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Child's Nervous System
Published in: Child's Nervous System 2/2017

01-02-2017 | Case Report

Tectal glioma presenting with clinical triad of obesity, amenorrhea and central cord syndrome with radiological pentad of hydrocephalus, empty sella, suprapineal diverticula, Chiari and syrinx

Authors: Sivashanmugam Dhandapani, Madhivanan Karthigeyan, Sushanta K Sahoo, Vivek Gupta

Published in: Child's Nervous System | Issue 2/2017

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Abstract

Background

Tectal gliomas (TG) are slow-growing tumors and generally present with features of increased intracranial pressure.

Case description

We describe an unusual case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled a pentad of hydrocephalus, empty sella, suprapineal recess diverticulum, secondary Chiari malformation and cervical syringomyelia, masking a relatively obscure tectal plate glioma. She was subjected to endoscopic third ventriculostomy (ETV). All of her symptoms improved after ETV and the tumor is being followed up. The possible pathogenesis of such a highly atypical clinico-radiological presentation is described.

Conclusion

This unusual syndromic presentation of tectal glioma without clinical features of increased intracranial pressure is probably due to increased compliance of third ventricular walls, and is relieved with ETV.
Literature
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Dhandapani M, Gupta S, Dhandapani S, Kaur P, Samra K, Sharma K, Dolma K, Mohanty M, Singla N, Gupta SK (2015) Study of factors determining caregiver burden among primary caregivers of patients with intracranial tumors. Surg Neurol Int. 6:160
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Dhandapani S, Srinivasan A (2016) Contiguous triple spinal dysraphism associated with Chiari malformation Type II and hydrocephalus: an embryological conundrum between the unified theory of Pang and the unified theory of McLone. J Neurosurg Pediatr. 17:103-6
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Hayhurst C, Osman-Farah J, Das K, Mallucci C (2008) Initial management of hydrocephalus associated with Chiari malformation type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg 108:1211–1214
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Tedeschi E, Rapanà A, Elefante A, De Liso M, Morrone R, Iaccarino C (2013) Expansion diverticulum of the suprapineal recess causing cerebellar ataxia. A case report. Neuroradiol J 26:163–167
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Vega RA, Harrison JF, Scott GR (2014) Tectal plate glioma: lessons learned from a patient with hydrocephalus, Chiari malformation-1 and concomitant aqueductal stenosis. Clin Neurol Neurosurg 119:50–53CrossRefPubMed
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Wu FZ, Fu JH, Chen JY, Lai PH (2010) Teaching neuroimages: acquired Chiari malformation with syringohydromyelia caused by posterior fossa tumor. Neurology 75:e59CrossRefPubMed
Metadata
Title
Tectal glioma presenting with clinical triad of obesity, amenorrhea and central cord syndrome with radiological pentad of hydrocephalus, empty sella, suprapineal diverticula, Chiari and syrinx
Authors
Sivashanmugam Dhandapani
Madhivanan Karthigeyan
Sushanta K Sahoo
Vivek Gupta
Publication date
01-02-2017
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 2/2017
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-016-3266-0

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