Vascular Anomalies: Advanced Practice Providers

1 in 3 newborns can present with some type of vascular birthmark

Vascular tumors are benign proliferative endothelial neoplasms that are typically not present at birth and are dynamic in their life cycle

Vascular malformations are errors of embryonic development, are congenital, classified based on clinical and histologic appearance of abnormal channels, and never involute

Venous malformations are composed of thin-walled dilated veins that lack normal smooth muscle, having only a single layer of muscle instead of the normal triple layer

Venous malformations are compressible on physical exam, expand with dependency, and can slowly enlarge with normal growth, often presenting problems during puberty

Venous malformations can be associated with superficial clotting that is painful, consumptive coagulopathy requiring preoperative hematology evaluation, and limb length differences

Blue rubber bleb nevus syndrome consists of multifocal venous malformations in skin, soft tissues, muscles, joints, bone, intestinal mucosa, and organ systems, with painful skin lesions and GI lesions that can lead to intractable bleeding requiring transfusion

In venous malformations, abnormal endothelial cells cannot respond to normal triggers that start the coagulation cascade, which is the cornerstone of the coagulopathy

Blood flow in venous malformations is so slow and stagnant that the normal coagulation response is negligible, and the stasis causes more damage to endothelial cells

Localized intravascular coagulopathy means the blood contained within large venous malformations is abnormal with extremely low fibrinogen and no ability to clot

Large multifocal venous malformations are at higher risk for bleeding than other types of malformations, with serious bleeding complications associated with any manipulation

Low molecular weight heparin is used at 1 mg per kilogram every 12 hours with adjustments based on response and fibrinogen level, targeting 0.5 to 1 international units per mL

In this case, the usual 1 mg per kilo dose was actually closer to 1.4 and 1.5 mg per kilo to control bleeding

Lovenox is held 12 hours prior to procedure to maintain fibrinogen levels in the desired range

Platelet count should be kept greater than 50,000 and fibrinogen levels above 100, ideally 120 to 150 in cases with active intervention

Five sclerotherapy and embolization procedures were performed over 10 months using ethanol, bleomycin, glue, and sodium tetradecyl sulfate

The blood volume in the lesion was estimated to be greater than the rest of the patient's body

The surgical team spent the first hour in the OR manually compressing the vascular malformation to push blood into central circulation, monitoring CVP, and removing blood via right internal jugular catheter into cell saver

About 80% of the mass was decompressed before proceeding with resection, using pledgeted sutures along the base to prevent refilling

Total blood loss was about 5 liters with phlebotomy of 2.25 liters

The patient was in ICU for 2 months postoperatively with multiple returns to OR for debridement and dressing changes, then one week on surgical floor before discharge

At 3+ years post-operation, the patient is walking, doing well, and performing all activities expected of a 5-year-old

About 75% of patients seen at Boston Children's vascular anomalies center receive sclerotherapy as first-line treatment rather than surgical resection

Bleomycin is used for microcystic lesions, defined as anything that you cannot put a needle in

Doxycycline has become the agent of choice for treating macrocystic or combined lymphatic malformations, with kids showing deflation after 2 or 3 sessions and long durable response

Bleomycin is particularly used for periorbital or retroorbital lymphatic malformations

Over 60% of patients referred to the Los Angeles vascular anomalies clinic come in with the wrong diagnosis, and at times wrong treatment has already been initiated