Orbital lymphangioma and persistent fetal vasculature

A 12-year-old girl had an orbital lymphangioma and ipsilateral persistent hyperplastic primary vitreous (recently termed persistent fetal vasculature). Persistent fetal vasculature, orbital combined venous lymphatic vascular malformations, and noncontiguous intracranial vascular malformations may reflect the same defect in embryonal vascular maturation occurring in different tissues. Physicians caring for patients with orbital lymphangiomas should search for subtle signs of ipsilateral microphthalmos and should consider neuroimaging to evaluate for intracranial vascular malformations.