Episode 78: Infantile Hemangioma.
Dr Shelat discusses with Dr Schlaerth and Dr Arreaza the definition, pathophysiology, diagnosis and treatment of infantile hemangioma.
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Infantile Hemangioma.
By Tejal Shelat, MD (Lady Hardinge Medical College).
Discussed with Katherine Schlaerth, MD; and Hector Arreaza, MD.
This is Rio Bravo qWeek, your weekly dose of knowledge brought to you by the Rio Bravo Family Medicine Residency Program from Bakersfield, California. Our program is affiliated with UCLA, and it’s sponsored by Clinica Sierra Vista, Let Us Be Your Healthcare Home.
What is infantile hemangioma?
Infantile hemangioma is vascular overgrowth that leads to tangled blood vessels that appear as a reddish plaque on the skin as early as days to weeks after birth.
It is the most common benign vascular tumor in infants, with a prevalence of 4-5% in mature neonates and is about 2.5 times more common in female (ratio female:male is 3:1) and Caucasian children.
Risk factors:
There are several risk factors, including prematurity, low birth weight less than 1000g, family history of infantile hemangioma, placental anomalies, and eclampsia.
Progression of infantile hemangioma.
Hemangiomas typically undergo three phases:
First is the proliferation phase that occurs between 0 to 6 months of age, with about 80% growing to their final size by age 3 months. During this time there is growth of a bright red, soft, raised, non-blanching plaque that is visible on the skin. This occurs to due proliferation of rapidly dividing endothelial cells in the blood vessels.
This is followed by a plateau phase.
Next is the involution phase, that occurs after 6 months of age. The lesion/s now turn deep red or violet and spontaneously begin to regress in size.
Pathogenesis.
Several hypotheses have been described to explain the reason behind the occurrence of hemangiomas. We now know that they occur due to dysregulation in angiogenesis and vasculogenesis.
The most likely trigger is thought to be hypoxia, which induces transcription of the Vascular Endothelial Growth Factor (VEGF) gene, leading to overexpression of angiogenic factors such as VEGF. This leads to differentiation of endothelial cells, influx of other cells such as mast cells, myeloid cells and also tissue inhibitors of metalloproteinases (TIMPs).
Regression.
The mast cells produce interferon and transforming growth factor, which, along with the TIMPs that we just talked about all work together to halt the proliferation of endothelial cells. The endothelial cells then become senescent and that leads to passive involution of the hemangioma.
Diagnosis.
The diagnosis of infantile hemangiomas is clinical. If you are not familiar with how a hemangioma looks, search in your favorite dermatology atlas.
A hemangioma may be red if it involves the papillary dermis (called superficial strawberry hemangiomas), but they can also be purple, blue, or colorless if they involve the reticular dermis or subcutaneous fat (called deep, cavernous hemangiomas).
Early white discoloration of infantile hemangioma may be an early sign of imminent ulceration.
Additional workup.
Further investigation is also required in specific situations:
If there are 5 or more cutaneous lesions, we would need a liver ultrasound to rule out involvement of the liver
For facial or segmental involvement, echocardiogram and MRI of the head are recommended to rule out posterior fossa malformations, hemangioma (usually localized on the face), arterial anomalies, cardiovascular anomalies, eye anomalies, sternal clefting and/or supraumbilical raphe
PHACE Syndrome: posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities. By definition, PHACE is diagnosed when there is at least one hemangioma >5 cm on head/scalp PLUS one major or two minor criteria OR hemangioma of any size on neck, upper trunk and proximal upper extremity plus two major criteria.
Major criteria include arterial anomalies such as anomaly of major cerebral or cervical arteries, retinal vascular anomalies, sternal defect.
Minor criteria include cerebral artery aneurysm, ventricular septal defect, etc.
Laryngoscopy should be done if there is cervicofacial involvement, i.e., beard distribution
Spinal ultrasound should be performed if the hemangioma is in the lumbosacral region
Management
-Most hemangiomas will not require treatment, and most need observation only.
-When treatment is needed, treatment is usually medical depending on severity, location, and extension of hemangioma/s, you may decide to go with topical or systemic therapy.
-Topical therapies include beta blockers (propranolol 1% applied TID for 1 year), corticosteroids, and imiquimod, but data on efficacy is limited.
-Systemic therapies: Beta blocker therapy (with propranolol by mouth) is indicated when there is concern for ulceration or scarring in large, facial, segmental and or rapidly growing hemangiomas, for visual impairment in periorbital involvement, high output heart failure in hepatic involvement and airway obstruction in subglottic involvement. The dose of propranolol is 1mg/kg to 3mg/kg in the form of an oral solution, depending on the response to therapy and weight of the child. Initiation of therapy may require hospital admission to watch for side effects to beta blockers.
-Second-line treatments include systemic corticosteroids
-Surgical intervention is rarely needed, and it´s usually avoided because surgical scars may be worse than the resulting lesion after spontaneous regression.
Prognosis.
The prognosis is very good for most uncomplicated hemangiomas, with about 50% undergoing complete involution by age 5 years and about 90% by age 9 years. Permanent cutaneous residua are seen for hemangiomas that involute slowly, after 6 years of age and hemangiomas involving the eyelid, nasal tip, ear and lip. Functional impairment or obstruction may occur when the hemangioma is located near natural orifices and/or in the head and neck area. In these cases, surgical intervention may be needed.
Conclusion.
You may find hemangiomas during routine physical exam of a newborn. It is important to remember the natural progression of uncomplicated hemangiomas. Make sure to educate parents about concerning features and how to determine if treatment is needed. In most cases, when treatment is needed, a dermatology evaluation is needed.
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[Music to end: JERUSALEMA]
Now we conclude our episode number 78 “Infantile Hemangioma.” We learned about the natural progression of most hemangiomas. They grow for up to 3 years, then remain unchanged until around 6 years of age when they gradually regress without treatment. In most cases, monitoring is all what’s needed. However, it’s important to identify the hemangiomas with concerning features that require additional work up or early treatment. Treatment is mainly medical. Surgery is rarely recommended or required. Even without trying, every night you go to bed being a little wiser.
Thanks for listening to Rio Bravo qWeek. If you have any feedback about this podcast, contact us by email RioBravoqWeek@clinicasierravista.org, or visit our website riobravofmrp.org/qweek. This podcast was created with educational purposes only. Visit your primary care physician for additional medical advice. This week we thank Hector Arreaza, Tejal Shelat. Audio edition: Suraj Amrutia. See you next week!
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References:
Léauté-Labrèze, C., Harper, J. I., & Hoeger, P. H. (2017). Infantile haemangioma. The Lancet, 390(10089), 85-94.
Kowalska, M., Dębek, W., & Matuszczak, E. (2021). Infantile Hemangiomas: An Update on Pathogenesis and Treatment. Journal of clinical medicine, 10(20), 4631.
Metry D.W. Infantile hemangiomas: Epidemiology, pathogenesis, clinical features, and complications. UpToDate. Accessed December 5, 2021. https://www.uptodate.com/contents/infantile-hemangiomas-epidemiology-pathogenesis-clinical-features-and-complications#H22.
Antaya R.J. Infantile Hemangioma. Medscape. Accessed December 5, 2021. https://emedicine.medscape.com/article/1083849-overview#a1.
