AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Capillary malformation (CM), usually referred to as a port-wine stain or nevus flammeus, is the most common type of vascular malformation. As a congenital malformation of the superficial dermal blood vessels, CM is present at birth and grows in size commensurate with the child; it remains present for life and has no tendency toward involution.

Past nosology of this lesion has resulted in much confusion, and an excessive number of descriptive terms have been applied to it. Confusion originated from difficulty in differentiating vascular malformations from vascular proliferative lesions, such as hemangiomas, and from the use of wholly clinical descriptions in categorizing these lesions. Vascular malformations are categorized by predominate vessel type, such as arterial, venous, lymphatic, capillary, or complex (a combination of different vessels). Although some CMs may be associated with other vessel malformations, most occur alone as venulocapillary malformations.

Pathophysiology

CM and other vascular malformations are the result of abnormal morphogenesis. CMs are characterized by ectatic papillary dermal capillaries and postcapillary venules in the upper reticular dermis, with some evidence of increased vessel density and no apparent proliferation of vessels. These ectatic vessels are lined by flat, benign-appearing endothelial cells, similar to the vessels of normal skin, with similar staining characteristics for endothelial antigens, including fibronectin, von Willebrand factor, and collagenous basement membrane proteins. The endothelial cells also exhibit cell turnover similar to normal vessels, supported by a paucity of mitoses or an uptake of tritiated thymidine. One study demonstrated a mean vessel depth of 0.46 mm in CMs, suggesting that most of the vessels are superficial.

Mounting evidence supports a neural role in both the development and progression of CMs. Animal studies show that the sympathetic nervous system influences the composition and functional properties of the vessel wall during development. Immunohistochemical studies of CMs reveal a significantly decreased density of perivascular nervous tissue in lesional skin, suggesting that inadequate innervation may be responsible for decreased vascular tone and progressive vascular dilatation.

Transcutaneous videomicroscopy reveals 2 distinct patterns of vascular ectasia in CMs. The type 1 abnormality is composed of superficial, tortuous, dilated end capillary loops in the superficial papillary dermis. The type 2 abnormality consists of dilated, ectatic vessels in the superficial horizontal vascular plexus. Some patients exhibit a combination of both abnormal patterns. Evidence suggests that the type 1 abnormality has a better response to 585-nm flashlamp-pumped pulsed dye laser (PDL) therapy than the type 2 abnormality. Moreover, one study found a correlation between the depth and pattern of the CMs and the location of the lesions, demonstrating improved responses to laser therapy with locations demonstrating a type 1 pattern (V3 region of the face, neck, and trunk).

An inactivating mutation of RASA1 on 5q has been detected in some kindreds with multiple, small, round-to-oval, pink CMs (Boon, 2005). These kindreds all had members who also had arteriovenous (AV) malformations or AV fistulae.

Frequency

United States

CM occurs in 0.3-0.5% of newborns.

International

Worldwide, CM occurs in 0.1-2% of newborns.

Mortality/Morbidity

• Isolated CMs do not appear to cause an increase in mortality; however, psychosocial disability secondary to facial disfigurement can be overwhelming. Several studies demonstrate that patients with facial CMs exhibit greater self-concern, ruminative self-doubt in interpersonal interactions, social inhibition, isolated and passive orientation in interpersonal relationships, stigmatization from society, and limitations of privileges and opportunities otherwise afforded to those without facial disfigurement.
• Any morbidity involved with CMs is associated with more extensive vascular malformations.

Race

According to Pratt's study, CM is more common in whites than in African Americans.

Sex

The sex distribution is equal.

Age

CMs are present at birth. Some lesions may not be readily observed at birth because of anemia or plethora. In certain lesions, some lightening of the lesions may occur during the first year of life; however, beyond that time, further lightening is generally not observed.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

History

Nearly all cases of CM can be diagnosed by taking a careful history and performing a physical examination.

• Onset: CMs are always present at birth, but they may not be apparent early in life because of neonatal anemia or plethora.
• Location
• • Of CMs, most involve the head and the neck.
  • Of facial CMs, 45% are more or less restricted to 1 of the 3 areas supplied by the divisions of the fifth cranial nerve.
  • Of facial CMs, 55% involve an area innervated by more than 1 division of the fifth cranial nerve, crossing the midline or occurring bilaterally.
• Growth
• • Growth in size is commensurate with that of the child.
  • CMs remain present for life.
  • They show no tendency toward involution.
• Evolution
• • CMs may change from pink in infancy to red in early adulthood to deep purple during middle age in some individuals.
  • The surface may become thickened with a cobblestonelike contour. Studies estimate that up to 65% of facial CMs develop these changes during adulthood.
  • Nodular vascular lesions may develop, usually in adulthood.
  • Pyogenic granulomas with bleeding may develop in CMs, even in childhood.
• CM may coexist with other vascular malformations. Geographic (ie, well-circumscribed, sharply bordered) cutaneous lesions carry a much higher probability of associated lymphatic malformations than blotchy stains, especially in patients with Klippel-Trenaunay syndrome.
  

