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Case Report

Colloid Cyst of the Third Ventricle Presenting with Features of Terson’s Syndrome

Mary K. Jacob, Anand S. K., Prasad George1

ABSTRACT Access this article online Website: This report describes a middle‑aged man presenting to the ophthalmologist with history of www.meajo.org seeing floaters before both eyes since 2‑weeks duration. A history of intermittent headache DOI: and dizziness of recent onset was elicited on questioning. Ocular examination showed bilateral 10.4103/0974-9233.142275 early papilloedema and mild vitreous hemorrhage. Brain computed tomography (CT) disclosed Quick Response Code: features suggestive of colloid cyst of the third ventricle in the region of foramen of Monro with moderate hydrocephalus. Emergency craniotomy and excision of the cyst was done, and the patient is doing well for the last 18 months after the surgical intervention. The mechanism of this presentation, importance of early investigations, and timely intervention are highlighted in order to avoid serious neurological sequelae. The literature was extensively reviewed for atypical presentations of intraventricular colloid cyst.

Key words: Colloid Cyst, Obstructive Hydrocephalus, Third Ventricle, Vitreous Haemorrhage

INTRODUCTION disclosed the presence of a colloid cyst in the third ventricle with moderate hydrocephalus. Retrospectively, it can be postulated olloid cyst is a benign neoplasm located in the rostral part that the presentation appears to be a variant of Terson’s syndrome. Cof third ventricle accounting for about 2% of all intracranial Our case is unique in its presentation. To the best of our tumors.1 It is believed to be originating from the diencephalic knowledge, this remains the first reported case of colloid cyst vesicle or from the persistence of the embryonic paraphysis. presenting with features suggestive of Terson’s syndrome. Occasionally, they are seen to be originating from locations like chiasma, sellar area, brain convexity, lateral ventricle, septum CASE REPO RT pellucidum, brain stem, and subarachnoid space.1,2 Histologically, these lesions are composed of epithelial cells and goblet cells, the A 41‑year‑old gentleman presented to the eye clinic complaining latter secreting proteinaceous material that accumulate in the of seeing floaters before both eyes since 2 weeks. On questioning, cyst.3.In more than 75% of cases, headache associated with nausea he admitted getting intermittent dull headache of mild to and vomiting are the presenting symptoms.4 Unusual presentations moderate intensity and dizziness on and off while walking since like gait disturbance, temporary loss of consciousness, drop attacks, 2 months. There was no associated nausea or vomiting or other quadriparesis, spasmodic torticollis, pseudoeclampsia, aseptic neurological symptoms. He was detected to be a hypertensive meningitis, blurred vision, psychiatric problems, dementia, and few months ago and was on treatment with lisinopril 5‑mg daily. so forth have been reported in the literature.1,5,6 Ocular examination revealed a best‑corrected Snellen’s visual This report describes a middle‑aged man presenting to the acuity of 6/6 p in both eyes. Anterior segment examination ophthalmologist complaining of seeing floaters before both eyes showed exotropia of about 45° in left eye that was alternating. and was found to have bilateral early vitreous hemorrhage and Rest of anterior segment examination including intraocular papilloedema. Computed tomography (CT) scan of the brain pressure was normal. Fundoscopy of the right eye revealed early

Departments of Ophthalmology and 1Medicine, Nizwa Hospital, Ministry of Health, Sultanate of Oman Corresponding Author: Dr. Mary K. Jacob, Department of Ophthalmology, Nizwa Hospital, P.B 1222, Nizwa‑611, Sultanate of Oman. E‑mail: [email protected]

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Jacob, et al.: Colloid Cyst of the Third Ventricle Presenting as Floaters

vitreous hemorrhage. The disc showed slight blurring of margins associated with peripapillary retinal hemorrhages [Figure 1a]. Fundus examination of the left eye showed early vitreous hemorrhage and blurring of disc margin. There was a small area of preretinal hemorrhage inferior to the disc [Figure 1b]. B scan ultrasound also confirmed early vitreous hemorrhage.

