Open Access
CC BY 4.0 · J Neurol Surg Rep 2025; 86(04): e206-e213
DOI: 10.1055/a-2713-5787
Case Report

Tetraventricular Hydrocephalus Due to Idiopathic Fourth Ventricle Outlet Obstruction: A Case Report and Literature Review

Authors

  • Guramritpal Singh

    1   Department of Neurosurgery, Shree Ram Neuro Centre, Jalandhar, Punjab, India

Funding Information The author declares that the contents of the article were written in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
 

Abstract

Introduction

Tetraventricular hydrocephalus happens due to the fourth ventricle outlet obstruction. Idiopathic fourth ventricle outlet obstruction (IFVOO) is a condition where no clear-cut etiology for fourth ventricle outlet obstruction can be found. The etiopathogenesis of IFVOO is unclear. There is no clear-cut consensus regarding the treatment practices for its management. These cases present a diagnostic dilemma to the treating neurosurgeon and are thus often managed inappropriately. This study aims to review the existing literature regarding this condition, illustrating with a case from our hospital.

Case Details

We present a case of a 50-year-old female who presented to us with the chief complaints of headache, difficulty in walking, with an inability to balance while standing and walking, diplopia, and three episodes of loss of consciousness for 6 months. A brain MRI was done, which was suggestive of dilatation of all ventricles with obstruction at the foramina of Luschka and Magendie. She underwent a right-sided, medium-pressure ventriculoperitoneal shunt at our hospital. Postsurgery, there was immediate improvement in her symptoms.

Conclusion

IFVOO is a rare cause of tetraventricular hydrocephalus with an unknown cause. Endoscopic third ventriculostomy (ETV) appears to have a higher risk of failure in such cases. Fenestration procedures after craniotomy and shunt procedures are still effective in their management. ETV is still an alternative to the above-mentioned procedures. To confirm these conclusions, larger studies involving multiple hospitals and institutes are required.


Introduction

Hydrocephalus is caused due to the accumulation of excess cerebrospinal fluid (CSF) in the ventricular system of the brain. In the event of obstruction of the fourth ventricle outlet (foramina of Luschka and Magendie), dilatation of all four ventricles occurs (tetraventricular hydrocephalus). Etiology includes intracranial bleed, intracranial infection (bacterial, tubercular, cysticercal meningitis) Dandy–Walker malformation, Arnold–Chiari malformation, Basilar invagination and other craniovertebral junction abnormalities, and tuberous sclerosis.[1] In rare cases, no causative factor can be identified that is causing fourth ventricle outlet obstruction (FVOO). Such a condition is called idiopathic fourth ventricle outlet obstruction (IFVOO). Congenital occlusion of the foramina of Luschka and Magendie is known to occur; however, the etiology is unknown.[2] [3] As such cases are rare, only a handful of them are described in the literature. In this paper, the author presents his own experience with IFVOO and reviews the relevant literature.


Case Details

Clinical Presentation

A 50-year-old female presented to our hospital with the chief complaints of headache, difficulty in walking, imbalance while standing and walking, diplopia, vertigo, and three to four episodes of loss of consciousness for the past 6 months. Initially, the patient had taken medications from a local physician for the same, but no symptomatic relief occurred. On examination, the patient had bilateral modified Friesen grade III papilledema, positive bilateral cerebellar signs. A brain MRI ([Fig. 1]) was done, which was suggestive of tetraventricular hydrocephalus with obstruction of the foramina of Luschka and Magendie. The preoperative radiological diagnosis was IFVOO. Laboratory findings were normal.

Zoom
Fig. 1 Brain MRI (A-D) showing tetraventricular hydrocephalus with obstruction of foramina of Luschka (yellow arrow) and Magendie (blue arrow).

Surgery

The patient underwent a right-sided medium-pressure ventriculoperitoneal shunt at the Keen's point. CSF came under high pressure, in a jet-like fashion, at the rate of 80 to 90 drops/minute.


Postoperative Course and Follow-Up

On postoperative day 1, the patient reported significant improvement in gait ataxia, vertigo, and diplopia. The rest of the postoperative course in the hospital was uneventful. At the 3-month follow-up, the patient had complete resolution of the presenting complaints.



Literature Review

A comprehensive review of the literature was undertaken to examine the management of IFVOO. We searched on the PubMed database using the terms “FVOO,” “tetraventriculomegaly,” and “hydrocephalus.” Inclusion criteria: Cases of tetraventricular hydrocephalus that did not have any associated congenital malformation or any other secondary cause, such as intracranial bleed, intracranial infection, or tuberous sclerosis. Excluding criteria: Cases of communicating hydrocephalus; secondary causes such as congenital malformation, intracranial bleed, intracranial infection, tuberous sclerosis; case reports that provided incomplete information regarding clinical presentation, radiological findings, management protocols, and functional outcomes. About 24 articles (a total of 63 cases of IFVOO) fulfilled our criteria and were included in this study, besides my own case ([Table 1]).