Physical

Early in life, the lesions appear as flat (macular), mostly well-circumscribed patches. The color varies from pink to red to purple. The color of the CM does not correlate with the capillary depth or diameter. Blanching with external pressure is variable. In infancy and childhood, the color darkens with crying, fever, or overheating. CMs are usually unilateral with fairly sharp midline cutoffs. The face is the most frequently affected site, followed by the upper part of the trunk.

Later in life, as the vasculature dilates the CM may evolve into a raised, thickened plaque. The CM becomes deep-red to purple. Lesions may become studded with vascular papules, imparting a cobblestonelike appearance. Vascular papules often form and may be prone to bleeding. Skin and underlying soft tissue or bony hypertrophy may be present. Lobulated capillary hemangiomas (pyogenic granulomas) may form, especially with intraoral lesions.

Associated findings are as follows:

• Glaucoma occurs in approximately 10% of patients with facial CMs, and no leptomeningeal involvement is present. Glaucoma affects 27-45% of patients when CMs involve the skin supplied by both the ophthalmic (CN V1) and the maxillary (CN V2) divisions of the fifth cranial nerve, the trigeminal nerve. Glaucoma is less frequent when the face is involved in only 1 of these upper divisions of the trigeminal nerve or if it is affected solely below the eye; however, the prevalence of eyelid involvement is increased. The prevalence may not be correlated with increased vascularity of the choroid or the bulbar conjunctiva. Glaucoma may be due to increased episcleral venous pressure with resultant elevated intraocular pressure, and it can occur without leptomeningeal involvement (eg, in the absence of Sturge-Weber syndrome).
• Other types of vascular malformations (venous, lymphatic, arterial, or mixed) may be present.
• • Sturge-Weber syndrome
  • • Sturge-Weber syndrome (encephalofacial or encephalotrigeminal angiomatosis) is characterized by the triad of CMs involving the upper facial dermis, the ipsilateral leptomeninges, and the ipsilateral cerebral cortex. Some authorities believe that only 2 features are necessary to make this diagnosis. The facial skin supplied by the ophthalmic branch (CN V1) of the trigeminal nerve must be involved with the CM for a patient to meet one of the criteria for Sturge-Weber syndrome.
    • Sturge-Weber syndrome occurs in less than 10% of patients with CMs on the upper eyelid or the forehead. Involvement of areas on the face supplied by only CN V2 or CN V3 does not carry an increased risk for Sturge-Weber syndrome.
    • Typically, CM associated with Sturge-Weber syndrome is more extensive than isolated CM, and it often has bilateral facial involvement. Complications include glaucoma, seizures, hemiplegia, mental retardation, cerebral calcifications, subdural hemorrhage, and an increased prevalence of underlying soft tissue hypertrophy. Large variability exists in the severity of associated symptoms.
  • Klippel-Trenaunay syndrome (angio-osteohypertrophy syndrome) manifests as a triad of CM, congenital varicose veins, and hypertrophy of underlying tissues, particularly skeletal overgrowth. The sex distribution is equal. The lower limbs are involved in 95% of patients, and involvement is unilateral in 85%. Most are asymptomatic at birth, but may experience problems later in childhood. Complications include varicose veins with venous thrombosis and pulmonary embolism; bleeding from varices, the rectum, or the bladder; skin ulceration; increased sweating overlying the CM; leg circumference or length discrepancy with resultant scoliosis; edema; and recurrent infections.
  • With Parkes-Weber syndrome, the diagnostic criteria include an AV malformation in addition to those listed above for Klippel-Trenaunay syndrome. AV fistulae are usually diffuse and difficult to ablate. Almost all patients present in childhood with an enlarged, warm extremity. The prognosis is worse than that associated with Klippel-Trenaunay syndrome. A positive bradycardic reaction (Nicoladoni-Branham sign) portends a poorer prognosis. This test is performed by occluding the arterial inflow by compression with a blood pressure cuff. In a limb with a hemodynamically significant AV malformation, this maneuver leads to reflex bradycardia secondary to a sharp rise in blood pressure. Complications include ulceration and severe lymphedema.
  • In Cobb syndrome (cutaneomeningospinal angiomatosis), a cutaneous vascular lesion in the skin overlying the spine is associated with vascular malformations (venous or AV) in the subjacent spinal meninges. Possible complications result from neurologic damage caused by mass effect on the spinal cord or nerves, bone erosion, and subarachnoid hemorrhage.
