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Infantile hemangiomas (IH) show great heterogeneity in size, morphology, growth, residua remaining after involution, and in degree of response to therapy.
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Propranolol is now preferred first-line therapy, given that its efficacy, tolerability, and safety are superior to that of oral corticosteroids.
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The period of most rapid proliferation is complete by 8 weeks after birth, suggesting that referral to specialists should occur early, within the first month of life for concerning IH.
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More infants are
Diagnosis and Management of Infantile Hemangiomas
Section snippets
Key points
Diagnosis
Proper diagnosis of IH hinges on a solid understanding of the classification structure for vascular anomalies, and the most comprehensive and widely accepted classification schema is put forth by the International Society for the Study of Vascular Anomalies (ISSVA). The classification rests primarily on distinguishing vascular tumors, of which IH are the most common, from vascular malformations (Fig. 1, Table 1). In all but a few cases, the diagnosis of IH can be determined by good history
Epidemiology
The incidence of IH has remained unclear, because they are not often present at the time of hospital discharge after birth (when most registries for birth defects are completed.) Current evidence from a prospective study that followed nearly 600 pregnant women and their infants until the infants were 9 months of age suggests an overall incidence of IH of 4.5%, and up to 9.8% in premature infants.7 Although the cause of IH is not yet fully known, there are several important risk factors for
Natural history
The growth cycle of IH is unique among vascular tumors and malformations, showing proliferation and preprogrammed involution. IH start with a nascent phase as either an area of pallor, a telangiectatic patch, or something mistaken for an ecchymosis or birth trauma.16 They may present anywhere on the body surface but are present most commonly on the head and neck, a finding which is also true of vascular malformations.17 Typically, most begin to proliferate to a noticeable degree at between 2
Complications
Most IH follow a benign course without the need for intervention aside from anticipatory guidance, but evidence shows that approximately 12% are complex and warrant referral for specialist evaluation; of those seen at tertiary care centers, nearly a quarter developed complications, ranging from ulceration to obstruction of the eye, airway, or auditory canal to cardiac compromise, in a study based on a cohort of more than 1000 infants.9 The same study documented that segmental IH are 11 times
Multifocal IH
Infants can present with multiple, often small, focal IH; clinically, some infants present with monomorphic small superficial His, sometimes numbering in the hundreds, whereas others present with multiple focal IH of various sizes and appearance. This phenomenon has been referred to as benign neonatal hemangiomatosis when it occurs without visceral involvement and as diffuse or disseminated neonatal hemangiomatosis (DNH) when it is accompanied by symptomatic IH internally, in the liver,
Complications associated with segmental IH
PHACE (OMIM 606519), a neurocutaneous syndrome of skin, brain, eye, and ventral body anomalies, is the most well-documented segmental IH syndrome occurring in the setting of segmental facial IH (Fig. 12), although a few cases have been reported with nonfacial IH and systemic complications relevant to PHACE.40, 41, 42, 43 Diagnostic criteria were published in 2009 and help to risk stratify patients into definite, possible, or probable PHACE syndrome, based on organ involvement at presentation.44
Therapy
Although there have been impressive and fundamental changes in our understanding of the heterogeneous presentations and pathogenesis of IH in the last decade, clearly the most dramatic changes have occurred in the realm of therapy, with the complete shift toward β-blockers as the standard of care. An important simultaneous but perhaps less obvious effect of the use of propranolol, which is widely accepted to be a safer and more well-tolerated drug than oral corticosteroids (OCS), is that
Summary
Improved insight into the natural history, associated complications, and response to propranolol has helped to advance IH research and practice. In many cases, the decision to treat is made obvious by associated complications. For most patients whose indication for therapy is risk of disfigurement, it is a fundamentally important concept to decide at which stage within the growth cycle of the IH to intervene with close monitoring, topical, systemic, adjunctive (laser or intralesional) therapy,
References (70)
- et al.
A prospective study of cutaneous findings in newborns in the United States: correlation with race, ethnicity, and gestational status using updated classification and nomenclature
J Pediatr
(2012) - et al.
Prospective study of infantile hemangiomas: demographic, prenatal, and perinatal characteristics
J Pediatr
(2007) - et al.
Approach to the patient with an infantile hemangioma
Dermatol Clin
(2013) - et al.
Diffuse neonatal hemangiomatosis: an evidence-based review of case reports in the literature
J Am Acad Dermatol
(2012) - et al.
