Episcleral hemangioma as an isolated finding.

Abstract

To report on a case of episcleral hemangioma in a child.
A 3-year-old boy presented with a 4-week history of limbal mass in the left eye and had an excision of that mass.
There was an 8 x 7 mm oval lesion, 3-4 mm below the inferior limbus. It was reddish, sharply elevated with smooth surface, and firmly attached to the underlying sclera with two large blood vessels over it. Histopathologic examination demonstrated a cellular lesion containing vascular spaces of various sizes. The solid appearance indicated an increase of cells that appear to be consistent with endothelial cells which formed a nest surrounded by PAS-positive strands. Small to moderate size capillaries were evident throughout the solid portion of the lesion. These findings were consistent with episcleral hemangioma of capillary type.
Episcleral hemangioma is a rare tumor and should be included in the differential diagnosis of episcleral tumors. To our knowledge, this is the first reported case of episcleral hemangioma in a child as an isolated finding.

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Episcleral hemangioma as an isolated finding
HUDA AL-GHADEER1, ALI A. AL-RAJ HI1, FENWICK C. RILEY2
1Anterior Segment Division
2Pathology and Screening Clinic, King Khaled Eye Specialist Hospital, Riyadh - Kingdom of Saudi Arabia
INTRODUCTION
Hemangiomas are the most common soft tissue tumors
of infancy. The rate of incidence stands at 5–10% in in-
fants (1, 2). They are defined as vascular tumors with a
growth phase, marked by hypercellularity and involutional
phase (1). They are more frequently found in girls than
boys ranging from a 3:1 to 5:1 ratio, and are thought to be
common in white patients than in black patients (1). We
report on a case of capillary type episcleral hemangioma
and its histopathologic findings.
Case report
A 3-year-old boy presented to the emergency room at
King Khaled Eye Specialist Hospital with a 4-week history
of painless swelling in the left eye, which had gradually in-
creased in size and with no change in visual acuity (VA).
The patient fell down from his bicycle 5 weeks prior to his
complaint. He was a product of full-term twin pregnancy
with no prenatal or postnatal complications. The family
history was unremarkable.
European J ournal of Ophthalmology / Vol. 19 no. 2, 2009 / pp. 292-294
1120-6721/292-03$25.00/0
© Wichtig Editore, 2009
PURPOSE. To report on a case of episcleral hemangioma in a child.
METHODS. A 3-year-old boy presented with a 4-week history of limbal mass in the left eye and
had an excision of that mass.
RESULTS. There was an 8 x 7 mm oval lesion, 3–4 mm below the inferior limbus. It was reddish,
sharply elevated with smooth surface, and firmly attached to the underlying sclera with two
large blood vessels over it. Histopathologic examination demonstrated a cellular lesion con-
taining vascular spaces of various sizes. The solid appearance indicated an increase of cells
that appear to be consistent with endothelial cells which formed a nest surrounded by PAS-
positive strands. Small to moderate size capillaries were evident throughout the solid portion
of the lesion. These findings were consistent with episcleral hemangioma of capillary type.
C ONCLUSIONS. Episcleral hemangioma is a rare tumor and should be included in the differential
diagnosis of episcleral tumors. To our knowledge, this is the first reported case of episcleral
hemangioma in a child as an isolated finding. (Eur J Ophthalmol 2009, 19: 292-4)
KEY WORDS. Capillary, Children, Episclera, Hemangioma, Vascular tumors
Accepted: August 14, 2008
Ocular examination showed apparently normal VA in both
eyes. Intraocular pressure was within normal limits, right
eye was normal. Left eye assessment revealed normal
lids, full extraocular movement, and regular round reactive
pupil. Both fundi were normal. Slit-lamp examination
showed clear cornea and lens, deep and quiet anterior
chamber. There was an 8 x 7 mm oval lesion, 3–4 mm be-
low the inferior limbus. It was reddish, sharply elevated
with smooth surface, and firmly attached to the underly-
ing sclera with two large blood vessels over it (Fig. 1). Ul-
trasound biomicroscopy of the left eye revealed a 6 x 7
mm lesion with two small rounded cystic spaces sur-
rounded by homogenous dense opacities. The sclera ap-
peared to be thin in the area of the lesion with no evi-
dence of ciliary body, scleral, or globe involvement. The
conjunctiva was dissected from the tumor from the limbal
side and the conjunctiva overlying the tumor was also dis-
sected until the boundaries of the tumor were identified
which were attached to the sclera. There were two large
blood vessels coming inferiorly, one from the nasal and
some telangiectatic blood vessels at the limbus, over
conjunctiva. These were cauterized and the tumor was
Presented as a poster at the annual Saudi Ophthalmology 2004 confer-
ence (March 7–10); Riyadh, Kingdom of Saudi Arabia.
SHORT COMMUNICATIONS & CASE REPORTS

