You are in: eMedicine Specialties > Plastic Surgery > SKIN Vascular, Lymphatic MalformationsArticle Last Updated: Feb 5, 2008AUTHOR AND EDITOR INFORMATIONSection 1 of 12
  Author: Meir Cohen, MD, MPS, Consulting Staff, Department of Plastic Surgery, Schneider Children's Medical Center of Israel, Tel Aviv University Meir Cohen is a member of the following medical societies: American Cleft Palate/Craniofacial Association and Plastic Surgery Research Council Coauthor(s): Shimon Maimon, MD, Head of Invasive Radiology Unit, Beilinson Campus, Rabin Medical Center, Israel; Dan Ben-Amitai, MD, Head of Pediatric Dermatology Service, Lecturer, Schneider Children's Medical Center of Israel; Eric Bensimon, MD, Clinical Instructor, Department of Surgery, University of Montreal 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; Wayne Stadelmann, MD, Stadelmann Plastic Surgery, PC; 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: VM, vascular lesion, lymphangioma, cystic hygroma, hemangioma, lymphatic malformation, lymph malformation, congenital skin lesion, acquired skin lesion, congenital vascular lesion, acquired vascular lesion, skin malformation, vascular malformation INTRODUCTIONSection 2 of 12
  Classification Vascular lesions of the skin can be divided into congenital and acquired lesions (see Image 1).
The classification of vascular lesions is confusing, and puzzling terms such as "capillary hemangiomas" are found in some textbooks. The classification presented here is based on the type of blood vessels, type of flow, and time of presentation.2 Exceptions exist to this division: some hemangiomas are congenital and some VMs are not present at birth. FrequencyLymphatic VMs are the most common bases for macrocheilia, macroglossia, macrotia, and macromelia.3 Combined lymphaticovenous malformation (LVM) occurs particularly in the craniofacial region.4 EtiologyThe vascular system is built by 2 processes, vasculogenesis and angiogenesis. In vasculogenesis, a primitive vascular plexus is established from endothelial precursors.5 The vascular plexus is connected to the developing heart tube, and after onset of the heartbeat, the vascular channels are perfused with blood and the primary circulation is established by the end of the third week of development. In angiogenesis, new vessels arise from these preexisting vessels by migration and proliferation of endothelial cells.5 In general, the endothelial cell's fate is determined by the combined effects of a large number of positive and negative signals simultaneously transduced by numerous receptors. Many molecules have been defined that regulate vessel growth in vivo and in vitro.5 In general, the formation and remodeling of blood vessels are controlled by paracrine signals; many are protein ligands that bind and modulate transmembrane receptor tyrosine kinases.5 Known negative regulators are angiostatin, endostatin, and thrombospondin. Positive regulators are vascular endothelial growth factor (VEGF), fibronectin, 5-integrin, vascular endothelial cadherin, and transforming growth factor-1 or TGF-1.5 The lymphatic system begins to develop at the end of the fifth week.5, 6 Lymphatic vessels develop as endothelial outgrowths from the venous system. First, 6 lymphatic sacs are formed.5 These lymph sacs sprout from large central veins. The exact modulator that causes lymphatic VM is not known yet; however, genetic studies provide insight into the effect of positive and negative signals on the formation of those lesions. By analyzing a 2-generation family, an autosomal dominant type of congenital hereditary lymphedema has been mapped to chromosome 5.5 The candidate region contains a gene that encodes the VEGR R-3 gene. Cervicofacial lymphatic VM further occurs in trisomy 13, 18, and 21 and in Turner syndrome.5 Some have suggested that sporadic cases of lymphatic VM are caused by de novo dominant somatic mutations and that germline mutations are lethal.5 Mapping of the human genome will help clarify the exact regulators that are involved in the formation of lymphatic VMs. PathophysiologySee Etiology. ClinicalThe diagnosis of a vascular lesion is based on medical history, physical examination, and imaging tests. The type of lesion usually can be determined easily based on the first 2 items. Imaging studies are mostly useful for confirming the clinical diagnosis, estimating the extent of the lesion, and determining the feasibility of surgical resection. Medical history When obtaining the medical history, ask the following 4 questions:
Clinical diagnosis of hemangiomas and venous, lymphatic, and arterial lesions can be made in a straightforward fashion based on the answers to the above questions (see Image 3). A common feature of lymphatic VM is episodic enlargement associated with systemic or localized infection. Image 4 shows a 5-year-old child with a right leg lymphatic VM. The right lower picture shows acute infection of the lesion with typical redness, swelling, local warmth, and high systemic fever. Physical examination The physical examination of a patient with a vascular lesion includes inspection, palpation, and transillumination. The diagnosis of hemangiomas and venous, lymphatic, and arterial lesions can be made simply based on the above questions (see Image 5). Lymphatic VM can have a small and localized or an extensive presentation. Lesions that are limited to the superficial layer of the skin are called lymphangioma circumscriptum (see Images 6-7). In the head and neck area, the lesions can involve the orbit and eyelids (see Images 8-10), cheek (see Images 11-12), tongue (see Image 13), and neck (see Images 14-16). In the extremities, lymphatic VM can present as a localized (see Images 17-18) or as an extensive extremity lesion associated with lymphedema and dysfunction (see Image 4, Images 19-21). Skeletal distortion and hypertrophy are also common features of limb and facial lymphatic malformations. Image 22 shows the effect of a cheek lymphatic malformation on the mandible. Assessment of asymmetry and deformity of the facial and limb skeleton should be part of the physical examination of patients with lymphatic VMs. Differential diagnosis Lymphatic VMs may be confused with deep hemangiomas or venous VMs. The presence of the lesion at birth supports the diagnosis of a VM, although congenital hemangiomas can be observed at birth. MRI can help distinguish between a VM and a hemangioma. The differential diagnosis also includes gliomas, benign tumors, and malignant tumors. Image 28 shows the differential diagnosis of orbital lymphatic VMs. INDICATIONSSection 3 of 12
Lymphatic VMs never involute. They expand or contract depending on the ebb and flow of lymphatic fluid and the occurrence of inflammation and intralesional bleeding.3 Several indications for treatment exist. The size and location of the lesion, recurrent infection, and pain are the most frequent indications for treatment.7 Vision An intraorbital lesion usually presents as proptosis. It may expand rapidly and cause optic nerve compression, disk swelling, and decreased vision. Cysts filled with blood ("chocolate" cysts) or lymph fluid may be aspirated under ultrasonographic guidance. Currently, no well-tested pharmacologic treatments for lymphatic VMs are available. Preliminary studies demonstrate that OK-432 may be useful for intralesional injection.8 Surgery may be indicated for superficial eyelid lesions and in selected patients with intraorbital lesions. Sufficient surgical excision of orbital tumors without injury to intervening structures is difficult. Image 9 shows a 14-year-old boy with a right upper eyelid lymphatic lesion that had caused ptosis of the right upper eyelid (images on left). Image 10 shows an MRI of the lesion. Improvement in his visual field was achieved after excision of the lesion (see Image 9, images on right). Breathing Sublaryngeal lesions may compress the soft tracheal rings of infants. Intralesional injection with alcohol may be successful in macrocystic lesions but also may be followed by acute swelling and airway obstruction.9 Tracheostomy may be required in selected patients. Limb function Localized limb lesions (see Images 17-18) are mostly of aesthetic concern. Large lesions (see Image 4, Images 19-20) may be associated with functional handicap. Treatment may be indicated in such individuals. Recurrent infections Infections as part of a systemic disease or as a localized problem are frequently observed in patients with lymphatic VMs. Image 4 (lower right) shows acute infection of a lower limb lesion. Long-term antibiotic treatment and compression stockings (see Image 4,) may decrease the incidence of infections. Hygiene Lymphatic lesions with a superficial component are associated with chronic shedding of small cysts, lymph leak, staining, and an unpleasant odor. Image 21 shows a 12-year-old boy with a lymphatic lesion involving the entire right leg. Good hygiene was very difficult to maintain at the toe area. Aesthetic concerns Lesions that involve the head and neck area may be associated with significant aesthetic deformity. Images 8-9 and Images 12-16 show some common head and neck lesions with aesthetic deformities. Parental concerns and the psychosocial effect of the deformity on the growing child should be taken into consideration and may be indications for early treatment.9 RELEVANT ANATOMYSection 4 of 12
Normal lymphatic capillaries consist of single-layer endothelial cells without surrounding pericytes and without valves. These lymphatic capillaries merge into collecting lymphatic vessels,5 which consist of a thin endothelial lining, surrounded by an incomplete layer of smooth muscle cells, with valves. Finally, either the thoracic or the right lymphatic duct delivers lymph to the venous system. Their walls contain a tunica intima, media, and adventitia, with the media containing bundles of smooth muscle cells.5 Lymphatic VMs are composed of dilated lymphatic channels.5 They are filled with a proteinaceous fluid and do not have connections to the normal lymphatic system. Lesions can be primarily macrocystic or microcystic. Thoracic lesions are usually macrocystic and cervicofacial lesions are usually microcystic.5 Lesions are located at the skin and subcutaneous tissue but may invade the floor of the mouth, cheek muscles, and other anatomic structures. Images 23-25 show an MRI of a 9-year-old child with a right facial lesion that extended into the floor of the mouth and cheek. CONTRAINDICATIONSSection 5 of 12
Sclerotherapy may be contraindicated in the following situations:
Surgery may be contraindicated in the following situations:
WORKUPSection 6 of 12
Imaging Studies
Histologic FindingsLymphatic malformations are composed of dysplastic vesicles or pouches filled with lymphatic fluid. They can be described as either microcystic, macrocystic, or combined forms. Lymphatic VMs have walls of variable thickness, composed of both striated and smooth muscle, with nodular collections of lymphocytes in the connective tissue stroma.3 TREATMENTSection 7 of 12
