Arnold Chiari – May 2016

May 2016

Case Presentation

A 24 year-old woman was referred to Illinois Retina Associates for blurry vision in her right eye.

She was otherwise healthy, known for occasional episodes of tachycardia not needing treatment, and taking only oral contraceptives. She complained of gradual vision loss in the right eye starting the prior day and recently worsening headaches.

Examination

Although her visual acuity was 20/20 in both eyes, she had a subjective complaint of decreased vision in her right eye. There was no afferent pupillary defect, and confrontational visual fields were normal. On posterior segment examination of the right eye, there were a few scattered peripapillary, flame-shaped and blot hemorrhages, mild disc edema, and mild venous dilation and tortuosity. There was mild elevation of the left optic nerve.

Differential Diagnosis

  • Disc Edema/Papilledema
  • Venous stasis retinopathy/impending central retinal vein occlusion
  • Intracranial hypertension, idiopathic or secondary to other causes
  • Diabetic retinopathy

Diagnostic Testing

Fundus photography revealed the blot and flamed-shaped hemorrhages in the right eye (Figure 1), while the left eye appeared normal. A fluorescein angiogram showed normal circulation patterns in both eyes (Figure 2).

Additional testing was performed by a hematologist, including a work-up for hypercoagulability disorders, but all the investigations were negative.

A brain MRI revealed an Arnold Chiari malformation and syringomyelia.

Figure1
Figure 1

Figure2
Figure 2

Diagnosis

Optic nerve edema and venous stasis retinopathy secondary to Arnold-Chiari malformation

Follow-Up

The patient’s visual symptoms subsided within a few months of presentation, and teh fundus hemorrhages resolved completely (Figure 3). Her headaches have significantly improved as well. She is currently followed by a neurosurgeon.

Figure3
Figure 3

Discussion

Arnold-Chiari is a structural defect of the cerebellum, resulting in its downwards placement within the skull, and can sometimes result in cerebrospinal fluid accumulation and increased intracranial pressure. Symptoms of high intracranial pressure include, among others, headaches and optic nerve swelling.

Syringomyelia is a disorder characterized by cerebrospnal fluid accumulation in a cyst form within the spinal cord, and is often associated with Arnold-Chiari.

References

Haaland Stone WJ, Ittner EA, Teitelbaum BA, Messner LV. Progressive, asymptomatic papilledema as the presenting sign of a Chiari I Malformation. Optometry. 2012 Mar 30;83(3):114-9

McVige JW, Leonardo J. Neuroimaging and the clinical manifestations of Chiari Malformation Type I (CMI) Curr Pain Headache REp. Jun;19(6):18

Zhang JC, Bakir B, Lee A, Yalamanchili SS. Papilloedema due to Chiari I Malformation. BMJ Case Rep.1002 Oct 16;2011