DOI: 10.363247/sjmcr.2018.v06i10.031

Pages: 856-861

Orbital Langerhans Cell Histiocytosis (LCH) is a rare disease, generally affects younger age group [1]. Presentation may resemble infective and malignant diseases, hence making diagnosis more challenging. We report a case of a young girl with sudden progressive right upper eyelid mechanical ptosis with superotemporal orbital swelling, which was highly suspicious of orbital childhood malignancy. Surprisingly, tissue biopsy confirmed an Orbital LCH. This 3 year-old Malay girl developed painless, sudden progressive drooping of right upper eyelid of three weeks duration, associated with swelling over superotemporal orbit and acute subconjunctival hemorrhage (SCH) three days prior to presentation. She had no history of fever, recent trauma or surgery prior to that. Her vision was normal. There was right upper eyelid and superotemporal fullness with no overlying inflammation, mild non-axial proptosis and SCH temporal and inferiorly. Fundus and left eye examination was unremarkable. Computed Tomography (CT) of brain and orbit revealed extraconal mass at lateral aspect of the right orbit, eroding adjacent lateral wall of orbit. Magnetic Resonance Imaging (MRI) showed hemorrhagic and enhancing mass at superolateral aspect of right orbit with adjacent bony erosion. Histopathological Examination (HPE) of the incisional biopsied tissues confirmed Orbital LCH. Chemotherapy was started according to the LCH therapy guidelines and she responded with marked improvement clinically and radiologically. Although Orbital LCH is rare, several cases have been reported with almost similar presentation [2,3]. Thus, it may be suspected in a child with orbital mass, but confirmation with tissue biopsy is mandatory.