CONGENITAL CYSTIC MALFORMATIONS OF THE LU NG - PRENATAL-DIAGNOSIS ANDFETAL-OUTCOME - A REVIEW OF 31 CASES

We present 31 cases of prenatally diagnosed cystic malformations of th e lung. The diagnosis was made between the 16(th) and 36(th) weeks of gestation. We found 14 cases of a congenital cystic adenomatoid malfor mation type I (CCAM), six cases of a CCAM type III, seven cases of an isolated lung or bronchogenic cyst, and four cases of a lung sequestra tion. The diagnosis was confirmed in 23 cases. The final diagnosis of the remaining eight cases confirmed postnatally were: enterogenic cyst (n = 3), lung sequestration (n = 2), bronchogenic cyst (n = 1), thora cic teratoma (n = 1)and mediastinal lymphangioma (n = 1 ). The conditi on of hydrops fetalis was found in ten cases, and a mediastinal shift in 12. All cases with hydrops fetalis and/or mediastinal shift had a p oor outcome (TOP/abortion n = 11, NND n = 3). Only one child with pleu ral effusion was born alive and is doing well up to now. Ten children were operated on successfully, and in five cases we found a spontaneou s resolution of the described malformation. The following prenatal man agement can be proposed: at first the exact sonographical diagnosis ha s to be made followed by serial ultrasound examinations to detect path ological conditions, such as hydrops fetalis or mediastinal shift. Dop pler sonography of the venous system could be helpful to detect a high cardiac preload. Invasive therapy is possible in special cases The mo st important prognostic factors are the size of the malformation and t he presence of additional complications. The delivery should be done a t a centre for perinatal medicine.