|Year : 2014 | Volume
| Issue : 4 | Page : 344-346
Colloid Cyst of the Third Ventricle Presenting with Features of Terson's Syndrome
Mary K Jacob1, SK Anand1, Prasad George2
1 Department of Ophthalmology, Nizwa Hospital, Ministry of Health, Sultanate of Oman
2 Department of Medicine, Nizwa Hospital, Ministry of Health, Sultanate of Oman
|Date of Web Publication||4-Oct-2014|
Mary K Jacob
Department of Ophthalmology, Nizwa Hospital, P.B 1222, Nizwa 611, Sultanate of Oman
Source of Support: None, Conflict of Interest: None
| Abstract|| |
This report describes a middle-aged man presenting to the ophthalmologist with history of seeing floaters before both eyes since 2-weeks duration. A history of intermittent headache and dizziness of recent onset was elicited on questioning. Ocular examination showed bilateral early papilloedema and mild vitreous hemorrhage. Brain computed tomography (CT) disclosed 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.
Keywords: Colloid Cyst, Obstructive Hydrocephalus, Third Ventricle, Vitreous Haemorrhage
|How to cite this article:|
Jacob MK, Anand S K, George P. Colloid Cyst of the Third Ventricle Presenting with Features of Terson's Syndrome. Middle East Afr J Ophthalmol 2014;21:344-6
|How to cite this URL:|
Jacob MK, Anand S K, George P. Colloid Cyst of the Third Ventricle Presenting with Features of Terson's Syndrome. Middle East Afr J Ophthalmol [serial online] 2014 [cited 2019 Aug 19];21:344-6. Available from: http://www.meajo.org/text.asp?2014/21/4/344/142275
| Introduction|| |
Colloid cyst is a benign neoplasm located in the rostral part of third ventricle accounting for about 2% of all intracranial tumors.  It is believed to be originating from the diencephalic vesicle or from the persistence of the embryonic paraphysis. Occasionally, they are seen to be originating from locations like chiasma, sellar area, brain convexity, lateral ventricle, septum pellucidum, brain stem, and subarachnoid space. , Histologically, these lesions are composed of epithelial cells and goblet cells, the latter secreting proteinaceous material that accumulate in the cyst. . In more than 75% of cases, headache associated with nausea and vomiting are the presenting symptoms.  Unusual presentations like gait disturbance, temporary loss of consciousness, drop attacks, quadriparesis, spasmodic torticollis, pseudoeclampsia, aseptic meningitis, blurred vision, psychiatric problems, dementia, and so forth have been reported in the literature. ,,
This report describes a middle-aged man presenting to the ophthalmologist complaining of seeing floaters before both eyes and was found to have bilateral early vitreous hemorrhage and papilloedema. Computed tomography (CT) scan of the brain disclosed the presence of a colloid cyst in the third ventricle with moderate hydrocephalus. Retrospectively, it can be postulated that the presentation appears to be a variant of Terson's syndrome. Our case is unique in its presentation. To the best of our knowledge, this remains the first reported case of colloid cyst presenting with features suggestive of Terson's syndrome.
| Case report|| |
A 41-year-old gentleman presented to the eye clinic complaining of seeing floaters before both eyes since 2 weeks. On questioning, he admitted getting intermittent dull headache of mild to moderate intensity and dizziness on and off while walking since 2 months. There was no associated nausea or vomiting or other neurological symptoms. He was detected to be a hypertensive few months ago and was on treatment with lisinopril 5-mg daily.
Ocular examination revealed a best-corrected Snellen's visual acuity of 6/6 p in both eyes. Anterior segment examination showed exotropia of about 45° in left eye that was alternating. Rest of anterior segment examination including intraocular pressure was normal. Fundoscopy of the right eye revealed early
vitreous hemorrhage. The disc showed slight blurring of margins associated with peripapillary retinal hemorrhages [Figure 1]a. 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 1]b. B scan ultrasound also confirmed early vitreous hemorrhage.
|Figure 1: (a) Fundus picture of right eye showing blurred disc margin (arrow head) 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|
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A diagnosis of bilateral early disc edema with mild vitreous hemorrhage was made, and he was investigated. Contrast-enhanced CT scan of brain showed a well-defined oval nonenhancing hyperdense lesion measuring 2 × 1.9 × 2.3 cm at the 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.
|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 of the outlet foramina of the lateral ventricle and causing moderate hydrocephalus. CT features suggestive of colloid cyst|
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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 follow-up after 1 month, he reported complete resolution of floaters and headache.
