Case Studies Series: Multiple Hemangiomas

This child was 3 months old when she presented to the emergency room with evidence of increased pressure inside of her head.  She had rapid head growth and the soft spot at the top of her head (anterior fontanelle) was tense.  Interestingly, she had multiple skin lesions that were consistent with cutaneous hemangiomas.

Based on her clinical condition, she underwent a CT scan of the brain, followed by an MRI of the brain. We could not do a contrast MRI since she was allergic to the contrast material for the MRI but we did a contrast CT and MRI without contrast. For a lot of tumors, it’s easier to see the tumors with the contrast dye but we had to proceed without it.  

We were not sure what these lesions were but they were causing significant irritation to the brain (as evidenced by the edema) and pressure on the brain which caused the hydrocephalus.  My recommendation to the parents was to start out by treating the most symptomatic lesion.  Since the pressure from the left cerebellum was causing the hydrocephalus, we chose that one for the initial surgery.

The next day she underwent a left posterior fossa craniotomy and resection of the tumor.  The pathological assessment of that lesion confirmed the diagnosis of an intracranial hemangioma.  This meant she had multiple infantile hemangiomas scattered throughout her brain (about 9 lesions total) in addition to multiple cutaneous (skin) lesions. This all was in addition to a lesion in the liver. 

By resecting the cerebellar lesion, we eliminated the cerebellar swelling and the obstruction to the flow of her spinal fluid.  Her hydrocephalus resolved.

Hemangiomas are benign vascular tumors mostly found in the skin.  They can be managed conservatively as they will often involute, or decrease in size, on their own over the first year of life.  If they don’t involute, then there are medical treatments (steroids and propranolol) that eradicate the lesions. They rarely need surgical resection.

We debated how to proceed in our tumor board.  The decision was to treat her lesions conservatively except for the left frontal lesion since it was causing so much edema. We were worried that it would damage the brain just because of the edema so she had a second surgery and the left frontal lesion was removed. 

She did very well after both surgeries.

Her remaining brain lesions and the lesions in her abdominal organs were treated medically with steroids and propranolol.  Some of her skin lesions were removed by plastic surgery.

She is now almost 4 years from her initial diagnosis and she has no intracranial disease.  She still has a small residual lesion in her liver.

Developmentally and neurologically, she is normal.  Actually, her development may even be slightly advanced.

We were able to tailor her treatment to minimize the damage to her brain either from excessive surgery or from allowing the swelling to persist too long.

She is a happy, healthy, and a very cute 4 year-old.

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