  • Wyburn-Mason syndrome (unilateral retinocephalic syndrome), also known as Bonnet-Dechaume-Blanc syndrome, manifests as facial CMs associated with unilateral AV malformation of the retina and the intracranial optic pathway. Physical findings include monocular amblyopia, mild proptosis, and dilatation of conjunctival vessels. CMs may occur anywhere on the ipsilateral face (not just the eyelids or periorbitally), and they may have associated facial hypertrophy or occasional involvement of the optic chiasm, the hypothalamus, the midbrain, and the basal ganglia, with associated mental retardation or neurologic signs and symptoms.
  • Nevus vascularis mixtus is the name given when a CM is paired with nevus anemicus, an example of didymosis or twin spotting.
• CM overlying the lumbar spine may be a marker for an underlying primary skeletal or neurologic anomaly, such as spinal dysraphism, tethered spinal cord, lipomeningocele, or diastematomyelia. The prevalence of underlying defects is increased when multiple abnormalities are present in the lumbar skin. Skin markers include acrochordons (skin tags), an abnormal tuft of hair (fawn's tail), lipomas, an irregular (usually deviated) gluteal cleft, or a dermal sinus tract or sacral dimple that is large or superior to the gluteal fold. Guggisberg et al found that none of 16 patients with an isolated CM showed occult spinal dysraphism (OSD), whereas 7 of 10 patients with CMs in combination with other lumbar congenital anomalies did have an OSD. Conversely, Tubbs et al found that 21 (17.5%) of 120 patients with an isolated CM harbored an OSD, and they recommended MRI for all patients who present with an isolated lumbar CM.
• Phakomatosis pigmentovascularis refers to the presence of a CM with a melanocytic or other type of nevus.
• • This phenotype is another example of twin spotting, with the pathogenesis hypothesized to include developmental abnormalities, perhaps paired mutations of varied neural crest–derived elements, such as vasomotor nerves and melanocytes.
  • The histopathologic findings of a CM in phakomatosis pigmentovascularis are the same as those for isolated CMs.
  • Four classification types are described. Type I is composed of CM and nevus pigmentosus et verrucosus or epidermal nevus. Type II is a CM and dermal melanocytosis with or without nevus anemicus; this is the most common type. It also includes nevus of Ota (oculocutaneous melanosis) and can be associated with Sturge-Weber syndrome and Klippel-Trenaunay syndrome. Type III is a CM and nevus spilus with or without nevus anemicus. Type IV is a CM, nevus spilus, and dermal melanocytosis with or without nevus anemicus. Subdivisions of each type include subtype a for cutaneous involvement only and subtype b for cutaneous and systemic involvement. No systemic involvement is reported for type I.
  • Recently, another classification scheme has been proposed by Happle. This includes 3 different distinct categories based on the type of associated lesion: phacomatosis cesioflammea (CM with bluish gray spots as observed with various lesions of dermal melanocytosis), phacomatosis spilorosea (pale pink telangiectatic CM associated with a nevus spilus), and phacomatosis cesiomarmorata (cutis marmorata telangiectatica congenita with blue spots). A final category includes others that cannot be included in one of the other 3 variants.

Causes

The exact mechanism remains unknown.

• Evidence for genetic influence is lacking. Postzygotic somatic mutations may account for the mosaic and twin-spotting phenotypes.
• CMs may result from a neural deficiency of sympathetic innervation of the superficial dermal blood vessels.

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Early infantile hemangiomas: Consider a diagnosis of infantile hemangiomas before the onset of rapid proliferation. Infantile hemangiomas are easily differentiated from CMs by the observation of rapid growth in the hemangiomas.

Abortive (telangiectatic) infantile hemangiomas: These usually are flat, pink patches with obvious telangiectases running throughout the lesion. These will involute slowly over time, albeit slower than typical infantile hemangiomas.

Nevus flammeus neonatorum: Synonyms include salmon patch, stork bite, angel kiss, nevus simplex, nevus flammeus nuchae, medial or midline telangiectatic nevus, and medial nevus flammeus. They occur on the midline glabella, the nose, the upper lip, the occipital scalp, or the eyelids. They are usually lighter pink than CMs, with rates of 42% in white neonates and 31% in black neonates. In most cases, the anterior facial lesions lighten or resolve by the time the patient is aged 1-2 years; however, the occipital and lumbar lesions may persist throughout life.

