PMC3396869_01
Massive proptosis with exposure keratopathy on 5th day of life
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The swelling rapidly filled the orbit and extruded the eye globe within 6 days of birth (Figure 1).
Computerised tomography showing the well circumscribed orbital tumour of mixed density with marked anterior displacement of the globe
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Computerised tomography scan revealed a well circumscribed left retroocular mass of mixed density with splaying of the orbital walls and with increased vascularity (Figure 2) and which did not show any intracranial extension.
Haematoxylin & eosin stained sections (x40): showing neuroepithelium, cartilage and cystic spaces
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The solid areas consisted of primitive neuroepithelial cells which comprised about 5% of the tumour and were forming tubules (Figure 3).
PMC3396869_02
PMC3396869_03
Contracted socket with lid notching and conjunctival exposure in same patient at six months of age having defaulted from follow-up
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Contact with the patient was only re-established through the help of social health department of the hospital at 6 months of age (Figure 4) by which time, the left socket was already contracting and it was impossible to fit even our smallest prosthesis.