Bilateral subperiosteal Orbital Hematomas and Henoch-Schönlein Purpura FREE

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 purpura.

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 initial visit.

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 reports^4- 6 of associated intracerebral, scrotal, intestinal, and adrenal gland hematomas. To our knowledge, there are no previous reports on Henoch-Schönlein purpura and orbital hematomas.

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 malignancy.

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 degradation.⁷

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).