Literature Review

Giant Leaking Colloid Cyst Presenting with Aseptic Meningitis: Review of the Literature and Report of a Case Mehrdad Hosseinzadeh Bakhtevari1, Guive Sharifi1, Reza Jabbari1, Misagh Shafizad2, Mona Rezaei1, Mohammad Samadian1, Omidvar Rezaei1

Key words Aseptic meningitis - Colloid cysts - Hydrocephalus - Neuroendoscopy -

Abbreviations and Acronyms CSF: Cerebrospinal fluid CT: Computed tomography MRI: Magnetic resonance imaging From the 1Department of Neurosurgery, Loghman e Hakim Hospital, Shahid Beheshti University of Medical Sciences, Tehran and 2Department of Neurosurgery, Emam Khomeini Hospital, Mazandaran University of Medical Sciences, Sari, Iran To whom correspondence should be addressed: Mehrdad Hosseinzadeh Bakhtevari, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2015). http://dx.doi.org/10.1016/j.wneu.2015.06.064 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2015 Elsevier Inc. All rights reserved.

INTRODUCTION Colloid cysts (also called neuroepithelial cysts) are relatively rare intracranial lesions, located in the anterior aspect of the third ventricle. Approximately 1% of all brain tumors in adults are colloid cysts (7). Colloid cysts are slow growing and the initial onset of symptoms usually occurs between 20 and 50 years of age, although they have been reported in younger patients (2). Colloid cysts are histologically benign yet potentially dangerous tumors because they often generate nonlocalizing symptoms and, in rare instances, are associated with sudden death, even in children (4). The optimal management of colloid cysts has always been a debatable issue over the past decades. Treatment options include cerebrospinal fluid (CSF) diversion procedures, stereotactic aspiration, microsurgical resection, and endoscopic removal of the colloid cyst. Transcallosal or transcortical craniotomy has been the traditional approach for microsurgical

- BACKGROUND:

Colloid cysts are benign third ventricle lesions that need to be diagnosed correctly because of their association with sudden death. Chemical or aseptic meningitis is a rare presentation of a colloid cyst.

- METHODS:

We present a case of a 69-year-old man with fever, alteration of mental status, and meningismus. Microbiological examination of the cerebrospinal fluid revealed aseptic meningitis. Brain imaging revealed a third ventricular colloid cyst with hydrocephalus.

- RESULTS:

The tumor was resected via endoscopic intervention. There were no persistent operative complications related to the endoscopic procedure.

- CONCLUSIONS:

Chemical or aseptic meningitis is an unusual clinical manifestation of a colloid cyst, complicating the differential diagnosis, especially in the elderly.

resection of colloid cysts for many years. Because of its capability of accomplishing complete resection, the microsurgical approach has been considered to be the gold standard technique for the treatment of colloid cysts (16). However, even though several studies have favored microsurgery over other techniques, it is associated with significant morbidity and prolonged postoperative hospital stay. Treatment is recommended for most cysts that are 1 cm or larger, or those associated with symptoms or signs (16). Continued improvements in endoscopic techniques and instruments, together with good long-term results in patients treated endoscopically, have established endoscopy as an alternative to microsurgical techniques and may even set a new standard for treatment (8). Although most patients with colloid cysts typically present with a history of headaches, lasting days to years, sudden neurological deficits related to the obstruction of CSF flow at the level of the foramen of Monro caused by hydrocephalus have been described (5, 15). Chemical or aseptic meningitis has been reported as a result of the growth and leakage of dermoid cysts, which could result in vasospasm, infarction, and death (3).

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To our knowledge, aseptic meningitis secondary to rupture of a colloid cyst in the third ventricle has not been reported yet. We present a case of a 69-year-old man with alteration of mental status and aseptic meningitis associated with a ruptured third ventricular colloid cyst. MATERIALS AND METHODS A 69-year-old man was referred to our emergency department with severe headache, dizziness, and diminished mental status. His symptoms started the day before presentation and aggravated gradually. The patient’s past medical and psychological history were unremarkable. The patient was febrile (temperature ¼ 38 C) and could not speak fluently. Neurological examination revealed bilateral papilledema as a sign of increased intracranial pressure on fundoscopic ophthalmic examination; Kernig and Brudzinski signs were positive. His blood investigations revealed normal kidney, liver, and thyroid function. Urine drug screen was negative. At lumbar puncture, the CSF sample showed increased white blood cells (150 cells/mL) with 90% polymorphonuclear cells, increased protein (100 mg/dL) and normal glucose (70 mg/dL) levels, which supported aseptic meningitis.

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LITERATURE REVIEW MEHRDAD HOSSEINZADEH BAKHTEVARI ET AL.

