eMedicine Specialties > Neurology > Pediatric Neurology

Incontinentia Pigmenti: Follow-up

Author: Celia H Chang, MD, Associate Health Sciences Clinical Professor, Department of Neurology, University of California at Davis
Contributor Information and Disclosures

Updated: Dec 3, 2009

Follow-up

Further Outpatient Care

  • 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

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.10

Patient Education

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

Miscellaneous

Special Concerns

  • Patients should have close ophthalmologic and dental follow-up.
  • Prenatal diagnosis is possible for affected woman or families with previously affected children.
 


More on Incontinentia Pigmenti

Overview: Incontinentia Pigmenti
Differential Diagnoses & Workup: Incontinentia Pigmenti
Treatment & Medication: Incontinentia Pigmenti
Follow-up: Incontinentia Pigmenti
Multimedia: Incontinentia Pigmenti
References
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References

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Further Reading

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Keywords

Bloch-Sulzberger syndrome, incontinentia pigmenti type 2, skin pigmentation disorder, X-linked inherited disorder, loss of melanin, incontinentia pigmenti type 1, hypomelanosis of Ito, IP, hypohidrotic ectodermal dysplasia with severe immunodeficiency, EDAID, anhidrotic ectodermal dysplasia, EDA-ID, osteopetrosis, lymphedema, hemangiomas, OL-EDA-ID, hyper-IgM syndrome, NEMO gene

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Contributor Information and Disclosures

Author

Celia H Chang, MD, Associate Health Sciences Clinical Professor, Department of Neurology, University of California at Davis
Celia H Chang, MD is a member of the following medical societies: American Academy of Neurology and Child Neurology Society
Disclosure: Nothing to disclose.

Medical Editor

David A Griesemer, MD, Professor, Departments of Neuroscience and Pediatrics, Medical University of South Carolina
David A Griesemer, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Epilepsy Society, Child Neurology Society, and Society for Neuroscience
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Pediatrics, Division of Pediatric Neurology, Department of Neurology, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

 
 
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