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Choroid plexus size in young children with Sturge-Weber syndrome
P D Griffiths1, S Blaser, M B Boodram
1Department of Neuroradiology, Newcastle General Hospital, Newcastle upon Tyne, United Kingdom.
Insights
Children with Sturge-Weber syndrome (SWS) show enlarged choroid plexus, particularly on the affected side. Choroid plexus size correlates with the extent of leptomeningeal involvement in SWS.
Area of Science:
- Pediatric Neurology
- Neuroimaging
- Cerebrovascular Disorders
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder characterized by a facial birthmark and neurological abnormalities.
- Choroid plexus abnormalities have been anecdotally reported in SWS, but systematic evaluation is lacking.
Purpose of the Study:
- To evaluate choroid plexus size in young children diagnosed with unilateral or bilateral Sturge-Weber syndrome.
- To compare choroid plexus dimensions between affected and unaffected hemispheres in SWS patients and healthy controls.
Main Methods:
- Retrospective analysis of MRI scans from 15 children (≤4 years) with SWS (11 unilateral, 4 bilateral).
- Choroid plexus diameters measured on contrast-enhanced axial MR images.
- Comparison with 15 age-matched controls without SWS.
Main Results:
- Significant enlargement of the choroid plexus was observed in hemispheres affected by SWS compared to unaffected sides and controls.
- A positive correlation was found between choroid plexus size and the extent of leptomeningeal enhancement indicating SWS involvement.
- Wide variation in choroid plexus size was noted across all SWS cases.
Conclusions:
- The choroid plexus enlarges early in the course of Sturge-Weber syndrome, irrespective of unilateral or bilateral presentation.
- Increased choroid plexus size is associated with the degree of leptomeningeal disease in pediatric SWS patients.
Purpose:
To assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome.
Methods:
Subjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal leptomeningeal enhancement on MR images. The diameters of the choroid plexus were measured on contrast-enhanced axial MR images. The choroid plexus of the affected and unaffected sides in these cases were compared with those of 15 age-matched children without Sturge-Weber syndrome who were being examined for seizures.
Results:
Our results show a wide variation in the size of the choroid plexus in children with Sturge-Weber syndrome; however, plexus associated with a hemisphere affected by Sturge-Weber syndrome were significantly larger than those on the unaffected side and in the age-matched control group. The size of the choroid plexus was positively correlated with the extent of leptomeningeal involvement as demonstrated by abnormal contrast enhancement.
Conclusion:
The choroid plexus is enlarged early in the course of Sturge-Weber syndrome in both unilateral and bilateral cases. There is a positive correlation between choroid plexus size and extent of leptomeningeal involvement in children with Sturge-Weber syndrome.