Chapter XXII.3. Hemangiomas, Vascular Malformations, and Nevi
Amity Tran
Janurary 2023

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The editors and current author would like to thank and acknowledge the significant contribution of the previous author of this chapter from the 2002 first edition Dr. Malcolm S. Michels. This current third edition chapter is a revision and update of the original author’s work.

A 12 month old female is brought in by her mother because she was afraid that her daughter has a skin tumor. What started as a small red spot on her cheek has recently grown to almost the size of a dime. It now sticks out and can be palpated with a bulging appearance. Mom is also concerned because other people have remarked about the lesion, but the child seems unconcerned as it seems to cause no pain.

The child's exam is normal except for a 1 cm bright red strawberry colored mass on her cheek which is elevated approximately 4 mm higher than the surface of her skin. You advise her parents that this is a strawberry hemangioma which should resolve on its own. Surgical removal is not advised since this will result in more scarring than letting it involute on its own.

Vascular birthmarks are common lesions that have a variable presentation (1). Various methods of categorizing congenital vascular lesions have arisen. One common grouping is to separate these entities into: 1) hemangiomas, sometimes termed vascular nevi, and 2) vascular malformations.

Hemangiomas are defined as benign neoplasms with proliferating vascular endothelium. This endothelium enlarges, stabilizes, and finally undergoes involution. Vascular malformations on the other hand, are hamartomas of mature endothelium that do not proliferate. Hemangiomas are also three times more common in females and are less common in Black and Asian children (1,2). Risk factors for infantile hemangiomas include multiple gestation, advanced maternal age, preeclampsia, and placenta previa (1). Additionally, prematurity has been found to be a significant risk factor for hemangiomas (2). Hemangiomas are usually single lesions, but cases of multiple lesions may also occur. Children with more than five cutaneous hemangiomas may experience lesions involving the internal organs (2).

Hemangiomas appear as superficial, deep, or mixed lesions. Superficial hemangiomas appear as bright red dome-shaped, plaque-like, or lobulated papules, plaques, and nodules (1). On palpation, superficial lesions feel rubbery or noncompressible (1). They may also blanch with pressure. Deep hemangiomas are more subcutaneous, partially compressible, and warm on palpation (1). Unlike their superficial counterpart, deep hemangiomas appear blue in color. Mixed lesions have a superficial and deep component, appearing with a superficial bright-red and deep blue component (1).

There are well-defined stages of hemangiomas: proliferative, stationary, and involutional phases (1). Characteristically the lesions grow and become more protuberant over a period of time and adopt the typical raised configuration of the strawberry hemangioma. They then slowly regress and in about 70% of cases, they completely disappear by age 7 (1). Overall spontaneous resolution occurs in greater than 90% (1). 50% of children may experience scarring, loose skin, or telangiectasias after resolution (2). Treatment is usually discouraged because a variety of techniques including surgery, cryotherapy, laser therapy, injection of sclerosing agents, and photocoagulation have all caused scarring that is unacceptable compared to the typical spontaneous resolution. In rare instances, vital functions are compromised when lesions are located near the anus, urethra, airway, or eye (2). For these cases and those that may require later surgery, propranolol is the first-line therapy which has a low-risk of adverse effects and long-term neurocognitive effects (2,3). It is also FDA-approved for this indication (2). Timolol has also been reported to be efficient in treating small, uncomplicated superficial hemangiomas (2).

Hemangiomas are usually not associated with extracutaneous syndromes (2). However, midline hemangiomas, particularly those in the lumbosacral area have been found to be associated with vertebral and spinal cord abnormalities;e.g., LUMBAR (Lower body hemangioma, Urogenital abnormalities / ulceration, Myelopathy, Bony deformities, Anorectal malformations / arterial anomalies, and Rectal anomalies), SACRAL (Spinal dysraphism, Anogenital, Cutaneous, Renal and urologic anomalies, associated with an Angioma of Lumbosacral localization), and PELVIS (Perineal hemangioma, External genitalia malformations, Lipomyelomeningocele, Vesicorenal abnormalities, Imperforate anus, and Skin tag) syndrome (1,2). Large, segmental hemangiomas of the face and scalp have been linked to PHACE syndrome (Posterior fossa brain malformations, Hemangiomas, Arterial anomalies, Cardiac defects, and Eye defects) (1, 2). It is suggested that in these cases, ultrasound or MRI studies be obtained to exclude these diagnoses (2).

One particular type of hemangioma should be mentioned, Kasabach-Merritt syndrome. In this condition, the hemangioma is large and extends to deep structures (1). Complications can include disfigurement; high output cardiac failure, infection and thrombocytopenia with acute or chronic consumptive coagulopathy as in DIC (disseminated intravascular coagulation) (1). Kasabach-Merritt syndrome commonly occurs within the first few weeks after birth with a rapidly enlarging vascular lesion (1). Other symptoms that may occur include ecchymosis, prolonged bleeding, epistaxis, hematuria, or hematochezia (1). The mortality rate of this syndrome ranges from 10% to 30% (1).

Vascular malformations make up about two thirds of all lesions in the newborn. Common vascular malformations include the "salmon patch" which is an extremely common benign capillary malformation (2). They usually spontaneously fade within the first year of life (2). Patches may persist or darken with crying, breath holding, and physical exertion; however, these do not require any further evaluation (2). Unlike salmon patches, the "port wine stain" or nevus flammeus, is permanent (2). It persists throughout childhood and typically darkens and thickens during adolescence and adulthood (2). When this occurs on the posterior neck, it is termed the "stork bite", or on the eyelids, the "angel's kiss". Most of the lesions on the face fade with time. Some of the occipital patches persist.

