Treatment and prognosis of fetal lymphangioma

Abstract

Objective: To investigate the treatment and prognosis of fetal lymphangioma and factors that inform treatment selection.

Study design: Retrospective analysis of 79 patients with fetal lymphangioma treated at our hospital. Treatment methods included medical termination (death in-utero), expectant treatment, surgery, and interventional sclerotherapy (including ex utero intrapartum treatment, EXIT). Methods of treatment were selected according to the location and size of the lymphangioma.

Results: Among the 133,322 fetuses, in 130,202 pregnant women, examined at our hospital, a lymphangioma was identified in 79. The lymphangioma was confirmed by ultrasound, magnetic resonance imaging and post-natal computed tomography, as appropriate, and pathological results obtained postoperatively or on autopsy. Septation of the mass was identified in 66 of the 79 cases (83.54%). 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 underwent expectant management and 7 surgical treatment. Medical termination of the pregnancy was performed in 32 cases, and 2 fetuses died in-utero. Of the 16 cases of expectant treatment, the lesions retrogressed during the intra-uterine period in 7 cases, before the post-natal age of 6 months in 4 neonates, and before the age of 2 years in 3 neonates, with no change in the size of the lymphangioma identified in 2 cases. Of the 7 neonates who were treated surgically, relapse occurred in 1 case, which required re-operation.

Conclusions: Several treatment options for lymphangioma are available, with treatment selection being based on the location and size of the lymphangioma.

Keywords: Ex utero intrapartum treatment (EXIT); Lymphangioma; Prenatal diagnosis; Treatment; Ultrasonography.