1 Classification of Vascular Anomalies


1 Classification of Vascular Anomalies

Francine Blei and Michael Wassef

1.1 Introduction

The key to understanding vascular anomalies is speaking a common language so that pathologists, clinicians, and researchers use a consistent terminology. This is important, as it is essential to appreciate that not every benign vascular lesion is a hemangioma. Classification systems importantly provide common terminology and serve as a guide for diagnosis, management, and research endeavors. Fig. 1.1 , Fig. 1.2 , Fig. 1.3 , Fig. 1.4 , Fig. 1.5 , Fig. 1.6 , Fig. 1.7 , Fig. 1.8 , Fig. 1.9 , Fig. 1.10 , Fig. 1.11 , and Fig. 1.12 demonstrate several types of hemangiomas and vascular malformations described throughout the text and tables.

Fig. 1.1 Hemangiomas. (a,b) Ulcerated superficial hemangioma of infancy involuting over time. (c) Large subcutaneous hemangioma of infancy. (d) Subcutaneous periorbital hemangioma of infancy with ptosis, deprivational amblyopia and astigmatism. (e,f) Segmental facial hemangiomas of infancy in two patients with PHACE syndrome.
Fig. 1.2 Rapidly involuting congenital hemangioma at birth (a) and after several months (b), spontaneous involution without treatment.
Fig. 1.3 Kaposiform hemangioendothelioma of neck and cheek: boggy vascular lesion with Kasabach Merritt phenomenon (profound thrombocytopenia, hypofibrinogenemia).
Fig. 1.4 Capillary malformation: facial trigeminal distribution warrants evaluation for potential Sturge-Weber syndrome.
Fig. 1.5 Extensive venous malformation: facial, intraoral, and cerebral.
Fig. 1.6 Lymphatic malformation. Left cheek lymphatic malformation has been present since birth and growing along with the child; the lesion is soft, compressible, and transilluminates.
Fig. 1.7 Hereditary hemorrhagic telangiectasia: multiple vascular lesions on tongue, lips, and skin, with intraoral bleeding and strong family history of similar findings.
Fig. 1.8 Blue rubber bleb nevus syndrome. Note the multifocal vascular malformations, which are frequently present throughout the gastrointestinal tract, causing bleeding.
Fig. 1.9 Phosphatase and tensin homolog gene PTEN hamartoma syndrome. Note the tricholemmas, hyperpigmentation, and papules on gums in a patient with PTEN mutation who is at risk for early malignancies of thyroid, breast, colon, and genitourinary tract.
Fig. 1.10 RASA-1 capillary malformation – arteriovenous malformation. Note the enlarged hand and arm resulting from arteriovenous malformation. Patient and father had several macular red–brown cutaneous capillary malformations.
Fig. 1.11 Glomovenous malformation. Ectatic veins in checkerboard pattern, with extensive blue–purple vascular discoloration.
Fig. 1.12 (a,b) Proteus syndrome. Skeletal distortion, cerebriform plantar surface of feet in a patient with cystic lesions typical of Proteus syndrome.

Malan and Puglionisi were the first to distinguish vascular malformations based on vessel type and flow characteristics. 1 ,​ 2 As a result of a workshop in Hamburg, Germany, in 1988, the Hamburg Classification evolved to replace commonly used eponyms, separate vascular malformations anatomically based on predominant vessel type, and further categorize into truncular (involving major axial vessels) or extratruncular (involving branches of major vessels) features. 3 This classification has been further refined and is shown in Table 1.1 . 4 ,​ 5 Abnormalities of truncular vessels include aplasia or obstruction, stenosis, coarctation, dilatation, agenesis, or aplasia on the one hand and aneurysms or persistent embryonic channels or aneurysm; extratruncular pathologies can be local or infiltrating. Extratruncular and truncular forms are believed to result from developmental arrest in early and later embryogenesis, respectively. 5

Table 1.1 Hamburg Classification of vascular anomalies 3 with modifications 4 ,​ 5

Predominant vessel




Aplasia or obstruction





Aplasia or obstruction





Aplasia or obstruction




Arteriovenous malformations with shunting

Deep arteriovenous fistula

Superficial arteriovenous fistula



Combined or mixed

Arterial and venous without shunt

Hemolymphatic with or without shunt


Infiltrative hemolymphatic

Limited hemolymphatic

Mulliken and Glowacki then proposed a classification ( Table 1.2 ) based on clinical and endothelial cell characteristics. 6 This classification provided the first fundamental separation of vascular anomalies into lesions with a proliferative component (vascular tumors) versus relatively static vascular malformations (based on principal anomalous vasculature) in an article that greatly influenced the field of vascular anomalies and is reported to be the most frequently cited article in the plastic surgery literature. 7

Table 1.2 Classification of vascular anomalies according to Mulliken and Glowacki 6


Vascular malformation

Proliferative phase


Involuting phase






Capillary venous


Capillary venous/lymphatic

The table was further refined by the 1996 workshop of the International Society for the Study of Vascular Anomalies (ISSVA), augmenting the more rudimentary previous classification and providing a basic separation of lesions based on histologic and rheologic features; new subcategories of diagnoses; and updated histologic, genetic information, and syndromes ( Table 1.3 ). These distinctions provide a framework to provide a common nomenclature and distinguish vascular anomalies into functional and descriptive categories, enabling optimal assessment and treatment for patients. 5 ,​ 8 ,​ 9

Table 1.3 International Society for the Study of Vascular Anomalies classification of vascular anomalies

