Hydrocephalus Caused by Primary Fourth Ventricle Outlet Obstruction: Our Experience and Literature Review

Krejčí, Ondřej; Krejčí, Tomáš; Mrůzek, Michael; Večeřa, Zdeněk; Šalounová, Dana; Lipina, Radim

doi:10.1016/j.wneu.2021.01.008

Cited by 7 publications

(5 citation statements)

References 20 publications

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“…Although it is more likely to encounter primary FVOO in the pediatric population, Longatti 2009 11 and Krejčí 2021 6 reported many adult cases, which found its expression in the demographic data of our systematic review, where FVOO was found to typically affect individuals in the first or sixth decades of life, with a median age of 26 years.…”

Section: Discussionmentioning

confidence: 72%

“…Historically, primary FVOO has been managed with suboccipital craniotomy with magendieplasty or VP shunt placement. 1 , 6 However, these approaches are invasive and associated with significant postoperative complications. In the case of VP shunt, they further require implants and cause shunt dependency.…”

Section: Discussionmentioning

confidence: 99%

“…More recent recommendations favor endoscopic third ventriculostomy (ETV) as a minimally invasive alternative treatment. Since the beginning of the 21st century, ETV has been considered a possible treatment of FVOO 1,2,6 . Because of the rarity of this condition, there is insufficient evidence about the efficacy of ETV in treating it, especially regarding the recurrence rate and the neurological outcomes through a long follow‐up.…”

Section: Introductionmentioning

confidence: 99%

“…Although primary FVOO was first described in the late 19th century by Wernicke and Macewen, 4,5 the medical literature still lacks sufficient studies of this rare condition and its best treatment modality. In the 20th century, neurosurgeons undertook suboccipital craniotomy with magendieplasty or ventriculoperitoneal shunt (VP shunt) implantation as standard treatments 1,6 ; however, these approaches are invasive and accompanied by high postoperative or lifelong complication rates. Since the hydrocephalus caused by FVOO is from the occlusive type, VP shunting was frequently applied as a standard treatment, especially in pediatric patients 3 .…”

Section: Introductionmentioning

confidence: 99%

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The endoscopic treatment of fourth ventricle outlet obstruction: Report of two children and systematic review

Mulhem,

Alsulaiman,

Hammersen

et al. 2023

Clinical Case Reports

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Key Clinical MessageFourth ventricle outlet obstruction (FVOO) is a rare cause of hydrocephalus. In the last century, the standard treatment was the suboccipital craniotomy with magendieplasty or ventriculoperitoneal shunt (VP shunt). Since the beginning of the 21st century, the endoscopic third ventriculostomy (ETV) has been considered a less invasive alternative. The medical literature lacks sufficient reports of FVOO cases and strong evidence about ETV's efficacy in treating this condition. We report two cases of FVOO treated with ETV and review published similar cases. Clinical and radiological findings of two FVOO cases with outcomes after ETV were presented. Moreover, we conducted a systematic review after protocol registration in PROSPERO (CRD42021281474). MEDLINE, Embase, Scopus, and Web of Science were searched from inception till December 31, 2022. Studies were included if they reported cases of FVOO treated initially with ETV. Cases with Chiari malformation, Dandy‐Walker malformation, tuberous sclerosis, trapped fourth ventricle, or space‐occupying lesions were excluded. Two reviewers independently examined title/abstract records in the first stage and full‐text publications in the second for eligibility. The primary outcome was the recurrence rate, defined by the need for re‐ETV or other invasive treatments (e.g., VP shunt or magendieplasty). Other outcomes included clinical state at follow‐up and mortality. Two cases, a 3‐year‐old male and 3.5‐year‐old female, with FVOO, were treated with ETV in our department by the same neurosurgeon (SH) in 2013 and 2021. Both cases improved significantly after ETV, and there was no recurrence through the follow‐up. Besides the present cases, we found 57 other cases of FVOO treated with ETV reported in 17 studies between 2001 and 2021. The median age was 26 years, with an IQR from 2.4 to 59 years, and 56% of cases were females. The recurrence rate was 32% in the sample (19 out of 59), with a 95% CI from 21% to 46%. The median time to recure was 2 months with IQR from 1.25 to 26. A VP shunt was the treatment for recurrence in 68% and a re‐ETV in 32%. At the follow‐up (41 ± 29 months), only one case died, and one deteriorated clinically. FVOO is a rare cause of hydrocephalus encountered mainly in the first or sixth decades of life. ETV provides the first reasonable treatment. Despite the moderate recurrence rate, the outcomes are favorable.

