Quadrigeminal arachnoid cyst with perinatal encephalocele

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Abstract

Introduction

Quadrigeminal arachnoid cyst (QAC) associated with encephalocele is rare; and while some treatments have been developed in recent years, no definite therapeutic approach for QAC has been established. Endoscopic treatment for arachnoid cyst is gaining popularity because it is relatively less invasive to the normal brain tissues.

Case presentation

The patient, a 4-year-old girl, presented with QAC associated with congenital occipital encephalocele. At the age of 1 month, repair of the perinatal encephalocele had been performed at another institute. An asymptomatic arachnoid cyst remained in the posterior fossa, which was closely monitored with follow up. At age 4 years, the patient started to complain of headache, which gradually increased in both strength and frequency. Magnetic resonance imaging (MRI) revealed cerebellar compression due to cyst enlargement. We performed neuroendoscopic cyst fenestration with an occipital bone approach. Post-operative MRI showed reduced size of the cyst, and the headache dramatically improved and resolved.

Discussion

The standard treatment of QAC is still controversial; however, our successful use of endoscopic fenestration toward the third ventricle indicates its efficacy and safety. QACs have been classified into 3 types based on their expansion mechanisms; our case might suggest another possible mechanism of QAC development.

Conclusion

In our case, endoscopic cyst fenestration was successful for QAC with perinatal encephalocele. However, long-term follow-up and analysis of similar cases are needed to determine its effectiveness.

Related collections

Most cited references 18

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Prevalence and natural history of arachnoid cysts in children.

Karin M Muraszko, Zackary Boomsaad, Hugh Garton … (2010)

Arachnoid cysts are a frequent finding on intracranial imaging in children. The prevalence and natural history of these cysts are not well defined. The authors studied a large consecutive series of children undergoing MR imaging to better define both the MR imaging-demonstrated prevalence and behavior of these lesions over time. The authors reviewed a consecutive series of 11,738 patients who were 18 years of age or younger and had undergone brain MR imaging at a single institution during an 11-year period. In the patients in whom intracranial arachnoid cysts were identified, clinical and demographic information was recorded and imaging characteristics, such as cyst size and location, were evaluated. Prevalence data were analyzed using univariate and multivariate logistic regression, linear regression, and ANOVA. All patients with sufficient data (repeat MR imaging studies as well as repeated clinical evaluation over at least 5 months) for a natural history analysis were identified. This group was assessed for any change in symptoms or imaging appearance during the follow-up interval. Three hundred nine arachnoid cysts (2.6% prevalence rate) were identified. There was an increased prevalence of arachnoid cysts in males (p < 0.000001). One hundred eleven patients met all criteria for inclusion in the natural history analysis. After a mean follow-up of 3.5 years, 11 arachnoid cysts increased in size, 13 decreased, and 87 remained stable. A younger age at presentation was significantly associated with cyst enlargement (p = 0.001) and the need for surgery (p = 0.05). No patient older than 4 years of age at the time of initial diagnosis had cyst enlargement, demonstrated new symptoms, or underwent surgical treatment. Arachnoid cysts are a common incidental finding on intracranial imaging in pediatric patients. An older age at the time of presentation is associated with a lack of clinical or imaging changes over time.

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Ultrastructure and pathogenesis of intracranial arachnoid cysts.

S S Rengachary, I. Watanabe (1980)

