Fetal Lymphangioma: Prenatal diagnosis on ultrasound, treatment, and prognosis

Abstract Objective To investigate the characteristics of fetal lymphangioma, including their location, imaging features, prenatal and differential diagnoses, treatment, and prognosis. Study design Imaging data of 79 patients with fetal lymphangioma treated at our hospital were obtained. Imaging modalities included prenatal and post-natal magnetic resonance imaging (MRI), prenatal and post-natal color Doppler ultrasound, and post-natal contrast-enhanced computed tomography (CT). Modalities of delivery and treatment were selected according to the location and size of lymphangioma. Results Among the 133,322 fetuses of 130,202 pregnant women examined at our hospital, 5 fetuses were misdiagnosed and the diagnosis was missed in 5 fetuses prenatally. Finally, 79 had lymphangioma, confirmed by ultrasound, MRI, post-natal CT, and pathological results obtained postoperatively or on autopsy. The diagnostic coincidence rate of lymphangioma was 88.1% (74/84 cases). Of the 79 fetuses with a lymphangioma, septation of the mass was identified in 66 cases (83.54%), with no evidence of septation in the remaining 13 fetuses (16.46%). With regard to location, the lymphangioma was located in the neck in 50 fetuses (63.29%). Interventional sclerotherapy, using bleomycin, was performed in 22 neonates, of which 3 underwent ex utero intrapartum treatment (EXIT) due to evidence of airway or esophageal obstruction (16 patients underwent expectant management; 7 surgical operation). Thirty-two fetuses underwent medical termination and 2 fetuses died in-utero. Of the 16 patients who had expectant treatment, the lesions retroregressed during the intra-uterine period in 7 fetuses, before the post-natal age of 6 months in 4 neonates, and before the age of 2 years in 3 neonates. Of the 7 neonates who were treated surgically, relapse occurred in 1 case, which required re-operation. Conclusions Prenatal ultrasound provides a clear differential diagnosis of fetal lymphangioma. Interventional therapy should be the first-choice treatment for neonates with a lymphangioma confirmed postnatally.