Multifocal lymphangioendotheliomatosis with thrombocytopenia
J Am Acad Dermatol
(2006) - et al.
Multifocal lymphangioendotheliomatosis with thrombocytopenia: phenotypic variant and course with propranolol, corticosteroids, and aminocaproic acid
J Am Acad Dermatol
(2012) - et al.
Efficacy of propranolol in hepatic infantile hemangiomas with diffuse neonatal hemangiomatosis
J Pediatr
(2010) - et al.
Lessons from a liver hemangioma registry: subtype classification
J Pediatr Surg
(2012) - et al.
Congenital cardiac, aortic arch, and vascular bed anomalies in PHACE syndrome (from the International PHACE Syndrome Registry)
Am J Cardiol
(2013) - et al.
PHACE: a neurocutaneous syndrome with important ophthalmologic implications: case report and literature review
Ophthalmology
(1999)
LUMBAR: association between cutaneous infantile hemangiomas of the lower body and regional congenital anomalies
J Pediatr
Treatment of infantile haemangiomas with atenolol: comparison with a historical propranolol group
J Plast Reconstr Aesthet Surg
Atenolol: a promising alternative to propranolol for the treatment of hemangiomas
J Am Acad Dermatol
Ulcerated hemangiomas: clinical characteristics and response to therapy
J Am Acad Dermatol
Efficacy of pulse dye laser therapy for the treatment of ulcerated haemangiomas: a review of 78 patients
Br J Plast Surg
Propranolol, a very promising treatment for ulceration in infantile hemangiomas: a study of 20 cases with matched historical controls
J Am Acad Dermatol
Propranolol: useful therapeutic agent for the treatment of ulcerated infantile hemangiomas
J Pediatr Surg
Propranolol for treatment of ulcerated infantile hemangiomas
J Am Acad Dermatol
Infantile hemangiomas: how common are they? A systematic review of the medical literature
Pediatr Dermatol
Propranolol for severe hemangiomas of infancy
N Engl J Med
Initiation and use of propranolol for infantile hemangioma: report of a consensus conference
Pediatrics
Timolol maleate 0.5% or 0.1% gel-forming solution for infantile hemangiomas: a retrospective, multicenter, cohort study
Pediatr Dermatol
RCT of timolol maleate gel for superficial infantile hemangiomas in 5- to 24-week-olds
Pediatrics
A controlled study of topical 0.25% timolol maleate gel for the treatment of cutaneous infantile capillary hemangiomas
Ophthal Plast Reconstr Surg
Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment
Pediatrics
Infantile hemangiomas: an emerging health issue linked to an increased rate of low birth weight infants
J Pediatr
Strawberry hemangioma in preterm infants
Pediatr Dermatol
Demographic and clinical characteristics and risk factors for infantile hemangioma: a Chinese case-control study
Arch Dermatol
Hemangioma in the newborn: increased incidence after chorionic villus sampling
Prenat Diagn
Maternal and perinatal risk factors for infantile hemangioma: a case-control study
Pediatr Dermatol
Familial clustering of hemangiomas
Arch Otolaryngol Head Neck Surg
Early growth of infantile hemangiomas: what parents’ photographs tell us
Pediatrics
Update on pediatric extracranial vascular anomalies of the head and neck
Childs Nerv Syst
Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development
Pediatrics
The nonrandom distribution of facial hemangiomas
Arch Dermatol
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2022, Journal of Pharmacological SciencesCitation Excerpt :IH tumors occurring within the first week of life grow rapidly over the first 3–6 IH tumors are often untreated.1,2 Most IHs occur in the periphery of the head and neck areas,3 which can seriously affect a child's appearance and psychology.4 Even when associated vital, functional, and cosmetic problems are mild, parents are often concerned about the cosmetic aspects because most IHs form on the body surface, and thus, the parents request treatment for IH.4
Infantile hemangiomas: risk factors for complications, recurrence and unaesthetic sequelae
2022, Anais Brasileiros de DermatologiaCitation Excerpt :Morphologically, IH are classified as superficial, deep, or mixed. Regarding their extension, they can be focal, segmental, or multifocal.1–3,6 The focal subtype is the most common, while the segmental subtypes – which occupy a subunit of the body – are often associated with complications.3,9–11
Management of High-Flow Vascular Anomalies
2020, Image-Guided Interventions: Expert Radiology Series, Third EditionCost-effectiveness of treating infantile haemangioma with propranolol in an outpatient setting
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Disclosure: Advisory Board Member, Pierre Fabre.