Severe microphthalmos with cyst and unusually huge dermolipoma

The purpose of this study was to report an unusual case of severe microphthalmos, together with an orbital cyst and huge ocular surface dermolipoma.

This is a clinical report relating clinical features as well as imaging and histopathologic findings, along with surgical management of the patient.

A 5-month-old Chinese male infant was referred, with 2 large masses in the left eye that were present since birth. Ocular examination results revealed a complete absence of any eye structures in the left orbit. In its place were 2 large masses between the left upper and lower palpebral fissure. One was a 3 × 3 × 2.5-cm spherical red tumor with a smooth surface. The other was a large solid spherical tumor, 4 × 4 × 5 cm, covered with normal skin located in the temporal region and attached to the red mass by a pedicle. Orbital magnetic resonance imaging examination findings confirmed that no eye structures were present in the left orbit. However, a cystic lesion was found in the left orbit, with a low signal on T1-weighted imaging and high signal on T2-weighted imaging, and another huge spherical heterogeneous mass was located "outside" the left orbit. Anterior orbitotomy by conjunctival incision was performed under general anesthesia. A spherical cystic mass of 1.5 × 1.5 × 1.6 cm, a small eye of 0.7 × 0.7 × 0.6 cm, and a huge dermolipoma were removed completely. Pathologic examination results confirmed the diagnosis of severe microphthalmos, together with orbital dermoid cyst and dermolipoma.

This rare case demonstrates that severe microphthalmos with a cyst may be completely covered by conjunctiva and associated with an unusually huge dermolipoma.