Hemangiomas and Vascular and Lymphatic Disorders

Cutis Marmorata


Figure 23-1

Cutis marmorata This term means “marbled skin” and is intended to describe the appearance of the skin in which the terminal vessels are so superficial and dilated that they are constantly visible in patterns that vaguely suggest veined marble. This condition is physiologic in the newborn and represents a vasomotor response to lowering of the environmental temperature. Persistent cutis marmorata is seen in Down syndrome and in trisomy 18.

Cutis Marmorata Telangiectatica Congenita


Figure 23-2

Cutis marmorata telangiectatica congenita This condition is present at birth and consists of accentuated vascular markings (cutis marmorata), along with areas of telangiectasia, and occasionally ulceration, and atrophy. The lesions may be localized, usually to a lower extremity or generalized.

Figure 23-3

Cutis marmorata telangiectatica congenita A small minority of children with this condition have other abnormalities, including hemiatrophy or hemihypertrophy. The mottling resolves gradually with age in most patients.

Livedo Reticularis


Figure 23-4

Livedo reticularis Livedo means a slate-gray blueness. Reticularis means “like a net.” The term describes a network of gray-blueness that is generally seen on the lower extremities. The idiopathic form of this disorder, most common in young women, carries a good prognosis. However, livedo reticularis is also seen in association with polyarteritis nodosa, SLE, and cryoglobulinemia.

Nevus Simplex (Salmon Patch)


Figure 23-5

Nevus simplex (salmon patch) Nevus simplex is by far the most common vascular lesion in the newborn. This midline or symmetrical pink macular lesion is most commonly seen on the eyelids, the nape of the neck, and the glabella. The last two are commonly described as “stork bite” and “angel’s kiss,” respectively. The glabellar lesion in Fig. 23-5 can be expected to resolve spontaneously, as do lesions on the eyelids. Rarely, a nevus simplex on the neck may persist into adult life.

Port-Wine Stain


Figure 23-6

Port-wine stain This unilateral vascular malformation has a markedly different histology, significance, and natural history from that of the nevus simplex. The port-wine stain is made up of capillary ectasias that may be present throughout the dermis and that gradually increase with age. The color may change from pink to purple as the patient grows, and the lesions may become nodular during adult life. Because port-wine stains show no tendency to involute, they may represent a significant, lifelong cosmetic problem.

Figure 23-7

Port-wine stain The tunable pulsed dye laser is a successful modality in the treatment of disfiguring port-wine stains. Success is related to the fact that hemoglobin preferentially absorbs laser energy at the 595 to 600 nm wavelength.

Figure 23-8

The very brief pulse duration prevents the heat from spreading from the blood vessels into the surrounding connective tissue, and the danger of scarring is thus minimized.

Sturge-Weber Syndrome


Figure 23-9

Sturge-Weber syndrome Certain port-wine stains, occurring on the face, may be associated with ocular and cerebral vascular malformations. It is now recognized that the danger of this occurrence correlates with the exact location of the skin lesions, and is related to the embryonic vasculature of the face.

Figure 23-10

Children with port-wine stain involving the forehead are at risk for seizures, neurodevelopmental abnormalities, and glaucoma. Port wine stains occurring on other parts of the face appear to carry little or no risk.



Figure 23-11

Hemangioma Infantile hemangioma is the most common benign vascular tumor that may occur during infancy. The vast majority of hemangiomas do not require treatment, and resolve spontaneously over time. Hemangiomas may be present at birth, or more commonly, develop during the first few months of life. The most rapid growth occurs during those months, and, for most, continues until 6 months of age. Some hemangiomas continue to increase in size until 1 year of age. Resolution of the hemangioma takes several years, and in some cases, may not be complete.

Figure 23-12

An additional important risk related to hemangioma is disfigurement and scarring. This is most significant for lesions involving the central face and, especially, the nose. In some cases, depending on the morphology, location, and growth stage of the hemangioma, early intervention is critical. The treatment of choice is oral propranolol, but topical timolol can be used to treat very superficial lesions.

Figure 23-13

Hemangioma Illustrated in Fig. 23-13 is an example of segmental hemangioma. In contrast the localized nodular lesions seen in previous figures, this lesion is a plaque from which areas of vascular proliferation arise.

Figure 23-14

Segmental hemangiomas in the diaper area may be associated with a variety of underlying anatomic abnormalities. The acronym LUMBAR syndrome denotes Lower body hemangioma and other cutaneous defects, Urogenital anomalies, ulceration, Myelopathy, Bony deformities, Anorectal malformations, arterial anomalies, and Renal anomalies.

Figure 23-15

Hemangioma The hemangiomas in Figs. 23-15 and 23-16 have a large deep component and a superficial overlying area of vascular discoloration. The lesion in Fig. 23-16 is beginning to show spontaneous involution of the superficial component. Note that the central part of the superficial component is lightening and becoming blue-gray.

Figure 23-16

When deep hemangiomas involute, redundancy of overlying skin may remain. Frequently no treatment is needed, but if necessary, and depending on the location, plastic surgery may be required to improve appearance. Pulsed dye laser is of value in treating the residual superficial component of such a lesion.

Figure 23-17

Hemangioma Hemangiomas that are close to the eye may result in astigmatism, strabismus, amblyopia, and even permanent vision loss. These changes result from the mass effect of the growing vascular lesion on the cornea, and from obstruction of the visual axis.

Figure 23-18

Infants with significant periorbital involvement should be managed in conjunction with an ophthalmologist.

Figure 23-19

Hemangioma Figures 23-19 and 23-20 illustrate the spontaneous regression of a hemangioma. Although the resolution of the vascular portion is complete, there remains a fibrofatty mass with abnormal skin surface at the location of the original lesion.

Figure 23-20

In general, depending on the size and location of the lesion, plastic surgery may be possible. In other cases, the patient and family may decide that surgical treatment is unnecessary.

Hemangioma (Ulcerated)


Figure 23-21

Hemangioma (ulcerated) During the growth phase of hemangiomas, especially in the groin area and buttocks (Figs. 23-21 and 23-22), ulceration may develop. The initial sign of ulceration, which is the most common complication of hemangioma, may be an area of crusting which may then enlarge and bleed. Ulcerated lesions on the scalp (Fig. 23-23) are more prone to bleeding given the increased vascularity of that area, and life-threatening bleeding has been reported in a number of hemangiomas.

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Jan 9, 2019 | Posted by in PEDIATRICS | Comments Off on Hemangiomas and Vascular and Lymphatic Disorders
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