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Sturge–Weber syndrome with intracerebral hemorrhage: a case report
INTRODUCTION: Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. However, intracranial hemorrhage in patients with SWS i...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer International Publishing
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5055513/ https://www.ncbi.nlm.nih.gov/pubmed/27795889 http://dx.doi.org/10.1186/s40064-016-3439-z |
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author | Chonan, Masashi Suzuki, Yasuhiro Haryu, Shinya Mashiyama, Shoji Tominaga, Teiji |
author_facet | Chonan, Masashi Suzuki, Yasuhiro Haryu, Shinya Mashiyama, Shoji Tominaga, Teiji |
author_sort | Chonan, Masashi |
collection | PubMed |
description | INTRODUCTION: Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. However, intracranial hemorrhage in patients with SWS is rare. We herein report a rare case of SWS with intracerebral hemorrhage derived from sinus thrombosis. CASE DESCRIPTION: A 62-year-old man suddenly fell into a coma and was admitted to our hospital. His neurological status was assessed as GCS 6 (E1V1M4) with right-sided hemiparesis. At birth, he had a right-sided facial port-wine stain typical of SWS that involved the ophthalmic division of the trigeminal nerve. Laboratory findings showed that he was dehydrated, and his serum D-dimer concentration was increased. Computed tomography revealed left thalamic hemorrhage with acute hydrocephalus and cortical calcification in the right occipital lobe. Magnetic resonance imaging displayed a vascular malformation of the right cerebral hemisphere consistent with SWS. Magnetic resonance venography showed steno-occlusion of the superior sagittal sinus, straight sinus, and left internal cerebral vein (ICV). Emergency ventricular drainage was performed. Seven days after surgery, his consciousness improved to GCS 14 (E4V4M6). Rehydration therapy was performed to prevent sinus thrombosis. DISCUSSION AND EVALUATION: His postoperative course was uneventful. Sudden congestion of the left ICV may have caused left thalamic hemorrhage. CONCLUSIONS: SWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments. |
format | Online Article Text |
id | pubmed-5055513 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Springer International Publishing |
record_format | MEDLINE/PubMed |
spelling | pubmed-50555132016-10-28 Sturge–Weber syndrome with intracerebral hemorrhage: a case report Chonan, Masashi Suzuki, Yasuhiro Haryu, Shinya Mashiyama, Shoji Tominaga, Teiji Springerplus Case Study INTRODUCTION: Sturge–Weber syndrome (SWS) is a rare congenital disease that affects the brain, skin, and eyes, and is a sporadically occurring neurocutaneous syndrome that affects intracerebral veins, which is associated with venous thrombosis. However, intracranial hemorrhage in patients with SWS is rare. We herein report a rare case of SWS with intracerebral hemorrhage derived from sinus thrombosis. CASE DESCRIPTION: A 62-year-old man suddenly fell into a coma and was admitted to our hospital. His neurological status was assessed as GCS 6 (E1V1M4) with right-sided hemiparesis. At birth, he had a right-sided facial port-wine stain typical of SWS that involved the ophthalmic division of the trigeminal nerve. Laboratory findings showed that he was dehydrated, and his serum D-dimer concentration was increased. Computed tomography revealed left thalamic hemorrhage with acute hydrocephalus and cortical calcification in the right occipital lobe. Magnetic resonance imaging displayed a vascular malformation of the right cerebral hemisphere consistent with SWS. Magnetic resonance venography showed steno-occlusion of the superior sagittal sinus, straight sinus, and left internal cerebral vein (ICV). Emergency ventricular drainage was performed. Seven days after surgery, his consciousness improved to GCS 14 (E4V4M6). Rehydration therapy was performed to prevent sinus thrombosis. DISCUSSION AND EVALUATION: His postoperative course was uneventful. Sudden congestion of the left ICV may have caused left thalamic hemorrhage. CONCLUSIONS: SWS with major sinus occlusion needs to be diagnosed with utmost caution in order to allow for preoperative neurological and radiological assessments. Springer International Publishing 2016-10-07 /pmc/articles/PMC5055513/ /pubmed/27795889 http://dx.doi.org/10.1186/s40064-016-3439-z Text en © The Author(s) 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. |
spellingShingle | Case Study Chonan, Masashi Suzuki, Yasuhiro Haryu, Shinya Mashiyama, Shoji Tominaga, Teiji Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title | Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title_full | Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title_fullStr | Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title_full_unstemmed | Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title_short | Sturge–Weber syndrome with intracerebral hemorrhage: a case report |
title_sort | sturge–weber syndrome with intracerebral hemorrhage: a case report |
topic | Case Study |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5055513/ https://www.ncbi.nlm.nih.gov/pubmed/27795889 http://dx.doi.org/10.1186/s40064-016-3439-z |
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