Diagnosis and Treatment of Infantile Hemangioma from the Primary Care Paediatricians to the Specialist: A Narrative Review
Abstract
:1. Epidemiology
2. Risk Factors
3. Classifications of IH
4. Etiopathogenesis
5. Natural History
- Rapid Proliferative Phase (0–1 year):
- Early proliferative phase characterised by rapid growth. It occurs within the first 3–5 months, with the fastest growth velocity typically seen between weeks 5 and 8. By the end of this phase, IHs reach about 80% of their final size.
- Late proliferative phase, which is slower, usually completing by 9–12 months of age, though growth may continue up to 36 months, marking late growth.
Only 3% of IHs continue to grow beyond 9 months. Generally, superficial forms grow until the 5th month, while deep and segmental forms appear later and continue to grow for a more extended period (up to 18 months) [20]. Superficial IHs are more prone to residual scarring than deep ones, significantly if the lesion lightens within the first 3 months, which can be an early sign of ulceration. - Involution Phase (1–5 years): This period is characterised by a softening of the lesion and fading of its colour, starting from the centre, with a progressive reduction in volume and decreased vascularisation.
- Involuted Phase (5–10 years): In this phase, complete or near-complete regression occurs, sometimes leaving residual scarring such as loose skin, atrophy, telangiectasias, and/or fibro-fatty tissue [21].
6. Diagnosis
- (1)
- Life-threatening risks, such as obstructive subglottic haemangiomas and large haemangiomas causing cardiac or hepatic failure [25]. Obstructive subglottic haemangiomas can lead to significant airway obstruction (Figure 1), while large haemangiomas might contribute to cardiac failure or hepatic dysfunction due to high-output cardiac failure.
- (2)
- Functional impact risks, such as periorbital haemangiomas, which may impede complete eye-opening, especially during the peak proliferation phase (2–3 months of life), potentially leading to permanent amblyopia, astigmatism, strabismus, proptosis, and optic nerve compression. Nasal, labial, or laryngotracheal haemangiomas can obstruct airways, posing life-threatening risk—haemangiomas on joints, which may limit the mobility of the affected segment, among other functional impairments.
- (3)
- Aesthetic risks with psychological implications, as haemangiomas located on the face (Figure 2), including the glabella, nose, philtrum, chin, cheeks, and lips, which may result in permanent deformity; on the mammary gland in females; on the genitals in both males and females; and ulcerated haemangiomas that do not respond to local treatments, causing pain and subsequent permanent aesthetic damage.
- (4)
- Ulcerations, the most common complication of haemangiomas, particularly between the 4th and 8th months of life, occurring in 10–25% of patients referred to specialised centres. The areas at highest risk of ulceration include the lips, head and neck region, skin folds, and buttocks. Clinically, approximately 50% of haemangiomas in the perineal region and 30% of those on the lower lip are at risk of ulceration.
- (5)
- -
- Complications or potential risk of complications (ulcerations, visual deficits, feeding difficulties, laryngeal stridor).
- -
- Location in the midface and/or ears, mammary region (in females), or lumbosacral midline.
- -
- Size ≥ 4 cm (focal or segmental).
- -
- Number of haemangiomas ≥ 5.
- -
- the location of the haemangioma,
- -
- the size of the most extensive haemangioma,
- -
- the child’s current age,
- -
- the growth of the haemangioma in the past two weeks.
7. Treatment
- -
- Topical Timolol: Timolol maleate 0.5%, a nonselective beta-blocker, is a well-tolerated, safe, and effective treatment for thin superficial IHs. It is as effective as oral propranolol for treating superficial IHs, with fewer systemic adverse events and superior efficacy to topical corticosteroids. It should be applied directly to the lesion using a 0.5% eye drop solution at a dose of 1–2 drops twice daily or as a 0.5% timolol maleate gel (15-min applications, 3 times a day). Treatment should continue for 6–9 months [32,33].