CSF culture and Gram stain were negative for bacteria and fungi. Because of his presentation, a computed tomography (CT) scan of the brain was performed; this showed a large high-attenuation lesion in the anterior third ventricle, with severe hydrocephalus (Figure 1A). Magnetic resonance imaging (MRI) of the brain showed a giant (anteroposterior diameter, 5 cm; right to left diameter, 4 cm; and height, 3.5 cm) intraventricular mass, which was isointense on T1-weighted and hyperintense on T2weighted MRI, with no enhancement after gadolinium injection (Figure 1BeF). The cyst appeared to be ruptured and the cystic fluid was leaking into the right occipital horn of the ventricle. We named this picture, the “bucket sign” (Figure 1B,C). The patient underwent surgery via an endoscopic approach. The procedure was performed using general anesthesia with the patient in a supine position with the head elevated approximately 30 above the horizontal plane to minimize CSF egress through the endoscope. The patient received intravenous antibiotic prophylaxis before skin incision. An access point offering the most direct intraventricular path to the tumor site was selected on the nondominant (right) side of the head, 5e6 cm anterior to the coronal suture at the

GIANT LEAKING COLLOID CYST

hairline and 3e4 cm lateral to midline. A 3-cm linear skin incision was made parallel to the midline at the selected entry site, and a 1 cm diameter burr hole was then created on the frontal bone to permit access to the ventricular space; the dura was opened after coagulation and a huge third ventricular colloid cyst was detected after entering with the endoscope. We did not use neuronavigation in this case. Our hospital’s endoscope system is a Storz endoscope and the access catheter that we used was a 0-degree rigid lens endoscope (Karl Storz, Tuttlingen, Germany), with an outside diameter of 6 mm and a 2-mm working channel. After coagulation of the choroid plexus with bipolar diathermy, the cyst wall was coagulated and then perforated with a dissector. The yellowish gelatinous content of the cyst was evacuated with suction aspiration, which extracted a volume of approximately 50 mL (probably with some CSF in it), and the cyst wall remnants were extirpated with bipolar diathermy and sharp dissection. An externalized ventricular drain was placed at the time of surgery for postoperative pressure monitoring; this was removed on the second postoperative day because of normal intracranial pressure (Figure 2). There were no persistent

Figure 1. Brain computed tomography scan revealed a huge intraventricular mass with hydrocephaly (A). Brain magnetic resonance imaging showed a large third ventricle colloid cyst. T1-weighted (B) and fluid attenuation inversion recovery imaging (C) show the tail of ruptured contents coursing into the occipital horn of the lateral ventricle; we called this the bucket sign. Axial (D), sagittal (E), and coronal (F) images after gadolinium injection.

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operative complications related to the endoscopic procedure. The patient was discharged on the 7th postoperative day, without any neurological deficit, except for short-term memory loss, which improved gradually over 6e8 months. Pathological examination showed gelatinous content positive for periodic acidSchiff, with a fibrous wall with a single layer of columnar ciliated epithelium with attachment of normal choroid plexus, confirming the diagnosis of colloid cyst. Follow-up imaging revealed no recurrence after 3 years (Figure 3) and the patient has been symptom free during this period. DISCUSSION In this report, we presented a 69-year-old man with alteration of mental status and aseptic meningitis as the first indication of a symptomatic ruptured third ventricular colloid cyst. He did not mention any history of neurological complaints. To our knowledge, this study is the first report of a spontaneous ruptured colloid cyst of the third ventricle presenting with aseptic meningitis. Colloid cysts are histologically benign but potentially dangerous tumors and, if untreated, they may lead to sudden neurological deterioration and death. Headache occurs in 68%e100% of patients and is often the presenting symptom. Other symptoms of colloid cysts include ataxia and gait abnormalities, impaired mental status, nausea and/or vomiting, blurred vision, incontinence, and dizziness. The main signs are papilledema, gait disturbance, hyperreflexia, incoordination, and nistagmus (7). Colloid cysts commonly occur in a supratentorial location and meningitis is an unusual clinical manifestation of colloid cysts. Chemical or aseptic meningitis has been reported due to growth and leakage of dermoid cysts, which could be lethal (15). Unlike in the case of a ruptured dermoid cyst, when the disseminated fat globules in the subarachnoid space can be easily demonstrated by fat-sensitive magnetic resonance sequences, the diagnosis of chemical meningitis due to a leaking neurenteric cyst or an epidermoid cyst can be difficult on imaging studies alone and requires close correlation with the clinical and laboratory data (14).

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LITERATURE REVIEW MEHRDAD HOSSEINZADEH BAKHTEVARI ET AL.

Figure 2. Postoperative brain computed tomography (CT) scan revealed resection of the cyst with a remnant of the cyst wall. Upper images: brain CT scan 1 day after endoscopic surgery; lower images, brain CT scan 42 months postoperatively.