Medical therapyNonsurgical therapyLocal pressure Elastic support stockings may help decrease the swelling and the functional handicap associated with lymphatic VMs of the extremities (see Image 4). Antibiotics Viral or bacterial infection can cause acute infection of a lymphatic VM. Infection may be associated with acute enlargement, pain, local warmth, redness, and elevated systemic fever. Intravenous (IV) antibiotics and nonsteroidal anti-inflammatory drugs (NSAIDs) are indicated during such episodes. Sclerotherapy Transcutaneous injection of a sclerosant such as alcohol may help decrease a lymphatic VM.7 It is mostly useful for macrocystic malformations and for combined venous-lymphatic lesions. The procedure is painful; therefore, it is performed by an invasive radiologist under general anesthesia. Initially, 5 mL of contrast fluid is injected through a venous catheter to delineate the anatomy of the lesion and to detect escape of contrast to the systemic circulation (see Image 29, upper left). Image 29 (upper middle) shows injection of contrast into a facial venous-lymphatic malformation. The catheter was relocated when escape of contrast to the facial vein was detected (upper right). When a lumen filled with lymphatic fluid is detected, the lymph is aspirated. Alcohol (100%) mixed with a small amount of contrast fluid (alcohol-to-contrast ratio of 20:5) is then injected through the same venous catheter. The total amount of alcohol injected into a lesion of a full-sized male is approximately 50 mL. Exceeding a dose of 1 mL/kg is not advised. The catheter is left in place following alcohol injection in case some of the alcohol needs to be withdrawn. Injection is discontinued when skin changes such as peau d'orange, erythema, and bruising are observed. Image 29 (upper left) shows bruising and peau d'orange changes following injection with alcohol of a chest venous-lymphatic VM. To decrease the swelling, 4 mg of dexamethasone (PO/IV) is administered 3 times every day for 3 days after the procedure. NSAIDs are administered for pain control. The patient usually stays in the hospital overnight for pain control and for monitoring of possible vascular or neurologic limb compromise. Systemic leakage of alcohol may cause myocardial depression. Injection of alcohol close to the skin or mucosa may cause skin slough or skin necrosis. Image 29 (lower right and left) shows necrosis of skin and mucosa following injection of alcohol to the forearm and tongue, respectively. Bleomycin and OK-432 Intralesional bleomycin and OK-432 have recently been reported to have dramatic results.11 Surgical therapySurgery is the only way to "cure" a lymphatic malformation. It should be considered in the following situations:
The surgery must be well planned. Most lesions cannot be resected completely; therefore, the extent of the resection needs to be defined before the procedure. In certain situations, such as eyelid surgery, performing the surgery under local anesthesia is better. This facilitates intraoperative navigation. Image 9 shows an upper right eyelid lesion before and after resection under local anesthesia. Lymphangioma circumscriptum is a superficial lymphatic malformation of the skin. This often can be resected completely and the defect reconstructed with a skin graft (see Image 6). Administration of hemostatic agents such as recombinant factor VIIa (rVIIa) may decrease bleeding and improve surgical efficiency.12 COMPLICATIONSSection 8 of 12
Vision Obstruction of vision during the first 6 months of life by a periorbital, cheek, forehead, or nasal VM may cause long-term visual damage. Early treatment with transcutaneous sclerosis and/or surgery is indicated in such patients. Breathing Lymphatic VM, which invades the neck, may compress the soft tracheal rings of infants and present as stridor. Direct excision of the lesions is impossible in most patients because of the close proximity of the lesion to vital structures. Consider tracheostomy in patients in whom pharmacologic treatment has failed.9 Psychosocial complications Facial lymphatic VMs may cause a significant facial deformity. Children with facial lymphatic VMs may be teased by their peers. Early surgery may be indicated in such patients. OUTCOME AND PROGNOSISSection 9 of 12
Lymphatic lesions gradually enlarge and worsen with time.3 Surgery is the only complete cure for this problem. However, this is not always possible, and the goal of treatment in many patients is improvement rather than cure. FUTURE AND CONTROVERSIESSection 10 of 12
A significant contribution to the understanding of vascular lesions is the introduction of a classification method by Mulliken and associates.2 This made diagnosis and treatment more accurate and predictable. However, confusing and occasionally misleading terms used by different subspecialties are still found. Improvement in patient monitoring and anesthesia during and after surgery made early excisions and transcutaneous sclerosis safer and more acceptable. A major controversy is the timing of operative procedures. No clear-cut answer exists to this question. The authors believe that decisions should be made according to the individual patient. The psychosocial consequences of growing up with a facial deformity always should be taken into consideration. Future research of specific genes and their angiogenic growth factor products will contribute to the understanding of the mechanism underlying the formation of lymphatic VMs and may provide new modalities of treatment at the gene level.5 MULTIMEDIASection 11 of 12
REFERENCESSection 12 of 12
Vascular, Lymphatic Malformations excerpt Article Last Updated: Feb 5, 2008 | ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
