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 Sivashanmugam Dhandapani1, Sushanta K Sahoo1, Madhivanan Karthigeyan1, Vivek Gupta2 Departments of Neurosurgery1 and Radiology2, Postgraduate Institute of Medical Education & Research (PGIMER), Chandigarh, India
Correspondence: Dr. Dhandapani SS Department of Neurosurgery PGIMER Chandigarh E mail-
[email protected] Contact No. +917087008028
Abstract We describe the case of a young girl who presented with clinical triad of obesity, primary amenorrhea and central cord syndrome. The radiology unveiled pentad of hydrocephalus, empty sella syndrome, suprapineal recess diverticula, secondary Chiari malformation and cervical syringomyelia, masking an obscure tectal plate glioma. All of her symptoms improved after endoscopic third ventriculostomy and the tumor is being followed up.
Key Words: tectal glioma; hydrocephalus; empty sella; Chiari; syringomyelia; endoscopic third ventriculostomy
INTRODUCTION Tectal plate gliomas are benign indolent tumors, which usually present with features of raised intracranial pressure due to their close proximity causing obstruction of aqueduct. Headache with
visual disturbances are the frequent symptoms. In this report, we describe a case of pediatric tectal glioma who presented with atypical features of obesity, primary amenorrhoea and central cord syndrome with the below described radiological pentad, who improved symptomatically after endoscopic third ventriculostomy.
CASE REPORT A 14-year-old girl presented to our outpatient department with history of increased weight gain and paresthesias of bilateral upper limbs of 6 months duration. She had delayed appearance of secondary sexual characteristics and was amenorrheic. There was no headache or visual obscurations. On examination, she was obese and had early papilledema, upgaze palsy and dissociated-suspended sensory loss from C4-D4 dermatomes. Endocrinological workup revealed hypogonadotropic hypogonadism with low levels of follicular stimulating hormone and luteinising hormone. Serum thyroid stimulating hormone and prolactin was normal. Craniospinal magnetic resonance imaging (Fig. 1. A-D) showed triventricular hydrocephalus, empty sella, suprapineal recess outpouching into quadrigeminal cistern, secondary Chiari malformation and cervico-dorsal syringomyelia. On meticulous evaluation, there was a non-enhancing lesion suggestive of tectal plate glioma causing aqueductal stenosis. She underwent endoscopic third ventriculostomy, following which she was relieved of all her symptoms. At 6-months follow-up, she had normalization of gonadotropin levels, attained menarche, and amelioration of paresthesias. Follow-up imaging (Fig. 1. E,F) revealed resolution of most abnormalities. She is on regular clinico-radiological follow up for the tectal plate lesion.
DISCUSSION In children, 5% of brainstem tumors are located in the tectal plate.5 These are slow growing tumors and may remain asymptomatic for long period. Often, they manifest with hydrocephalus, visual disturbances and Parinaud’s syndrome causing considerable hardship.1,5 To the best of our knowledge, the ‘symptom-complex’ as in the present case, with triad of obesity, hypogonadotropic hypogonadism and central cord syndrome, and the radiological pentad of hydrocephalus, empty sella, suprapineal recess diverticula, and secondary Chiari malformation with cervico-dorsal syrinx has not been described in literature.
Herniation of third ventricle into the sella in cases of aqueductal stenosis and brain tumors may present with clinical features of endocrine abnormalities1. However, the association of empty sella syndrome with tectal glioma is unusual. Chiari malformation and syrinx secondary to hydrocephalus has been reported, with the management of hydrocephalus alone sufficient in reversing the clinical symptoms.
[2, 3, 5]
Secondary Chiari has been seen rarely with posterior fossa tumors. [6] Tedeschi et al. has reported cerebellar ataxia secondary to an expansion diverticulum of the suprapineal recess.[4] However, suprapineal recess diverticulum causing pressure on posterior fossa structures and causing secondary Chiari is a very rare phenomenon and has not been described earlier. The possible pathogenesis for the clinico-radiological manifestation in our case is detailed in Fig. 2. We believe that the primary inciting factor behind such a presentation without raised intracranial pressure is the increased compliance of third ventricular walls. It is plausible that in a yielding ventricular wall, the raising pressure within caused the cerebrospinal fluid (CSF) to track along pathways of least resistance (i.e.third ventricular recesses). The CSF herniation into sella turcica caused the empty sella syndrome. A similar pathophysiology led to the formation of diverticulum of the suprapineal recess causing tonsillar herniation and syringomyelia. Despite such a complex presentation, the management is focused on hydrocephalus, a treatable entity, and the indolent tumor is conservatively followed up with serial imaging. In our case, the patient was primarily evaluated by physicians for endocrine abnormality as she presented with weight gain and amenorrhoea. All the medical professionals need to be aware of this unusual clinical triad which can be reversed with a simple minimally invasive neurosurgical procedure (endoscopic third ventriculostomy).
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2. 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.17:103106 3. Hayhurst C, Osman-Farah J, Das K, Mallucci C. Initial management of hydrocephalus associated with Chiari malformation Type I-syringomyelia complex via endoscopic third ventriculostomy: an outcome analysis. J Neurosurg. 2008 Jun;108(6):1211-4. 4. 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 5. 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 119:50–53 6. Wu FZ, Fu JH, Chen JY, Lai PH (2010) Teaching neuroimages: acquired Chiari malformation with syringohydromyelia caused by posterior fossa tumor. Neurology 75:e59
Fig. 1. A-C) Pre-operative T2-weighted MRI showing hydrocephalus, herniation of third ventricle into sella and Chiari with cervico-dorsal syrinx. D) Gadolinium enhanced MRI shows small non-enhancing tectal plate glioma (white arrow). E-F) Post-operative T2 weighted MRI showing resolution of hydrocephalus and CSF flow across the Foramen magnum with regression of syrinx.
Fig 2. Flow chart for possible pathogenesis of the clinical triad and the radiological pentad
