ISSVA World Congress 2022: The Latest in Vascular Anomalies

Vancouver – British Columbia – Canada 10-13 May, 2022 Meeting Abstracts Disclaimer The opinions or views expressed in this supplement are those of the authors and do not necessarily reflect the opinions or recommendations of ISSVA or Wolters Kluwer. Although published, these abstracts have not undergone serious scientific scrutiny and standard peer review process outside of being reviewed and accepted by the Scientific Committee of the ISSVA World Congress. To maintain the purity of the abstracts submitted, the following published abstracts are unedited material as provided by the authors. All authors were offered the opportunity to withdraw or keep their abstract in this publication. Previously published works, and abstracts the authors ask to withdraw have been omitted from this abstract supplement. Any information, methods, compounds or experiments described herein should be verified independently by the reader. No responsibility is assumed by the publisher or ISSVA for any injury and/or damage to persons or property from any use or operation of any methods, products, instructions, or ideas contained in the material herein. Oral Abstracts Session 1: Vascular Tumors Infantile Hemangioma Masqueraders Ashley Ng (University of Wisconsin - Madison); Eric Monroe (University of Wisconsin - Madison); Kara Gill (University of Wisconsin - Madison); Catharine Garland (University of Wisconsin - Madison); Jason Pinchot (University of Wisconsin); Carol Diamond (University of Wisconsin - Madison); Beth Drolet (University of Wisconsin - Madison); Lisa Arkin (University of Wisconsin School of Medicine) Purpose: Vascular anomalies in children encompass a wide spectrum of diseases with overlapping features. Many vascular anomalies are mistaken for infantile hemangiomas, which are characterized by high-flow vascular lesions that appear within weeks of birth, grow rapidly within the first few months of age, and typically begin to involute around the first year of life. The purpose of this presentation is to describe a series of infantile hemangioma “masqueraders” with an educational focus on useful strategies for the highly trained specialist to correctly diagnose vascular anomalies in children. Methods: Patients were identified by physician members of the multi-interdisciplinary birthmarks and vascular anomalies clinic at the University of Wisconsin-Madison. Cases included those with the presumed diagnosis of infantile hemangiomas where further workup and evaluation established an alternative diagnosis. Results: We herein describe a series of 6 cases of hemangioma masqueraders, most without response to oral propranolol (Table 1). Clinical and radiographic pearls to differentiate these lesions from infantile hemangiomas will be discussed. Conclusion: Clinical history, physical exam, radiologic imaging, and interdisciplinary assessment can help with the appropriate diagnosis of hemangioma masqueraders. Clinical history of rapid growth in the first few months of life may still accompany other vascular anomalies, and clinicians should stay attuned to alternative diagnoses with further workup, particularly when beta-blocker treatment produces minimal improvement. Analysis of therapeutic decisions for infantile hemangiomas: A prospective study comparing the Hemangioma Severity Scale with the Infantile Hemangioma Referral Score Tong Qiu (University of Sichuan at China); Yi Ji (West China Hospital of Sichuan University) Purpose: In view of the high incidence of infantile hemangioma (IH) in infants and young children, a comprehensive and reasonable evaluation scale for treatment is urgently needed. This study compared the influence of the Hemangioma Severity Scale (HSS) and the Infantile Hemangioma Referral Score (IHReS) on treatment decisions for infantile hemangioma patients. We aimed to establish a reliable and effective evaluation method for treatment decisions. Methods: This study conducted a prospective cross-sectional study to determine whether treatment was needed for IH patients after evaluation with HSS and IHReS. Results: A total of 266 consecutive referred IH patients were evaluated for the risk of IH, and the treatment rate was 80.8%. The area under the curve (AUC) of the subject receiver operating characteristic curve (ROC) of treatment decision-making after assessment by the HSS was 0.703 (95% CI: 0.634-0.772), and that after assessment by the IHReS was 0.892 (95% CI: 0.824-0.960). Conclusion: For decisions regarding the treatment of IH patients, the IHReS has a higher efficiency and sensitivity than the HSS. However, the specificity of the IHReS is lower than that of the HSS. Infantile hemangioma sensitivity to propranolol treatment relies on unique cellular and extracellular features Sandra Oucherif (U1035 BMGIC University of Bordeaux); Priscilla Kaulanjan-Checkmodine (Bordeaux University); Sorilla Prey (Bordeaux University Hospital); Muriel Cario-Andé (Bordeaux University); Christine Léauté-Labreze (Bordeaux University Hospital); Alain Taieb (INSERM 1035 Bordeaux); Hamid Reza Rezvani (Bordeaux University); Francois Moisan (Bordeaux University) Purpose: Propranolol, a nonselective β-adrenergic receptor blocker, is the first-line treatment for severe infantile hemangiomas (IH). The antitumor effect of propranolol is still largely misunderstood, due to the lack of a validated in vitro model of IH. Aquaporin-1 (AQP1), an aqueous channel modulated in angiogenesis and in tumor cell migration, was decreased in propranolol treated mice and correlated with tumor growth inhibition, whereas overexpression was associated with increased tumor growth. We therefore looked at AQP1 in IH, which made it possible to identify interstitial cell named telocytes (TC). Theses recently described interstitial cells are characterized by long cytoplasmic processes, the telopods, which form a three-dimensional communication network between cells, in multiple organs including the skin, especially around dermal vessels. Methods: The localization and markers of TC have been studied in a panel of HI, healthy skin and other vascular tumors. Then from fresh IH tissue, three cell types were isolated and cultured for functional study: endothelial cells, pericytes and telocytes. To study the functional role of TC in the pathophysiology of IH and its response to propranolol, we performed capillary like tube formation assays, seeding IH endothelial cells, pericytes and telocytes together as an IH model. We then studied the response to propranolol with AQP1 downregulated by shRNA. Moreover we quantified circulating molecules in IH tissues in order identify other critical factors for propranolol response. Results: Our immunostaining analysis revealed that expression profile AQP1 is different in IH compared to other vascular tumors. Furthermore, AQP1 expression is exclusively restricted to perivascular TC in IH. Our in vitro IH model was then instrumental into identifying the roles of AQP1 and other critical factors in propranolol response. Conclusion: Altogether, we show that sensitivity of our IH model to propranolol relies on the crosstalk between vascular and perivascular lesional cells when specific microenvironmental factors are present. Non-β-Blocker Enantiomers of Propranolol and Atenolol Inhibit Vasculogenesis in Infantile Hemangioma Caroline Seebauer (University Hospital Regensburg); Matthew S. Graus (University of Sydney); Lan Huang (Boston Children’s Hospital); Alex McCann (The University of Queensland); Jill Wylie Sears (Boston Children’s Hospital); Frank Fontaine (Gertrude Biomedical Pty Ltd); Tara Karnezis (Gertrude Biomedical Pty Ltd); David Zurakowski (Boston Children’s Hospital); Steven J. Staffa (Boston Children’s Hospital); John B. Mulliken (Boston Children’s Hospital); Joyce Bischoff (Boston Children’s Hospital); Mathias Francois (The University of Sydney) Purpose: Propranolol and atenolol, current therapies for problematic infantile hemangioma (IH), are composed of R(+) and S(-) enantiomers: the R(+) enantiomer is largely devoid of β-blocker activity. Methods: We show both R(+) propranolol and R(+) atenolol inhibit hemangioma stem cell (HemSC) to endothelial differentiation. Furthermore, both R(+) enantiomers inhibit the formation of IH-like blood vessels from HemSC in a murine xenograft model. Results: As our previous work implicated the transcription factor SOX18 in propranolol-mediated inhibition of HemSC to endothelial differentiation, we tested a known SOX18 small molecule inhibitor (Sm4) and show that this compound inhibits HemSC vessel formation in vivo in a similar manner as the R(+) enantiomers. To uncover mechanism(s), we examined how R(+) propranolol alters SOX18 transcriptional activity. We show that R(+) propranolol directly interferes with SOX18 target gene trans-activation, disrupts SOX18-chromatin binding dynamics and reduces SOX18 dimer formation. Conclusion: With these new results, we propose that β-blockers propranolol and atenolol may act independently of β-adrenergic receptors and instead the mechanism of drug action for both propranolol and atenolol when used to treat IH includes R(+) enantiomer targeting of SOX18 transcriptional activity. The use of the R(+) enantiomers could increase safety and efficiency by reducing β1- and β2-related side effects in the treatment of infantile hemangioma and possibly in other types of vascular anomalies in which SOX18 plays a role. SEGMENTAL NON-INVOLUTING CONGENITAL VASCULAR ANOMALY WITH ATROPHY, ULCERATION AND SCARRING (SNICVAUS): FURTHER EVOLUTION OF THE SPECTRUM OF “CONGENITAL HEMANGIOMA” Marta Ivars (University Clinic of Navarra); Ilona Frieden (UC San Francisco); Lauren Provini (University of California, San Francisco); Lisa Weibel (Pediatric Dermatology Department, University Children’s Hospital Zurich); Martin Theiler (Children’s Hospital Zurich); of of and Hospital); of and Hospital University Hospital); and Children’s Hospital of Beth Drolet of and for and OF Purpose: The ISSVA of vascular anomalies is a that vascular and vascular tumors. 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