This videoconference can be viewed in its entirety here:
http://insidekp.kp.org/ncal/tpmgphysicianed/media/video/pediatric_hemangiomas/index.html
Also, one may wish to review this newsletter's previous article on the use of Propranolol for Infantile Hemangiomas.
Some important highlights of the videoconference include:
- Infantile hemangiomas grow most rapidly 2 to 6 weeks of life. Early referral to a pediatric dermatologist is important.
- Most Infantile Hemangiomas are just observed closely. However, hemangiomas that could lead to disfigurement, functional impairment or life threatening complications are treated. Topical and systemic Propranolol are now first line therapies.
- The key window for optimal intervention is 2 to 4 weeks of age. Pediatricians are encouraged to take a photo of their patient's lesion and upload it to the Health Connect chart. The Pediatric Dermatologist can view this photo prior to referral.
- All children with hemangiomas on the face should be referred for early evaluation.
- In general, 16% of all hemangiomas will ulcerate. Locations at risk for ulceration include the perioral area, flexion areas such as the elbow and groin, and areas of friction (such as the diaper region).
- Ulcerations tend to occur at 4 to 6 months of age. Early whitening of the hemangioma may be a precursor to ulceration.
- Hemangiomas in the diaper area are exposed to friction, urine and feces. These lesions should be generously lubricated with emollients to avoid abrasions. In addition, diapers should be changed frequently. Lesions in this area are painful and can ulcerate readily and should be referred for early evaluation.
- Pain management can include topical emollients, oral Tylenol and a small amount of 5% Lidocaine gel (max 5 times per day). Lidocaine gel will relieve pain for only a short while, but can be useful on lip hemangiomas for feeding, and lesions in the groin during bathing. Covering lesions with xeroform gauze will also help control pain.
- Eyelid hemangiomas can cause functional impairment leading to amblyopia and should be watched carefully.
- Babies who have more than 5 hemangiomas anywhere on their body have an increased risk of liver involvement. These patients should have an ultrasound with doppler flow of the liver.
- If liver hemangiomas are seen ultrasound, a referral to pediatric dermatology for possible systemic propranolol treatment is indicated.
Segmental Hemangiomas, which involve more than one entire segment of the face or body, pose high risks of complications.
Segmental hemangiomas tend to grow more rapidly and for a longer period of time than do simple hemangiomas. Approximately 30% of segmental hemangiomas are associated with PHACE syndrome. PHACE syndrome includes one or all of the following:
- Posterior fossa malformations, usually present at birth
- Hemangioma that covers a large area, generally on the face, neck or head and extends more than 5 cm (also called "segmental hemangiomas").
- Cerbral artery lesions
- Congenital cardiac defects, most commonly coarctation of the aorta
- Eye abnormalities
Infants with a "beard" distribution hemangioma on the face have increased risks of airway hemangiomas. These infants will need an ENT evaluation and appropriate imaging. Families should be trained to recognize symptoms of airway obstruction such as stridor, respiratory distress, and swallowing difficulties.
The treatment of Infantile Hemangiomas has changed dramatically over the past few years. In 2008, propranolol was identified as a medication that can prevent growth of the lesions as well as promote their involution. Timolol, an ophthalmic preparation of propranolol, is used topically for small and superficial hemangiomas anywhere on the body.
Oral propranolol, used for deeper and more serious lesions, can cause cardiovascular side effects such as bradycardia and hypotension. Small infants receiving systemic propranolol are also at risk for hypoglycemia.
Differential Diagnoses for Infantile Hemangiomas:
Port wine Stain > flat telengatatic vessels occupying a trigeminal nerve distribution (dermatomes)
Venous malformation > abnormally formed vessels that will not proliferate rapidly, yet grow over time
Congenital tumors > rare hemangiomas, fully formed at birth
Editors Commentary:
While most infantile hemangiomas require no intervention, those infants with lesions on the face, groin, or whose lesions are greater than 5 cm in size should all be referred early to a pediatric dermatologist. The pediatrician should anticipate those at risk of ulceration and treat with emollient skin barriers. Photos of the hemangiomas can be taken and uploaded into health connect at various visits, documenting their progression.
Any facial segmental hemangiomas greater than 5 cm and/or in the beard distribution qualify for a PHACES workup.
The Children's Hospital of Wisconsin's website describes segmental hemangioma lesions and PHACE Syndrome: https://www.chw.org/display/PPF/DocID/28483/router.asp
Patients can be referred to any of the following Pediatric Dermatologists:
Dr. Deborah Goddard at San Jose
Dr. Pristine Lee at San Rafael
Dr. Linda Beets-Shay at Oakland
Dr. Glenda Swetman at Pleasanton / Walnut Creek
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