Beckwith-Wiedemann syndrome

Coats disease: Retinal telangiectasia (usually unilateral) is rarely reported with an ipsilateral facial CM.

Cobb syndrome: See Physical.

Parkes-Weber syndrome: See Physical.

Phacomatosis pigmentovascularis

Roberts syndrome: This is characterized by facial CM, hypomelia, hypotrichosis, cleft lip, and growth retardation.

TAR syndrome: This is characterized by congenital thrombocytopenia, bilateral absence or hypoplasia of the radius, and CMs.

Sturge-Weber syndrome: See Physical.

von Hippel-Lindau disease

Wyburn-Mason syndrome: See Physical.

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Imaging Studies

• Imaging studies should be performed if Sturge-Weber syndrome is suspected. MRI with gadolinium enhancement is the optimal diagnostic imaging technique for the screening of Sturge-Weber syndrome.
• • CT scan or MRI findings may be absent during the first few years, unless intravenous contrast is administered. However, even if intravenous contrast is used, some milder lesions may still not be detected. Positive findings include gyral enhancement, enlargement and enhancement of the ipsilateral choroid plexus, progressive cortical atrophy, and gyral calcification. Accelerated myelination in the involved hemisphere also may be an early diagnostic feature before age 6 months in some infants.
  • Cerebral angiography can detect parenchymal contrast stasis and abnormal cortical veins.
• Debate continues regarding the need for and type of diagnostic imaging for patients who present with an isolated CM. Some authorities believe that in low-risk lesions such as an isolated CM, either no imaging or ultrasonography (age <5 mo) is reasonable. Imaging studies should be performed in all patients who have a CM and another lumbosacral cutaneous anomaly, because the risk is much higher. Some authors recommend MRI for all children who present with an isolated lumbar CM.

Other Tests

• An ophthalmologic evaluation with tonometry to exclude glaucoma in infants with CN V1 and CN V2 or eyelid involvement should be performed semiannually for the first 3 years of life and annually thereafter.

Histologic Findings

Histologically, the abnormal features are difficult to appreciate in tissue samples obtained from children younger than 10 years. With time, the affected vessels become progressively more ectatic and filled with erythrocytes. The ectasia appears to progress from the superficial dermis to the deeper dermis and subcutaneous tissues. The endothelial cells of a CM do not stain for GLUT-1, a specific marker for infantile hemangiomas.

TREATMENT

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical Care

• Affected areas can be tattooed with a skin-colored pigment; however, this is not routinely performed.
• Use of a cosmetic cover-up is an alternative. Opaque makeup, sold commercially without a prescription under brand names like Dermablend and Covermark, disguises CMs but does not treat them.

Surgical Care

• Flashlamp-pumped PDL therapy is the treatment of choice for CMs. It uses selective photothermolysis with ultrashort pulses of monochromatic yellow light (585-600 nm), which are preferentially absorbed by oxyhemoglobin in the abnormally dilated superficial dermal blood vessels. It causes selective destruction of these superficial target blood vessels, inducing intravascular coagulation and rupture of some smaller vessels, and it limits surrounding dermal and epidermal destruction. Kono et al recently published a thorough review of PDL therapy for CMs (Kono 2006), which examines many of the idiosyncrasies of this treatment modality.
• • Skin treated with a PDL immediately develops edema and purpura, the latter of which generally last 1-2 weeks. It is relatively safe; major risks include pigmentation alterations (hyperpigmentation, which is always transient, and potentially permanent hypopigmentation), crusting, and, rarely, scarring. Immediate adverse events associated with PDL therapy are pain (a hot snapping sensation that can increase with repeated pulses) and combustion (especially of hair-bearing areas and in the presence of increased ambient oxygen). PDL therapy can be performed on patients with skin types V and VI, but with a potentially higher risk of pigmentary complications.
  • Some authorities advocate treatment beginning as early as 7-14 days of life, yet the age at which laser treatment achieves maximal response is unknown. Some studies indicate a better response if treatment commenced before the patient is aged 1 year, while others reveal no difference. From a psychosocial perspective, treatment commencing in infancy is most beneficial.
  • The response rate depends on several variables. The first is anatomical location, with rates in descending order of location from best to worst as follows: neck, torso, face, and hand and arm; and with rates in descending order of location from best to worst for facial CMs as follows: forehead, lateral part of the face, temple, and central part of the face. The second variable is size; smaller lesions appear to have higher response rates. These variables are influenced by the histologic features of CMs. Recently, it was demonstrated using videomicroscopy that CMs with smaller vessels deep in the dermis were the most difficult to eradicate with PDL (Sivarajan, 2006).
• Earlier laser therapies that caused an unacceptably high rate of scarring are not recommended; these therapies include carbon dioxide laser, copper vapor laser, and argon laser. Other modalities, such as intense pulsed light and the long-pulse (Yang, 2005) and frequency-doubled Nd:YAG laser (Pence, 2005), are reported to be effective in certain instances, but they are generally considered to have higher complication rates than the PDL.
• Previous nonlaser treatments determined unreliable or deleterious include surgical excision, radium implants, cryosurgery, electrocautery, sclerotherapy, and Grenz rays radiotherapy.

