Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Cutaneous Hemangiomas and Vascular Malformations Case Presentation: HW is a 67-year-old white male presented to the VA ENT Clinic for evaluation of the lower lip and tongue lesion. Patient states that he has had these lesions since birth. He states that they have grown slowly throughout his life and he has difficulty keeping liquids in his mouth due to the lower lip lesion. These are non-painful and enlarge when dependent. His past medical history is significant for hypertension, cardiomyopathy, coronary artery disease, and atrial fibrillation. Past surgical history is none. For his atrial fibrillation, he is on Coumadin. Other medications include digoxin, metoprolol, and lisinopril. On exam of his oral cavity and oropharynx, about 80% of his lower lip contained a compressible and non-painful bluish mass. His left tongue also contained a compressible non-painful mass. These enlarge when we placed his head in the dependent position, in Trendelenburg position. Remainder of his head and neck examination is within normal limits. A diagnosis of lower lip and venous malformation was made clinically. Since the patient is on Coumadin for his atrial fibrillation, his best modality for treatment would be sclerotherapy to lower lip. Historically, mothers were thought to have children with these venous malformations and hemangiomas due to their aversion or liking for certain foods during their pregnancies such as strawberries, mulberries, wine, or things they could not eat. Historically, nomenclature included the name of Virchow, the pathologist who named these after their architecture such as angioma simplex, angioma cavernosum, and angioma racemosum. Hemangiomas are classified into three categories: superficial, deep, and compound. Superficial ones are pigmented about 65% of the time and located in the papillary dermis. The deep type, which you can see here, is a nasal hemangioma. Reticular dermis account for 15%; they have a bluish hue. The compound or combined type, which you can see here, has a bluish hue combined with a dark intense red stain. Vascular malformations are classified into: low flow, high flow, and the third, mixed. The low flow category includes venous, venular, capillary, port-wine stains, and lymphatic. In a recent grand rounds, Dr. Cordes presented lymphatic malformations, so we will not further pursue that topic today. High-flow includes the arterial venous, while mixed includes venous or lymphatic. Here you can see a port-wine stain in a 2-year-old child. These are postcapillary venule malformations. These slides demonstrate the epidemiology of hemangiomas versus vascular malformations such as port-wine stains. The incidence of hemangiomas is about 10% in the first year of life whereas the incidence of port-wine stains is about 0.3% of those. Females are more likely to have hemangiomas, whereas vascular malformations occur about equally in females and males. Hemangiomas typically present within the first few weeks of life, whereas vascular malformations are always present at birth. Sixty percent of hemangiomas occur in the head and neck, while 80% of vascular malformations occur in the head and neck. There is cellular proliferation or hyperplasia of these cells in hemangiomas, whereas in vascular malformations such as the port-wine stain, there is no cellular proliferation. They do hypertrophy, but there is no hyperplasia. Most hemangiomas regress, whereas the vascular malformations demonstrate commensurate growth with the child. Hemangiomas: There are two phases: the proliferative phase and the involution phase. In the proliferative phase, they first appear blanched, which you can see here over the glabella region. This is due to plump endothelial cells with very little vascular channels and very little red cells, which you can see here. These later become bright red and raised. The proliferative phase lasts for approximately 12 to 18 months. You can see frequent mitoses here. There is type IV collagenase and multiple growth factors occurring. If you notice multiple hemangiomas greater than 3 to 4 in number, an abdominal ultrasound is necessary to rule out any visceral hemangiomas. In the involution phase, there is a cessation of growth of these hemangiomas. You can see flat endothelial cells and the lesion itself will turn dark maroon or an ashen grey. You will see atrophic changes, residual scar, redundant skin, hypopigmentation, and a lot of fibrofatty tissue. Finn studied over 200 or 300 hemangiomas in 1983 and, in terms of involution, approximately 50% involute by 5 years of age and 70% involute by 7 years of age. He states that of these 50% that involute by about 5 years of age, 40% will end up with unacceptable aesthetics deformity, which you can see here, redundant tissue and fibrofatty tissue. The other 50% then involute later after 6 years of age. They have an 80% unacceptable aesthetic deformity. You can see here the early involuters and the late involuters. Ulceration is the most common complication with hemangiomas, occurring in 5% to 12% of these lesions. These occur with rapidly proliferating hemangiomas. They