Congenital cystic adenomatoid malformation – diagnostic and therapeutic procedure: 8-year experience of one medical centre

INTRODUCTION: Congenital cystic adenomatoid malformation (CCAM) is a rare anomaly. The mechanisms and the time at which the abnormality develops are still unclear. The malformation is characterized by the presence of single large or multiple but smaller cysts. AIM: To present the experience of our medical centre, the Polish Mother’s Memorial Hospital – Research Institute. MATERIAL AND METHODS: We analysed the medical records of 32 neonates hospitalized in 2008–2017 at the Department of Paediatric Surgery and Urology ICZMP due to pre- or postnatally diagnosed congenital cystic adenomatoid malformation. RESULTS: In 2008–2017 32 children were hospitalized at our department due to prenatally diagnosed CCAM. An intrauterine procedure of thoracoamniotic shunting was performed in 13 fetuses. All newborns had chest computed tomography. Surgery had been performed in 21 children. Histopathological analysis of specimens prepared from the resected lung tissue confirmed type 1 CCAM in 4 cases, type 2 CCAM in 11 cases and type 3 CCAM in 1 case. Four patients were diagnosed with a bronchogenic cyst and one with extralobar pulmonary sequestration. CONCLUSIONS: Postnatal management of CCAM depends on the clinical status of the newborn. Newborns without clinical symptoms require radiological diagnostics to confirm the presence of malformation. Surgical treatment is recommended before the age of 10 months. Resection of the malformed lung fragment in patients with CCAM is the first-line treatment. It should be emphasized that despite the tremendous advances in diagnostic imaging, the final diagnosis of CCAM is made based on histopathological findings.