J Korean Ophthalmol Soc.  2007 Oct;48(10):1438-1444. 10.3341/jkos.2007.48.10.1438.

Papilledema Associated with Anemia in a Patient with Paroxysmal Nocturnal Hemoglobinuria

• Woo KJ
• Kim SK

• KMID: 2211002
• DOI: http://doi.org/10.3341/jkos.2007.48.10.1438

Abstract

PURPOSE: To report a case of bilateral optic disc edema associated with hemolytic anemia and paroxysmal nocturnal hemoglobinuria (PNH).
METHODS
A 51-year-old woman visited our ophthalmologic clinic complaining of metamorphopsia. Twenty eight years ago, she had been diagnosed with PNH and hemolytic anemia and had received blood transfusion on an irregular basis. The best corrected visual acuity was initially 0.5 in the right eye and 1.0 in the left eye. Light reflex was intact and no afferent pupillary defect was found. Fundus examination revealed severe optic disc swellings with indistinct margins in both eyes. Papillary and peripapillary retinal hemorrhages were also present.
RESULTS
A visual field test revealed the enlarged physiologic scotoma in both eyes. Fluorescein angiograms showed hyperfluorescence of the optic disc and blocked fluorescence due to the papillary hemorrhages. Optical coherence tomograms of the optic disc showed the loss of physiologic disc cupping and severe elevation. There was no evidence of an intracranial lesion upon brain magnetic resonance imaging. These findings were compatible with optic disc edema associated with anemia and the management was oriented towards the anemia. At the 2-months follow-up, the best corrected visual acuity of both eyes had improved to 1.0 and optic disc edema markedly decreased. However, the patient's overall physical condition deteriorated and she expired due to dyspnea and hepatic coma.

Keyword

Anemia; Optic disc edema; Paroxysmal nocturnal hemoglobinuria

MeSH Terms

Anemia*
Anemia, Hemolytic
Blood Transfusion
Brain
Dyspnea
Edema
Female
Fluorescein
Fluorescence
Follow-Up Studies
Hemoglobinuria, Paroxysmal*
Hemorrhage
Hepatic Encephalopathy
Humans
Magnetic Resonance Imaging
Middle Aged
Papilledema*
Pupil Disorders
Reflex
Retinal Hemorrhage
Scotoma
Vision Disorders
Visual Acuity
Visual Field Tests
Fluorescein

Figure

• 

  Figure 1. Photograph of peripheral blood smear. The erythrocytes are relatively normal in size and in hemoglobin content, but they are insufficient in number. These findings correspond to normocytic normochromic features of hemolytic anemia (Wright-Giemsa stain, χ400).

• 

  Figure 2. Fundus photographs show bilateral disc edema at the first visit. The disc margin is indistinct and papillary and peripapillary retinal hemorrhages are accompanied.

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  Figure 3. Automated Humphrey visual field test demonstrates enlarged physiologic scotoma and irregular peripheral visual field defects.

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  Figure 4. Early (A, B) and late (C, D) phase fluorescein angiograms show continued hyperfluorescence of the disc accompanied blocked fluorescence by papillary and peripapillary hemorrhages.

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  Figure 5. Optical coherence tomographs of the right (A) and left (B) optic disc show the loss of physiologic disc cupping and elevated optic disc.

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  Figure 6. Orbit (A) and brain (B) MRIs of the patient reveal no evidence of retrobulbar lesion and brain metastasis or hydrocephalus. The superior ophthalmic vein (arrow) is not dilated.

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  Figure 7. Abdomen CT shows diffuse swelling of the liver (*) and spleen (**) with left portal vein thrombosis (arrow).

• 

  Figure 8. Fundus photographs after 2 months show improved papilledema.

Reference

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