can be painful. They can lead to infection, bleeding, and scarring. There are few possible mechanisms. One is that the hemangioma is actually expanding faster than the skin can heal or that the hemangioma has developed necrosis in the center of the lesion. Treatment: The main treatment for hemangiomas, if there are symptoms, is local wound care and pulsed dye laser. Other modalities include steroids, a plethora of drug growth factors. One syndrome to keep in mind with hemangiomas is Kasabach-Merritt. Rapid enlargement and proliferation of this hemangioma may lead to sequestration of platelets and clotting factors, which results in profound thrombocytopenia, DIC, and you will see, on exam, ecchymoses and numerous petechiae. So, when treating these hemangiomas, if you notice that they are proliferating at a rapid rate or they are in a location that may cause impingement on vital structures (the eye) or cause a functional impairment, then we may need to treat these. If there is a presence of ulceration or a prediction of poor future aesthetic result, then these are indications for treatment. Poor aesthetic results may occur in the nose, lips, and ears with a hemangioma. Children demonstrate self-perception of themselves at approximately 18 to 24 months. So, many advocate treatment at approximately 2 years of age. The goals of treatment are to prevent the loss of function, relieve the pain and bleeding from ulceration, and provide an acceptable aesthetic result. In this algorithm for treatment for the proliferative phase from a study from Williams, you can see that if there is a functional problem with this ulceration, the main stay of treatment is the pulsed dye laser. If the eyelid is involved, make sure to get an ophthalmologic evaluation to rule out amblyopia. The unaffected eye can be patched, and if there is corneal exposure, optic nerve compression, or amblyopic threat, then systemic or intralesional steroids, and surgical debulking are required. If there is rapid proliferation of the superficial lesions, pulsed dye laser is the treatment. For rapid proliferation of the deep lesions, oral steroids can be used and, if it is diffuse, large, or disfiguring, oral steroids can be used. Otherwise, in insignificant hemangioma, we can just watch these and most will involute. Options for treatment in the involutional phase:If there is a functional problem, at involution, Finn states that surgical excision at 18 to 24 months, when the child his self-perception, should be considered. Otherwise, observe and consider dye laser for treatment of dermal ectasia. For the late involuters, if there is minimal deformity, no treatment is required. For ectasia and superficial deformities, or fibrofatty subcutaneous residual tissue, you can consider dye laser or surgical excision. The treatment options are steroids, oral, intralesional, topical. Interferon is another treatment, as well as laser and specifically, the pulsed dye laser, and surgical excision. Other modalities of treatment may include Vincristine or cyclophosphamide, however there are only case reports for these, and in cases that were not responsive to steroids. Cryotherapy does not have extreme case result and radiation treatment can lead to systemic growth and other induced malignancies. In terms of oral steroids, these will only work in the proliferative phase and they are mainly used for diffuse, large, or disfiguring lesions. The mechanisms of action stop, but they may potentiate the actions of vasoconstrictors on vascular smooth muscles and inhibit angiogenesis. With this does, one should see an effect within one to two weeks. If you do not, then you should probably stop the steroids, as it is most likely not worth the side effects. A third will improve on oral steroids, a third will stay about the same and stop proliferating, and a third will continue to proliferate. This slide shows a patient approximately two months after steroids. When you sense that this is a rapidly proliferating lesion, the earlier you treat, the better results the patient may have. Intralesional steroids with triamcinolone or betamethasone demonstrate similar results with oral steroids, although they can still have the systemic side effects that oral steroids have. You have to be concerned about injection of steroids around the eye for retinal artery occlusion. Topical steroids are not efficacious and demonstrate much slower results. This is a patient after oral and intralesional steroids; this is approximately 1 year later. Interferon inhibits the process of proliferation, and angiogenesis as opposed to steroids that can be used both in the proliferating and in involution stage. It works much more slowly than steroids and lasts about 8 months, and it also has its own side effects. In terms of the laser, the flash length pulsed dye laser is probably the best laser and surgical modality. It photocoagulates vessels while keeping overlying skin and mucosa intact - as opposed to the argon, which is a continuous laser and causes a lot of damage, scarring, and hypopigmentation around the area. It penetrates approximately 1 to 2 mm, requiring about 6 treatments every two to