A detailed light, transmission, and scanning electron microscopic study of the arachnoid cyst wall was made in four cases and compared with that of the normal arachnoid mater in the human. Two hundred and eight reported cases of arachnoid cysts were analyzed to evaluate the anatomical distribution of these lesions and to get r an insight into their pathogenesis. The structural features of the arachnoid cyst wall that distinguish it from the normal arachnoid membrane are as follows: (1) splitting of the arachnoid membrane at the margin of the cyst, (2) a very thick layer of collagen in the cyst wall, (3) the absence of traversing trabecular processes within the cyst, and (4) the presence of hyperplastic arachnoid cells in the cyst wall, which presumably participate in collagen synthesis. The distribution of arachnoid cysts in two hundred and eight reported cases was as follows: Sylvian fissure, 49%; cerebellopontine angle, 11%; supracollicular area, 10%; the vermis, 9%; sellar and suprasellar area, 9%; interhemispheric fissure, 5%; cerebral convexity, 4%; and the clival and interpeduncular area, 3%. At each site, except possibly on the cerebral convexity, the cyst was associated with a normal subarachnoid cistern. This striking and nearly invariable association of arachnoid cysts with normal subarachnoid cisterns prompts the authors to hypothesize that arachnoid cysts represent a congenital anomaly of the developing subarachnoid cisterns in early intrauterine life. It is postulated that, during the process of the complex folding of the primitive neural tube and the formation of normal subarachnoid cisterns, an anomalous splitting of the arachnoid membrane occurs.

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Intracranial arachnoid cysts in children: a review of pathogenesis, clinical features, and management.

Jayaprakash Gosalakkal (2002)

Arachnoid cysts are developmental anomalies that are most often diagnosed in childhood. They are often discovered as incidental findings found on imaging. Occasionally they may produce symptoms because of expansion or bleeding. There may be underlying maldevelopment of the cortex especially the temporal lobe. There is controversy regarding the role and the type of surgery indicated in its treatment. Recent descriptions of aphasia and attention-deficit disorders associated with these cysts indicate that we do not fully understand this entity. There is also no acceptable explanation for the male preponderance and increased incidence on the left side. The distribution, clinical features, treatment modalities, and some unusual syndromes associated with arachnoid cysts in children are discussed in this review.

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Author and article information

Contributors

Takao Tsurubuchi: t-tsurubuchi@md.tsukuba.ac.jp

Journal

Journal ID (nlm-ta): Childs Nerv Syst

Journal ID (iso-abbrev): Childs Nerv Syst

Title: Child's Nervous System

Publisher: Springer Berlin Heidelberg (Berlin/Heidelberg )

ISSN (Print): 0256-7040

ISSN (Electronic): 1433-0350

Publication date (Electronic): 23 April 2020

Publication date PMC-release: 23 April 2020

Publication date (Print): 2020

Volume: 36

Issue: 7

Pages: 1393-1397

Affiliations

[1 ]GRID grid.428872.3, ISNI 0000 0004 0378 1711, Division of Pediatric Neurosurgery, , Ibaraki Children’s Hospital, ; Mito, Ibaraki Japan

[2 ]GRID grid.20515.33, ISNI 0000 0001 2369 4728, Department of Neurosurgery, Faculty of Medicine, , University of Tsukuba, ; 1-1-1 Tennodai, Tsukuba, Ibaraki Prefecture 305-8575 Japan

[3 ]GRID grid.20515.33, ISNI 0000 0001 2369 4728, Department of Neurosurgery, Faculty of Medicine, , University of Tsukuba, ; Tsukuba, Ibaraki 305-8575 Japan

Article

Publisher ID: 4626

DOI: 10.1007/s00381-020-04626-2

PMC ID: 7300100

PubMed ID: 32328704

SO-VID: c8327193-8815-4dc6-933f-bda99860c2ca

License:

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

History

Date received : 10 February 2020

Date accepted : 16 April 2020

Custom metadata

ScienceOpen disciplines: Neurology

Keywords: quadrigeminal arachnoid cyst,endoscopic surgery,cyst fenestration,encephalocele

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ScienceOpen disciplines: Neurology

Keywords: quadrigeminal arachnoid cyst, endoscopic surgery, cyst fenestration, encephalocele

Quadrigeminal arachnoid cyst with perinatal encephalocele

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Abstract

Introduction

Case presentation

Discussion

Conclusion

Related collections

Stroke MRI

Most cited references 18

Prevalence and natural history of arachnoid cysts in children.

Ultrastructure and pathogenesis of intracranial arachnoid cysts.

Intracranial arachnoid cysts in children: a review of pathogenesis, clinical features, and management.

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Contributors

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