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Al-Ghadeer et al
293
dissected from the underlying sclera. It was found to be
firmly attached to the sclera. The tumor, in our case, was
excised completely (Fig. 2) and obtained for histopatho-
logic study.
Histopathologic findings demonstrated a cellular lesion
containing vascular spaces of various sizes which was
surrounded on three sides by a dense fibrous pseudo-
capsule. No epithelium was associated with the lesion.
The solid appearance indicated an increase of cells
that appear to be consistent with endothelial cells
which formed a nest surrounded by PAS-positive
strands (Fig. 3A). Small to moderate size capillaries
were evident throughout the solid portion of the lesion
(Fig. 3B). In some areas, these vascular spaces be-
come a bit larger and appeared to have a venous ar-
chitecture. In half of the specimen, a large vessel
seems to be extending through the deeper portion of
the lesion. These lesions were lined with flattened en-
dothelial cells which appeared to be associated with
neural cells. They were consistent with a large venule.
Lymphocytes were scattered all through the lesions.
Immunohistochemistry was done for von Willebrand
factor (factor VIII), CD 34, and smooth muscle actin.
Both von Willebrand and CD 34 were positive in the ar-
eas surrounding the vascular spaces, suggesting the
origin is vascular endothelium.
Fig. 1 - Feeding blood vessel over the lesion as indicated by the
arrow.
Fig. 2 - After excision.
Fig. 3 - (A) Peripheral
areas with capillary
proliferation and cap-
sule (H-E x 100). (B)
Capillary proliferation
(H-E x 200).

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Episcleral hemangioma
294
DISCUSSION
Episcleral tumors are common but tumors of the sclera
are rare. They may originate from the episcleral, vascular,
or nervous tissues which pass through the extension of
the lesions of conjunctiva or inner eye or may be a mani-
festation of systemic neoplastic process (3).
In early stages of the vascular tumors, they can be seen
as a dilatation of some of the vessels of the deep episcle-
ral network. In some congenital varieties, these anomalies
can be extensive. They regress with age as can be seen
in the cases of skin hemangioma (4). However, angiomas
arising within the episclera resemble an amelanotic
melanoma which, sometimes, cannot be distinguished
except by biopsy. The new vessels which are formed in
the tumor itself appear to radiate from the mass rather
than evade it, as in the inflammatory conditions (4).
Hence, it is suggested that trauma may be responsible for
their clinical appearance or increase in growth (4).
Episcleral hemangioma is regarded as one of the ocular
manifestations of Sturge-Weber syndrome. These lesions
are often not clinically apparent in early childhood but be-
come noticeable upon slit-lamp examination in the sec-
ond half of the first decade. There were no signs of this
syndrome in our patient.
Takahashi reported two cases of simple scleral heman-
gioma, one occurring in a 56-year-old woman, and the
other in a 19-year-old man. The latter was associated with
an incomplete form of Sturge-Weber syndrome. Slit lamp
microscopy had revealed that these cases seem to be
telangiectasias of capillaries with microaneurysms along
the limbus (5). Currently, they are thought to result from an
arrest in the development of the mesenchymal primordial
in the earliest stage of vascular system formation (6).
Chang et al reported a case of diffuse neonatal heman-
gioma with eyelid, conjunctival, and iris hemangioma. The
findings of histopathology disclosed capillaries of varying
size which was similar to the findings in our case (7).
Absence of typical symptoms and signs of inflammatory
conditions such as nodular episcleritis led to the decision
to excise the tumor rather than attempt topical therapy.
The histopathologic and immunohistochemical results
support our diagnosis.
There was no recurrence of this lesion in our patient 3
years after presentation.
In conclusion, episcleral hemangioma, a rare tumor,
should be included in the differential diagnosis of episcle-
ral tumors. We are unaware of prior reports of episcleral
hemangioma in a child as an isolated finding.
ACKNOWLEDGEMENTS
The authors thank Dr. Abdullah Al-Kandari for assistance
with immunohistochemistry.
None of the authors has any proprietary or financial interest or conflicts
with respect to any of the material presented in the article.
Reprint requests to:
Huda Al-Ghadeer, MD
Member, Anterior Segment Division
King Khaled Eye Specialist Hospital
PO Box 7191
Riyadh 11462, Kingdom of Saudi Arabia
hudagh2001@yahoo.com
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