Table 1

Literature review of Idiopathic fourth ventricle obstruction cases

S.No

Study, year

Age/Sex (M/F)

Number of patients

Clinical presentation

Surgery

Outcome

Resurgery

Mean follow-up

1

Coleman and Troland, 1948[2]

17 y/M

1

Headache

Craniotomy

Improved

N/A

N/A

2

Holland and Graham, 1958[17]

31 y/F

1

Blurred vision

Nausea

Headache

Weakness of right leg

Craniotomy

Dead

N/A

N/A

3

Amacher and Page, 1971[24]

21 y/F

1

Headache Vomiting

Nausea

Craniotomy + VC shunt

Improved

N/A

1 year

4

Yoshioka et al, 1985[25]

Mean age = 35.3 y/M = 3

3

Gait disturbance

Cerebellar ataxia

Craniotomy + shunt

Improved

N/A

1 year

5

Rifkinson-Mann et al, 1987[18]

Mean age = 47 y/M = 2

2

Hemiparesis

Hemianopsia

Headache

Vomiting

Blurred vision

Craniotomy

Improved

N/A

1 year

6

Aesch et al, 1991[16]

35 y/M

1

Ataxia

Headache

Nausea

VP shunt

Improved

N/A

2 months

7

Osaka et al, 1995[19]

20 y/F

1

Headache

Nausea

Papilledema

Craniotomy

Improved

N/A

N/A

8

Hashish et al, 1999[26]

Mean age = 51.5 y

F = 2

2

Headache

Gait disturbance

Nausea

Memory disturbance

Craniotomy + VC shunt

Improved

N/A

6 years

9

Suehiro et al, 2000[29]

27 y/F

1

Dizziness

Headache

Nausea

ETV

Improved

N/A

N/A

10

Huang et al, 2001[20]

15 y/F

1

Headache

Nausea

Vomiting

Amenorrhea

Craniotomy

Improved

N/A

14 months

11

Carpentier et al, 2001[10]

58 y/F

1

Visual impairment

Dizziness

Headache

Nausea

Vomiting

Gait disturbance

ETV

Improved

N/A

3 years

12

Inamura et al, 2001

9 months/M

1

Macrocephaly

Arrest of mental development

VP shunt

Improved

N/A

3 months

13

Karachi et al, 2003[11]

Mean age = 47.3 y/M = 1, F = 2

3

Headache

Vomiting

Papilledema

Vertigo

Nausea

Gait disturbance

Sphincteric disorders Impairment of higher functions

ETV

Improved

N/A

36 months

14

Mohanty et al, 2008[1]

>2 years

12

N/A

(not specified in respect to PFVOO cases)

ETV

N/A (in respect to PFVOO cases)

Failed to describe (not specified in respect to PFVOO cases)

4.2 years

15

Longatti et al, 2009[4]

60.6 years

10

Ideomotor slow down

Gait disturbance

Depression

Dizziness

Memory impairment

Incontinence

Visual impairment

Headache

Vomiting

ETV = 8

ETV + aqueductoplasty = 1

Endoscopic magendieplasty = 1

Improved = 9

Lost to follow-up = 1

N/A = 7

1. Recurrence after 12 years, re-ETV done

2. Recurrence after 3 years/ VP shunt done

3. Recurrence after 2 months, re-ETV done

47.7 months

16

Hashimoto et al, 2014

20 months/M

1

Syndrome of inappropriate antidiuretic hormone secretion

ETV

Improved

N/A

N/A

17

Torres-Corzo et al, 2014[6]

18.5 years/M = 2, F = 5

7

Lethargy

Bulging fontanel

Headache

Gait disturbance

Seizures

Blurring of vision

Nausea

Vomiting

ETV + magendieplasty = 5

ETV + magendieplasty + aqueductoplasty = 1

Endoscopic magendieplasty = 1

Improved

N/A

26.5 months

18

Ishi et al, 2015[15]

3 y/M

1

Headache

Vomiting

ETV

Improved

Recurrence after 1 year, re-ETV done

32 months

19

Kasapas et al, 2015[21]

37 y/F

1

Headache

Blurred vision

Vomiting

Phonophobia

Recent memory loss

Bilateral papilledema

Craniotomy

Improved

N/A

2 weeks

20

Duran et al, 2017[22]

19 y/F

1

Headache

Diplopia

Intracranial hypertension

Craniotomy

Improved

N/A

N/A

21

Pérez et al, 2019

41 y/F

1

Headache

Imbalance

Nausea

Vomiting

ETV

Improved

N/A

6 months

22

Bai et al, 2019[23]

