The authors present six cases (seven eyes) of congenital microphthalmia and orbital cyst. These cases were newborn mainly with one aged 20 years. There were four females and two males and two of seven eyes had a visual potential of light perception or better after intraorbital surgery to centralise the position of the microphthalmic globe. These cases had maximal follow-up of 10 years. In four eyes, the cyst was left in place to stimulate orbital growth. At the end of follow-up, three patients had a good cosmetic result without cyst deterioration or transformation. The authors developed a treatment algorithm, starting with early investigation within weeks of birth to establish visual potential and other associated ocular abnormalities. Where visual potential is present, they recommend centralising the affected globe if possible to maximise visual stimulation. If absent visual potential, their aim is to maintain facial symmetry. They discuss options for cyst removal where this is indicated and management of orbital volume. They conclude it is possible in many cases to avoid enucleation, if treated early.
Management options for microphthalmia with orbital cyst with / without visual potential
Reviewed by Fiona Rowe
The management of congenital microphthalmia with orbital cyst: a case series.
CONTRIBUTOR
Fiona Rowe (Prof)
Institute of Population Health, University of Liverpool, UK.
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