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”.
| Syndromes | Anomalies |
|---|---|
| Klippel-Trénaunay syndrome | CM + venous malformation +/- lymphatic malformation + extremity overgrowth |
| Parkes Weber syndrome | CM + multiple arteriovenous fistulas / AVM + extremity overgrowth |
| Sturge-Weber syndrome | Facial 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 syndrome | CM + lymphatic malformation + venous malformation +/- arteriovenous malformation + asymmetric (lipomatous, osseous) overgrowth |
| Proteus syndrome | CM + 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.
