Original Article

Isolated Intra Fourth Ventricular Neurocysticercosis

^*Divye Prakash Tiwari, ^*Vivek Sharma, ^*Deepak Patil, ^*Amrita Ghosh

• ^*Department of Neurosurgery and Pathology, Institute of Medical Sciences, Varanasi, India

• Submitted: December 05, 2013
• Accepted: December 09, 2013
• Published: December 15, 2013

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction

Neurocysticercosis is one of the most frequently observed central nervous system parasitic infection caused by the larvae of the pork tapeworm, Taenia solium. The fourth ventricle is the most frequent location of intraventricular neurocysticercosis that carries a higher risk for CSF blockage and resulting in hydrocephalus.

Methods

We report a series of cases of 13 patients with symptomatic obstructive hydrocephalus due to cysticercus in the fourth ventricle presenting to us over a period of 7 years.

Conclusion:

The excision of fourth ventricular cysts typically requires a microsurgical approach via suboccipital craniotomy.

Key words

neurocysticercosis, fourth ventricle, hydrocephalus, Neurosurgical procedure.

Introduction

Neurocysticercosis (NCC) is a parasitic disease caused by infection with the larval form of Taenia solium or pork tapeworm and is the most frequent parasitic infestation of the central nervous system.[1,2] Neurocysticercosis is acquired when T. solium eggs are consumed, either via fecal–oral contact with a tapeworm carrier or ingestion of food or water contaminated by T. solium eggs. Persons harboring adult tapeworms in their intestines may also acquire neurocysticercosis via fecal–oral autoinfection. After ingestion, the tapeworm egg hatches and penetrates the intestine, and travels through the bloodstream to develop into cysticerci in the central nervous system, muscles, or eyes. Although the medical management of neurocysticercosis with cysticidal drugs is controversial, neurosurgical resection of fourth ventricular neurocysticercosis cysts is the treatment of choice. In this series we focus on the management of neurocysticercosis presenting in the fourth ventricle with resultant hydrocephalus.

Patients and Methods

All cases of fourth ventricular neurocysticercosis reported to neurosurgery in last 7 years were taken into study. They were analysed in respect to age, sex, symtoms, radiology and management. Their incidence, a etiology and treatment protocol were discussed.

Observation

We have encountered 13 cases of fourth ventricular neurocystercercosis presenting with acute hydrocephalus. The maximum incidence (6 cases) were found in the age group 21-30 years, while 2 cases were found in 0-10 years group, 3 cases were in 11-20 years group, whereas 2 cases were in 31-40 years group.[Table 1]

Table 1:Incidence of intrafourth ventricular neurocysticercosis.

Age(years)	Sex	Incidence
0-10	Male	1
	Female	1
11-20	Male	1
	Female	2
21-30	Male	2
	Female	4
31-40	Male	1
	Female	1

Eleven patients presented with headache, nine patients with vomiting, and unconsciousness, decreased visual acuity in five patient, and blindness in one patient. [Table 2]

Table 2:Clinical findings

Clinical features	No. of patients
Headache	11
Vomiting	09
Unconsciousness	01
Blurred vision	05
Blindness	01

Management

MRI is the investigation of choice depicting the well defined cyst with or without small enhancing dot with CSF all around. [Fig.1,2,3] There may be associated hydrocephalus depending upon the degree of block.

Figure 1: Coronal section of T1 weighted MRI showing cyst in fourth ventricle

Figure 2: Axial section of MRI T1 weighted showing well defined cyst in 4th ventricle

Figure 3: Sagittal section of brain

The treatment of fourth ventricular NCC is defined by the clinical presentation. The first thing to define is the presence or risk of acute hydrocephalus thereby the option of ventriculostomy or ventriculoperitoneal shunt. [Table 3] Intraoperative ultrasonography may help in localising the cyst. [Fig.4] Histopathology confirmation was done in all cases. [Fig.5]

Table 3 Surgical options

Procedure	No. of patients
Ventriculoperitoneal shunt followed by posterior fossa decompressive craniotomy and cyst removal	4
Direct posterior fossa decompressive craniotomy and cyst removal	9

Figure 4: Intraoperative ultrasound of brain

Figure 5 : Histopathological examination of cyst wall

Discussion

Isolated fourth ventricular neurocystercercosis can cause acute hydrocephalus by mass effect resulting in CSF outflow obstruction. Isolated fourth ventricular neurocysticercosis presents with headaches in 77% to 100% of patients, whereas parenchymal neurocysticercosis have seizure as presenting feature in 70% of patients. Isolated intra fourth ventricular neurocysticercosis presents as throbbing headache and blurred vision, syncopal event, bradycardia as it is in close proximity to the floor of the fourth ventricle. [3-16].

The diagnosis of neurocysticercosis is made by classic imaging finding of a cystic lesion containing a ‘‘nodule’’, which represents the ‘‘scolex’’ of the T. solium larva.

MRI is the preferred in a patient with possible neurocysticercosis, as it is more sensitive and specific than CT scan. The fourth ventricular neurocysticercosis is often isodense or isointense with CSF, posing difficulty in diagnosis. Occasionally, the scolex can be seen as a hyperintense dot within the cyst being diagnostic of cysticercal cyst. [19] The cornerstone of most neurocysticercosis management is symptomatic therapy. This includes the use of anti-epileptic drugs for seizure control, treatment of hydrocephalus, and the use of corticosteroids to attenuate the host immune response, especially when giving cysticidal therapy (albendazole, praziquantel) or attempting cyst resection. Indication for neurosurgical resection of neurocysticercosis cyst is generally limited to intraventricular cyst, giant subarachnoid cyst (usually located within the Sylvian fissure), and cyst causing spinal cord compression.

In a series of 45 patients with intraventricular cysticercosis, the fourth ventricle was the most common location for cyst (53%) followed by the third ventricle (27%), lateral ventricles (11%) and Sylvian aqueduct (9%). [20] Hydrocephalus associated with intraventricular cyst usually does not require shunt surgery after excision of the cyst. Shunt has also been known to be the cause of migration of fourth ventricular cyst into the third or lateral ventricles due to a siphon effect. Excision of fourth ventricular cyst typically requires microsurgical approach via suboccipital craniotomy. [17, 18]

The available treatment options are: medical, external cerebro-spinal fluid diversion, microsurgical, or endoscopic removal alone or in combination. Albendazole (15 mg/kg/day for 3-4 weeks) is recommended for IVNCC even after surgical procedures. [21] Praziquantel (100 mg/kg/day for 3-4 weeks) may cause rapid regression of large cyst of the fourth ventricle thus avoiding the need for surgery.[22,23] Direct surgical excision through sub- occipital approach is better option than shunt as shunt associated complication rate is high. [24] Shunt surgery only may be contemplated if pericystic enhancement without mass effect are seen in MRI. [25] Endoscopic neurosurgery is a minimally invasive technique enabling removal of intraventricular cyticercal cyst from all locations, thus avoiding major craniotomy/posterior fossa exploration and shunt. [26]

Conclusion

The intra fourth ventricular cysticercosis is relatively uncommon and prersent with raised intracranial pressure due to hydrocephalus. The investigation of choice is MRI. Surgical option is better than medical but there are few reports where lesion disappeared on antihelmenthic treatment. The excision of fourth ventricular cyst typically requires microsurgical approach via suboccipital craniotomy.

Conflict of Interest

The authors declare that they have no conflict interests.

Authors' Contribution

VS: Concept of study and final approval

DPT: Concept and analysis of study, literature search and preparation of manuscript

DP: Concept and design of study

AG: Histopathological examination

Ethical Considerations

Not declared

Funding

None declared

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