Primary intraosseous venous malformation

Orpha code: 140436OMIM code: 606893

Definition

Primary intraosseous venous malformation is a rare, genetic vascular anomaly characterized by severe blood vessel expansion (most frequently within the craniofacial bones) with painless bone enlargement (usually of mandibule, maxilla and/or orbital, nasal, and frontal bones), typically resulting in facial asymmetry and contour deformation. Midline abnormalities, such as diastasis recti, supraumbilical raphe, and hiatus hernia, are commonly associated. Additional features reported include gingival bleeding, ectopic tooth eruption, exophthalmos, loss of vision, nausea, and vomiting.

Disease data
Classification

Disease

Synonyms
Intraosseous hemangioma
Malformacja naczyniowa kości
Naczyniak wewnątrzkostny
Osseous venous malformation
ORPHA code
140436
OMIM code
606893
ICD10 code
D18.0
ICD11 code
-

No additional description.

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