four weeks. Lasers can be used in hemangioma during both the proliferative phase and the involution phase. The YAG laser is used for deeper lesions or surgical therapy is used for deeper hemangiomas. For appropriate changes after involution, surgery is recommended, as in this involuting hemangioma. You can see it is starting to turn dark with an ash and grey and you can do an M-plasty to shorten the length of the scar and obtain quite a good result. CO2 laser can also be used in the involution stage. When excising these hemangiomas, if they are tense and rapidly proliferating, it is important to maintain the subunits of the nose, an external rhinoplasty approach can be used here, making sure to maintain the integrity of the lower lateral cartilages, and reapproximate these afterwards. With lip hemangiomas, it is important to maintain the white line and just perform a debulking procedure, if necessary. Vascular Malformations: The most simple kind is a nevus simplex or telangiectatics or small nevus flammeus, also known as salmon patch or stork bite, occurring in about 25% to 40% of Caucasian neonates. Forty percent of these will occur on the nape of the neck and 50% of those that occur on the nape of the neck will regress completely. Twenty percent will be seen on the eyelid or the glabella. The eyelid ones fade by 6 to 12 months, whereas the glabella ones will fade much later, at approximately five to six years of age. The most common vascular malformations, port-wine stains, most commonly in the head and neck, occur in the V2 distribution. They are postcapillary venule malformations, otherwise called large nevus flammeus or capillary vascular malformations. These are congenital lesions due to inborn errors of vascular morphogenesis and when they first appear, they are flat, a peak red hue at birth, and later, as the child grows, they will grow with the child and will start to darken to this maroon color, developing this nodular appearance called cobbling. There is again no cellular proliferation of these vascular malformations; they simply hypertrophy, and again, here you can see the nodularity. They are characterized into four grades. As they grow, they become thicker, purple, and cobbled. It is thought that there is a diminished perivascular nerve density in these port-wine stains, leading to vascular ectasia due to decreased sympathetic nerve stimulation. Again, these will grow with puberty and with pregnancy, as you can see here, based on this chart. One syndrome to remember with port-wine stain is the Sturge-Weber syndrome. V1 distribution is definitely a necessary diagnosis. These may or may not have V2 or V3 involvement. The CNS abnormalities that may be seen include cerebral atrophy, leptomeningeal angiomas, and cortical calcifications. These patients will frequently have seizures, mental retardation, and they develop glaucoma. In order to diagnose Sturge-Weber syndrome, an MRI must be performed after the first six months of life. You would not see the CNS abnormalities prior to the first six months, and if there are no CNS findings after two years of age in a patient with a V1 port-wine stain, then you can exclude the diagnosis of Sturge-Weber syndrome. Venous malformations are less common, and occur one in every 5000 to 10,000 patients. They are blue or purple, soft, compressible, nonpulsatile masses, and may develop swelling of the lesion. The key term is calcified phleboliths, which you may see on x-ray modality or palpation. These enlarge when the patients are dependent and are typically asymptomatic, unless impinging on vital structures. Two important syndromes to remember are blue rubber-bleb nevus syndrome, where you may see skin and GI tract malformations. This patient has a deep venous malformation of his right elbow in addition to GI tract malformations. The other is Maffucci syndrome, which demonstrates venous malformations in addition to long bone nodules. Arteriovenous malformations are pretty uncommon in head and neck. They are most common intracranially. These may also expand with puberty, pregnancy, trauma or infection. Radiologically, diagnosis is with ultrasound, MRI or MRA. Treatment is typically only warranted when endangering signs and symptoms develop. Treatment may include sclerotherapy and embolization, followed by surgery. Treatment Modalities for Vascular Malformations: The argon laser used in the 60s and 70s is not currently used as often as it was. It is a continuous wave laser with 0.5-micrometer wavelength. It dissipates heat to the surrounding structures, which leads to scarring and hypopigmentation, which has led to its decreased use. Currently, the pulsed dye laser is probably the most common modality used for these vascular lesions. It uses a theory called selective photothermolysis, which basically is a site-specific thermally mediated injury of microscopic pigmented tissue by selectively absorbed pulses of radiation. The characteristic here is that the wavelength is preferentially absorbed by the target structure and this laser is able to penetrate deeply enough to get to these layers of the papillary and reticular dermis. Now, the longer wavelengths