Colloid cyst of the third ventricle: a clinical review of 39 cases

Colloid cyst of the third ventricle: a clinical review of 39 cases

JOCN-25.QXD 6/21/01 9:03 AM Page 328 Journal of Clinical Neuroscience (2001) 8(4), 328–331 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.08...

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Journal of Clinical Neuroscience (2001) 8(4), 328–331 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0800, available online at http://www.idealibrary.com on

Clinical study

Colloid cyst of the third ventricle: a clinical review of 39 cases R. L. Jeffree MBBS MSc, M. Besser FRACS FRCSC FACS Department of Neurosurgery, Royal Prince Alfred Hospital, Missenden Rd, Camperdown, NSW 2050, Australia and the University of Sydney

Summary This paper is a retrospective review of all patients treated for a colloid cyst of the third ventricle at Royal Prince Alfred Hospital over an 18-year consecutive period. Our experience is fairly typical compared with other published series. Colloid cysts made up 1.6% of tumours treated by the neurosurgical unit. Patients presented with non-specific neurological symptoms and signs, commonly suggestive of raised intracranial pressure. Microsurgical excision was carried out via the transcallosal route in 34 cases and the transfrontal approach in four cases. There was no apparent difference in results from the different surgical approaches. In 86% of patients reviewed after more than 6 months good long term outcome was achieved. These benign tumours can be safely cured if the diagnosis is made early and the cyst removed by careful microsurgical techniques. © 2001 Harcourt Publishers Ltd Keywords: colloid cyst, familial occurrence, third ventricle, transcallosal surgery, management outcome

INTRODUCTION In spite of their rarity, colloid cysts remain one of the most interesting cerebral tumours. They are curable yet potentially fatal, the etiology is unknown and the management is both challenging and controversial. Colloid cysts make up between 0.5% and 2% of brain tumours.1,2,3 They are an infrequent yet well recognised cause of sudden death4,5,6 but may also be found incidentally at postmortem.7 Historically it was thought that colloid cysts arise from the paraphysis,8 a rudimentary embryonic structure in the roof of the third ventricle, but several recent studies show immunohistochemical evidence that the epithelium is endodermal in origin.9,10,11 Although these cysts are probably developmental, the mean age at presentation is in the 3rd and 4th decade.3,12 Current management of colloid cysts includes observation or shunting alone,13,14 stereotactic aspiration13,15,16 or excision using endoscopic17 or microsurgical techniques. The relative success and safety of the various approaches remains controversial. There is a risk of recurrence and catastrophic hydrocephalus if the cyst is left in situ14 but surgery has associated morbidity and mortality. Microsurgical excision can be carried out by either a transcallosal approach,3,12,20,21 with specific risks of disconnection syndrome and memory problems,22,23,24 or a transfrontal approach which involves a breach of normal cortex and a theoretical increased risk of seizures.1,12,12,22,25,26 Here we report a retrospective review of all patients treated for a colloid cyst of the third ventricle at the Royal Prince Alfred Hospital between 1979 and 1997. The patients’ notes were reviewed and their General Practitioners contacted to determine presenting condition, treatment, morbidity and current functional and medical status. PATIENT CHARACTERISTICS A search of the hospital computerised database identified 39 patients who were treated for a colloid cyst after the purchase of Received 6 June 2000 Accepted 21 July 2000 Correspondence to: Associate-Professor M. Besser, Suite 146, R.P.A.H Medical Center, 100 Carillon Avenue, Newtown NSW 2042, Australia. Tel.:;61 2 519 9669; Fax:;61 2 517 2503

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the computed tomography (CT) scanner in 1979. The rate of presentation did not change over the 18 years of the study. These 39 patients represent 1.6% of all patients with brain tumours and 3.6% of patients with benign brain tumours presenting to the hospital during this time. This is similar to Hernesniemi and Leivo’s report that patients with colloid cysts make up 2% of tumour patients in eastern Finland.3 The patients ranged in age from 21 to 63 years (mean 39.9 years) at the time of diagnosis. There were 20 women. Although some series report a preponderance of males,16,21 others have equal numbers11,13 as we do. Two of the patients were siblings, a 22 year old male with an incidental colloid cyst and his sister who presented with drop attacks several years later at the age of 24. No other family members have symptoms suggestive of a colloid cyst. There are several other reports of first degree relatives with colloid cysts, including a father and son,27 a mother and son,16 three sisters,28 two brothers29 and identical twins.30 This is evidence that the etiology is genetic or developmental.

