You are in: eMedicine Specialties > Plastic Surgery > SKIN Vascular, Venous MalformationsArticle Last Updated: Sep 11, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 11
  Author: Silvio Podda, MD, Craniofacial and Plastic Surgeon, Department of Plastic and Reconstructive Surgery, St Joseph's Regional Medical Center and Children's Hospital Silvio Podda is a member of the following medical societies: American Medical Association, American Society of Maxillofacial Surgeons, American Society of Plastic Surgeons, and Northeastern Society of Plastic Surgeons Coauthor(s): Frederick J Duffy, Jr, MD, FACS, Clinical Assistant Professor, Department of Plastic Surgery, University of Texas Southwestern Medical School Editors: Shahin Javaheri, MD, Chief, Department of Plastic Surgery, Martinez Veterans Affairs Outpatient Clinic; Consulting Staff, Advanced Aesthetic Plastic & Reconstructive Surgery; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics; Nicolas (Nick) G Slenkovich, MD, Practice Director, Colorado Plastic Surgery Center at Swedish Medical Center; Jorge I de la Torre, MD, FACS, Professor of Surgery and Physical Medicine and Rehabilitation, Residency Program Director, Division of Plastic Surgery, University of Alabama at Birmingham; Director, Center for Advanced Surgical Aesthetics Author and Editor Disclosure Synonyms and related keywords: venous malformation, vascular malformation, vascular venous malformation, cavernous malformation, VM, vascular birthmark, port wine stain, strawberry hemangioma, vascular birthmark, birthmark, venous thrombosis, thrombosis venous, vascular anomaly, nevus maternus, mother’s mark, turner syndrome, Turner syndrome, cerebral cavernous VM, cavernous venous malformation, venous anomaly INTRODUCTIONSection 2 of 11
  History of the ProcedureThe history of vascular birthmarks and anomalies is marked by misconceptions, confusing nomenclature, and folklore extending centuries into the past.1 Birthmarks were believed to be secondary to "maternal impressions." The unborn child could be imprinted with the mother's past experiences, fears, emotions, or objects of desire. Mothers were therefore to blame for the "nevus maternus" or mother's mark. These beliefs continue to exist in many cultures around the world. The past 2 decades have seen great advances in understanding of the pathophysiology, classification, nomenclature, and treatment of all vascular lesions.2, 3 In that vein, avoid using synonyms, as they have confused the diagnosis, classification, and management of these vascular tumors. Use the appropriate terminology instead, as advocated with passion by Dr. Mulliken.4 ProblemVenous malformations are always present at birth. They may manifest clinically in infancy, childhood, or adulthood. Depending on their location, they may remain asymptomatic throughout life. FrequencyVenous malformations are the most common of all vascular anomalies. Overall incidence of venous malformations is reported in 1-4% of the population. No predilection exists for either sex. EtiologyOf venous malformations, 100% are present at birth, although not all are clinically apparent. Venous malformations are developmental errors composed of dysmorphic channels lined by flattened endothelium exhibiting slow turnover. They are usually singular and solitary isolated events but may occur in multiple areas. If they are present in multiple areas, take a family history, because autosomal dominant transmission has been described for a subtype of venous malformation termed multiple glomangiomas. In addition, at least one mutation for venous malformations has been identified in a gene that codes for an endothelial receptor on chromosome 9p.5 PathophysiologyVenous malformations usually manifest by childhood or early adulthood. They grow commensurately with the developing child. Unlike hemangiomas, they do not regress. They are by definition "slow-flow" lesions and sometimes are not obvious at birth. They can expand in response to trauma, following incomplete surgical resection,7 or in altered hormonal states (pregnancy, puberty, steroid use). They also may expand following thrombosis or in sepsis. The following cellular characteristics are important to remember:
ClinicalVenous malformations present in various ways, from a vague blue patch to a soft blue mass. They are easily compressible and usually swell in the dependent position or when venous pressure increases (ie, when a child cries). They may be relatively localized or quite extensive within an anatomic region. Venous malformations typically involve the skin of the face, limbs, or trunk but also are found in the internal viscera and bones. They have also been identified in skeletal muscle.8 Episodic thromboses commonly occur in venous malformations. These are low-flow lesions. Phleboliths, secondary to phlebothrombosis, have been observed in patients as young as 2 years. They might be recognizable with plain radiography. INDICATIONSSection 3 of 11