Consultations

• Consult a pediatric dermatologist, a general dermatologist, or a specialist at a laser center or a vascular lesion center for evaluation and early intervention if indicated.
• Consult an ophthalmologist for tonometry to exclude glaucoma in infants with CN V1 and CN V2 or eyelid involvement. An ophthalmologist can also evaluate for evidence of ocular CM in patients with Sturge-Weber syndrome.
• Consult a radiologist or refer the patient to a vascular lesion center if underlying complicating vascular, neural, or skeletal malformations are suspected. If a leg-length discrepancy is apparent in a patient with a lower extremity CM, consult a pediatric orthopedic surgeon.
• Consult a neurologist if Sturge-Weber syndrome is suspected.

FOLLOW-UP

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Patient Education

• Patients and parents benefit from an organized, informative, multidisciplinary effort to address psychological, medical, and surgical needs for optimal outcomes from medical care, especially surgical or laser procedures.

MISCELLANEOUS

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Although difficult to treat, glaucoma associated with periorbital CMs can occur early in childhood; thus, early referral for high-risk patients is important.
• CM overlying the lumbar spine may be a marker for an underlying primary skeletal or neurologic anomaly, such as spinal dysraphism, tethered spinal cord, lipomeningocele, or diastematomyelia. The frequency of underlying defects is increased when multiple abnormalities are present in the lumbar skin. Skin markers include acrochordons (skin tags), an abnormal tuft of hair (fawn's tail), lipomas, an irregular (usually deviated) gluteal cleft, or a dermal sinus tract or sacral dimple that is large or superior to the gluteal fold. Guggisberg et al found that none of 16 patients with an isolated CM showed OSD, whereas 7 of 10 patients with CM in combination with other congenital lesions did have an OSD.

Special Concerns

• In the author's experience, a frank discussion with the affected child's classmates early in the school year is most beneficial. A brief classroom discussion, which can be facilitated by the patient's parent or teacher, offers an opportunity to educate the child's classmates about the birthmark and to reassure them that it is neither painful nor contagious. Once the CM has been explained adequately and questions answered, most children cease to further question the child. This helps to alleviate some of the patient's psychological burden.

MULTIMEDIA

Section 9 of 10  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Histopathologic features of a capillary malformation (nevus flammeus) showing telangiectatic vessels lined by mature-appearing endothelial cells.
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Media file 2:  Capillary malformation on the left preauricular aspect of the cheek, the ear, and the neck in a neonate (same patient as in Images 3-4).
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Media file 3:  Same patient as in Images 2 and 4 immediately after test spots with the pulsed dye laser at 585 nm. Note the purpuric macules where the laser impacted in a linear distribution on the preauricular aspect of the cheek.
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Media type:  Photo

Media file 4:  Same patient as in Images 2-3 after 4 treatments with the pulsed dye laser. Treatments were given at 2-month intervals in an outpatient setting using topical anesthetic.
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Media type:  Photo

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Follow-up
• Miscellaneous
• Multimedia
• References

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• Sivarajan V, Maclaren WM, Mackay IR. The effect of varying pulse duration, wavelength, spot size, and fluence on the response of previously treated capillary vascular malformations to pulsed-dye laser treatment. Ann Plast Surg. Jul 2006;57(1):25-32. [Medline].
• Tubbs RS, Wellons JC 3rd, Iskandar BJ, Oakes WJ. Isolated flat capillary midline lumbosacral hemangiomas as indicators of occult spinal dysraphism. J Neurosurg. Feb 2004;100(2 Suppl Pediatrics):86-9. [Medline].
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• Yang MU, Yaroslavsky AN, Farinelli WA. Long-pulsed neodymium:yttrium-aluminum-garnet laser treatment for port-wine stains. J Am Acad Dermatol. Mar 2005;52(3 Pt 1):480-90. [Medline].

Article Last Updated: Mar 19, 2007