15 y

1

Headache

Vomiting

Craniotomy

Improved

N/A

1 year

23

Rosa et al., 2021[14]

7 y/M

1

Abdominal pain

Vomiting

Sixth and seventh cranial nerve palsy

VP Shunt twice

VA Shunt once

Deteriorated

ETV after 10 months

6 years

24

Krejčí et al, 2021[7]

Mean age = 40.9 y/M = 3, F = 5

8

Headache

Vertigo

Gait disturbance

Diplopia

Vomiting

Papilledema

ETV = 5

Craniotomy = 2

Acute ventricular drainage = 1

Improved = 7

Death = 1

1. VP shunt in 1 patient

2. Recurrence after 6 weeks, re-ETV done

75.4 months

25

This study

50 y/F

1

Headache

Difficulty in walking

Imbalance while standing and walking

Diplopia

Vertigo

Papilledema

Three to four episodes of loss of consciousness

VP shunt

Improved

Not done

3 months

26

Summary

Mean age = 28 years/M = 17,

F = 24

Sex N/A = 23

Total = 64 patients

Headache 50% (n = 32)

Vomiting 31.3% (n = 20)

Gait abnormalities 31.3% (n = 20)

Diplopia 6.25% (n = 4)

Vertigo 9.38% (n = 6)

Papilledema 12.5% (n = 8)

Cranial nerve palsy 1.6% (n = 1)

Raised intracranial pressure 3.1% (n = 2)

Memory loss = 6.25% (n = 4)

Seizure = 1.5% (n = 1)

Phonophobia = 1.5% (n = 1)

Loss of consciousness = 1.5% (n = 1)

Lethargy = 1.5% (n = 1)

Bulging fontanel = 1.5% (n = 1)

Vision abnormalities = 9.4% (n = 6)

Syndrome of inappropriate antidiuretic hormone secretion = 1.5% (n = 1)

Amenorrhea = 1.5% (n = 1)

Incontinence = 3.1% (n = 2)

Abdominal pain = 1.5% (n = 1)

Depression = 1.5% (n = 1)

Craniotomy = 10

Shunt = 7

ETV = 23

Endoscopic magendieplasty = 8

Endoscopic aqueductoplasty = 2

Improved = 48 (75%)

Deteriorated = 1 (1.5%)

Death = 2 (3%)

Lost to follow-up = 1 (1.5%)

N/A = 12 (19%)

ETV = 5 (7.8%)

Shunt = 2 (3.1%)

Mean follow-up = 20.9 months

Abbreviations: ETV, endoscopic third ventriculostomy; F, female; M, male; PFVOO, Primary fourth ventricle outlet obstruction; Ventriculo-cisternal shunt; VP, ventriculoperitoneal shunt; y, years.



Discussion

Etiopathogenesis and Demography

Tetraventricular hydrocephalus resulting from FVOO is caused due to multiple conditions. Hydrocephalus due to intracranial hemorrhage or intracranial infection is more common, occurring due to CSF malresorption, leading to lower endoscopic third ventriculostomy (ETV) success rates in these cases.[1] In IFVOO, hydrocephalus is principally obstructive in nature owing to the membranous occlusion of the foramina of Luschka and Magendie. The underlying mechanism leading to this occlusion is unknown. It may be congenital or acquired.[4] Congenital occlusion of the foramina of Luschka and Magendie is well-documented.[2] [3] [5] [6] However, it fails to explain the exact pathogenesis leading to congenital cases of IFVOO becoming symptomatic. In acquired causes, the mechanism that has been suggested is underlying inflammation leading to scarring of the arachnoid layers in the cisterns and ventricles. This was supported by the findings of anomalous membranous proliferation in the interpeduncular cistern and thickening of the floor of the third ventricle.[7] In cases of ETV failure, signs of scarring in the interpeduncular cistern were found.[8] [9] Multiple studies have shown an increased number and toughness of the membranes in the interpeduncular cistern, abnormal third ventricle floor rigidity, and changes in the choroidal plexus in the ventricle, leading to a higher risk of ETV failure in such cases.[1] [4] [6] The other explanation is that the increased intracranial pressure leads to a dilated fourth ventricle, which causes the membranes of foramina of Luschka and Magendie to come into contact with the dura mater, interrupting the CSF flow.[10] This might be the underlying mechanism in patients with acute hydrocephalus. However, there seems to be a lack of consensus regarding the primary cause. IFVOO is typically seen in adults, with no gender predilection.[7] The authors propose the fact that since the majority of the affected patients are adults, there appears to be an ongoing subclinical inflammatory process in the patients of IFVOO over a prolonged time period. This might lead to IFVOO with the congenital anomaly being present in the background.


