Venous Malformations

Published on 09/04/2015 by admin

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Last modified 09/04/2015

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Chapter 19 Venous Malformations

Vascular malformations are probably the single most misdiagnosed entity in the vascular system. Essentially, vascular malformations are errors in vasculogenesis with the particular characteristics of the lesion determined by the vessel in the vascular system that is involved. As a result, these malformations can include arteries, veins, lymphatic vessels, or capillaries. These lesions occur in about 1.5% of the population and over 90% are present at birth. Venous malformations are the most common vascular anomalies.

While these lesions can have mass effect on adjacent structures, they are not tumors. A clearly organized classification system was presented by Mullikan, Glowacki, and colleagues in 1992, and this classification system was adopted in 1996 by the International Society for the Study of Vascular Anomalies (ISSVA). The classification system clearly separates tumors (e.g., hemangioma) from the vascular spaces that characterize a vascular malformation.1

What are Hemangiomas?

Hemangiomas are childhood masses characterized histologically by high endothelial cell turnover and are characterized by cell markers (GLUT-1, merosin, Lewis Y) that are otherwise found only in human placental tissue and that display phasicity characterized by a rapid proliferative phase, plateau phase, and slow involutional phase. The proper nomenclature for these lesions is infantile hemangioma. Infantile hemangiomas are benign vascular tumors that are not usually present at the time of birth but instead become evident within the first 2 to 3 weeks of life. They are the most common benign tumor of infancy. There is a subgroup of hemangiomas that present fully formed at birth known as congenital hemangiomas. These congenital hemangiomas (as opposed to infantile hemangiomas) do not exhibit the expected accelerated postnatal growth. Some congenital hemangiomas involute rapidly over the first year of life and are called rapidly involuting congenital hemangiomas (RICHs), while some may persist indefinitely without treatment and are called noninvoluting congenital hemangiomas (NICHs).1

The majority of infantile hemangiomas are localized and, although disconcerting to parents and care providers, are nonthreatenting.2 For these lesions, observation and routine monitoring by the pediatrician or dermatologist are acceptable treatment options. A minority of infantile hemangiomas can, however, cause significant morbidity. These require early recognition, timely referral to a specialist, and prompt intervention to minimize complications. Worrisome presentations include multiple hemangiomas and sensitive locations such as beard distribution, periocular, perioral, nasal tip, large regions of the face and neck, and the lumbosacral spine region. In general, larger hemangiomas located on the face are more likely to require treatment. One of the strongest indications for the use of the laser is the presence of ulceration. Other symptoms necessitating therapeutic intervention include congestive heart failure, airway obstruction, dysphagia, infection, failure to thrive, external auditory canal occlusion, visual axis impairment, and severe facial deformity.

Muscular skeletal hemangiomas are not hemangiomas. They are venous malformations that occur in the muscle. With the correct clinical history, a properly performed magnetic resonance imaging examination should be almost pathognomonic. Vertebral body hemangiomas are also not hemangiomas; they are venous malformations that occur in bone. Review of the literature demonstrates numerous studies demonstrating “increased vascularity” of these lesions. However, the increased vascularity is from the venous pooling in the lesion and not from arterial hypertrophy and neovascularity. Liver hemangiomas are not hemangiomas; they are venous malformations that occur in the liver.

Hemangiomas in adults are not hemangiomas; a hemangioma is a childhood-only birthmark. If a vascular birthmark was not present in the childhood stage, then it is not a hemangioma. Hemangiomas are NOT arteriovenous malformations (AVMs), and vice versa. While there is arterial inflow that identifies both these lesions, hemangiomas demonstrate typical tumor vascularity with a central arterial pedicle, and there is fairly minimal, if any, shunting identified in the outflow vessels. An AVM, on the other hand, may have significant venous shunting, with resultant low resistance arterial inflow and arterialized pulsatility in the venous outflow vessels. Venous enlargement is also common in AVMs, resulting from the pressurized shunted arterial flow into the nidus.