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There are few reports of nontraumatic bilateral subperiosteal orbital
hematomas in the ophthalmic literature.1-3 None
exist on subperiosteal orbital hematomas associated with Henoch-Schonlein
Report of a Case
A 5-year-old boy with a known case of infantile spasms since birth visited
with bilateral exophthalmos of a few days' duration. Ocular examination revealed
bilateral upper eyelid ecchymosis and exophthalmos mostly on the right side,
with no afferent pupillary defect (Figure
1). There was no history of trauma. Physical examination revealed
a purpuric rash on the buttocks and lower extremities, abdominal tenderness,
and bilateral ankle swelling and tenderness. Computed tomography (CT) scans
of the orbits revealed well-defined, nonenhancing, bilateral superior orbital
masses with no bone erosion (Figure 2).
Hematuria was noted on urinalysis, but systemic workup, including coagulation
studies, was normal. A diagnosis of Henoch-Schönlein purpura with bilateral
orbital hematomas was entertained. Orbital magnetic resonance imaging (MRI)
at follow-up confirmed the diagnosis and showed improvement. There was an
uneventful recovery, with marked resolution of the exophthalmos 2 weeks following
Bilateral upper eyelid ecchymosis
with exophthalmos more prominent on the right side.
Computed tomographic scan shows
bilateral, well-defined superior orbital masses with no bone erosion.
Henoch-Schönlein purpura is a systemic hypersensitivity disease
of unknown origin, with common vasculitis in childhood. The clinical diagnosis
relies on a classic tetrad of purpuric rash, arthralgias, abdominal pain,
and renal involvement. A kidney or skin biopsy specimen demonstrating IgA
deposition can be helpful in atypical cases. Although Henoch-Schönlein
purpura is not usually associated with frank bleeding, there are reports4-6 of associated
intracerebral, scrotal, intestinal, and adrenal gland hematomas. To our knowledge,
there are no previous reports on Henoch-Schönlein purpura and orbital
Orbital hematomas are most commonly traumatic in origin but can occasionally
be spontaneous or secondary to increased central venous pressure, extension
from chronic sinusitis, and blood dyscrasias. Few reports of bilateral nontraumatic
orbital hematomas are found in the literature, and they are mainly in association
with alcoholic liver disease,1 scurvy,2 and disseminated intravascular coagulation.3 The resulting exophthalmos in children can sometimes
pose a diagnostic challenge, as the rapid presentation might resemble an orbital
The subperiosteal space is a potential space bridged by small irregular
diploic vessels. Bleeding into this space commonly manifests as painful proptosis
with eyelid edema and conjunctival chemosis. The most common location for
subperiosteal hematomas is the superior orbit. However, rare reports of medial
and lateral subperiosteal orbital hematomas are present. Orbital hematomas
most commonly occur in children and young adults because the periorbita is
less firmly adherent to bone in this age group. On CT scan, appearance of
acute subperiosteal orbital hematoma is a biconvex, well-defined, nonenhancing
mass of homogeneous density that is slightly higher than that of the brain.
In most cases, CT scan images are sufficient to seal the diagnosis. Confirmation
can be obtained on MRI, which can characterize the different stages of blood
Because most of these hematomas usually resolve within a few weeks,
conservative management is acceptable as long as normal optic nerve function
is documented. Fine-needle aspiration and surgical drainage should be considered
in cases of compression of the optic nerve.
Corresponding author: Riad N. Ma'luf, MD, Department of ophthalmology,
American University of Beirut Medical Center, 113-6044, Beirut, Lebanon (e-mail: rmaluf@cyberia .net.lb).
Ma'luf RN, Zein WM, El Dairi MA, Bashshur ZF. Bilateral subperiosteal Orbital Hematomas and Henoch-Schönlein Purpura. Arch Ophthalmol. 2002;120(10):1398–1399. doi:
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