PRESENTING SYMPTOMS AND SIGNS The most common presentation was with symptoms or signs of raised intracranial pressure. Headache, associated with nausea and vomiting, neurological disturbance or a change in conscious state was the presenting symptom in 17 patients (44%). The remaining patients had non-specific symptoms (Table 1). In three cases the

Table 1

Symptoms on presentation

Symptom Headache or ‘head pressure’ Nausea or vomiting Visual disturbance Confusion, memory loss or cognitive decline Disturbance of balance or gait Collapse Incidental finding Body ache or numbness Seizures Disturbance of hearing

Incidence (n:39) (%) 90 41 28 21 21 18 08 08 05 05

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discovery of the cyst was incidental: CT scans were performed on patients after an overdose, a minor motor vehicle accident (passenger) and a psychiatric admission. On examination 28 patients (72%) were normal. Only eight patients had signs of raised intracranial pressure, the commonest being papilloedema (Table 2). Overall, nearly half of the cysts could not have been suspected on clinical grounds and were only found due to easy availability of brain imaging techniques. The nature of presentation in the current series reflects the international experience,12,13,16,21,26 although a greater proportion of our patients had only minimal symptoms and signs. RADIOLOGICAL FINDINGS CT scan revealed a hyperdense cyst in 10 out of 14 cases (Fig. 1A). Three cysts were isodense, one hypodense, one enhancing and one calcified. Magnetic resonance imaging (MRI) showed a hyperintense cyst on T1-weighted images in three out of three cases (Fig. 1B). During the early years under review, a number of Table 2

Physical signs at presentation

Sign Papilloedema Ataxia Confusion Up going plantar reflexes Abrasions from falls No signs

patients underwent angiograms which were normal (5/10) or showed ventricular enlargement (4/10), and two patients had a 3rd ventricular filling defect diagnosed on pneumoencephalogram. Overall 18 patients had hydrocephalus. Although most colloid cysts can be diagnosed on CT, MRI contributes to accurate preoperative diagnosis, particularly for isodense cysts.21,31 SUDDEN DEATH One patient presented unconscious with fixed dilated pupils on a background of one week of nausea and vomiting without headache. He died in spite of emergency ventricular drainage. At postmortem the cyst was found to be full of hemorrhage. Mathiesen et al.16 propose ‘colloid cyst apoplexy’ as the term to describe hemorrhage into a cyst causing sudden deterioration, as in this case. There are many reports demonstrating that poor clinical condition at the time of diagnosis is likely to result in a poor outcome from colloid cyst treatment.3,13,16,18 Similarly, precipitous death from a symptomatic but undiagnosed colloid cyst is well documented.32,33 Early diagnosis is clearly an important factor in the successful treatment of a colloid cyst.

Incidence (n:39) (%) 21 08 08 05 03 72

TREATMENT In three cases a ventriculo-atrial shunt was the primary surgical management. These patients all underwent microsurgical excision of their cyst when they represented with signs of raised intracranial pressure (in one case, coma). The mean time to re-presentation was 8 years. Two other patients had an external ventricular drain inserted several days before excision of their colloid cyst. In total, 38 patients underwent microsurgical excision of their colloid cyst. In 34 cases transcallosal microsurgery was performed via a right frontal craniotomy: a 1.5 to 2.5 cm incision was made in

Fig. 1 (A) Brain CT scan of a 24 year old woman presenting with drop attacks. In the anterior third ventricle between the foraminae of Monroe a 9 by 6 mm hyperdense colloid cyst is seen. (B) T1- weighted, coronal MRI scan of the brain of a 63 year old man who presented with confusion. A hyperintense colloid cyst is obstructing the foramina of Monroe bilaterally causing hydrocephalus.

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the corpus callosum and the foramen of Munro approached lateral to the fornix. In four cases transfrontal excision was performed: the ventricle was cannulated (in two cases using stereotactic guidance) and the cyst approached along the cannula tract.

has been carried out on patients within 2 months of transcallosal excision of their colloid cyst. In the four patients in whom this has been completed, memory and planning deficits have been identified but these have not prevented the patient returning to their previous function.

