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Editorial

The Origin of the Field of Vascular Anomalies

Mulliken, John B.,*

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Journal of Vascular Anomalies: December 2020 - Volume 1 - Issue 1 - p e001
doi: 10.1097/JOVA.0000000000000001
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We must change the truth a little in order to remember it.

George Santayana (1863–1952)

The Last Puritan (1935)

In 1974, my chief, Dr Joseph Murray, gave me an academic push and suggested I study a common disorder, such as hemangioma or skin cancer. In the clinic, I noted that every vascular lesion, whether pink, red, or blue, was called “hemangioma.” Textbooks were confusing as were the recommended treatments. I began working in Dr Judah Folkman’s Vascular Biology Laboratory and recruited Dr Julie Glowacki to join me. Our purpose was to understand the biology of vascular lesions using special histopathologic stains. Dr Folkman suggested we ask a critical question of Mother Nature: “What cells are upregulated in vascular anomalies?”

About this time, I met Tony Young, a surgical fellow at the Peter Bent Brigham Hospital. We expressed a mutual fascination with vascular anomalies. Tony wrote his Cambridge thesis (“Mixed Vascular Deformities of the Limbs”) under the guidance of Prof. John Kinmonth at St. Thomas’s Hospital, London.

Dr Murray suggested that Tony and I organize an “International Workshop for the Study of Vascular Anomalies.” The first two biennial meetings were in Boston (1976, 1978), followed by London (1980), Paris (1982), Milan (1984), Boston (1986), Hamburg (1988), and Amsterdam (1990). The irrepressible Wayne Yakes was chairman at the 9th meeting in Denver (1992) when we agreed to change the name from “Workshop” to “The International Society.” In Budapest (1994), the ISSVA bylaws were accepted as written by the Swiss vascular surgeon, Robert Schobinger, and registered in Le Moniteur Belge, the official journal of the Belgium government (Figure 1). We voted to adopt the broken red rectangle as our logo, designed by Schobinger.

Figure 1
Figure 1:
10th ISSVA meeting in Budapest, 1994. Left to right: John Mulliken, Laurence Boon, Miikka Vikkula, and Romain van Wicjk from Université catholique de Louvain, Brussels.

Most memorable was the 11th ISSVA meeting in Rome (1996). Attendees recognized that our field was encumbered by a chamber of terminological horrors and eponymous syndromes. Our interdisciplinary specialty could not advance until we communicated in a common nosologic tongue. There were two competing schools of classification: the allied French-American team and the German contingent from Hamburg. The respected Robert Schobinger was elected our first president (Figure 2). Discussion at the business meeting was heated. Professor Schobinger gave everyone a chance to speak—he fluently spoke all three languages—and “The ISSVA Classification” carried the day (Figure 3). Major papers presented in Rome included “PHACE association” by Ilona Frieden, “Congenital hemangiomas” by Laurence Boon, and “Kasabach-Merritt phenomenon with kaposiform hemangioendothelioma—not infantile hemangioma,” independently given by Odile Enjolras and the author.

Figure 2
Figure 2:
President Robert Schobinger (center); back row: Marie-Claire Riché, Anthony Young, and author, Rome, 1966.
Figure 3
Figure 3:
First ISSVA Classification, accepted at 11th Meeting in Rome, 1996. ISSVA indicates xxx.

Centers for the care of vascular anomalies began to rise worldwide in many pediatric and adult hospitals. The revolution in molecular genetics has had a major impact. Cellular events in proliferation and regression of infantile hemangiomas are better understood. The causal hypothesis is a somatic mutation in a hemangioma stem cell, but the precise genetic mechanism remains elusive. Families with inheritable vascular anomalies began to cluster in centers; this facilitated discovery of germline mutations. New genetic technology revealed somatic mutations in the main categories of vascular malformations, particularly those associated with skeletal and soft tissue overgrowth. Knowledge of molecular pathways opened a new era of targeted pharmacologic therapy to manage complications and to control recurrence of a vascular anomaly after radiological and surgical interventions.

I am fortunate to have been guided toward the field of vascular anomalies and grateful to all my colleagues who helped me fossick in its fertile soil. ISSVA has grown from a few fervent members to become a mature scientific organization that merits its own journal.

© 2020 by Lippincott Williams & Wilkins, Inc.