Seminars in Pediatric Surgery 23 (2014) 158161

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Seminars in Pediatric Surgery

journal homepage: www.elsevier.com/locate/sempedsurg

ISSVA classication
Roshni Dasgupta, MD, MPHa,n, Steven J. Fishman, MDb
a
b

Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnett Ave, Cincinnati, Ohio 45229
Department of Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts

a r t i c l e in f o

abstract

Keywords:
ISSVA Classication
Vascular tumors
Vascular malformations

Mulliken and Glowacki, in 1982 created a classication system of vascular anomalies which divided
vascular anomalies into tumors and malformations which provided the framework for great advances in
the management of these patients. This classication system was recently expanded at the 2014 ISSVA
workshop in Melbourne. This revision again provides much greater detail including newly named
anomalies and identied genes to account for recent advances in knowledge and clinical associations.
& 2014 Published by Elsevier Inc.

Mulliken and Glowacki,1 in 1982, created a classication system

of vascular anomalies. This classication was later adopted in 1996
by the International Society for the Study of Vascular Anomalies
(ISSVA) at the 1996 meeting in Rome.2 This schema divided
vascular anomalies into tumors and malformations and provided
the framework for great strides in research and treatment in the
eld. The classication system was recently expanded at the 2014
ISSVA workshop in Melbourne.3 This revision was essential to fully
encompass the advances in knowledge. This new system again
divides vascular anomalies into tumors and malformations but
provides much greater detail, including newly named anomalies

and identied genes. In addition, clinical associations such as

coagulopathies have been incorporated (Figure 1).
Vascular tumors are divided into benign, locally aggressive,
and malignant entities (Figure 2). Hemangiomas are further
sub-classied by pattern and location to encompass syndromic
associations such as PHACES and LUMBAR (Figure 3). Vascular
malformations are divided into simple and combined, based on
vessel types that are involved. Each simple malformation (capillary, venous, lymphatic, lymphedema, and arterio-venous) is then
subdivided into known subtypes. The combined vascular malformations are named specically for the vessels involved in the

Fig. 1. ISSVA classication for vascular anomalies.

Corresponding author.
E-mail address: roshni.dasgupta@cchmc.org (R. Dasgupta).

http://dx.doi.org/10.1053/j.sempedsurg.2014.06.016
1055-8586/& 2014 Published by Elsevier Inc.

R. Dasgupta, S.J. Fishman / Seminars in Pediatric Surgery 23 (2014) 158161

Fig. 2. ISSVA classication for vascular tumors.

Fig. 3. Infantile hemangioma.

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R. Dasgupta, S.J. Fishman / Seminars in Pediatric Surgery 23 (2014) 158161

Fig. 4

Fig. 5

R. Dasgupta, S.J. Fishman / Seminars in Pediatric Surgery 23 (2014) 158161

malformation, allowing greater consistency with nomenclature

and ease of communication between specialists (Figure 4).
Vascular malformations that are associated with other anomalies such as overgrowth in KlippelTrenaunay syndrome are also
categorized separately (Figure 5). This identies the type of
vascular malformation and the associated anomalies. Recognition
of associated features is important for the comprehensive care of
the patient, as many of these associated anomalies require intervention and long-term follow-up.
The gures presented here are adapted from the ofcial ISSVA
Classication. The entire comprehensive Classication including
further details of currently identied causal genes and clinical

161

associations of vascular anomalies is available on the ISSVA

website (www.issva.org). The online presentation of the Classication is in a dynamic format, allowing the reader to link between
tables to see greater detail about any particular lesion.
References
1. Mulliken JB, Glowacki J. Hemangiomas and vascular malformations in infants
and children: a classication based on endothelial characteristics. Plast Reconstr
Surg. 1982;69:412422.
2. Enjolras O, Mulliken JB. Vascular tumors and vascular malformations (new
issues). Adv Dermatol. 1998;12:375.
3. www.issva.org.