Epibulbar complex choristoma with simultaneous involvement of eyelid: a case report

Abstract

BackgroundEpibulbar complex choristoma, a rare congenital epibulbar tumor, has many diverse forms. Reviewing the literature, it can present clinically as either a circumferential or isolated epibulbar mass, limbal tumor, lateral canthal mass, aggregate of ectopic cilia in the upper eyelid, eyelid mass mimicking chalazion, or lacrimal caruncle mass. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. Here, we report an atypical presentation of epibulbar complex choristoma with simultaneous eyelid involvement.Case presentationA 1-month-old full-term boy was brought to our clinic with congenital epibulbar mass of the right eye with simultaneous eyelid involvement. Dilated fundus examination was unremarkable. Survey for linear nevus sebaceous Jadassohn was negative. Due to concerns of possible amblyopia and cosmetics, lamellar keratectomy, sclerotomy, and conjunctivoplasty were performed to remove the epibulbar lesion. The eyelid defect was reconstructed with 6–0 Vicryl sutures. Histopathological examination reported complex choristoma. Upon three-year follow-up, low astigmatism and favorable cosmetics results were achieved.ConclusionsCongenital complex choristoma can present clinically as an epibulbar mass with eyelid involvement. The management depends on the extent of involvement, the risk of amblyopia, and cosmetic concerns. The method of eyelid reconstruction should be tailored according to the residual eyelid defect.

Epibulbar osseous choristoma with dermolipoma: A case report and review of literature

“…Full-text, free-access, Englishprinted articles with pathologically confirmed epibulbar osseous choristoma and dermolipoma identified 14 cases (Table 1). [8,[11][12][13][17][18][19][20][21][22][23][24][25][26][27] Including our case, age range at presentation was from birth to 38 years with mean age of 11.6 years. Ten cases (66.7%) were females, and 5 cases (33.3%) were males.…”

“…[9,10] While it occurs most commonly in supratemporal subconjunctival space attached to or separate from sclera, it can also be found at various locations such as perilimbal area, lateral canthus, extraocular muscle insertions, and tarsal plate. [11][12][13][14] Some explanations for the of epibulbar osseous choristoma have been proposed. Duke Elder first proposed the existence of secondary ossification centers of the orbital bones which very rarely could remain isolated, giving rise to small bones identified as osseous choristoma.…”

“…In review of literature, CT images of epibulbar osseous choristoma revealed a cystic mass with fat-like low-density and a high-density radio-opaque mass which is indicative of a calcium component. [8,11,12,[17][18][19][20][21][22][23][24]26] This differentiates epibulbar osseous choristoma from low-density lesions like fat, dermolipoma, or epibulbar dermoid and helps predict the diagnosis.…”

“…[9,10] While it occurs most commonly in supratemporal subconjunctival space attached to or separate from sclera, it can also be found at various locations such as perilimbal area, lateral canthus, extraocular muscle insertions, and tarsal plate. [11–14]…”

“…We conducted a systematic literature review on previously reported cases via PubMed using the keywords “congenital subconjunctival mass,” “epibulbar osseous choristoma” and “epibulbar dermolipoma.” Full-text, free-access, English-printed articles with pathologically confirmed epibulbar osseous choristoma and dermolipoma identified 14 cases (Table 1). [8,11–13,17–27] Including our case, age range at presentation was from birth to 38 years with mean age of 11.6 years. Ten cases (66.7%) were females, and 5 cases (33.3%) were males.…”