HEMANGIOMA

Hemangiomas, colloquially termed "strawberry marks", are the most common benign tumor of
infancy with an incidence of approximately 4–5% , 3:1 females Caucasic , and are caused by
endothelial cell proliferation. And sometime been associatedwith low birth weight and
premature infants .

Infantile hemangiomas present shortly after birth most often as well-demarcated, flat, and
erythematous red patches. At this stage, hemangiomas may be confused with other red
lesions of birth, but rapid proliferation and vertical growth will trigger the diagnosis

Hemangiomas follow a predictable course with three distinct developmental phases:

proliferation, quiescence, and involution. In most hemangiomas, eighty percent of
proliferation occurs by three months of life but may last longer [8]. During proliferation, rapid
growth can lead to exhaustion of blood supply with resulting ischemia, necrosis, ulceration,
and bleeding. Following proliferation, hemangiomas enter a slower or no growth phase,
known as quiescence. This phase typically lasts from 9 to 12 months of age. The final and
unique phase of the hemangioma lifecycle is involution. This phase is marked by graying of the
overlying skin and shrinking of the deeper component

Hemangiomas can be superficial, deep, or compound.

The superficial hemangioma is red and nodular with no subcutaneous component.

A deep hemangioma presents as a protrusion with an overlying bluish tint or telangectasia.

Compound hemangiomas have both deep and superficial components (Figure 1(b)). This new
nomenclature helps eliminate confusing older terms

Old nomenclatura New nomenclature

Strawberry or capillary Superficial hemangioma

hemangioma

Cavernous hemangioma Deep hemangioma

Capillary cavernous hemangioma Compound hemangioma

Another subclassification for hemangiomas is focal versus segmental disease. Focal

hemangiomas are localized, unilocular lesions which adhere to the phases of growth and
involution. Multifocal hemangiomatosis also exists, and infants with greater than 5 lesions
should undergo workup to rule out visceral involvement. Segmental hemangiomas are more
diffuse plaquelike and can lead to untoward functional and aesthetic outcomes. The limb and
face are common locations for disease (Figure 2). Head and neck lesions frequently coincide
with the distribution of the trigeminal nerve. A beard-like distribution is associated with a
subglottic hemangioma 60% of the time

La mayor parte del encogimiento de un hemangioma infantil ocurre cuando el niño tiene entre
3 1/2 y 4 años. Casi la mitad de todos los niños con un hemangioma infantil pueden quedar
con tejido cicatricial o vasos sanguíneos adicionales en la piel

Se hace una distinción primaria entre un tumor vascular, que crece por hiperplasia celular, y
una malformación vascular, que representa un defecto localizado en la morfogénesis vascular.
Debido a las diferencias biológicas

Congenital hemangiomas are visible at birth whereas infantile hemangiomas appear later. This
activity describes the evaluation and management of hemangiomas and highlights the role of
the interprofessional team in caring for patients with hemangiomas.

Se plantea diagnóstico diferencial con malformaciones capilares, hemangiomas congénitos no

involutivos (NICH) y otras anomalías vasculares

Se demostra mediante el estudio histológico y la expresión de GLUT-1 que los HA son

verdaderos hemangiomas5

Despite being a “benign” tumor with eventual involution and possible resolution, IHs can
develop complications and require medical intervention. One study reports 24% of patients
presented with a complication and 38% of patients required treatment.7 Different morbidities
are associated with proliferative phase versus involuting/involuted phase IH. Therefore, the
treatment modalities may be different based on the morbidity that has developed. However,
the timing of intervention may or may not correlate with the time that a morbidity has
developed. For example, an urgent ulceration or bleeding of a proliferating IH will require
intervention at the time of development of the complication. By contrast, a large IH that
results in a residual contour deformity can be treated more electively, with less urgency to
immediate intervention.

