2/2/14 INTRODUCTION. Vascular Anomalies: An Introduction to Classification and Coordination of Care. VASCULAR ANOMALIES: History and Classification

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1 INTRODUCTION Vascular Anomalies: An Introduction to Classification and Coordination of Care Cindy Kerr,RN,MSN,CPNP Erin Spera,RN,MSN,CPNP Mary Beth Sylvia,RN,MS,FNP-BC 1in 3 newborns has a vascular birthmark such as nevus flammeus neonatorum ( stork bite or angel kiss ); most are of no consequence and predictably fade with time Vascular tumors and malformations can be present for years-to-lifelong and cause symptoms that affect daily life and require treatment VASCULAR ANOMALIES: History and Classification Beliefs about maternal diet and imprinting fetuses with maternal longings or fears Biological classification: Cellular features correlated with clinical characteristics and natural history Vascular tumors Vascular Malformations VASCULAR TUMORS Benign, proliferative endothelial neoplasms Arise by endothelial hyperplasia Dynamic life cycle VASCULAR MALFORMATIONS TUMORS AND MALFORMATIONS Errors of embryonic development Classified based on clinical/histologic appearance of the abnormal channels Generally sporadic; rarely familial Congenital lesions, but may not be apparent until later in life Localized or diffuse Never involute TUMORS Infantile Hemangioma Congenital Hemangioma RICH NICH Kaposiform Hemangioendothelioma (KHE) Cutaneous Angiomatosis with Thrombocytopenia (CAT) PHACE Association Others MALFORMATIONS Capillary (CM) Venous (VM) Lymphatic (LM) Arteriovenous (AVM) Combination KTS (CLVM) Parkes Weber syndrome Sturge-Weber syndrome CM-AVM CLOVES syndrome 1

2 Vascular Tumors can be difficult to distinguish from Vascular Malformations. Are these lesions the same or different? Lymphatic malformation vs. Kaposiform Hemangioendothelioma (KHE) Are these lesions the same or different? Hemangioma vs. Venous Malformation Vascular Anomalies Center Interdisciplinary team made up of 18 different specialties Based on the Boston Campus Clinics run first 3 Fridays/month and all day on 5 th Fridays-also filter to each MD clinic NP Clinics-twice monthly Weekly interdisciplinary conference on Wednesday evenings 5:30pm in Surgical Library Patient base is primarily outpatient both national and international patients Inpatient consults/rounds 2

3 2/2/14 VAC Specialties General Surgery-Director, Dr. Steve Fishman Plastic Surgery-Director, Dr. John B. Mulliken Interventional Radiology (also Neurointerventional and diagnostic)-director, Dr. Ahmad Alomari Orthopedic Surgery Dermatology Cardiology Hematology/Oncology Pathology Endocrinology Gastroenterology Neurology Neurosurgery ORL Pulmonology Basic Science-multidisciplinary Anesthesiology Social Work Nursing VENOUS MALFORMATION Compressible, expand with dependency Slowly enlarge with normal growth of patient; often enlarge during puberty Associated with: superficial clotting consumptive coagulopathy requiring perioperative LMWH LLD Mesenteric/portal venous anomalies with GI VMs Case Study: Patient S. VENOUS MALFORMATION Present at birth, but not always evident Rarely hereditary (TIE 2) Composed of thin-walled, dilated veins that lack normal smooth muscle Slow flow lesions Range: simple ectasias to discrete spongy masses to diffuse network s of complex channels that involve tissues or organs Blue Rubber Bleb Nevus Syndrome (BRBNS) Multi-focal venous malformations Locations include the skin, soft tissues, muscles, joints, bone, intestinal mucosa, and some organs Skin lesions can be quite painful; GI lesions can lead to intractable GI bleeding. Mother Lesion Sclerotherapy/Embolization/Operative management Early Patient History Large sacral mass-28wks prenatal US Left buttock mass 22x22cm at birth (11/6/08) Multiple 2-4mm purple small lesions over body Plts 85K and Fibrinogen <50transfused DX: Hemangioma, LVM, and VM Referred to VAC first week of life Conf:? BRBNS v. VM v. LM-Biopsy VAC Clinic: BRBNS Plan of Care Sclerotherapy Surgical Resection 3

