Periocular Hemangioma of Childhood

By Paul T. Finger, MD

Description

periocular-hemangioma-full;size$350,248.ImageHandler
A large periocular hemangioma involves both the upper and lower eyelids. The eye is able to open but there is an astigmatism induced by a mass effect from the lower eye lid.

Children can either be born with or develop reddish “strawberry” colored tumors on or around their eyes. This is one of the most common tumors of infancy. It is 3 times as frequent in girls and can run in families.

Symptoms

Periocular hemangioma of childhood can be large, and commonly grow during the first year of life, but also tend to get smaller (involute) over the following 2 years.

Periocular hemangioma of childhood can extend into the orbit  (behind the eye) and push the eye forward (proptosis), make the eyes misaligned (strabismus), or can cause the eyelid to droop (ptosis).

Children with periocular hemangioma of childhood can have hemangiomas in other parts of their bodies, so a pediatric consultation is necessary. If the hemangiomas are multiple or on the jaw or neck, a pulmonary consultation is necessary to rule out upper respiratory tract involvement. Consider the PHACES syndrome (anomalies of the Posterior fossa, Hemangiomas, the Arteries, Cardiac, Eye, Sternum) which is more commonly seen in girls.

Diagnosis

Hemangioma can be diagnosed by ocular examination with magnetic resonance imaging (MRI). Rarely, a small biopsy may be required to confirm the clinical diagnosis.

Treatments

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Histopathology reveals multiple well differentiated vascular channels. No atypical cells are seen.

Since periocular hemangioma of childhood is a benign tumor (not a cancer), immediate treatment is often not necessary. In fact, after an initial growth phase, many of these tumors will get smaller by themselves. Most patients can be followed for evidence of spontaneous remission.

Treatment is urgently indicated if the periocular hemangioma of childhood is found to harm the proper development of vision in the affected eye in infants and young children (amblyopia), and for psychosocial reasons in older children and adults.

Let me explain Amblyopia: In order for an eye to achieve its best possible potential for vision, two things are necessary. First, proper images must be focused on the retina and second, the brain must receive those images. During the first 10 years of life, there is a process where images are collected and the brain learns to understand those images. If an eye is blocked by the tumor (or the eye lid), or if the eyes are not aligned, or if the eye is not able to focus images on the retina, the child’s eye-brain connection will not develop. That is, the child will not learn to see from that eye (a problem called amblyopia). Urgent treatment of periocular hemangioma of childhood can be necessary be to prevent amblyopia.

Periocular hemangioma of childhood has been treated with surgery, laser-surgery, radiation, and drugs (intralesional steroids and systemic beta-blockers). When possible, treatment of periocular hemangiomas of childhood involves injections of steroid into the tumor. In comparison to taking the medicine by mouth (PO) or by vein (IV), this approach has the advantage of putting the medicine right into the tumor. An acute effect, but does carry risk of tumor and orbital hemorrhage.

References

  1.  Haik B, Karcioglu Z, Gordon RA, Pechous BP. Capillary hemangioma (infantile periocular hemangioma). Survey of Ophthalmology 1994;38:399-426.
  2.  Kushner BJ. Hemangiomas. Archives of Ophthalmology 2001;118;835-836.

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Cavernous Hemangioma of the Orbit

By Paul T. Finger, MD

Description

Computed tomography (CT) – Coronal Section demonstrates displacement of the optic nerve (medially) by the orbital hemangioma.

Hemangioma is a benign tumor that is found to grow within the orbit. Most commonly located behind the eye globe, it can push the eye forward causing eye-bulging doctors call proptosis.

Symptoms

Cavernous hemangioma of the orbit is most commonly seen in middle-aged women. Most are found within the muscle cone, but can be found anywhere in the orbit.

These orbital tumors can indent the back of the eye causing choroidal folds, or push on the optic nerve causing damage (atrophy).

Rarely, the tumor can push the eye so far that the cornea cannot be covered by the eye lids. In these cases, corneal exposure problems (keratitis, superficial punctate keratopathy, ulceration, even perforation) can occur.

Diagnosis

Cavernous hemangioma of the orbit is usually a slow-growing tumor. If the tumor has not damaged the eye, cavernous hemangioma can be observed for growth prior to considering intervention. Should tumor growth occur, it will be measured by eye examinations including (but not limited to) visual acuity, color vision assessment, Hertel exophthalmometry (a measure for proptosis), as well as an evaluation for double vision (strabismus), corneal exposure, retinal damage, vascular damage, and optic neuropathy.

Treatment

Treatment of orbital hemangioma is indicated when there is evidence of growth, optic nerve compression, and corneal exposure (with secondary keratitis sicca), or evidence of vision loss.

The goal of orbitotomy for choroidal hemangioma should be complete removal of the tumor. This usually involves careful dissection of the tumor to protect the tumor’s capsule (as possible). Connecting vascular feeder vessels should be identified and cauterized. A lateral orbitotomy can be required to keep the large tumor intact.

A fundus photograph demonstrates choroidal folds induced by the choroidal hemangioma indenting the posterior eye-wall.
Histopathologic sections with elastin-stain also demonstrate the large loosely distributed vascular channels associated with orbital hemangioma. The tumor’s capsule is noted on the right.

Benign Eyelid Tumors – Capillary Hemangioma

By Paul T. Finger, MD

Description

These tumors typically grow and then spontaneously decrease in size.
These tumors typically grow and then spontaneously decrease in size.

Congenital “strawberry-spot” which usually will spontaneously get smaller “involute.”

Treatment is indicated for the prevention of amblyopia (loss of vision) and strabismus (misdirected eyes). These tumors naturally grow and then spontaneously decrease in size. Therefore, if the tumor is not harming the child, it can be observed untreated for spontaneous regression.

Symptoms

Capillary hemangiomas are typically found at birth. They will grow during the first decade (10 years) and most will shrink (involute). If they involve the eyelids, they can cover the eye and cause loss of vision (amblyopia). It is very important that children with capillary hemangiomas be seen by a pediatric ophthalmologist and eye cancer specialist immediately.

Diagnosis

Capillary hemangioma is diagnosed by clinical examination. It has a typical appearance and biopsy is rarely needed. It appears as a reddish tumor or mass beneath the skin. The eye care specialist will order a radiographic scan (MRI or CT) to see how deep the tumor extends into the orbit (around the eye). The child’s pediatrician should be advised and the child inspected for hemangiomas on other parts of the body.

Treatments

Observation for spontaneous resolution is commonly performed. If the tumor is blocking the eye and vision causing amblyopia, then it can be treated with an oral beta-blocker medication (e.g. propranolol) or injected with a steroid solution. This will shrink the tumor in an effort to uncover the eye. Surgery may be required to remove very large tumors. The child’s pediatric ophthalmologist may suggest that the other “good” eye be periodically covered with a patch to strengthen the tumor affected eye and prevent amblyopia-related vision loss.

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"Very well treated by Dr. Finger. He explained everything I needed to know about my issue with detail and attention, putting me at ease and giving me confidence to handle this problem for the rest of my life.”
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