External hydrocephalus associated with dural sigmoid sinus arteriovenous fistula: a case report

External hydrocephalus (EH) is a recognised sub-type of hydrocephalus associated with macrocephaly in infancy. EH is characterised by the enlargement of subarachnoid spaces (so-called subarachnomegaly) with a normal ventricular system on brain imaging. EH is traditionally considered benign and self-...

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Bibliographic Details
Published in:British journal of neurosurgery 2024-10, Vol.38 (5), p.1167-3
Main Authors: Barrit, Sami, El Hadwe, Salim, Lubicz, Boris, De Witte, Olivier
Format: Article
Language:English
Subjects:
Central Nervous System Vascular Malformations - complications
Central Nervous System Vascular Malformations - diagnostic imaging
Cerebral Angiography
Cranial Sinuses - abnormalities
Cranial Sinuses - diagnostic imaging
Cranial Sinuses - pathology
Humans
Hydrocephalus - diagnostic imaging
Hydrocephalus - etiology
Hydrocephalus - surgery
Infant
Magnetic Resonance Angiography
Male
Megalencephaly
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Summary:External hydrocephalus (EH) is a recognised sub-type of hydrocephalus associated with macrocephaly in infancy. EH is characterised by the enlargement of subarachnoid spaces (so-called subarachnomegaly) with a normal ventricular system on brain imaging. EH is traditionally considered benign and self-limiting, yet its pathophysiology remains puzzling. Mounting evidence for an association between EH and hydrovenous disorders reshapes our understanding of this condition and its management. To our knowledge, we report the first association between EH and dural arteriovenous fistula (dAVF) in a 17-months-old boy. As dAVF may be a life-threatening condition, early diagnosis and optimal treatment are critical. This case epitomises the intricacies of EH's aetiology and associated conditions requiring careful management. Therefore, we recommend considering MR angiography in EH's workup and long-term follow-up. Our experience supports the ongoing reconsideration of EH's presumed benignity.
ISSN:0268-8697
1360-046X
1360-046X
DOI:10.1080/02688697.2022.2101616