Skip to main content

Glossary

  • Capillary Malformation: In previous ISSVA classifications this term has been attached to both a specific diagnosis of an isolated red cutaneous stain, usually caused by a somatic pathogenic variant in the gene GNAQ, and to a general category of congenital cutaneous vascular malformations characterized by small vessels on pathology. In the current classification the specific lesion has been renamed as ‘port wine capillary malformation’; the term ‘capillary malformation’ is now a subcategory of slow-flow malformations, and may sometimes be applied non-specifically in the process of seeking a specific diagnosis.
  • Capillary-lymphatic-venous-malformation (CLVM): A type of combined vascular malformations. CLVM with hypertrophy is designated as Klippel-Trenaunay syndrome.
  • Central Conducting Lymphatic Anomaly (CCLA): Central conducting lymphatic anomaly (CCLA) encompasses disorders that are caused by dysfunction of the thoracic duct and/or cisternae chylae with subsequent reflux and leakage of lymphatic fluid, most commonly into the lungs and/or abdomen. Pleural and pericardial effusions, ascites, and generalized edema are common and can result in organ dysfunction, protein loss, and infections.
  • CLA: Complex lymphatic anomalies, includes lymphatic malformations that present structural complexity, multisystem involvement and functional impairment.
  • CLOVES Syndrome: CLOVES is now considered to be a part of the PIK3CA-related overgrowth spectrum. It is a rare, sporadic disorder characterized by tissue overgrowth and complex vascular anomalies. The manifestations are variable ranging from mild to severe anomalies. These abnormalities are typically present at birth, however some, such as fatty overgrowth, progress with age.
  • CM: capillary malformation
  • CNS: central nervous system
  • CVM: CM + VM. Capillary-venous malformation. A type of combined vascular malformations.