4 Birth Birth Birth Birth First Clinic Visit: Age 7 months First Clinic Visit Age 7 months 4

5 Treatment Goals Safely remove the large venous lesion Step 1: Close anomalous veins in the lesion using a minimally invasive approach -Sclerotherapy and Embolization Step 2: Surgical excision Challenges: Coagulopathy Massive size of the lesion and associated vessels Pathogenesis of Coagulopathy in Venous Malformations Not fully understood Multifactorial 1. Abnormal vascular endothelial cells -endothelial cells do not respond to coagulation stimulus -formation of basic coagulation components is hindered Pathogenesis of Coagulopathy in Venous Malformations 2. Blood Flow -slow flow inhibits transport of blood components responsible for clot formation -slow flow and stasis can damage the endothelium 3. Localized intravascular coagulopathy -coagulopathy limited to the blood contained within the vascular anomaly Pathogenesis of Coagulopathy in Venous Malformations Venous malformations are at risk for both hemorrhage and thrombosis Higher risk of coagulopathy in lesions that are larger and multifocal Serious bleeding complications associated with interventional and surgical manipulation Management of Risks Laboratory assessment CBC: anemia and platelet count PT/INR/PTT: status of coagulation cascade Fibrinogen: reduced in setting of venous malformations D-dimer: used to evaluate extent of coagulopathy Low Molecular Weight Heparin Lovenox (Enoxaparin) 1 mg/kg Q 12 hrs with adjustments based on response and fibrinogen levels LMWH levels range between IU/ml 5

6 Management Strategies Topical Aminocaproic Acid (Amicar) Lovenox held 12 hours prior to procedures Platelet count > 50,000 Platelet infusions immediately prior to and during procedures Fibrinogen level < 100 mg/dl cryoprecipate infusions PT within normal limits Fresh frozen plasma administration Sclerotherapy and Embolization Five sclerotherapy procedures completed over a period of 10 months Vessels within the malformation measure as large as 4cm in diameter Innumerable small venous channels just under the surface of lesion and along the skin edges Agents used: Ethanol, Bleomycin, n-butyl cyanoacrylate (glue), Sodium tetradecyl sulfate Final procedure focused on the left sciatic nerve region PREOPERATIVE PLANNING Interdisciplinary effort started one month prior to admission Services included: Anesthesiology, Blood Bank, Hematology, Interventional Radiology, Wound Care, Critical Care, General Surgery, Operating Room, Vascular Anomalies Center, Cardiology PREOPERATIVE PLANNING ISSUES/PLAN: Consumptive coagulopathy on chronic LMWH since age 6 weeks Labs and transition to UFH Size of lesion/blood volume in lesion Compression of lesion using CVP monitoring and phlebotomy for high volume cardiopulmonary issues and coagulation monitoring; phlebotomized blood into cell saver PREOPERATIVE PLANNING Intraoperative blood loss Initial request for 75 units PRBCs and 30 units platelets with additional FFP and cryoprecipitate Placement of pledgets and sutures for compartmentalization of decompressed lesion Possible ECMO, by-pass, circulatory arrest if needed in OR Positioning in the OR Postoperative wound care Proposed VAC dressing 6

7 2/2/14 PREOPERATIVE ADMISSION Admitted to ICU for hemodynamic monitoring Laboratory studies Transitioned from LMWH to UFH Cardiology workup CXR, EKG, Echocardiogram Hematology and Nutrition Consultations Placement of arterial and venous lines Mass gradually compressed over one hour period with CVP monitoring 2.25 liters of blood removed via right internal jugular catheter into cell saver Mass sutureligated with pledgeted sutures Positioning in the OR 80% of mass decompressed Mass resected 7

8 2/2/14 Wound closed VAC dressing applied Total blood loss 5 liters Estimated phlebotomy 2.25 liters Blood products administered: 9 units PRBC, 9 units platelets, 9 units cryoprecipitate, 200 ml of cell saver, 5 liters crystalloid POSTOPERATIVE CARE Multiple debridements and VAC dressing changes in the OR Final wound revision in the OR Remained in ICU for 2 months before transfer to surgical inpatient unit Transferred one week later to local hospital in Kansas City for a single overnight stay before discharge home SUMMARY High risk patient requiring interdisciplinary care and coordination of services for optimum surgical outcome Age 5 years, 3+ years postop 8