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CC BY-NC-ND 4.0 · Asian J Neurosurg 2017; 12(04): 746-750
DOI: 10.4103/ajns.AJNS_231_16
Case Report

Subependymal giant cell astrocytoma: Associated hyperproteinorrhachia causing shunt failures and nonobstructive hydrocephalus - Report of successful treatment with long-term follow-up

Ekkehard Kasper
Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA
,
Yosef Laviv
Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA
,
Mohammed-Adeeb Sebai
Department of Surgery, Beth Israel Deaconess Medical Center, Division of Neurosurgery, Harvard Medical School, Boston, MA
,
Ning Lin
1   Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA
,
William Butler
1   Department of Neurosurgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA
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Subependymal giant cell astrocytomas (SEGAs) are histologically benign tumors most frequently associated with tuberous sclerosis complex (TSC). Despite their benign histopathological appearance, they may cause unfavorable outcomes due to their intraventricular location. Rarely, SEGA may be associated with hyperproteinorrhachia (high levels of proteins in the cerebrospinal fluid [CSF]), which causes malresorptive, communicating hydrocephalus; certainly, this scenario makes shunt obstruction likely in this patient population. In this report, we illustrate the case of hyperproteinorrhachia in an SEGA patient with known TSC, who presented repeatedly with shunt failure from proteinaceous shunt obstruction. Subsequent surgical resection of the main intraventricular lesion resulted in a dramatic drop in the CSF protein levels and has since prevented further shunt failures. Different treatment concepts and possible pathophysiology are discussed and the pertinent literature is reviewed.

Key-words:

Communicating hydrocephalus - hyperproteinorrhachia - subependymal giant cell tumor - tuberous sclerosis


Publication History

Article published online:
20 September 2022

© 2017. Asian Congress of Neurological Surgeons. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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