Coagulation issues in vascular anomalies

https://doi.org/10.1016/j.sempedsurg.2020.150966Get rights and content

Abstract

Vascular anomalies, comprised of vascular tumors and malformations, are frequently associated with coagulopathy. Recognition of and familiarity with these vascular anomaly-associated hematologic abnormalities prior to surgery or interventional procedures is essential for pre-operative pre-operative planning. Complicated coagulopathies present within the framework of either Kasabach-Merritt phenomenon (KMP) or localized intravascular coagulopathy (LIC), and their management benefits from the expertise of a hematologist for optimal intra- and peri‑operative care. Furthermore, with the recent broadening of understanding of vascular anomalies and the addition of new classification sub-groups, distinctions of these two classic coagulopathy phenotypes have been recognized. This review summarizes the main features of these coagulopathies, described according to their vascular anomaly type, highlighting clinical aspects relevant to surgical management.

Introduction

Vascular anomalies comprise a heterogeneous group of disorders characterized by abnormal growth or development of arterial, venous, capillary, and/or lymphatic vessels. In one end of the spectrum, vascular tumors are characterized by the abnormal proliferation of endothelial cells and aberrant blood vessels, with the growth of abnormal vascular structures peaking before the first year of life. At the other end of the spectrum, vascular malformations are networks of abnormal blood and/or lymphatic vessels that form during fetal development and have normal endothelial cell turn over. In contrast to vascular tumors, the growth of vascular malformations occurs in parallel to the growth of the patient.

Hematologic disorders, and specifically coagulopathy, occur in many types of vascular anomalies but until recently, their recognition and treatment were hampered by an unclear characterization of the underlying vascular anomaly. This reality changed when Drs. Glowacki and Mulliken proposed a classification system and launched a new interpretation of how vascular anomalies behave according to endothelial cell kinetics, catapulting our current understanding of vascular lesions.1, 2, 3 This review summarizes the main features of the coagulopathies, described according to vascular anomaly type, highlighting key diagnostic and therapeutic aspects and enumerating unanswered questions relevant to the surgical management of those patients.

Section snippets

Kaposiform hemangioendothelioma / Tufted angioma

Kaposiform hemangioendothelioma (KHE) and tufted angioma (TA) are rare non-malignant vascular tumors that typically present in infancy or early childhood, although initial symptoms have been reported in adulthood (Fig. 1A and 1B). In the revised 2018 classification, KHE and TA were included in the vascular tumor category, appearing in the subgroup of lesions with locally aggressive or borderline clinical behavior.4 KHE and TA have similar histopathologic and clinical features and thus, are

Kaposiform lymphangiomatosis

Kaposiform lymphangiomatosis (KLA) is a recently recognized lymphatic anomaly with features of both vascular malformations and tumors. KLA most commonly involves the soft tissue, thoracic cavity, spleen and bones and is associated with high morbidity and mortality17 (Fig. 2A and 2B). In the revised ISSVA classification, this entity appears in the vascular malformation category, within the subgroup of lymphatic malformations.4 On histopathology, the defining features of KLA are focal areas of

Slow-flow vascular malformations

Vascular malformations are congenital networks of abnormal and misshapen blood and/or lymphatic vessels, which commonly cause progressive symptoms and complications over time. Slow-flow vascular malformations (SFVM) are those that lack an arterial component. In the 2018 ISSVA classification, these lesions are listed in the vascular malformation category and include lymphatic (LM), venous (VM), venous-lymphatic (VLM), capillary-venous (CVM), and capillary-lymphatic-venous (CLVM) malformations.4

Multifocal lymphangioendotheliomatosis with thrombocytopenia / Cutaneovisceral angiomatosis with thrombocytopenia

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), also called cutaneovisceral angiomatosis (CAT), is a recently described and rare multifocal vascular malformation involving the skin and GI tract with associated thrombocytopenia (Fig. 4A and B). Historically, MLT/CAT has been associated with high morbidity and mortality,49 but this trend appears to be changing based on better recognition of the disease as well as earlier treatment with sirolimus. The pathogenesis of this

Conclusion

Coagulopathies are commonly found in individuals with vascular anomalies. Some of these hematologic abnormalities are relatively benign or transient while others are life-threatening and require specialized management. Surgical manipulation of or trauma to certain vascular anomalies, specifically KHE, KLA, and SFVM, may worsen the underlying coagulopathy. For perioperative planning, hematology consultation is recommended for patients with vascular anomaly-associated coagulopathy or large venous

Declaration of Competing Interest

The authors declare no conflicts of interest.

Acknowledgments

The authors are grateful to the staff of the Cincinnati Children's Research Foundation, to the Vascular Anomaly Canada (CanVAC) group, and to the patients and families for participation in research and those willing to share photographs to improve understanding and familiarity of these disorders. We would also like to thank Prof. Laurence M. Boon, director of the Vascular Malformation Center, St. Luc University Clinic, Division of Plastic Surgery, Louvain Catholic University, Brussels, Belgium,

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