-operative complications and planned at a young age in patients with a high risk of developing clinical signs later in life (20). Patients with CPAM appear to have better perioperative outcomes before the beginning of symptoms, including shorter operating times, shorter post-operative mechanical ventilation, shorter chest drainage duration and shorter post-operative hospital stays (23).…”