Austin Smith* was a 3-year-old who had a problem that wouldn’t go away. Over the course of a month, his mother noted some redness above his left eye with a swollen upper lid. When it did not resolve within this time, she took him to his pediatrician who thought he had allergies and gave him a topical antihistamine. His mother noted that the redness and swelling persisted, and she returned to the pediatrician who then thought he might have preseptal cellulitis. Austin was started on Augmentin (amoxicillin and clavulanate potassium), which seemed to help initially. But after a week of therapy, he still had the redness and swelling, which seemed to be getting worse.
We Get a Look
He was referred to our clinic for preseptal cellulitis that was not responding to therapy. We thought his history seemed a little inconsistent with an allergy or preseptal cellulitis. We would have expected an allergic problem to be bilateral. For a diagnosis of preseptal cellulitis, he should have had a skin lesion from which the infection entered or an orbital focus.
We reviewed his history and examined him. He was a well-nourished, healthy boy with no previous medical or ocular history. His mother said he had been a little fussy lately, but he had neither fever nor chills. Examination showed his vision to be equal in each eye at 20/25. There was no relative afferent pupillary defect. Extraocular motility exam was full, and his slit-lamp examination was normal. External examination showed edema at the left lateral brow overlying the superior-temporal orbital rim. The area was exquisitely painful to manipulation. There was a palpable left preauricular lymph node.
A CT scan of the orbits showed an osteolytic lesion of the superior-temporal orbital rim. The patient underwent an uncomplicated incisional biopsy of the lesion through an upper eyelid crease incision. Frozen section examination was consistent with an eosinophilic granuloma.
What’s Your Diagnosis? |
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| (Top) Patient on presentation showing edema at the area of the left superior-temporal orbital rim. (Below, left) Coronal CT showing an ostelytic process of the left superior-temporal orbit. (Below, right) Three-dimensional reconstruction. |
About the Diagnosis
Previously termed histiocytosis X, Langerhans cell histiocytosis has a broad clinical spectrum. There are acute disseminated (Letterer-Siwe disease), multifocal (Hand-Schüller-Christian disease) and unifocal (eosinophilic granuloma) forms. The disease process involves proliferating pathologic Langerhans cells, which exert a mass effect. Orbital involvement most often represents unifocal disease.
Austin proved to be the poster child for an eosinophilic granuloma. Typically, the lesions occur in the superior-temporal quadrant of the orbit with an osteolytic defect of the orbital roof.1 There is a male preponderance, and the lesion usually occurs in the first to second decade. The lesion usually causes upper eyelid edema and brow pain, and is tender to palpation.
Differential diagnosis of the lesion includes metastatic lesions (e.g., neuroblastoma, Ewing’s sarcoma and Wilms’ tumor), lacrimal gland disorders, a ruptured dermoid cyst and primary bone tumors (e.g., giant cell reparative granuloma, aneurysmal bone cyst, cholesterol granuloma and brown tumor of hyperparathyroidism).2
Langerhans cell histiocytosis disorders have a predilection for hematopoietically active bone marrow, and lesions are thought to occur around the orbit due to the age-related distribution of active marrow within normal orbital bones. The pathology of eosinophilic granuloma shows Langerhans cells, chronic inflammatory cells and eosinophils, which stain for the surface antigens CD1a and S-100. Electron microscopy shows the pathognomonic Birbeck granules.
The Treatment
We chose to treat Austin with curettage and an intralesional injection of 125 milligrams of methylprednisolone. Multiple treatment modalities have been employed for eosinophilic granuloma, including low-dose irradiation, intralesional corticosteroid injection, simple biopsy and subtotal curettage. No modality has been proven to be more effective than the other, but these lesions overall resolve after minimal intervention with a good prognosis.1 (One study reported an orbital eosinophilic granuloma that recurred six weeks after biopsy and curettage.3)
In addition, the patient should be referred to a pediatric oncologist, and a skeletal survey should be performed to rule out systemic involvement. A bone marrow aspiration is not necessary. For follow-up, the patients have a periodic physical exam, orbital CT and skeletal survey. The bone will undergo reossification.
After Treatment |
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| Five-month follow-up with: (Top) no evidence of recurrence, and coronal CT with evidence of superior-temporal orbital rim reossification (bottom). |
Austin’s case is instructive in the sense that the initial diagnoses were not consistent with his history. And if preseptal cellulitis was seriously considered, a CT scan should have been pursued since there was no external lesion. Fortunately, a delay in diagnosis did not adversely affect his eventual recovery.
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*
Patient name is fictitious.
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1 Woo, K. I. and G. J. Harris.
Ophthal Plast Reconstr Surg 2003;19:429–439.
2 Selva, D. et al.
Surv Ophthalmol 2004; 49:328–342.
3 Song, A. et al.
Ophthal Plast Reconstr Surg 2003;19:140–144.
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Dr. Allen is a fellow in oculoplastic surgery and Dr. Nerad is a professor of ophthalmology; both are at the University of Iowa.
