Orbital lymphangioma with intracranial extension

We report a case of biopsy-proven orbital lymphangioma with contiguous spread intracranially in a 14-year-old boy. The patient had a history of congenital orbital lymphangioma and hydrocephalus. Multiple functional and cosmetic periorbital surgeries were done over the years. Severe unresponsive amblyopia developed along with disfiguring anterior orbital and brow invasion of tumor. Since the age of 4 years, the patient had had 12 episodes of periorbital and orbital cellulitis, often after upper respiratory infections. CT and MRI scans have shown progression of the tumor posteriorly beyond the orbital confines. The tumor, now present in the superior and inferior orbital fissure and the paranasal sinuses, extends extradurally into the middle cranial fossa. This case is unique, with documentation of the benign tumor's progressive posterior extension intracranially, along with repeated episodes of severe periorbital cellulitis.