Fourth ventricle epidermoid cyst

Intracranial epidermoid cysts are rare, comprising 0.2% to 1.8% of all primary intracranial expanding lesions, of which <5% occur within the fourth ventricle.

In a systematic review of 23 studies that reported 37 patients. Mean age was 42.8 years. Mean length of time between symptom onset and diagnosis was 4.43 years. Age (P = 0.049) and duration of symptoms (P < 0.001) were significantly different between patients who experienced symptom improvement and those who did not. Duration of symptom onset to diagnosis was a significant predictor of poor outcomes (odds ratio, 1.19, 95% confidence interval, 1.003-4.785) ¹⁾.

In a retrospective analysis of 50 cases of posterior fossa epidermoid cyst surgically treated between 1997 and 2007, patients with fourth ventricle epidermoids had features of raised intracranial pressure (ICP) and gait ataxia (69.2% each) ²⁾.

Symptoms can include hearing impairment, trigeminal neuralgia, facial tics, headaches, double vision, and facial palsy. H

A case report presented the cognitive profile of a woman after resection of an epidermoid cyst in the fourth ventricle. The 49-year-old patient underwent neuropsychological assessment after removal of the cyst, completing a comprehensive set of cognitive tests of estimated premorbid intelligence, attention, memory, social cognition, language, visual perception, and executive functioning. Test results indicated executive function impairment and reduced visuospatial perception in the acute stage after surgical removal of the epidermoid cyst. These findings suggest that cognitive deficits can occur after resection of space-occupying lesions in brain regions not typically associated with cognition. This is the first report of the neuropsychological consequences of surgical removal of a congenital epidermoid cyst in the fourth ventricle. An understanding of the neuropsychological sequelae of this rare cerebral cyst will allow patients, families, and health professionals to better anticipate and manage postoperative difficulties ³⁾.

Magnetic resonance is the standard diagnostic study, but it could lead to confusion with neurocysticercosis ⁴⁾.

see Fourth ventricle epidermoid tumor: radiologic, intraoperative, and pathologic findings ⁵⁾.

The extirpation and the treatment of the hydrocephalus are indicated.

Incomplete removal of the capsule, in case it would be adherent to the floor of the fourth ventricle, may be necessary to avoid morbidity and mortality ⁶⁾.

The endoscope is a safe and effective adjunct to the microscope in facilitating additional inspection and further resection of epidermoid tumors. Endoscopic-assisted surgery is particularly useful for identifying and removing additional tumor located around surgical corners ⁷⁾.

The review found that in patients with fourth ventricular epidermoid cysts, unfavorable outcomes were predicted by older age and by longer intervals from symptom onset to diagnosis. Clinicians should maintain a high index of suspicion for fourth ventricular epidermoid cysts in patients presenting with cerebellar signs and visual disturbances ⁸⁾.

The prognosis is favourable with a complete surgical resection ⁹⁾.

Xu et al., reported a case with epidermoid cyst (EC) in the fourth ventricle. The insinuation and scalloping growth pattern were the distinctive imaging characteristics of EC in the fourth ventricle on magnetic resonance imaging. The accurate identification of the punctate enhancement of vessels around the lesion may be helpful for its differential diagnosis from hemangioblastoma ¹⁰⁾.

Epidermoid cyst of the fourth ventricle in a child ¹¹⁾.

A 21-year-old woman presented with 3 months of headaches and visual obscurations and was diagnosed with a multilobulated mass in the fourth ventricle. The patient underwent suboccipital craniotomy. Complete removal of the mass was achieved and the final diagnosis was consistent with an epidermoid cyst. The systematic review identified 23 studies that reported 37 patients. Mean age was 42.8 years. Mean length of time between symptom onset and diagnosis was 4.43 years. Age (P = 0.049) and duration of symptoms (P < 0.001) were significantly different between patients who experienced symptom improvement and those who did not. Duration of symptom onset to diagnosis was a significant predictor of poor outcomes (odds ratio, 1.19, 95% confidence interval, 1.003-4.785).

The review found that in patients with fourth ventricular epidermoid cysts, unfavorable outcomes were predicted by older age and by longer intervals from symptom onset to diagnosis. Clinicians should maintain a high index of suspicion for fourth ventricular epidermoid cysts in patients presenting with cerebellar signs and visual disturbances ¹²⁾.

A 47-year old woman admitted for intracranial hypertension syndrome associated with walking disorders. The diagnosis of fourth ventricle epidermoid cyst was evoked based on diffusion MRI data then confirmed intraoperatively and by histologic examination. Subtotal surgical excision was performed due to capsule adhesion to the upper part of the V4 floor. After a 36-month follow-up, the patient showed no signs of tumor recurrence ¹³⁾.

case 1: a female 22 year old presented with an intense headache with a history of 3 months. At the hospital entry, symptoms and signs of high intracranial pressure were found. Tomography images showed hydrocephalus with high pressure in the FV. She was treated with a shunt from ventricular to peritoneal cavity. After that an encapsulated neoplasm was drawn. It had a pearled aspect. The histology report showed an EN originating in the FV. Case 2: a female 44 year old with a history of five years of dizziness; three years before admission she presented intermittent diplopia and dysphagia. At the hospital admission the patient presented paresis of the 6th and 7th cranial nerve. The tomography and the magnetic resonance studies showed a mass in the FV. The neoplasm was extirpated ¹⁴⁾.

In a report a retrospective study of four cases of epidermoid cysts of the cisterna magna and the fourth ventricle. The data was collected from January 2000 to December 2006 from to series of 18 cases of epidermoid cysts of posterior cranial fossa (14 cases were localised at the cerebellopontine angle). All the patients had a physical examination and a complete neuroradiological imagery. The treatment was surgical. The follow-up was at least 9 months.

There were two men and two women. The mean age was 47.75 years. All patients presented with cerebellar syndrome. Three patients had intracranial hypertension. Neuroradiological explorations showed a cystic lesion developed in the cisterna magna in two cases, in the fourth ventricle in one case and in the two locations in one case. Two patients had hydrocephalus. All patients had surgery with a posterior approach and one patient had first ventriculoperitoneal shunt. The diagnosis was confirmed by histological examination. Postoperatory outcome was favourable in all cases. One patient developed bilateral chronic subdural hematoma, which was surgically removed. A long time follow-up was good in all cases ¹⁵⁾.