penetrate deeper, but they actually have less energy, therefore requiring more fluence or energy delivered per unit area. The key here is that the pulsed duration or target exposure time is less than or equal to the time necessary to cool the overlying skin (this is called the thermal relaxation time), and with even increased pulse duration, the lesion gets larger with larger vessels. A sufficient amount of influence is necessary to reach a damaging temperature for target lesion. Here you can see a patient with a port-wine stain with a V1 distribution and you can see some lightening here after six months of pulsed dye laser treatment. Again, the wavelength that is typically used for the pulsed dye lasers is 585nm to 600nm, and as we increase the wavelength, there is increased energy. The pulse duration is typically 450 microseconds and you can use skin-cooling techniques to further prevent heat to the overlying skin, scarring, and hypopigmentation. There is also less discomfort and decreased healing time using the skin-cooling techniques. One simple technique is ice. Some of these skin photolysis have dynamic cooling devices with a cryogen spray. Most of these lesions, vascular malformations, can typically start at about six months. Most physicians use general anesthesia on these children when they perform the pulsed dye laser treatments. Anywhere from two to eight sessions have been documented, approximately four to eight weeks apart, and actually, it has been shown that younger patients, less than 7 years of age, will require fewer laser treatments. Approximately 65% of patients will have good results and achieve a 50% to 90% lightening of these lesions. Here you can see that same patient with a port-wine stain after four treatments with a pulsed dye laser, approximately six weeks later. Sclerotherapy is another modality which can be used for venous malformations. It is typically reserved for patients with large dilated veins, and can be performed prior to surgery. The most typically used substances that I found in the literature are 5% ethanolamine oleate, which can be used for esophageal varices, and 95% ethanol. Lee demonstrated a 95% initial success using this 95% ethanol; however, his follow-up was fairly short. Here you can see a ring-wall venous malformation and here is the lesion approximately six months after sclerotherapy. In summary, the nomenclature surrounding hemangiomas and vascular malformations can be confusing. Therefore, the most important factor to remember is that vascular malformations all present at birth. They lack proliferating cells, they only hypertrophy, and they exhibit commensurate growth with the child; whereas hemangiomas are typically present within the first few weeks of life, and are typically not present at birth. They have a proliferating and involuting phase, and most of them regress. Treatment options for hemangiomas include steroids, interferon, laser therapy, and surgery. Treatment options for vascular malformations include sclerotherapy, laser therapy, surgery, and embolization. Case Presentation: HW is a 67 year-old white male who presented to the VA ENT clinic for evaluation of a lower lip and tongue lesion. The patient states that he has had these lesions since birth. He has noticed that he has difficulty keeping liquids in his mouth due to the lower lip lesion. They are non- painful and enlarge when dependent. He has a past medical history of hypertension, cardiomyopathy, coronary artery disease, and atrial fibrillation for which he takes coumadin and digoxin. On exam, 80% of his lower lip contains a compressible and non-painful bluish mass. His left oral tongue also contains a compressible and non-painful mass. A diagnosis of lower lip and lingual venous malformation was made clinically. It was decided that since the patient is on coumadin for atrial fibrillation, his best modality for treatment would be sclerotherapy to the lower lip. We are currently in the process of obtaining sclerotherapy for this lower lip lesion from the vascular surgery department. Bibliography: Astner S, Anderson RR. Treating vascular lesions. Dermatology Therapy 2005;18:267-281. Batniji RK, Buckingham ED, Williams EF III. An aesthetic approach to facial hemangiomas. Arch Facial Plast Surg 2005;7:301-306. Ceisler EJ, Santos L, Blei F. Periocular hemangiomas: What every physician should know. Pediatr Dermatol 2004;21:1-9. Chiller KG, Passaro D, Frieden IJ. Hemangiomas of infancy: Clinical characteristics, morphologic subtypes, and their relationship to race, ethnicity, and sex. Arch Dermatol 2002;138:1567-1576. Chan YC, Giam YC. Guidelines of care for cutaneous haemangiomas. An Acad Med Singapore 2005;34:117-123. David LR, Malek MM, Argenta LC. 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Williams EF III, Stanislaw P, Dupree M, Mourtzikos K, Mihm M, Shannon L. Hemangiomas in infants and children. Arch Facial Plast Surg 2002;2:103-111. Wirth FA, Lowitt MH. Diagnosis and treatment of cutaneous vascular lesions. http://www.aafp.org/afp/980215ap/wirth.html. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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