CASE REPORT
A 12-month-old male with bilateral anophthalmia presented with an increasing “bump” of the right upper eyelid noted 2 months prior. Examination revealed a soft, palpable fullness of the right upper eyelid, with conformers in the appropriate position bilaterally and no discharge or periorbital erythema or edema. MRI of the orbits revealed a 2.8-cm lobular mass in the superolateral right extraconal orbit with mild intrinsic T1 shortening, fat suppression, and diffusion restriction most consistent with a dermoid cyst (). Histopathologic review following anterior orbitotomy with excisional biopsy revealed an thin- walled encapsulated lesion lined by compressed keratinized squamous epithelium and an abundant keratinaceous debris consistent with a dermoid cyst.
FIGURE 1. MRI showing 2.8-cm lobular mass in the superolateral right pre-septal orbit extending post-septally in the extraconal orbit above the lacrimal gland towards the posterior orbit with mild intrinsic T1 shortening, fat suppression, diffusion restriction most consistent with a lobular dermoid cyst.
COMMENT
Dermoid cysts represent the most common benign space-occupying mass of the orbit in children.Citation1 The incidence of anophthalmia or microphthalmia ranges from 0.4–1.4 per 10,000 live births.Citation2–4 Most dermoids are idiopathic, but an association has been noted in some heritable genetic syndromes, and in association with rubella, alcohol, thalidomide, retinoic acid,Citation5,Citation6 hydantoin,Citation7 and LSD. Dermoids arise from sequestration of embryonic epithelium between orbital bones, are slow growing, most commonly occur in the superotemporal or superonasal quadrants, and typically present as an enlarging mass.Citation8,Citation9 Imaging often reveals a fluid signal on MR and restriction on diffusion weighted imaging.Citation10 Definitive diagnosis is made through histopathologic review.Citation11 Although dermoid cysts have never been reported in the setting or bilateral anophthalmia, they should be considered in the differential diagnosis in the appropriate clinical setting.
Declaration of interest
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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