1. Vascular Tumors and Malformations aka ”Vascular Anomalies”
Steven M. Dean, DO, FACP, RPVI
Professor of Clinical Internal Medicine
Division of Cardiovascular Medicine
The Ohio State University Wexner Medical Center

2. Classification
Vascular Anomalies
Tumors
Malformations

3. Vascular Malformations
Hemangioma
Cavernous hemangioma
Birthmarks (naevi)
Port-wine stains
Cystic hygroma
Archaic Terminology:
The reason for this confusion is the inconsistent…

4. Mulliken JB, Glowacki J (1982) Plast Reconstr Surg 69:412–422.
Vascular Anomalies
Hemangiomas are true neoplastic disorders/vascular tumors and pathohistologically demonstrate increased endothelial cell turn over rate; not present at birth & appear later in infancy.
Vascular malformations arise by dysmorphogenesis without increased endothelial proliferation; always present at birth [may not be clinically apparent]
Mulliken JB, Glowacki J (1982) Plast Reconstr Surg 69:412–422.

5. Hemangioma of Infancy [HOI]
Classification
Vascular Anomalies
Tumors
Malformations
Hemangioma of Infancy [HOI]
Congenital hemangiomas
Other

6. Hemangioma of Infancy Retroauricular infantile hemangioma:
Not present during birth but appear later in infancy
Proliferative phase during the first year of life
Spontaneous involution typically by age 10
Retroauricular infantile hemangioma:
1 year
6 years
Boon LM, Enjolras O, Mulliken JB.. J Pediatr 1996;128:

7. Vascular Malformations
Definition
“Embryologically developed, inborn errors of vascular morphogenesis leading to true structural anomalies”1
Capillary
Arterio venous
Venous
Let’s start out by clarifying exactly what we mean by the term “vascular malformation” The definition is “……” Clinically, this translates into a diverse family of syndromes and lesions. The arteriovenous malformation, or AVM, the low flow malformations, including venous, capillary, and lymphatic, and the syndromes, such as Klippel-Trenaunay, that combine several of these elements
Lymphatic
KTS
1. Mulliken JB, Glowacki J (1982) Hemangiomas and vascular malformations in infants and children. A classification based on endothelial characteristics. Plast Reconstr Surg 69:412–422.

8. Vascular Malformations
Etiology:
Abnormal cell signaling in 1st trimester
Genetic predilection1 in some but not all
Spectrum of disorders ranging from minimal to fatal
Overall Incidence = %
The etiology of these entities has not been clearly delineated, but there is a definited genetic component. Molecular studies demonstrate that VMs are caused by “…..”
1. Cohen, M.M., Jr., Vasculogenesis, angiogenesis, hemangiomas, and vascular malformations. Am J Med Genet, (4): p

9. Vascular Malformations
Classification:
Extratruncular VMs: originate during early stages of embryogenesis masses of abnormal small/mid-sized vessels infiltrating tissues -tendency to progress/recur
Truncular VMs: originate during late stages of embryogensis defects of fully formed and often named vessels hemodynamic significance
Both types can coexist

10. Modified Hamburg Classification of Congenital Vascular Malformations
Below ST. Sem Vasc Surgery 1993;6:

11. Modified Hamburg Classification of Congenital Vascular Malformations
Both types can coexist
Below ST. Sem Vasc Surgery 1993;6:

12. Classification
Vascular Anomalies
Tumors
Malformations
Fast Flow (AVM/AVF)
Low Flow
Combined
Lymphatic
Venous
As a group, Low Flow Malformations are less aggressive than High Flow Malformations, and grow with the patient (not progressive)
Capillary

13. Capillary Malformations

14. Lee, B.B.,. Ann Vasc Surg, 2004. 18(3): p. 380-92.
Venous Malformations
Clinical Features:
Pain, swelling
Varicosities or bluish skin discoloration
Soft and easily compressible lesions
Drain w/elevation
Bleeding and/or thrombosis
May be trivial or extensive
Osteomuscular hypertrophy
Lee, B.B.,. Ann Vasc Surg, (3): p

15. Low Flow Vascular Malformations
Klippel-Trenaunay Syndrome Triad:
Osteomuscular hypertrophy
Capillary malformations (“port wine stain”)
Venous + lymphatic lesions
Gloviczki, P., et al., Surgery, (3): p

16. Lateral or marginal embryonic vein
Lateral aspect of extremity
Truncular VM subtype
Avalvulosis
Severe reflux
DVT risk
Hallmark of KTS –venous component
Lee BB. Phlebolymphology 2012;19(4):

17. Low Flow Malformations: Diagnosis
Aplasia or hypoplasia of deep venous trunks
Present in 8 % of VM patients (with venous predominance)3
Prevalence of deep venous anomalies is even higher in patients with KTS
Lee, B.B., Ann Vasc Surg, (3): p

18. High Flow Malformations
Lidsky, Shortell, et al JVS in press

19. 11/9/2008 20