Today, a new patient, a nine years old child with a history of ROP came to the office. The left eye was phthisical with severe band keratopathy and the right eye had a normal anterior segment the posterior retina is seen above. The child had a degree of spasticity in her walk but was quite charming.
Her vision was around 20/100.
She was too light sensitive for an extended peripheral exam and certainly not for scleral depressed exam She had nystagmus and poor control of her eye. I did not see any fibrosis anteriorly or any detachment however my view was poor.
The macular heterotopia frequently is associated with peripheral traction retina detachment and many of the fibrous elements that cause the early detachment and macula heterotopia are risk factors for retina detachment throughout life. The rate of detachment is impossible to know because we do not have a cohort of ROP patients followed beyond 15 years. My personal estimate is about 20% of patients with stage 4A.
ROP is a life-long disease. Especially, if there are retinal residua like macula heterotopia, tractional elements or even inactive cicatrix.
I try to train the children to tolerate a full exam and in cases with only one eye and particular risks I do exams under anethesia. I have a few ROP "autistic" and developmental delay children that I follow with exams asleep every 3-4 months.
These cases are technically demanding and I am caring for three adults with ROP with late Retinal Detachment now. They are very difficult because the vitreoretinal interface is not clean and requires careful attention to strip and retinal breaks without stripping are likely to fail. I use a few special techniques that I hope to discuss in the future. As is often the case in rare diseases, the techniques are not published.
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