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Section: Discussionmentioning

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

The endoscopic treatment of fourth ventricle outlet obstruction: Report of two children and systematic review

Mulhem,

Alsulaiman,

Hammersen

et al. 2023

Clinical Case Reports

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“…However, this approach seems to be more invasive with a more severe muscular and osseous involvement, a longer duration of surgery and a potential higher blood loss, which should be considered especially in pediatric patients. In the eight cases in our review all preoperative described symptoms resolved in 50% of cases [19,24], residual symptoms were described in 37.5% [25][26][27][28][29]. Additional surgery was necessary in 25% [26,27].…”

Section: Open Suboccipital Approachmentioning

confidence: 75%

Primary Occlusion of the Fourth Ventricle: Case Report and Review of the Literature

Wilbers,

Zawy Alsofy,

Schipmann

et al. 2023

Surgeries

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Idiopathic obstruction of the outlets of the fourth ventricle (FVOO) is a rare cause of hydrocephalus, which can be misdiagnosed as communicating hydrocephalus due to the enlargement of all four ventricles. Different surgical approaches are discussed in the literature. We present a case report of a 25-year-old male admitted with headache, vertigo, and nystagmus. The MRI scan showed a tetraventricular hydrocephalus with a patent aqueduct. After endoscopic third ventriculostomy (ETV), symptoms resolved. We performed a systematic review of the literature, covering 26 years, with the aim to investigate the symptoms, therapy, and outcome of primary FVOO, according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analysis) guidelines. We found 9 case reports and 2 case series and could extract a total of 34 cases. After ETV all symptoms resolved in 10 of 23 cases (43.5%), and in 13 of 23 cases (56.5%) symptoms improved partially. Seven cases (30.4%) required additional surgery. A decrease in ventricular volume occurred in most cases. In the 10 patients who were operated via fenestration, all symptoms resolved in 6 cases. ETV seems to be an effective treatment option for patients with idiopathic FVOO in a majority of cases. In special cases, fenestration of the foramen of Magendie may be suitable.

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The prepontine block and its relevance for the development and treatment of hydrocephalus

Richetta,

Shiran,

Constantini

et al. 2024

Childs Nerv Syst

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Objective Pulsatile CSF flow patterns include flow through the ventricles to the subarachnoid space and cisterns and from the infra- to the supratentorial subarachnoid space. In this study, we demonstrate how an obstruction at the level of the prepontine space may lead to obstructive hydrocephalus with specific radiological characteristics, as well as the implications for treatment options. Methods We retrospectively collected data of patients who underwent surgery between February 2010 and December 2022 for hydrocephalus secondary to a suspected prepontine block. One additional patient diagnosed with prepontine block who did not undergo surgery was also included. We excluded patients with a background of previous unrelated neurosurgical procedures or CNS infections. Results Six children and two adults were included. Three presented with hydrocephalus on imaging, without any other underlying pathology. Five had a suprasellar arachnoid cyst, with its lower border abating the pons and occluding the spinal subarachnoid space (SAS). All cases had an open aqueduct on T2 sagittal sequences, as well as an infracerebellar or retrocerebellar CSF collection. In most cases, a horizontal web was identified in the prepontine region. Seven cases were treated with an endoscopic fenestration. One patient subsequently underwent a shunt surgery. All the operated children reached normal developmental milestones after surgery. Conclusions This paper describes a rather small series of cases where clear obstruction was observed at the level of the prepontine subarachnoid space. We believe this anatomical subtlety adds to a better understanding of CSF pathways and the role of ETV in treating hydrocephalus, focusing on a small subgroup of patients without a clear obstruction.

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Hydrocephalus Caused by Primary Fourth Ventricle Outlet Obstruction: Our Experience and Literature Review

Cited by 7 publications

References 20 publications

The endoscopic treatment of fourth ventricle outlet obstruction: Report of two children and systematic review

The endoscopic treatment of fourth ventricle outlet obstruction: Report of two children and systematic review

Primary Occlusion of the Fourth Ventricle: Case Report and Review of the Literature

The prepontine block and its relevance for the development and treatment of hydrocephalus

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