A diagnosis of bilateral early disc edema with mild vitreous hemorrhage was made, and he was investigated. Contrast‑enhanced a b CT scan of brain showed a well‑defined oval nonenhancing Figure 1: (a) Fundus picture of right eye showing blurred disc margin (arrow head) hyperdense lesion measuring 2 × 1.9 × 2.3 cm at the and peripapillary retina hemorrhage (up arrow). (b) Blurred disc margin (arrow head) and an area of small preretinal hemorrhage (up arrow) inferior to the disc anterosuperior aspect of third ventricle bulging through the right foramen of Monro and compressing the left foramen of Monro causing dilatation of both lateral ventricles with cerebrospinal fluid (CSF) seepage [Figure 2]. Fourth ventricle appeared normal. Moderate 0.8‑cm midline shift was also seen to the left side.

A presumptive diagnosis of colloid cyst causing moderate obstructive hydrocephalus was made, and he was referred urgently to the department of neurosurgery. He underwent emergency craniotomy and cyst excision. Histopathological examination of the excised tissue showed cyst contents lined by cyst wall lined by ciliated cuboidal epithelium resting on fibrocollagenous tissue consistent with colloid cyst. Postoperative CT scan showed pneumocephalus and subdural collection in the right frontotemporal region that resolved spontaneously after few days.

The patient improved symptomatically following surgery. On Figure 2: Contrast-enhanced brain CT scan showing a well-defined round hyperdense lesion (arrow head) in the roof of the third ventricle. Lesion is causing obstruction follow‑up after 1 month, he reported complete resolution of of the outlet foramina of the lateral ventricle and causing moderate hydrocephalus. floaters and headache. CT features suggestive of colloid cyst

Fundus evaluation showed resolving vitreous hemorrhage and disc edema. Follow‑up after 3 months showed complete resolution of disc edema and vitreous hemorrhage [Figure 3c and d]. He is being followed up closely for recurrence of symptoms. He has remained asymptomatic for the last 18 months after the intervention.

DISCUSSION c d Figure 3: (c and d) Fundus picture of right and left eye showing the complete resolution of disc edema and retinal hemorrhages after surgical excision of the Colloid cysts are rare intracranial tumors mostly seen in the colloid cyst third ventricle, first described by Wallmann in 1858.7 They are supposed to originate from either the diencephalic vesicle or loss of consciousness, drop attacks, quadriparesis, hemorrhage, remnant of embryonic paraphysis.6,8 They usually have a sporadic CSF rhinorrhea, hypopituitarism, diabetes insipidus, spasmodic origin though few familial cases have also been reported.1,2,5 torticollis, pseudoeclampsia, aseptic meningitis, blurred vision, Presentation is usually at the age of 30-50 years.8 Headache is psychiatric problems, dementia, and so forth have been reported usually the presenting symptom in 68-100% of cases, which in the literature.1,5,6Other reported unusual visual presentations is typically severe, intermittent, and relieved by lying flat.4,5,7,8 related to colloid cyst are normal tension glaucoma,9 nonspecific visual disturbance,10 and superonasal quadrantic visual field defect.11 Classic symptoms of colloid cyst are increased intracranial pressure, sudden onset of headache, neuropsychiatric Foramen of Monro can act like an anatomical choke point of manifestations, normal pressure hydrocephalus, coma, and sudden the third ventricle. Usually, a pedunculated colloid cyst at this death.1 Unusual presentations like gait disturbance, temporary location disturbs the circulation of cerebrospinal fluid and

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Jacob, et al.: Colloid Cyst of the Third Ventricle Presenting as Floaters