El HA estaba presente al nacer en el 71% y apareció durante las dos primeras semanas de vida
en el 29%.

with a proliferative phase in the first year of life, more intense during the
first three months, followed by the involution phase
During the proliferative phase, blood vessels are disorganized and
composed of immature non-permeable ECs, possibly due to alpha-smooth
muscle actin (α-SMA). In the involution phase of the tumor, the blood
vessels mature and grow in size, but are reduced in number. The vascular
tissue is replaced with fat, connective tissue, and fibroblasts.
IH can be harmless, which is why conservative measures are
recommended, but in rare cases when complications such as obstruction,
bleeding, ulceration, or superinfection occur (Figures (Figures99 and
and10),10), therapeutic intervention is required [7, 14, 42]. Roughly for
every 10 cm2 increase in lesion size, there is a 4% increase in the need for
treatment, and 5% increased chances for complication [42]. In the wake of
the novel coronavirus disease 2019 (COVID-19) pandemic, the
Hemangioma Investigator Group (HIG) released a classification of IH based
on size and location useful in evaluating the probability for complications

Highest High Medium Low

▪ >5 cm or ▪ Large IH on ▪ Localizes perineal IH ▪ IH <2 cm on trunk

facial/scalp; trunk/extremities without ulceration; of extremities in areas
easily covered by
▪ Large or segmental ▪ Facial IH ≥2 cm (>1 ▪ Trunk of extremity IH clothing;
lumbosacral or cm if ≤3 months of >2 cm especially in
perineal; age); growth phase or if abrupt ▪ IH on trunk of
transition from normal to extremities >2 cm if
▪ Multifocal IHs ▪ Nasal tip or lip IH affected skin. gradual transition
(≥5) and abdominal even if <1 cm; from normal to
hemangiomas (US); affected skin.
▪ Oral;
▪ Periocular IH with
potential impact on ▪ Neck or scalp IH >2
visual axis. cm during growth
phase;

▪ Breast;

▪ Ulcerated
hemangioma.

Una de las principales características de los HI está relacionada con su peculiar ciclo de
crecimiento. En líneas generales, se considera que estos tumores están ausentes o son muy
sutiles al nacimiento (fase inicial), comienzan a crecer durante las primeras semanas-meses de
vida y esta fase proliferativa se extiende, en promedio, hasta alrededor del año de vida (con un
crecimiento acelerado entre los 3 y los 5 meses de vida, uno más lento entre los 6 y los 9
meses, y un período de meseta donde alcanzan su tamaño y distribución definitivos que
transcurre entre los 9 y los 12 meses de edad). Luego comienzan a retrogradar lentamente
(fase posproliferativa), a lo largo de los 10 años siguientes. La tasa de involución anual
descripta es del 10%.3 En el 80% de los casos se trata

Risk stratification depending on IH morphological characteristics, number

of lesions, location [3]
Based on the factors involved in the pathogenesis of IH, over time various
targeted therapies have been used: corticotherapy, interferon (IFN),
sclerosing agents, β-blocker therapy and laser therapy.
Corticosteroid therapy

Corticosteroid (CS) therapy has been widely used in the treatment of IH.
Systemic therapy using Prednisolone 2–3 mg/kg/day every morning had a
33% success rate, with only one third of lesions regressing, one-third
remaining stable, and the last third showing no response [26, 39, 43].
Growth delay, skin hypopigmentation, hypertrichosis, Cushingoid
appearance, behavior alteration, irritability, glaucoma, cataract are all
complications associated with systemic administration of CSs [41, 43,44].
Intralesional or topical regimes are used for

El síndrome PHACE se describió por primera vez en 2005 y consiste en malformaciones

cerebrales de la fosa posterior, hemangioma facial segmentario grande, anomalías arteriales,
anomalías cardíacas y anomalías oculares.

Cuando un paciente presenta un HI facial segmentario grande, existe un riesgo de ~20 % de

síndrome PHACE.41 En estos pacientes pueden estar indicadas intervenciones no relacionadas
con los hemangiomas. Por ejemplo, la cirugía cardíaca puede estar indicada si hay una
coartación de la aorta42 o una encefaloduroarteriosinangiosis, donde la arteria temporal
superficial se injerta directamente en la piamadre del SNC, lo que puede estar indicado para
proporcionar una perfusión adecuada al cerebro.