Clinical Presentation

Headache and gait difficulties are the most commonly encountered symptoms in IFVOO. Krejčí et al reported that primary surgery was successful in patients who had headaches in the preoperative period.[7] Symptoms of normal-pressure hydrocephalus were described in patients with obstructive tetraventricular hydrocephalus in the literature.[11] It becomes difficult to distinguish patients with normal pressure hydrocephalus (NPH) from IFVOO, as NPH is a more common entity.


Radiologic Findings

Both IFVOO and communicating tetraventricular hydrocephalus present with Hakim's triad—progressive gait impairment, cognitive deficits, and urinary urgency and/or incontinence. It is difficult to distinguish them on clinical grounds. Brain MRI is the imaging modality of choice in such cases. Krejčí et al reported that the presence of ballooning of the fourth ventricle, decreased prepontine cistern volume, decreased retrocerebellar space, and concomitant anterior displacement of the brainstem were found to be specific for IFVOO.[7] However, these radiological features appear to be diagnostic of FVOO rather than IFVOO. As IFVOO leads to an obstructive type of hydrocephalus, preoperative MRI findings such as third ventricle bowing and concomitant fourth ventricle ballooning appear to indicate the diagnosis of IFVOO. Resolution of third ventricle bowing and fourth ventricle ballooning was associated with successful management of IFVOO.[7] [12] [13] The presence of a widely dilated aqueduct of Sylvius differentiates IFVOO from a trapped fourth ventricle.[4]



Treatment and Outcome

Treatment modalities for IFVOO include ETV,[13] [14] [15] shunt surgery,[3] [16] open fenestration via suboccipital craniotomy,[2] [17] [18] [19] [20] [21] [22] [23] or a combination of the two.[24] [25] [26]

Suboccipital craniotomy and wide opening of the membrane were performed based on the fact that by removing the obstruction of the fourth ventricular outlets, CSF flow could be normalized.[17] [24] Frequent recurrences of the hydrocephalus were seen, although the patients showed initial clinical improvement with ventricle sizes becoming near normal.[27] [28]

The use of ETV in FVOO was first described by Mohanty et al, where they reported an overall success rate of 65%, with favorable outcomes (91% success) in patients of age more than 2 years, with failure in all patients younger than 6 months of age.[1] [15] Other studies have also reported successful outcomes using ETV for IFVOO, although in one study, redo ETV was done following recurrence of hydrocephalus.[15] [29]

In the literature, the majority of patients with IFVOO underwent endoscopic intervention. In patients with IFVOO, ETV failure can occur at any time, in comparison to the other types of hydrocephalus, in which failure typically occurs in the first few weeks.[4] [30] Literature review has found that in cases of IFVOO, ETV has a higher failure rate compared with the rest of the treatment options.[7]

Some cases of IFVOO present with a substantial increase in the volume of the fourth ventricle, which may lead to small prepontine and suprasellar cisterns due to the brainstem being pushed anteriorly. This may lead to the basilar artery being pushed closer to the third ventricle floor, making ETV an unsafe procedure. In such cases, Endoscopic fourth ventriculostomy as described by Giannetti et al can be performed. ETV has its own advantages as it is safer to perform. In endoscopic fourth ventriculostomy, there may occur extra manipulation of the third ventricle, cerebral aqueduct, and fourth ventricle with the increased risk of damage to the surrounding neurovascular structures. Hence, it should be considered a surgical option only in cases when ETV is not possible.[31]

Ventriculoperitoneal shunts have been utilized for the management of IFVOO, although long-term follow-up is not available in such cases.[3] [16] Hence, it is difficult to assess the efficacy of ventriculoperitoneal shunts in such cases.


Conclusion

The precise mechanism by which IFVOO develops and causes tetraventricular hydrocephalus is still unknown. Obstructive hydrocephalus results from IFVOO; hence, ETV is considered to be the treatment of choice in such cases. But in such cases, the risk of ETV failure remains higher. Literature review suggests long-term follow-up in patients undergoing ETV, as the failure can occur at any time. The initial treatment procedures, such as open fenestration via craniotomy and shunt surgery, remain still relevant and effective procedures for IFVOO. An alternate line of treatment for it would be endoscopic fenestration of the fourth ventricle outlets. Larger studies involving multiple hospitals are required to confirm these findings.



Conflict of Interest

The authors declare that they have no conflict of interest.


Correspondence

Guramritpal Singh, MCh
Department of Neurosurgery, Shree Ram Neuro Centre
Jalandhar 144001, Punjab
India   

Publication History

Received: 01 May 2025

Accepted: 21 September 2025

Article published online:
13 October 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Zoom
Fig. 1 Brain MRI (A-D) showing tetraventricular hydrocephalus with obstruction of foramina of Luschka (yellow arrow) and Magendie (blue arrow).