- -
- Oral Propranolol: First recognised as effective in 2008, oral propranolol is now considered the first-choice treatment for IHs at risk of complications. The effective dose ranges from 2 to 3 mg/kg body weight per day, with a treatment duration of at least 6 months, continuing until the child is 12–18 months old. Although the initiation of propranolol is recommended during the proliferative phase, it can still lead to improvement when started after 9–12 months of age. The mechanism of action of propranolol, beta-blocker, is yet to be elucidated; however, theories include vasoconstriction, the inhibition of angiogenesis, the induction of apoptosis, the inhibition of nitric oxide production, and the reduction of renin levels and consequently of the renin–angiotensin axis.
- -
- Steroids: Considered a second-line treatment if there is no response to propranolol, but perhaps the first choice for patients with cardiovascular disease. The most commonly used drug is oral prednisolone, administered at a dose of 2–3 mg/kg/day for 12 weeks, followed by a gradual tapering until the child is 9–12 months old. Intralesional injections of triamcinolone and/or betamethasone (every 4–6 weeks) may be considered for bulky focal haemangiomas in the proliferative phase or in specific locations (e.g., parotid gland, lips) [37].
- -
- Additional systemic treatment options include captopril, sirolimus, and intravenous vincristine; however, their use is limited in infants due to the risk of adverse reactions and side effects.
- -
- Laser or Traditional Surgery: Residual lesions after propranolol therapy, usually telangiectatic, may require surgical or laser treatment at specialised centres with expertise in haemangioma surgery. Most studies report the use of pulsed dye laser or long-pulse Nd laser. The combination of laser with systemic beta-blockade has demonstrated superiority over monotherapy [24].
8. Operative Algorithm for Propranolol Therapy
9. Hub and Spoke: Management of IH from Primary Care to Specialist Centres
- -
- Informational Leaflet: An informational flyer for primary care paediatricians summarises patient management by the multidisciplinary team, including the appropriate age for referral and key treatment indications. The leaflet also includes the IHReS scale to aid clinical decision making and outlines diagnostic tests and patient follow-up procedures.
- -
- Annual Courses/Meetings: Organised through significant professional associations to update paediatricians on new guidelines and therapeutic approaches.
- -
- Electronic Medical Records Integration: Adding a specific section in the database system used by primary care paediatricians. When the term “infantile haemangioma” is entered, an automatic link to the IHReS scale is provided, allowing paediatricians to complete, save, print, or email the form to the specialist centre.
- -
- Website Updates: The emangioma.net website has been updated with information for both parents and clinicians, including a map of referral centres with contact details.
10. Direct Referral Pathway for Patients with IH to a Specialist Referral Centre
11. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
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Bellinato, F.; Marocchi, M.; Pecoraro, L.; Zaffanello, M.; Del Giglio, M.; Girolomoni, G.; Piacentini, G.; Rigotti, E. Diagnosis and Treatment of Infantile Hemangioma from the Primary Care Paediatricians to the Specialist: A Narrative Review. Children 2024, 11, 1397. https://doi.org/10.3390/children11111397
Bellinato F, Marocchi M, Pecoraro L, Zaffanello M, Del Giglio M, Girolomoni G, Piacentini G, Rigotti E. Diagnosis and Treatment of Infantile Hemangioma from the Primary Care Paediatricians to the Specialist: A Narrative Review. Children. 2024; 11(11):1397. https://doi.org/10.3390/children11111397
Chicago/Turabian StyleBellinato, Francesco, Maria Marocchi, Luca Pecoraro, Marco Zaffanello, Micol Del Giglio, Giampiero Girolomoni, Giorgio Piacentini, and Erika Rigotti. 2024. "Diagnosis and Treatment of Infantile Hemangioma from the Primary Care Paediatricians to the Specialist: A Narrative Review" Children 11, no. 11: 1397. https://doi.org/10.3390/children11111397
APA StyleBellinato, F., Marocchi, M., Pecoraro, L., Zaffanello, M., Del Giglio, M., Girolomoni, G., Piacentini, G., & Rigotti, E. (2024). Diagnosis and Treatment of Infantile Hemangioma from the Primary Care Paediatricians to the Specialist: A Narrative Review. Children, 11(11), 1397. https://doi.org/10.3390/children11111397