Academic Journal
Pseudotumor cerebri syndrome in a child with Alagille syndrome: intracranial pressure dynamics and treatment outcome after ventriculoperitoneal shunting
| العنوان: | Pseudotumor cerebri syndrome in a child with Alagille syndrome: intracranial pressure dynamics and treatment outcome after ventriculoperitoneal shunting |
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| المؤلفون: | Polemikos, Manolis, Hermann, Elvis J., Heissler, Hans E., Hartmann, Hans, Krauss, Joachim K. |
| المصدر: | http://lobid.org/resources/99370677088806441#!, 37(9):2899-2904. |
| سنة النشر: | 2021 |
| المجموعة: | Publisso (ZB MED-Publikationsportal Lebenswissenschaften) |
| مصطلحات موضوعية: | Idiopathic intracranial hypertension, Humans [MeSH], Papilledema [MeSH], Treatment Outcome [MeSH], Pseudotumor Cerebri/surgery [MeSH], Pseudotumor Cerebri/complications [MeSH], Male [MeSH], Pseudotumor cerebri, Alagille Syndrome/complications [MeSH], Intracranial Pressure [MeSH], Alagille Syndrome/surgery [MeSH], Ventriculoperitoneal shunting, Alagille syndrome, Case Report, Child, Preschool [MeSH] |
| الوصف: | Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. The occurrence of pseudotumor cerebri syndrome (PTCS) in patients with AS has been reported only exceptionally. Owning to its rarity and a mostly atypical presentation, the diagnosis and natural history of affected patients remain uncertain. We report an atypical case of PTCS in a 4-year-old boy with a known history of AS who presented with bilateral papilledema (PE) on a routine ophthalmological examination. Visual findings deteriorated after treatment with acetazolamide. Continuous intracranial pressure (ICP) monitoring was then utilized to investigate ICP dynamics. Successful treatment with resolution of PE was achieved after ventriculoperitoneal shunting but relapsed due to growth-related dislocation of the ventricular catheter. This report brings new insights into the ICP dynamics and the resulting treatment in this possibly underdiagnosed subgroup of PTCS patients. It also demonstrates that ventriculoperitoneal shunting can provide long-term improvement of symptoms for more than 10 years. |
| نوع الوثيقة: | article in journal/newspaper |
| اللغة: | English |
| Relation: | https://repository.publisso.de/resource/frl:6451087; https://doi.org/10.1007/s00381-021-05043-9; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8423640/ |
| DOI: | 10.1007/s00381-021-05043-9 |
| الاتاحة: | https://repository.publisso.de/resource/frl:6451087 https://doi.org/10.1007/s00381-021-05043-9 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8423640/ |
| Rights: | https://creativecommons.org/licenses/by/4.0/ |
| رقم الانضمام: | edsbas.9D20125F |
| قاعدة البيانات: | BASE |
| DOI: | 10.1007/s00381-021-05043-9 |
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