The most common indication for medical or surgical treatment of a venous malformation is pain. Pain is likely secondary to thrombosis of the malformation, but depends on the size and location of the lesion. Discomfort and stiffness, particularly in the morning, are associated with many larger and deep cutaneous or intramuscular malformations. Intraoral venous malformations can bleed, distort speech or dentition, or obstruct the airway. Venous malformation involving the GI tract or internal viscera can bleed, requiring intervention. Finally, treatment of venous malformations may be indicated to improve appearance or function. Symptoms associated with venous malformations and indications for intervention vary with the organ system involved. A 3-cm venous malformation of the thigh may be asymptomatic, while the same size intracranial lesion may thrombose and lead to swelling and a life-threatening mass effect requiring emergency intervention. RELEVANT ANATOMYSection 4 of 11
Venous malformations represent vascular developmental errors and can occur anywhere. Their management becomes increasingly complex as they involve structures with significant neurovascular function. CONTRAINDICATIONSSection 5 of 11
Treatment of venous malformations, particularly surgical resection, is often greatly complicated by their deeper involvement with critical neurovascular structures. This is particularly true in the head and neck, intracranial, and extremity malformations. Surgery is often contraindicated if risks associated with the resection outweigh the presumed improvement in appearance or function that may be derived from surgery. WORKUPSection 6 of 11
Lab Studies
Imaging Studies
Diagnostic Procedures
Histologic Findings
TREATMENTSection 7 of 11
Medical TherapySclerotherapy is the primary form of nonsurgical intervention for venous malformations.9 Larger lesions usually are treated with 95% ethanol, while cutaneous and smaller lesions are treated with sodium tetradecyl sulfate (1%). Sclerotherapy is often performed by an interventional radiologist under general anesthesia. Multiple sclerotherapeutic sessions often are needed. Venous malformations have a propensity for recanalization and recurrence. An alternative to standard sclerotherapy using sclerosant foam has recently been described.10 Compression garments are a mainstay of treatment for extremity venous malformations, particularly the lower extremity. Venous malformations of the GI tract also have been managed by sclerotherapy or endoscopic banding. Laser therapy has shown promise in selected situations. Argon and yttrium-aluminum-garnet (YAG) lasers have been used to treat intraoral lesions.11 This approach seems more appropriate for smaller lesions. Surgical TherapySurgery is indicated in isolated, symptomatic venous malformations or following sclerotherapy to improve form or function. Surgical results are a function of the size and location of the malformation. Recurrence following surgery is more common with diffuse malformations and when excision is incomplete.7 In general, surgery or sclerotherapy is more successful when dealing with pure venous malformations than when dealing with combined malformations. Follow-upComplicated or large venous malformations are best treated at a referral center staffed by a multidisciplinary team of diagnostic and interventional radiologists, plastic surgeons, and interested ablative surgeons (eg, neurosurgery). COMPLICATIONSSection 8 of 11
The type and severity of complications depend on the size and location of the malformation and type of intervention chosen. Greater complications are seen with more difficult resections that involve vital structures. Recurrence is a common complication of therapy. OUTCOME AND PROGNOSISSection 9 of 11
The outcome and prognosis are most closely related to the size and location of the venous malformation. The likelihood of significant perioperative morbidity and recurrence increases with more diffuse malformations and with malformations intimately involving vital neurovascular structures. FUTURE AND CONTROVERSIESSection 10 of 11
The future holds great promise for the diagnosis and treatment of all vascular malformations, including venous malformations. Advances in molecular genetics are adding to the understanding of vascular malformations and hopefully will elucidate the mechanism of origin of the developmental abnormalities associated with these anomalies.12 Several inherited disorders have been identified and defective genes have been located.5 Additional information is expected as work on the human genome continues. This new knowledge hopefully will elucidate the pathogenesis of vascular malformations and lead to fresh approaches to therapy. 12 The field of angiogenesis continues to mature, and new antiangiogenesis drugs are in clinical trials that may lead to fresh treatment modalities for these vascular anomalies.12 REFERENCESSection 11 of 11
Vascular, Venous Malformations excerpt Article Last Updated: Sep 11, 2008 |
