Abstract

Objective: To evaluate the radiological and clinical findings of Sturge Weber Syndrome in pediatric patients in the light of literature data and to contribute to the early diagnosis of SWS, in patients especially with facial port wine birthmark.

Material and Methods: Fifteen patients who underwent Cranial Computed Tomography or Magnetic Resonance Imaging with the diagnosis of SWS in our hospital; between January 2010 and February 2019 were included in this study. Radiologic examinations of the patients were evaluated retrospectively by two experienced pediatric radiologists in a concensus reading. Demographic information and clinical findings were obtained from the medical records.

Results: The patients’ age ranged from 2 and 185 months (74.07 ± 74.08 months); 9 female and 6 male patients were evaluated. The most common presenting symptoms were seizure (40%) and facial port wine birthmark (26.66%). Radiological findings compatible with SWS were observed in nine patients (60%) and one of was bilateral. 7 patients had leptomeningeal enhancement and 8 patients had calcification areas. Transmedullary and cortical veins were observed in all patients except one patient with focal involvement. ADC values were decreased on pathological areas compared to normal hemisphere. None of the patients had accelerated myelination / white matter inversion or diffusion restriction.

Conclusion: SWS is a rare sporadic neurocutaneous syndrome with typical clinical and radiological findings. Although contrast-enhanced MRI is the most sensitive method in the diagnosis of pial angiomas, susceptibility-weighted imaging may contribute to the detection of calcifications and prominent venous collaterals.

Keywords: Çocuk, epilepsi, MRG, manyetik duyarlı MRG, nöroradyoloji

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Copyright © 2020 The Author(s). This is an open access article distributed under the Creative Commons Attribution License (CC BY), which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.

How to cite

1.
Alımlı AG, Yılmaz Ş, Özdemir Gökce A, Arıbaş Öz N. Radiologic Findings of Sturge-Weber Syndrome in Pediatric Population. Turk J Pediatr Dis [Internet]. 2020 Jan. 27 [cited 2025 Aug. 23];14(1):57-65. Available from: https://turkjpediatrdis.org/article/view/724