Management
Standard management of blisters and skin infections; dental care by a pedodontist; dental implants in childhood as needed; care by a speech pathologist and/or pediatric nutritionist if dental abnormalities interfere with chewing and/or speech; cryotherapy and laser photocoagulation of retinal neovascularization to reduce risk of retinal detachment; standard management of retinal detachment; referral to a pediatric neurologist for management of seizures, spasticity, or focal deficits; brain MRI for functional neurologic abnormalities and/or retinal neovascularization; developmental programs and special education as needed for developmental delay.
Standard measures to reduce the risk of skin infection; evaluate for retinal detachment if vision decreases, strabismus appears, or head trauma occurs.
Surveillance
Eye examination: monthly until age four months, then every three months from age four months to one year, every six months from age one to three years, and annually after age three years. Assessment of neurologic function at routine visits with pediatrician, pediatric neurologist, or developmental pediatrician. Routine evaluation by a pedodontist or dentist.