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Dural sinus malformation

Disease definition

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.

ORPHA:97339

Classification level: Disorder
  • Synonym(s):
    • Cranial dural arteriovenous fistula
    • Cranial dural arteriovenous malformations
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Antenatal, Neonatal, Infancy
  • ICD-10: Q28.3
  • ICD-11: LA90.2Y
  • OMIM: -
  • UMLS: C3839148
  • MeSH: -
  • GARD: -
  • MedDRA: -

  A summary on this disease is available in Deutsch (2020) Español (2020) Français (2020) Nederlands (2020)

Additional information

Further information on this disease

Patient-centred resources for this disease

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Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.