Neurologic Manifestations of Incontinentia Pigmenti Follow-up

Updated: Dec 11, 2018
  • Author: Celia H Chang, MD; Chief Editor: Amy Kao, MD  more...
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Further Outpatient Care

See the list below:

  • Routine ophthalmologic follow-up is essential to prevent blindness. Wong et al recommended frequent examinations, following the schedule below:

    • As soon as possible after birth

    • Monthly until the infant is 3-4 months of age

    • Every 3 months until the child is 1 year old

    • Twice a year until the child is 3 years old

    • Yearly thereafter

  • Ophthalmologic treatments may include the following:

    • Photocoagulation for fibrovascular proliferation

    • Vitreoretinal surgery for retinal detachments

  • Dental (See Physical.)

  • Neurologist, only if neurologic abnormalities are present



Prognosis can be quite variable. Bryant et al reported that a child with neonatal seizures and persistent subcortical and periventricular white matter abnormalities on brain MRI had resolution of the seizures and was successfully tapered off anticonvulsants at 7 months of age. At age 11, the child had a normal neurologic examination and she was adequate to advanced in her academic skills for her age. [17]


Patient Education

Because incontinentia pigmenti is an X-linked dominant disease, genetic counseling regarding the risk of having affected offspring is very important.