Fundus evaluation showed resolving vitreous hemorrhage and disc edema. Follow-up after 3 months showed complete resolution of disc edema and vitreous hemorrhage [Figure 3]c and d]. He is being followed up closely for recurrence of symptoms. He has remained asymptomatic for the last 18 months after the intervention.
|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 cyst|
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| Discussion|| |
Colloid cysts are rare intracranial tumors mostly seen in the third ventricle, first described by Wallmann in 1858.  They are supposed to originate from either the diencephalic vesicle or remnant of embryonic paraphysis. , They usually have a sporadic origin though few familial cases have also been reported. ,, Presentation is usually at the age of 30-50 years.  Headache is usually the presenting symptom in 68-100% of cases, which is typically severe, intermittent, and relieved by lying flat. ,,,
Classic symptoms of colloid cyst are increased intracranial pressure, sudden onset of headache, neuropsychiatric manifestations, normal pressure hydrocephalus, coma, and sudden death.  Unusual presentations like gait disturbance, temporary loss of consciousness, drop attacks, quadriparesis, hemorrhage, CSF rhinorrhea, hypopituitarism, diabetes insipidus, spasmodic torticollis, pseudoeclampsia, aseptic meningitis, blurred vision, psychiatric problems, dementia, and so forth have been reported in the literature. ,, Other reported unusual visual presentations related to colloid cyst are normal tension glaucoma,  nonspecific visual disturbance,  and superonasal quadrantic visual field defect. 
Foramen of Monro can act like an anatomical choke point of the third ventricle. Usually, a pedunculated colloid cyst at this location disturbs the circulation of cerebrospinal fluid and can cause acute obstruction of the third ventricle resulting in intracranial hemorrhage, brain herniation, coma, and death. ,, Hence, early recognition and prompt management is mandatory to decrease the mortality and morbidity.
Our case is unique as the patient sought medical help with floaters as presenting symptom due to early vitreous hemorrhage without any classical symptoms of raised intracranial pressure. History of intermittent headache and giddiness was elicited only on questioning. This combination of papilledema, vitreous hemorrhage, and small preretinal hemorrhage in the presence of colloid cyst may be considered as a variant of Terson's syndrome.
The association of vitreous hemorrhage with any form of intracranial hemorrhage was named as Terson's syndrome after Albert Terson, a French ophthalmologist. Later, the definition has been expanded by some authors to include intraretinal hemorrhage. , It has been postulated that Terson's syndrome may be related to acute elevation of intracranial pressure, independent of its causes and has been reported with similar incidence in severe brain injury. 
In our patient, it may be hypothesized that the acute rise in intracranial pressure resulting in hydrocephalus due to obstruction of the foramen of Monro by the colloid cyst might have resulted in acute rise in intraocular venous pressure resulting in rupture of peripapillary and retinal capillaries along with vitreous hemorrhage. Intraocular bleeding was minimal as the patient presented at an early stage. Relatively accurate diagnosis can be made by CT or magnetic resonance imaging (MRI) scans. Total resection of the colloid cyst is mandatory to prevent recurrence. 
A high index of suspicion and detailed evaluation of patients with no other obvious predisposing cause for bilateral vitreous hemorrhage like diabetes, trauma, or blood disorders may help in the early diagnosis and management of potentially life-threatening conditions of the brain as in this case.
| References|| |
|1.||Hamlat A, Casallo-Quilano C, Saikali S, Adn M, Brassier G. 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. Paediatric colloid cyst of the third ventricle: Management considerations. Acta Neurochir (Wein) 2010;152:451-61. |
|3.||Carrasio R, Pascual JM, Medina-Lopez D, Burdaspal-Moratilla A. Acute haemhorage in a colloid cyst of the third ventricle: A rare case of sudden deterioration. Surg Neurol Int 2012;3:24. |
|4.||Sharp MC, Macarther DC. An unusual presentation of colloid cyst-implications for lifestyle advice. Br J Neurosurg 2011;25:284-5. |
|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 pseudoeclampsia. Arch Gynecol Obstet 2009;280:1019-21. |
|7.||Spears RC. Colloid cyst headache. Curr Pain Headache Rep 2004;8:297-300. |
|8.||Armao D, Castillo M, Chen H, Kwock L. Colloid cyst of the third ventricle: Imaging-pathologic correlation. AJNR Am J Neuroradiol 2000;21:1470-7. |
|9.||Patel DK, Ali NA, Iqbal T, Subrayan V. Colloid cyst of the third ventricle mimicking normal tension glaucoma. Ann Ophthalmol (Skokie) 2008;40:177-9. |
|10.||Lowenstein A, Gaton DD, Reider-Grasswasser I, Bracha R, Lazar M. Deformed optic chiasma and colloid cyst in a patient with visual disturbances. Metab Pediatr Syst Ophthalmol 1993;16:9-11. |
|11.||Killer HE, Flammer J, Wicki B, Laeng RH. Acute asymmetric upper nasal quadrantanopsia caused by a chiasmal colloid cyst in a patient with multiple sclerosis and bilateral retrobulbar neuritis. Am J Ophthalmol 2001;132:286-8. |
|12.||Choudhari KA, Pherwani AA, Gray WJ. Terson's Syndrome as the sole initial presentation of aneurismal rupture. Br J Neurosurg 2003;17:355-67. |
|13.||Medele RJ, Stummer W, Mueller AJ, Steiger HJ, Reulen HJ. Terson's syndrome in subarachnoid hemhorrage and severe brain injury accompanied by acutely raised intracranial pressure. J Neurosurg 1998;88:851-4. |
[Figure 1], [Figure 2], [Figure 3]