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Linear nevus sebaceous syndrome. A review
I Alfonso1, C Howard, P F Lopez
1Department of Neonatal Neurology, Miami Children's Hospital, Florida 33155.
Journal of Clinical Neuro-Ophthalmology
|September 1, 1987
Summary
Linear nevus sebaceous syndrome can present with extensive eye calcification. Neurological function remained normal despite brain abnormalities in this reported case.
Area of Science:
- Ophthalmology
- Neurology
- Medical Genetics
Background:
- Linear nevus sebaceous syndrome (LNSS) is a rare congenital disorder.
- It is characterized by the triad of nevus sebaceous, skeletal abnormalities, and central nervous system (CNS) malformations.
Observation:
- This case details a patient with LNSS exhibiting extensive calcification in the posterior segments of both eyes.
- The calcification involved multiple retinal and choroidal layers.
Findings:
- Despite significant dysplastic brain changes observed on imaging, the patient maintained a normal neurological examination.
- This highlights a potential dissociation between CNS structural abnormalities and neurological function in some LNSS cases.
Implications:
- The findings suggest that extensive ocular calcification is a possible manifestation of LNSS.
- Understanding the embryopathogenesis of LNSS is crucial for predicting and managing its diverse clinical presentations.
- Further research is needed to elucidate the relationship between brain dysplasia and neurological status in LNSS.