Vascular tumors

Vascular malformations

Infantile hemangiomas

(hemangiomas of infancy)

(GLUT-1 positive)

Slow-flow vascular malformations:

CM (port-wine stain, telangiectasia, angiokeratoma)

Venous malformation (VM) (common sporadic VM, Bean syndrome, familial cutaneous and mucosal VM, glomovenous malformation, Maffucci syndrome, lymphatic malformation

Congenital hemangiomas

Rapidly involuting congenital hemangioma (RICH)

Noninvoluting congenital hemangioma (NICH)

Fast-flow vascular malformations:

arterial malformation, ateriovenous fistula, AVM

Tufted angioma (with or without Kasabach-Merritt syndrome)

Complex-combined vascular malformations:


Kaposiform hemangioendothelioma (with or without Kasabach-Merritt syndrome)

Spindle cell hemangioendothelioma

Other rare hemangioendotheliomas (epitheliod, composite, retiform, polymorphous, Dabska tumor, lymphangioendothelioma, etc.)

Dermatologic-acquired vascular tumors (pyogenic granuloma, targetoid hemangioma, glomeruloid hemangioma, microvenular hemangioma, etc.)

Abbreviations: AV, arteriovenous; AVM, arteriovenous malformation; C, capillary; CM, capillary malformation; GLUT1, erythrocyte glucose transporter protein 1; V, venous, L, lymphatic; M, malformation; V, venous.

Used with permission from Enjolras O, Wassef M, Chapot R. Color Atlas of Vascular Tumors and Vascular Malformations. 1st ed. New York: Cambridge University Press; 2006.

One publication examined the significance of histopathologists to incorporate this classification in the evaluation of vascular anomaly samples, underscoring the impact of ISSVA classification in attaining the appropriate diagnosis. 10 However, vascular anomalies represents a spectrum, and there may be overlapping features of a vascular tumor and malformation in one patient. 11 Additionally, more accurate diagnoses can be achieved by providing adequate clinical information to radiologists and pathologists who are aligned with this terminology. 12 ,​ 13 An updated ISSVA classification, which incorporates new entities, genetic and pathology information, was approved by the ISSVA membership in April 2014 and is available for citation from the ISSVA website, www.issva.org.

Complementary to the ISSVA classification are staging systems for specific vascular malformations, which some clinicians find advantageous in treatment planning. Schobinger presented a staging schema for arteriovenous malformations (AVMs) based on clinical behavior, from quiescent to destructive ( Table 1.4 ). 14

Table 1.4 Schobinger staging for arteriovenous malformations 14






Pink–blue stain, warmth, arteriovenous shunting



Stage I, plus enlargement, pulsations, thrill, bruit, tortuous/tense veins



Stage II, plus dystrophic skin changes, ulceration, bleeding, persistent pain, or tissue necrosis ± lytic osteolysis



Stage III, plus congestive cardiac failure

Two groups endorsed a staging system for cervical lymphatic malformations based on disease extent, suggesting a correlation between stage, complication rate, clinical or surgical outcome, and prognosis. 15 ,​ 16 In addition to providing guidelines for management by forecasting prognosis, the use of consistent terminology can provide uniformity in clinical, surgical, and radiologic research reporting 17 ( Table 1.5 ).

Table 1.5 Staging for cervical lymphatic malformations 15 ,​ 16




Unilateral infrahyoid


Unilateral suprahyoid


Unilateral infrahyoid and suprahyoid


Bilateral suprahyoid


Bilateral infrahyoid and suprahyoid


Bilateral infrahyoid

Other groups have suggested classifications with potential prognostic and treatment-related implications; for example, venous malformations can be classified according to the degree of dysplasia and drainage pattern, based on clinical severity, degree of valvular incompetence, and other measureable changes, or the CEAP (Clinical, Etiological, Anatomical, and Pathological elements) classification. 18 ,​ 19 ,​ 20

Nonetheless, a thorough history (including family history), physical examination, and appreciation of anatomical patterns of distribution of vascular lesions facilitate ascertainment of the correct diagnosis, evaluation, and management plan. Incorporating the age of presentation (prenatal, congenital, postnatal), clinical behavior (static, proliferative, involuting), location, and number of lesions and appreciating existing and impending morbidities are important in determining the extent and pace of further evaluation and potential intervention. Ascertaining the correct diagnosis remains a problem, however, and it is not unusual to receive a radiology or pathology report stating that the diagnosis is most compatible with hemangioma when clearly it is a vascular malformation.

Multidisciplinary vascular anomalies teams have been established in many institutions, providing a structured forum for expert subspecialists to review diagnostic studies and assess patients with vascular anomalies, most of whom require input from more than one medical discipline. This need is underscored by the high incidence of incorrect diagnoses of referred patients. 21 One study approximated that PubMed-listed publications during 2009 containing the term hemangioma was used to describe a vascular anomaly in 71.3% of publications, independent of medical subspecialty, concluding that “terminological imprecision is prevalent among both medical and surgical fields. Inaccurate designation of the vascular anomaly is associated with an increased risk of erroneous management.” 22 Correspondingly, the same researchers reported an incorrect referral diagnosis in 47% of patients referred to their vascular anomalies multidisciplinary referral center. 23 Classification of vascular anomalies can also be viewed by the time of presentation: prenatal, postnatal, and evolving over time.

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Jun 15, 2020 | Posted by in HEAD AND NECK SURGERY | Comments Off on 1 Classification of Vascular Anomalies
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