Venolymphatic malformations: prenatal diagnosis using magnetic resonance imaging, perinatal outcomes and long-term follow-up.

Abstract

Venolymphatic malformations are benign. Fetal MRI can more precisely demonstrate an infiltrative pattern of malformations than US. To evaluate perinatal outcomes and long-term follow-up of fetal venolymphatic malformations treated in different medical facilities using fetal MRI. This retrospective cohort study evaluated 20 pregnant women between 22weeks and 37weeks of gestation who were referred from different institutions. They presented with fetuses with various diagnoses of cystic masses on routine US. The cases were studied using MRI. We analyzed prenatal data, perinatal outcomes and long-term follow-up. We reviewed the MRI scans of 20 patients with venolymphatic malformation. Referral diagnosis was changed in 40% (8/20) of cases, with postnatal concordance of 100% (20/20). Moreover, 65% (13/20) presented with venolymphatic malformation in more than one body segment. The neck was affected in 70% (14/20) of fetuses, while the head and thorax were affected in 30% (6/20) and 45% (9/20), respectively. There were intrathoracic lesions in 35% (7/20), lesions in the abdomen in 30% (6/20), and lesions in the perineum and extremities in 10% (2/20) each. Tracheal displacement, neck deflection and anatomical displacement caused by tumoral compression were present in 15% (3/20) of cases. Moreover, 25% (5/20) of newborns required neonatal intensive care unit admission, and all presented with cervical or thoracic venolymphatic malformation. Furthermore, 50% (10/20) of cases presented with complete resolution after medical therapy. The intrathoracic and cervical residuals (35%, 7/20) were monitored and treated. MRI showed good correlation with postnatal examination of venolymphatic malformation, was useful in the differential diagnosis of fetal cysts on US, and presented a significant postnatal correlation with thoracic infiltration. The outcomes of prenatally diagnosed venolymphatic malformations are good despite the varying protocols among medical facilities.