can cause acute obstruction of the third ventricle resulting in REFERENCES intracranial hemorrhage, brain herniation, coma, and death.4,5,7 Hence, early recognition and prompt management is mandatory 1. Hamlat A, Casallo‑Quilano C, Saikali S, Adn M, Brassier G. to decrease the mortality and morbidity. Huge colloid cyst: Case report and review of unusual forms. Acta Neurochir (Wein) 2004;146:397‑401. 2. Kumar V, Behari S, Kumar Singh R, Jain M, Jaiswal AK, Jain VK. Our case is unique as the patient sought medical help with Paediatric colloid cyst of the third ventricle: Management floaters as presenting symptom due to early vitreous hemorrhage considerations. Acta Neurochir (Wein) 2010;152:451‑61. without any classical symptoms of raised intracranial pressure. 3. Carrasio R, Pascual JM, Medina‑Lopez D, Burdaspal‑Moratilla A. History of intermittent headache and giddiness was elicited Acute haemhorage in a colloid cyst of the third ventricle: A rare case of sudden deterioration. Surg Neurol Int 2012;3:24. only on questioning. This combination of papilledema, vitreous 4. Sharp MC, Macarther DC. An unusual presentation of hemorrhage, and small preretinal hemorrhage in the presence colloid cyst–implications for lifestyle advice. Br J Neurosurg of colloid cyst may be considered as a variant of Terson’s 2011;25:284‑5. syndrome. 5. Algin O, Ozmen E, Arslan H. Radiologic manifestation of colloid cyst: A pictorial essay. Can Assoc Radiol J 2013;64:56‑60. 6. Dhar H. Colloid cyst of the third ventricle presenting as The association of vitreous hemorrhage with any form of pseudoeclampsia. Arch Gynecol Obstet 2009;280:1019‑21. intracranial hemorrhage was named as Terson’s syndrome after 7. Spears RC. Colloid cyst headache. Curr Pain Headache Rep Albert Terson, a French ophthalmologist. Later, the definition 2004;8:297‑300. has been expanded by some authors to include intraretinal 8. Armao D, Castillo M, Chen H, Kwock L. Colloid cyst of the hemorrhage.12,13 It has been postulated that Terson’s syndrome third ventricle: Imaging‑pathologic correlation. AJNR Am J Neuroradiol 2000;21:1470‑7. may be related to acute elevation of intracranial pressure, 9. Patel DK, Ali NA, Iqbal T, Subrayan V. Colloid cyst of the independent of its causes and has been reported with similar third ventricle mimicking normal tension glaucoma. Ann incidence in severe brain injury.13 Ophthalmol (Skokie) 2008;40:177‑9. 10. Lowenstein A, Gaton DD, Reider‑Grasswasser I, Bracha R, In our patient, it may be hypothesized that the acute rise Lazar M. Deformed optic chiasma and colloid cyst in a patient with visual disturbances. Metab Pediatr Syst Ophthalmol in intracranial pressure resulting in hydrocephalus due to 1993;16:9‑11. obstruction of the foramen of Monro by the colloid cyst might 11. Killer HE, Flammer J, Wicki B, Laeng RH. Acute asymmetric have resulted in acute rise in intraocular venous pressure upper nasal quadrantanopsia caused by a chiasmal colloid resulting in rupture of peripapillary and retinal capillaries cyst in a patient with multiple sclerosis and bilateral retrobulbar along with vitreous hemorrhage. Intraocular bleeding was neuritis. Am J Ophthalmol 2001;132:286‑8. 12. Choudhari KA, Pherwani AA, Gray WJ. Terson’s Syndrome minimal as the patient presented at an early stage. Relatively as the sole initial presentation of aneurismal rupture. accurate diagnosis can be made by CT or magnetic resonance Br J Neurosurg 2003;17:355‑67. imaging (MRI) scans. Total resection of the colloid cyst is 13. Medele RJ, Stummer W, Mueller AJ, Steiger HJ, Reulen HJ. mandatory to prevent recurrence.1 Terson’s syndrome in subarachnoid hemhorrage and severe brain injury accompanied by acutely raised intracranial pressure. J Neurosurg 1998;88:851‑4. A high index of suspicion and detailed evaluation of patients

with no other obvious predisposing cause for bilateral vitreous Cite this article as: Jacob MK, Anand SK, George P. Colloid Cyst of the Third hemorrhage like diabetes, trauma, or blood disorders may Ventricle Presenting with Features of Terson's Syndrome. Middle East Afr J help in the early diagnosis and management of potentially Ophthalmol 2014;21:344-6. life‑threatening conditions of the brain as in this case. Source of Support: Nil, Conflict of Interest: None declared.

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