-
What
is capillary hemangioma?
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How is it
acquired? What is its natural course?
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Are there any complications associated with this condition?
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How does the tumor look? Are there special investigations necessary for its
diagnosis?
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What is the most appropriate treatment? Are there any side effects to watch
out for during treatment?
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How do I get
more information?
What is
capillary hemangioma?
Capillary hemangioma or “strawberry nevus”, an
abnormal growth of vascular tissue, is the most common vascular ocular tumor in
children. It is more commonly seen in females with a ratio of 3:2, and its
incidence is higher in premature babies. There is no apparent familial
inheritance pattern.
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How is it
acquired? What is its natural course?
This condition is congenital. A recent study
suggested that capillary hemangioma could be a sequestered tissue of placental
origin that grows rapidly due to post-natal escape from the intra-uterine
factors, controlling placental growth.
Its natural course is unique. In one-third of
cases, the vascular lesion is present at birth. It is usually small during the
first few weeks of life. Then, it progressively increases in size over the next
3-6 months. This phase is followed by a period of stabilization and a period of
spontaneous regression by the age of 4-7 years. Thirty percent regress by 3
years old, 60% by 4 years, and 70% by 7 years.
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Are
there any complications associated with this condition?
Most of the lesions are benign, but amblyopia may
occur if the mass causes ptosis of the eyelid, blocking the visual axis. Even a
small amount of ptosis can cause significant amblyopia. The prevalence of
amblyopia ranges from 40-60% in orbital lesions. Bulky tumors often cause
distortion of the eyeball causing corneal astigmatism. The astigmatism may
resolve partially, particularly if the tumor regresses early. Prolonged
occlusion of the visual axis may also lead to myopia, and the resultant
inequality in the refractive error of the two eyes (anisometropia) may be
another contributory factor in the development of amblyopia. Secondary
strabismus may develop as a result of interruption of binocular vision.
Systemic complications of capillary hemangiomas are
rare. An example is the Kassabach-Merrittt syndrome, a blood clotting problem
due to consumption and entrapment of blood components within a large vascular
hemangioma, often located in the viscera. It usually responds to platelet
replacement and systemic corticosteroids. PHACE syndrome (posterior fossa
malformations, hemangiomas, arterial anomalies, coarctation of the aorta and
cardiac defects, and eye abnormalities) is not a complication of capillary
hemangioma. It is a rare condition that proves an association between large
facial capillary hemangiomas and cerebral and ocular malformations. “Morning
glory disc” is an eye abnormality associated with capillary hemangiomas. This
optic disc abnormality has visual implications.
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How does the tumor look? Are there special investigations necessary for its
diagnosis?
It is very important that an accurate diagnosis is
reached to plan treatment for this self-limiting condition. This will certainly
help in reassuring anxious parents of children with cosmetically obvious lesions
that no active treatment is necessary initially.
Clinical diagnosis is often enough in majority of
cases. The lesion is commonly found in the upper lid or orbit, and its
appearance depends on the depth of involvement. Superficial tumors have a red
lobulated strawberry appearance, which gave rise to the name “strawberry
nevus”. They may increase in size or become bluish with crying. Deeper tumors
appear bluish or may show no significant skin color change. The diagnosis can
be facilitated by everting the eyelid to visualize the vascularity of the
hemangioma. Approximately one-third of lesions is located deep in the orbit,
and may present with proptosis only, without any associated skin discoloration.
A helpful diagnostic sign is an increase in proptosis with crying. Occasionally
the proptosis may be severe enough to cause corneal exposure. In other cases,
the enormous growth may obliterate facial structures.
Neuroimaging such as CT scan and MRI are recommended
for cases that are difficult to diagnose based on clinical findings. These
investigations will document the extent of the tumor. MRI will delineate the
tumor better.
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What is the most appropriate treatment? Are there any side effects to watch out
for during treatment?
Treatment is conservative as much as possible, with
treatment of significant refractive error and amblyopia, while awaiting
spontaneous regression. Active treatment to reduce the size of the lesion is
indicated only if there is occlusion of the visual axis or if a posterior lesion
results in progressive proptosis with evidence of optic nerve compression,
corneal exposure and significant and progressive amblyopia brought about by
obscuration of vision or astigmatism. The list of choices for treatment is not
long.
Corticosteroid injection is the most effective
treatment for large hemangiomas that block the visual axis. There have been a
few isolated reports of visual loss secondary to retrograde embolization of the
central retinal artery during intralesional steroid injection but this can be
avoided if the volume of injection is limited to a minimum. Steroid injections
are most effective when done early in infants younger than 8 months of age.
Repeat injections can be given every 6 weeks; however, long-term local steroid
injections can cause hormonal imbalance and growth retardation.
Sight-threatening lesions which failed to respond to
intralesional steroid may be amenable to treatment with interferon. However, it
involves daily injections for several weeks and the response to this treatment
is slow.
Some advocate systemic corticosteroids, however,
they can cause significant side effects and do not deliver a large enough dose
to significantly shrink most tumors. Lesions not limited to the orbital region
are treated in conjunction with the dermatologist, who (after discussing side
effects) gives oral steroids. Once again, early treatment appears to be more
effective. Oral steroids should be tapered slowly because rebound phenomenon
has been noted after abrupt discontinuation, with an increase in the size of
capillary hemangioma.
Surgical excision is reserved for small localized
lesions. It is usually avoided in larger lesions because of the risk of
considerable bleeding, and because the infiltrative nature of the lesion
requires removal of large amounts of skin. Surgery is indicated after failure
with steroids.
Laser procedures are limited by the scarring that
they produce.
References:
North et al. A unique microvascular phenotype
shared by juvenile hemangiomas and human placenta. Arch Dermatol 2001; 137:
559-570.
Frieden et al.
The association of posterior fossa brain malformations, hemangiomas, arterial
anomalies, coarctation of the aorta and cardiac defects, and eye
abnormalities. Arch Dermatol 1996; 132: 307-311.
Kushner BJ. Intralesional corticosteroid injection
for infantile adnexal hemangioma. Am J Ophthalmol 1982; 92: 496-506.
Esekowitz et al.
Interferon alpha-2a therapy for life-threatening hemangiomas of infancy. N Engl
J Med 1992; 326: 1456-1463.
Fledelius et al.
Interferon-alpha treatment of facial infantile hemangiomas: with emphasis on the
sight-threatening varieties. A clinical series. Acta Ophthalmol Scand 2001;
79: 370-373.
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EYE REPUBLIC
Ophthalmology Atlas
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EYE REPUBLIC Ophthalmology Clinic
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CAPILLARY HEMANGIOMA information compiled by
Dr. Barbara L. Roque
and initially uploaded on August 16, 2005.
Last updated on
September 13, 2007. |
