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W(h)ither orbital pseudotumor?

Hakan Agir1, Neil Aburn, Charles Davis

  • 1Head & Neck and Skull Base Surgery/Oncology Programme, Wellington Regional Plastic, Maxillofacial & Burns Unit, Hutt Hospital, Wellington, New Zealand.

The Journal of Craniofacial Surgery
|October 4, 2007
PubMed
Summary
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Orbital pseudotumor, an idiopathic inflammatory condition, is challenging to diagnose. Biopsy is crucial when corticosteroid treatment fails, as other orbital diseases may mimic its presentation.

Area of Science:

  • Ophthalmology
  • Pathology

Background:

  • Orbital pseudotumor is a nonspecific, idiopathic benign inflammatory process involving lymphoid infiltrate and fibrosis.
  • It presents with diverse ophthalmologic symptoms like swelling, proptosis, and visual loss.

Observation:

  • A retrospective review of six patients with initial orbital pseudotumor diagnosis highlighted diagnostic challenges.
  • Clinical findings, imaging, and initial corticosteroid response were used for diagnosis.
  • Only one of three patients initially treated with corticosteroids received a confirmed orbital pseudotumor diagnosis.

Findings:

  • Two patients initially diagnosed with orbital pseudotumor had Miller-Fisher syndrome and thyroid ophthalmopathy.
  • Biopsies revealed non-Hodgkin lymphoma in two of three patients initially suspected of orbital pseudotumor.

Related Experiment Videos

  • Orbital pseudotumor is a diagnosis of exclusion within a spectrum of lymphocytic infiltrative orbital conditions.
  • Implications:

    • The diagnosis of orbital pseudotumor is provisional and requires a high index of suspicion.
    • Failure to resolve with corticosteroids warrants biopsy to rule out other conditions like lymphoma.
    • Accurate diagnosis, even with histopathology, can be difficult.