Infantile Hemangiomas and Vascular Malformations

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Infantile Hemangiomas and Vascular Malformations





Infantile Hemangioma (IH)



Benign vascular neoplasm that represents the most common tumor of infancy.


Affects ~5% of infants, with a predilection for girls and premature neonates (see Table 85.1).


IHs appear during the first few weeks of life, with a characteristic course:


Proliferation for 6–9 months, tending to ‘mark out their territory’ early on and then grow primarily in volume; overall, ~80% of growth is in the first 5 months of life.


Subsequent involution gradually over several years (e.g. 30% completed by 3 years of age and 50% by 5 years), with softening and a duller or lighter color followed by flattening (Fig. 85.3).



Some IHs involute with little or no visible sequelae, whereas others (especially pedunculated or exophytic lesions) leave atrophic, fibrofatty, or telangiectatic residua in addition to scars at sites of previous ulceration (discussed later; Fig. 85.4).



Divided into three clinical subtypes based on the depth of cutaneous involvement:


Superficial lesions (~50%; upper dermis) are bright red with a finely lobulated surface (‘strawberry’ hemangiomas) during proliferation, changing to a mixture of purple-red and gray during involution (Fig. 85.5).



Deep lesions (~15%; lower dermis and subcutis) present as warm, ill-defined, light blue-purple, rubbery nodules or masses (Fig. 85.6); often become evident later and proliferate ~1 month longer than superficial lesions.



Mixed lesions (~35%) have superficial and deep components – e.g. a well-defined red plaque overlying a poorly circumscribed bluish nodule.


IHs have two major distribution patterns:


Focal lesions arise from a localized nidus (see Fig. 85.5A).


Segmental lesions cover a broader area or developmental unit and are more likely to be associated with regional extracutaneous abnormalities – e.g. PHACE(S) and LUMBAR syndromes (discussed later) (Fig. 85.7).



DDx: precursors and early lesions: capillary malformation, telangiectasias; well-developed lesions: pyogenic granuloma, other vascular tumors, venous/lymphatic malformation, and rare entities such as nasal glioma and soft tissue sarcomas (see Table 53.1); multiple lesions: glomuvenous malformations, blue rubber bleb nevus syndrome, multifocal lymphangioendotheliomatosis with thrombocytopenia, ‘blueberry muffin baby’ (see Chapter 99).


When an atypical clinical appearance or natural history leads to consideration of entities in the DDx of an IH, additional evaluation may include ultrasonography, other imaging, and biopsy.



Complications of IHs



Ulceration: occurs in ~10% of lesions, especially those on the lip and in the anogenital region or other skin folds, at a median age of 4 months (Fig. 85.8); whitish discoloration of an IH in an infant <3 months of age may signal impending ulceration; results in pain, a risk of infection (relatively uncommon), and eventual scarring (see Fig. 85.4A,B).



Disfigurement and interference with function due to the IH’s location and/or large size.


Periocular IH – risk of astigmatism if puts pressure on globe, amblyopia if obstructs the visual axis, and strabismus if affects orbital musculature (see Fig. 85.7A,B); requires ophthalmologic evaluation.


IH on nasal tip, columella, or lip (especially if crosses the vermilion border) – high risk of distortion of facial structures (as well as ulceration if on the lip) (Fig. 85.9).



IH on breast (in girls) – may affect underlying breast bud, so early surgery should be avoided.


Associated extracutaneous abnormalities.


IHs in ‘beard’ area of the lower face – often associated with airway IHs, which may present with noisy breathing or biphasic stridor; otolaryngologic evaluation is needed for infants with symptoms or when the IH is likely to be actively proliferating (i.e. <4–5 months of age) (Fig. 85.10).



Large facial IHs, particularly segmental lesions – risk of PHACE(S) syndrome: P, posterior fossa malformations; H, hemangioma; A, arterial abnormalities (cervical, cerebral); C, cardiac defects, especially coarctation of the aorta; E, eye anomalies; S, sternal defects and supraumbilical raphe (Table 85.2; Figs. 85.11 and 85.12; see Fig. 85.7A,B).





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Apr 22, 2016 | Posted by in Dermatology | Comments Off on Infantile Hemangiomas and Vascular Malformations

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