In early stages, port wine stains may be difficult to distinguish from hemangiomas (1). However, port wine stains are composed of mature capillaries that are limited to the dermis alone. Additionally, hemangiomas tend to rapidly proliferate and thicken during the first few months of life, unlike port wine stains which develop over the course of years (1). As the number of vessels increases and age, the color can change from bright pink to dark purple. As facial port wine stains age, the vessels undergo progressive ectasia, developing a dark violet hue and often a roughening skin texture (1, 2). This can lead to considerable emotional impact on the part of the child and the parents as well. Laser treatment is available for this condition (1). Certain parts of the skin act as targets that absorb specific wavelengths of light. The light is converted to heat, which destroys the target tissue. Careful selection of the wavelength administered can direct precise treatment with minimal peripheral destruction. This tends to decrease scarring, although some degree of scar formation is always possible.

There are several syndromes that can be associated with vascular malformations. The most common of these is Sturge-Weber Syndrome. Externally, this presents with a port wine stain over areas of the face innervated by the first division of the trigeminal nerve known as the V1 nerve (1). The significant hidden finding is the ipsilateral leptomeningeal angiomatosis, which usually presents with seizures during the first year of life (1). Classically this produces railroad track calcifications of cortical vessels seen on plain skull films. CT may show these cortical calcifications earlier in life than routine X-ray. Other concomitant findings include developmental delay, cognitive impairment, and various eye findings including glaucoma, visual field defects, optic atrophy, cataracts, retinal detachment and heterochromia of the iris (1). For this reason, MRI and CT are advised for detecting the cerebral atrophy and cortical calcifications classically seen in this syndrome (1). Serial ophthalmologic examinations are necessary to screen for glaucoma.

Nevi are collections of normal melanocytes. Non-medical people call these moles. The greatest concern about them comes from the fact that some undergo malignant transformation to melanoma. Nevi falls into several classes.

Congenital melanocytic nevi are pigmented macules or plaques that are usually accompanied by hair growth (2). Giant congenital nevi are defined as being greater than 20 cm in diameter and are associated with a 2% to 6% lifetime risk of melanoma (2). Malignant change typically occurs in the first 10 years of life (2). Therefore, most authorities feel that they should be removed if at all possible. Sometimes the location or extent makes removal very difficult, which means close observation and periodic MRI or CT scans are required (2). Some practitioners advocate the removal of all congenital nevi, regardless of size because of this theoretic danger.

Acquired melanocytic nevi, or common moles can appear at any time after birth. In early stages, the nevi are called junctional nevi, appearing flat and located at the dermal-epidermal junction (1). The nevi then enlarge, become papular, and migrate into the dermis, becoming compound nevi (1). They frequently are raised but smooth bordered. Over time, nevi may become fleshy or pedunculated (1). These nevi are intradermal nevi with the nevus cells residing completely within the dermis. There is a possibility of malignant transformation in these nevi as well, but as long as the clinical appearance does not rapidly change, they do not need to be removed and only observation is needed.

Lentigines are small, (less than 1 cm), hyperpigmented macules which unlike freckles, can develop on sun exposed skin but also on unexposed areas of the body. The risk of malignancy is low, but lentigines are a sign of significant sun exposure (2). Several syndromes may be associated with lentigines. The most well known of these is Peutz-Jeghers syndrome. This entity also includes characteristic pigmented lesions on the lips and oral cavity. It is dominantly inherited, but what is most problematic are the associated intestinal polyps which may cause cramping or bleeding. A rare autosomal dominant multisystem disease called LEOPARD syndrome (Lentigines, ECG conduction abnormalities, Ocular hypertelorism, Pulmonic stenosis, Abnormal genitalia, growth Retardation, neural Deafness) has also been associated with lentigines (2).

Café au lait spots are another type light brown hyperpigmented lesions that appear at birth or during childhood (2). Lesions vary in size and can present on any site on the skin (2). Most children who present with café au lait spots are healthy, but if there are more than 5 or 6 in number this can be indicative of neurofibromatosis or von Recklinghausen disease (2). Café au lait spots are not specific to neurofibromatosis and have been linked to a number of other disorders such as tuberous sclerosis, McCune-Albright syndrome, and epidermal nevus syndrome (2).

1. True/False: Proliferating vascular endothelium lesions can be treated with propranolol.

2. True/False: The concerned parent whose child has a protuberant, growing vascular lesion in early childhood can often be reassured that the lesion will involute with time.

3. Common manifestations of Sturge-Weber Syndrome include all of the following except:
   a. Meningeal vascular malformations
   b. Choanal atresia
   c. Visual field defects
   d. Developmental delay

4. True/False: Kasabach-Merritt Syndrome can cause thrombocytopenia.

5. True/False: Lentigines occur in sun exposed areas.

6. True/False: Peutz-Jeghers syndrome sometimes diagnosed when hyperpigmented macules are found on the lips of children with chronic abdominal pain.

1. Mancini AJ, Paller AS. Chapter 12. Infantile hemangiomas, vascular malformations, and other vascular disorders of infancy and childhood. In: Mancini AJ, Paller AS (eds). Hurwitz’s Clinical Pediatric Dermatology: a Textbook of Skin Disorders of Childhood & Adolescence, 6th edition. 2022. Elsevier, St. Louis. pp:326-368.
2. Püttgen KB, Cohen BA. Chapter 2. Neonatal Dermatology. In: Cohen BA (eds). Pediatric Dermatology, 5th edition. 2022. Elsevier, Philadelphia. pp:14-67.
3. Hagen R, Ghareeb E, Jalali O, et al. Infantile hemangiomas: what have we learned from propranolol? Curr Opin Pediatr. 2018;30(4):499-504. doi: 10.1097/MOP.0000000000000650

Answers to questions
1.True, 2.True, 3.b, 4.True, 5.True, 6.True

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