Subarachnomegaly—venous congestion of infancy
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Date
2021-11
Publication Type
Journal Article
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yes
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Abstract
Purpose
Multiple names within the literature refer to a clinical picture affecting infants and consisting of a large or fast growing head circumference with enlarged cortical subarachnoid spaces (CSAS) while cranial sutures are open. This myriad of terms demonstrates the confusion about the entity, that may even group together different etiological processes. In this review, we aim to shed light on this matter in an effort to restate the defining features of the clinical picture and sum the evidence and current understanding of its pathophysiology and related imaging findings.
Methods
Extensive and updated review of the literature with special focus on defining features, clinical history with long term evaluation and pathophysiological process.
Results
Functional and molecular CSF studies as well as clinical evidence challenges the common pathophysiological theory based on non-functional arachnoid villi. Conversely, there is increasing evidence supporting cerebro-venous system abnormalities as the main pathophysiological factor. Additionally, long term cohorts studies show that it may have subtle but irreversible neurodevelopmental consequences.
Conclusion
Subarachnomegaly is an age-related condition of the infancy with radiological enlargement of CSAS and often self limiting course. However, considering the evidence on pathophysiology as outlined herein and long term outcome reports, further research effort is needed to assess the consequences of venous outflow impairment and enlarged CSAS and how this relates to imaging findings and neurodevelopment test results later in life.
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published
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Journal / series
Volume
37 (11)
Pages / Article No.
3455 - 3463
Publisher
Springer
Event
Edition / version
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Date collected
Date created
Subject
Subarachnomegaly; Macrocephaly; Cortical subarachnoid space; Venous sinuses; MR phlebography
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Notes
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Related publications and datasets
Is referenced by: 10.1007/s00381-021-05425-z