PMC9152410_01
(a) RE congenital cystic eyeball in a 14-year female: LE microcornea with inferior scar. (b) Cyst shows a positive transillumination test. (c) B-scan USG of the RE showing a stump of optic nerve-like structure in the posterior aspect of a large cyst. (d) Histopathology of the excised cyst showing cyst lined by neuroglial tissue. No specific remnants of the eye could be identified.
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RE was no perception of light (NPL) whereas the vision in the left eye (LE) was 1/60 on the Snellen distant visual acuity chart. RE examination revealed a large cystic swelling predominantly in the lower eyelid but no visible eyeball in the palpebral aperture (Figure 1). The swelling was single, soft, smooth, bluish-tinged, nontender, nonpulsatile, and brilliantly transilluminating (Figure 1(b)). Examination of the LE suggested a microcornea, inferior corneal scar, iris coloboma, and chorioretinal coloboma involving the optic disc and macula (Figure 1). Typically, a stump of the optic nerve-like structure was seen in the posterior aspect of the cyst (Figure 1(c)). The cyst was sent for histopathological examination which revealed an irregular cyst lined externally by a connective tissue layer and internally by glial tissues (Figure 1(d)) without a histologically identifiable eyeball, which confirmed the diagnosis of the congenital cystic eyeball.