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Normal Size Eye with Light Perception
(A True Scleral Prosthesis)

This case presents a rare congenital aberration (Sturge-Weber Syndrome) which consists of benign tumors seen in the manner of heavily dilated and newly formed blood vessels.  As shown, it usually occurs unilaterally, and in this case involved half the face and all of the bulbar conjunctival tissue in the affected eye.  Beyond this noticeable deficiency, the light perceptive eye had a normal clear cornea and a deep anterior chamber, but when comparing the blue pigmentation in the iris in normal room lighting, its pupil remained constricted, which allowed its stroma to show more vibrant lighter striations.  This condition is called anisocoria, where the pupils are not of equal size.  Imbalance in iris color would only be detectable when the normal eye's pupil becomes dilated, which causes its stroma pigments to appear darker in color tones.

Inasmuch as the main concern was to detract from the abnormal inflammation of the conjunctiva, it was elected to produce what can be described as a (true) scleral cover shell prosthesis, or more appropriately called a 'donut' scleral shell prosthesis since the corneal portion of the shell is not required.  This would also eliminate any chance of causing corneal ulceration were the prosthesis to be worn over an extended period of time that included sleeping.  A Phase I clear acrylic moulded trial shell was fitted over the sclera and checked for blanching (pressure points) and displacement during normal eye excursion.  Any nonconforming areas would quickly be seen as white sclera and any displacement of the shell would rub against the cornea.  The Phase II 'true' scleral shell duplicated the trial shell in white acrylic and only required a limited amount of red threads and tinting to duplicate the sclera and vascular pattern of the normal eye.

Figure 9A Sturge-Weber Syndrome affecting the skin and conjunctival tissue of the eyeball.

Figure 9B The completed (True) scleral prosthesis in position covers most of the affected conjunctival tissue without compromising normal eye movement.

Figure 9C The impression moulded scleral prosthesis is similar in design to that of the empirically fit clear acrylic 'donut' shaped conformers worn post-operatively after removal of (symblepharon) adhesions between the eyelids and eyeball.



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