Ocular trauma as the first presentation of Langerhans cell histiocytosis

Ocular trauma occurs disproportionately in children on an annual basis. However, because of this frequency, other diagnoses, such as orbital neoplasms, can easily be ignored.

We report on a two-year-old boy who presented with a dark purple, irregularly shaped lesion on his lower left eyelid. The patient had suffered injuries twice to that area.

Axial computed tomography images demonstrated an ill-defined and inhomogeneous soft tissue mass in the lateral and posterior region of the left orbit, with bony destruction and absorption of the adjacent orbital wall. Magnetic resonance imaging of the orbit showed a lesion involving the greater sphenoidal wing, the supraorbital wall, and the frontal area. Enlarged nuchal and inguinal lymph nodes were detected by sonographic examination. Histopathological examination of a surgical biopsy showed an accumulation of characteristic Langerhans cells. The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemical examinations positive for CD1a and SP-100.

Orbital Langerhans cell histiocytosis is rarely encountered in ophthalmic practice, so the ophthalmologist needs to be familiar with its presentation and work-up, and has to be aware of possible OLCH diagnosis when a child (or even an adult) presents with prolonged and persistent eyelid edema, even with a history of ocular trauma.