PMC6311540_01
(a-d) Clinical photographs: (a, b) pre-operative, (c) post-operative and (d) orbital soft tissue mass.
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She also had left peri-ocular nodular swellings, multiple left-sided facial skin defects, left lateral canthal tag/cyst, left alar nasi cleft and a left orbital mass with no identifiable left eyeball (Figure 1(a)-(c)). A firm well-circumscribed tissue with a long stalk was enucleated and sent for histopathological examination (Figure 1(d)).
(a-c) Cranial CT scan axial cuts; (d) sagittal reconstruction. (a) Left orbital mass; the purple arrow on the remnant of the left eye. (b) Left-sided posterior fossa cyst communicating with the fourth ventricle (yellow arrow). (c) Colpocephaly. (d) Note the absence of the corpus callosum.
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Cranial computerised tomography scan and magnetic resonance imaging showed a left orbital mass, left micro-ophthalmia, colpocephaly, corpus callosal agenesis, giant dysplastic tectum, anteverted midbrain, near horizontal aqueduct of Sylvius, hypoplastic left cerebellar hemisphere, vermian aplasia, large left retrocerebellar cyst communicating with the fourth ventricle (Figures 2(a)-(d) and 3(a)-(d)). The left orbital 'cyst' represented remnant eye tissue surrounded by mixed density/intensity orbital mass (Figures 2(a) and 3(a)).
(a-d) Photomicrographs of H&E sections of left orbital soft tissue mass. (a) Uveal tract with hamartomatous features. (b) Stratified squamous epithelium of the conjunctiva (yellow arrow), focus of chondroid metaplasia (black arrow) and an apocrine gland (purple arrow). (c) Bundles of skeletal muscle. (d) Section showing blood vessels with foci of calcification of the tunica intima (black arrows).
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Histology of the orbital mass (Figure 4(a)-(d)) revealed fragments of fibrocollagenous tissue, rudimentary glial tissue, nests of pigmented cells and prominent vascular spaces within the stroma reminiscent of the uveal tract.