Syndromes with capillary malformation

  • Chapter: Capillary malformations

    Article: 13 of 14

    Update: Mar 30, 2021

  • Author(s): Ott, Hagen

Capillary malformations (CM) can be associated with a variety of other anomalies in the sense of a syndrome. These associations of capillary malformations with other abnormalities are summarized in the current ISSVA classification under the heading “vascular malformations associated with other anomalies”.

Association of capillary malformation with other anomalies

Klippel-Trénaunay syndromeCM + venous malformation +/- lymphatic malformation + extremity overgrowth
Parkes Weber syndromeCM + multiple arteriovenous fistulas / AVM + extremity overgrowth
Sturge-Weber syndromeFacial and leptomeningeal CM + abnormalities of the eyes +/- bone and/or soft tissue overgrowth
Macrocephaly-CM syndrome (MCAP)CM + macrocephaly + polymicrogyria
Microcephaly-CM syndrome (MICCAP)CM + microcephaly + epilepsy
CLOVES syndromeCM + lymphatic malformation + venous malformation +/- arteriovenous malformation + asymmetric (lipomatous, osseous) overgrowth
Proteus syndromeCM + venous malformation and/or lymphatic malformation + asymmetric extensive circumscribed overgrowth
PTEN hamartoma syndrome (Cowden/Bannayan-Riley-Ruvalcaba syndrome)CM + venous malformation +/- arteriovenous malformation + adipose tissue hyperplasia + hamartomas


It is common for dysplasias of other vessels, especially venous and lymphatic malformations, to be associated with the capillary malformation. These are then referred to as combined malformations and need not be associated with other anomalies. Thus, patients with a capillary malformation should always be evaluated for the presence of additional vascular anomalies.