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Multiple pericallosal lipomas in two siblings with frontonasal dysplasia
Mohammad A Alzoum1, Ibrahim A Alorainy, Muneera Al Husain
1Department of Radiology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
AJNR. American Journal of Neuroradiology
|April 16, 2002
Summary
This study describes two siblings with frontonasal dysplasia (FND) and unusual multiple pericallosal lipomas. These findings highlight a previously unreported association between FND and specific patterns of brain malformations.
Area of Science:
- Neuroscience
- Medical Genetics
- Developmental Biology
Background:
- Frontonasal dysplasia (FND) is a congenital condition characterized by craniofacial abnormalities.
- Pericallosal lipomas are benign tumors typically found near the corpus callosum.
- Previous reports have linked FND with anterior pericallosal lipomas of the tubulonodular type.
Observation:
- Two siblings presented with frontonasal dysplasia.
- Both siblings exhibited multiple pericallosal lipomas in similar, distinct locations.
- Lipomas were observed in relation to both the posterior corpus callosum and the rostrum.
Findings:
- This is the first report of multiple pericallosal lipomas associated with frontonasal dysplasia.
- The observed lipomas were curvilinear and located in both anterior (rostrum) and posterior regions of the corpus callosum.
- This contrasts with previously described tubulonodular anterior pericallosal lipomas in FND cases.
Implications:
- The findings suggest a potential broader spectrum of central nervous system involvement in frontonasal dysplasia.
- This association may warrant further investigation into the genetic and developmental mechanisms underlying FND and associated brain malformations.
- Understanding these associations can aid in more comprehensive diagnosis and management of patients with FND.