P19.04: A rare case of congenital lobar emphysema mimicing cystic adenomatoid malformation prenatally

Abstract

Objectives: Fetal lymphangiomas are uncommon congenital malformations of the lymphatic system that involve the skin and subcutaneous tissues. Lymphangiomas can occur anywhere in the skin and the mucous membranes.The aim of this study was to review the ultrasound findings in the prenatal diagnosis, the outcome and treatment of the NNL. Methods: We analyze our database with 1843 cases of congenital anomalies between 2003 to March of 2012. The definition for the prenatal diagnosis of the Lynphangiomas were cystic mass who involve the without vascular supply and include in the soft tissue. We exclude the nuchal localization. The diagnosis was confirmed after birth and each one was follow. The variables include in the analysis were the localization, associate malformations, postnatal outcome, treatment. Results: Thirteen cases were diagnosed with NNL. Postnatal assessment was obtained in all the cases. The anatomic localization was: fetal thorax 2 cases, axillar 3 cases, facial 2 cases, anterior neck 3 cases; 1 of them with a big mass with obstruction of the air way, arm 1 case, gluteus 1 case an 1 case with lymphangiomatosis. 2 (15%) died in the perinatal period 1 of them the lymfangiomatosis and the other the anterior neck with air way obstruction. 2 (15%) of the cases had spontaneous resolution the localization of this cases was arm and 1 case in the anterior neck. 9 cases require postnatal treatment with medical therapy with Bleomicin and surgical reduction. None were associated with chromosomal anomalies. None of them produced functional limitation. The facial lynphangiomas were related with aesthetic facial scarring sequelae. Conclusions: The prenatal diagnosis of the NNL require a sequential analysis of the localization, size, multiloculation and mass effect. The Involvement of the upper airways one of the prognostic factor the prenatal diagnosis allows the preparation for birth. In cases of lymphangiomatosis the prognosis is poor. As to its other locations lymphangiomas the prognosis are well but the the sequelae commitment to aesthetic or function must be taken into account when counseling parents.