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Start free with EleplanDural sinus malformation with arteriovenous shunt
ORPHA:97339Morphological anomaly
Also called Cranial dural arteriovenous fistula · Dural sinus malformation with arteriovenous fistulae
What it is
A rare neurovascular malformation characterized by massive dilation of one or more dural sinuses typically associated with arteriovenous shunts. Anatomic types are the lateral type involving the jugular bulb, which presents with minimal symptoms, and the usually symptomatic midline type involving the confluens sinuum (torcular Herophili) and adjacent posterior sinuses. Complications include sinus thrombosis, venous infarction, and cerebral hemorrhage, as well as cardiac failure, macrocrania, and hydrocephalus. Spontaneous regression of the malformation may occur.
Key facts
- Age of onset
- Antenatal, Infancy, Neonatal
- Classified as
- Morphological anomaly
Signs and symptoms
Common30–79%
5Sometimes5–29%
28- Abnormal cerebellum morphology
- Abnormal facial vein morphology
- Abnormality of the posterior cranial fossa
- Abnormality of vision
- Carotid cavernous fistula
- Cerebellar hemorrhage
- Cerebral hemorrhage
- Chemosis
and 20 more in this range
Rare1–4%
15- Abnormality of speech or vocalization
- Apathy
- Ataxia
- Cerebral edema
- Cranial nerve paralysis
- Dementia
- Dilatation
- Diplopia
and 7 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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