PATHOLOGICAL FINDINGS One cyst was found between the leaves of the septum pellucidum, the remainder were in the anterior third ventricle. The cyst size varied from 4 to 30 mm diameter (mean 14 mm). Almost all cysts (79%) contained a firm mucoid or gelatinous material. In four cases (14%) there was a solid component and in only one (4%) were the contents completely liquid. One cyst was calcified and in three (11%) the contents were brown or green, suggestive of hemorrhage. At histopathology, the epithelial lining of the cysts was columnar (42%) (Fig. 2) or cuboidal (58%). Many specimens showed specialized features such as cilia (21%), goblet cells (8%) or microvilli (4%). These features are consistent with immunohistochemical evidence that colloid cyst epithelium has the same developmental origins as sinus or respiratory epithelium.9,10,11 SURGICAL MORBIDITY No patient died as a result of his or her operation. Six patients developed wound infections or meningitis (Table 3), necessitating the removal of the bone flap in four cases. Early neurological deficit was a problem in eight patients, including memory, cognitive or frontal lobe dysfunction in seven patients. Three patients were re-operated for CSF leak, a chronic subdural hematoma and, in one case, because the colloid cyst could not be found at the first (transcallosal) operation. Both the nature and frequency of complications in our cohort are similar to that reported by other groups.12,13,16 Recently a routine neuropsychological assessment

Fig. 2 Paraffin section of a colloid cyst wall showing pseudostratified, columnar epithelium with cilia on the inner surface (top) (H & E stain). Table 3

% total cases

Number of patients Transcallosal (n:34)

Infection Neurological deficit Return to theatre Seizures Endocrinological Other

16 21 8 11 3 8

There was very little long term morbidity. Three patients suffered ongoing seizures, one of whom had experienced seizures prior to diagnosis. One patient required a shunt several months postoperatively. One patient had memory problems (on presentation) which prevented him from returning to work but not from enjoying an active retirement. There were no cyst recurrences. Of the 22 patients who were followed up for a minimum of 6 months (mean 5.8 years) (Table 4), 19 were independent without disability. Two patients were independent on the invalid pension; both of these patients had multiple other medical problems preoperatively. One man died of unknown causes 15 years after transcallosal excision of his colloid cyst (see legend Table 4). These results are comparable to other published series. Hernesniemi and Leivo have reviewed 245 published cases of colloid cyst treated by microsurgical excision. This world series has a 92% good outcome, 6% fair or poor outcome and 2% mortality.3

APPROACHES TO MANAGEMENT We feel that all symptomatic colloid cysts should be removed. In our experience, shunting alone is insufficient as all our patients represented, one comatose. Kondziolka & Lundsford34 and Mathiesen et al.16 have advocated stereotactic aspiration but the failure rate is up to 90%16 and in one series of 16 patients, four represented with evidence of coning and one died after primary treatment of their cyst by aspiration alone.15 Microsurgery is the current gold standard for colloid cyst treatment but there is ongoing debate about whether the transcallosal or transcortical approach is better. Transcallosal approach is through natural tissue planes but there is a risk of retraction damage to bridging veins and to the fornix as it runs around the foramen of Munro. The transcortical approach is a direct trajectory through the foramen of Munro but there is supposedly an increased risk of seizure disorders. In the current study there is no difference in complications or outcomes between the two groups and this is supported by other recently reported series.12,13,21,22,23 The newest reported treatment is endoscopic aspiration and excision. The colloid is aspirated via a transcortical transventricular route, as much capsule as possible is removed and the remainder cauterised.18,19 The maximal published follow up of these patients is 28 months, which is a short period in the natural history

Table 4

Long term* outcome (22 patients)

Outcome

Short term complications

Complication

LONG TERM RESULTS

Transfrontal (n:4)

6 6 3 4 1 1

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2

2

Good recovery Independent but disabled Dead

Number of patients 19 02 01#

* Mean follow up 5.8 years, minimum 6 months. # This patient originally presented in 1971, was investigated with a pneumoencephalogram and treated with a ventriculo-atrial shunt and radiotherapy. His course was complicated by multiple shunt infections and hypo-pituitarism. Following excision of the cyst in the 1980s he suffered seizures and memory problems. Shortly before his death he was living independently but unable to work. The cause of death is unknown.

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of colloid cysts, so it remains to be seen whether sterile ventriculitis or cyst recurrence are a limitation of this management. Stereotactic guidance has been used to increase the precision of the cortical incision.1,17,25 It could also be used to optimise the approach through the corpus callosum, although this has not been reported. This would minimise the risk of damage to the fornix or of disconnection syndrome from the callosal incision. Overall the most important factor in outcome from a colloid cyst is early diagnosis and careful, precise surgery using an approach with which the surgeon is comfortable. Currently microsurgical techniques enable a safe and complete removal of these rare tumours. In future endoscopy and stereotaxis are likely to be increasingly used to make treatment even safer.

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ACKNOWLEDGEMENTS Many thanks to the surgeons who managed some of the cases: Dr I. Johnston, Dr N. Dorsch, Dr M. McGee-Collett, Dr M. Morgan and Dr J. Segelov (deceased). Also to Jacqui Pearse from Medical Records and Dr Diane Caine, neuropsychologist, for her assessment of patients. REFERENCES 01. 02. 03.

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