Motoyama et al. (12) reported the case of an 83-year-old man who presented with gait disturbance, dementia, and urinary incontinence that had progressed over 2 months. Brain CT revealed hydrocephalus caused by a well-demarcated, round, hyperdense mass in the third ventricle, which was not enhanced by contrast agent. Ten days after the initial evaluation, CT revealed that the cyst in the third ventricle had disappeared. MRI revealed spontaneous rupture of the lesion and remnants of the cyst wall anchored to the anterior roof of the third ventricle. Thereafter, the symptoms of hydrocephalus subsided. However, 6 months later the patient’s condition gradually deteriorated and the ventricles dilated, without any evidence of tumor regrowth (12). Acute bleeding within a colloid cyst of the third ventricle is a rare event, causing a

sudden increase in the cyst volume that may lead to acute hydrocephalus and rapid neurological deterioration. Ogbodo et al. (13) reported a case of spontaneous rupture of a hemorrhagic third ventricular colloid cyst and its management. A 77-year-old man presented with unsteady gait, incontinence, and gradually worsening confusion over a 3-week period. The findings from a brain CT scan were highly suggestive of a third ventricular colloid cyst with intraventricular rupture. He underwent excision of the cyst and histopathology confirmed the radiological diagnosis with evidence of hemorrhage within the cyst. A ventriculo-peritoneal shunt was performed for delayed hydrocephalus. Surgical management of these patients must include emergency ventriculostomy, followed by prompt surgical removal of the hemorrhagic cyst (13).

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GIANT LEAKING COLLOID CYST

Wang et al. (18) presented a 46-year-old man with a ruptured colloid cyst of the fourth ventricle extending into the subarachnoid space; the patient underwent suboccipital craniectomy and removal of the cyst. Postoperatively, the patient had a fever and, after lumbar puncture and CSF examination, aseptic meningitis was diagnosed (18). There are different modalities for treatment of colloid cysts. The optimum management of patients with asymptomatic colloid cysts of the third ventricle remains unclear. Annamali et al. (1) reported a case of an asymptomatic colloid cyst of the third ventricle in a 35-year-old man, which, on follow-up MRI at 15 months, appeared to have spontaneously resolved (1). A 65-year-old patient who was managed conservatively with neuroimaging surveillance showed spontaneous regression of the cyst 19 months after the initial diagnosis (6). Chemical meningitis can be treated. Kudesia et al. (10) reported iatrogenic aseptic meningitis in the postoperative period in their case, attributing it to spillage of the contents of the cyst (10). Patients diagnosed with asymptomatic colloid cysts can be cared for safely; observation and serial neuroimaging are recommended. If a patient becomes symptomatic (the cyst is enlarging or hydrocephalus develops), prompt neurosurgical intervention is necessary to prevent the occurrence of neurological decline from these benign tumors. Microsurgical resection of colloid cysts is associated with a higher rate of complete resection, lower rate of recurrence, and fewer reoperations than with endoscopic removal. However, the rate of morbidity is higher with microsurgery than with endoscopy (17). Sharifi et al. (16) reported on results of 71 endoscopic resections of colloid cysts and concluded that endoscopic resection of third ventricular colloid cysts in patients without hydrocephalus seems to be feasible, effective, and not contraindicated. In a retrospective study in which 77 cases of primary endoscopic resection were identified, of which 20 resections were performed in patients with an incidental diagnosis and 57 in symptomatic individuals, Margetis et al. (11) compared the presenting characteristics and surgical

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GIANT LEAKING COLLOID CYST

Figure 3. Follow-up magnetic resonance imaging 1 month (A) and 42 months postoperatively showed no recurrence. (A) T1-weighted image with gadolinium; (B) axial T1-weighted image; (C) axial fluid attenuation inversion recovery image; and (D) coronal T1-weighted image.

outcomes between the incidental and symptomatic groups. Their conclusion was that age and cyst diameter were not correlated with the absence or presence of symptoms in patients with a colloid cyst of the third ventricle. Operative results were highly favorable in both groups and did not reveal a higher risk of morbidity in the patients presenting with an incidental lesion. The results support endoscopic resection as a legitimate therapeutic option for patients with incidental colloid cysts. Generalization of the operative results should be made cautiously because this is a limited series and the results may depend on the degree of neuroendoscopic experience (11). Hoffman et al. (9), in their review of 67 procedures for endoscopic resection of

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colloid cysts, concluded that endoscopic colloid cyst resection results in a low overall recurrence rate. Immediate postoperative MRI was insufficient for assessing the degree of resection and was a poor predictor of recurrence. Ablation of cyst remnants, rather than total removal, is associated with a significantly higher rate of recurrence. The primary goal of endoscopic surgery should, therefore, be removal of all cyst contents and wall remnants. Aseptic meningitis, or sterile meningitis, is a condition in which meninges become inflamed and a pyogenic bacterial source is not to blame. Meningitis is diagnosed on a history of characteristic symptoms and certain examination findings, such as the Kernig sign. Investigations should show an

increase in the number of leukocytes present in the CSF, obtained via lumbar puncture (normal is

Giant Leaking Colloid Cyst Presenting with Aseptic Meningitis: Review of the Literature and Report of a Case.

Colloid cysts are benign third ventricle lesions that need to be diagnosed correctly because of their association with sudden death. Chemical or asept...
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