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.

Keywords: Lymphangioma; Magnetic resonance imaging; Prenatal diagnosis; Tomography; Ultrasonography; X-ray computed.