Optic Disk Swelling and Retinal Hemorrhages in Both Eyes: Etiology, Pathophysiology, Imaging, and Management

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Introduction

Optic disk swelling and retinal hemorrhages are critical ophthalmologic findings that often signify an underlying neurological or systemic disorder. When both eyes are affected simultaneously, the differential diagnosis must urgently include conditions associated with increased intracranial pressure (ICP), venous obstruction, or systemic vascular disease.

In this article, we examine a representative case of a 25-year-old obese female who presented with headache, blurred vision, and transient visual obscurations. Neurological examination revealed bilateral optic disk swelling (papilledema) with retinal hemorrhages, and subsequent MRI/MRV findings demonstrated empty sella, flattened posterior globes, and bilateral transverse sinus stenosis without thrombosis ([Figure 1], [Figure 2]).

We will explore the cause, etiology, pathophysiology, epidemiology, clinical presentation, imaging features, treatment, and prognosis of this condition, with emphasis on idiopathic intracranial hypertension (IIH), a disease strongly associated with obesity.

1. Cause and Etiology

Idiopathic Intracranial Hypertension (IIH)

The most likely cause in this patient is idiopathic intracranial hypertension (IIH), also historically termed pseudotumor cerebri.

  • Definition: A syndrome of increased ICP without an identifiable structural lesion, hydrocephalus, or infection.

  • Risk Factors:

    • Severe obesity (especially in women of childbearing age).

    • Endocrine abnormalities (e.g., polycystic ovary syndrome, hypothyroidism).

    • Certain medications: tetracyclines, vitamin A derivatives, corticosteroid withdrawal, and growth hormone therapy.

    • Venous sinus stenosis or impaired CSF absorption.

Other Etiologies to Consider

While IIH is most common, other differential diagnoses include:

  1. Venous sinus thrombosis (excluded in this case by MRV).

  2. Intracranial mass lesions (tumors, abscesses, cysts).

  3. Infectious meningitis or encephalitis.

  4. Hypertensive retinopathy.

  5. Systemic hematological disorders (anemia, leukemia).

2. Pathophysiology

The pathophysiology of optic disk swelling and retinal hemorrhage in IIH involves:

  1. Elevated Intracranial Pressure

    • Increased CSF pressure is transmitted to the optic nerve sheath.

    • This impairs axoplasmic flow within the optic nerve head, leading to optic disk swelling (papilledema).

  2. Venous Congestion

    • Raised ICP compresses the central retinal vein, reducing venous outflow.

    • This results in retinal hemorrhages and dilated tortuous retinal veins.

  3. Secondary Vascular Leakage

    • Impaired perfusion pressure leads to microvascular leakage and hemorrhage, visible on fundoscopy.

  4. Transverse Sinus Stenosis

    • MRI/MRV often shows bilateral transverse sinus narrowing.

    • Whether this is cause or effect of IIH remains debated, but venous outflow restriction perpetuates elevated ICP.

3. Epidemiology

  • Age and Sex: IIH is most common in women aged 20–40 years, with a peak in reproductive age.

  • Obesity: Over 90% of patients are overweight or obese.

  • Incidence:

    • General population: ~1 per 100,000.

    • In obese women of childbearing age: ~20 per 100,000.

  • Global Burden: Rising in parallel with the obesity epidemic worldwide.

4. Clinical Presentation

Symptoms

  • Headache (most common, present in >90%).

  • Transient visual obscurations (brief episodes of vision loss, seconds in duration).

  • Diplopia (due to abducens nerve palsy).

  • Pulsatile tinnitus.

  • Photopsia or blurred vision.

Signs

  • Bilateral optic disk swelling (papilledema) – hallmark finding.

  • Retinal hemorrhages due to venous congestion.

  • Enlarged blind spot on visual field testing.

  • Visual field loss (classically arcuate or nasal step defects).

  • Normal neurological exam except for possible sixth nerve palsy.

5. Imaging Features

MRI and MRV are essential both to exclude secondary causes and to identify supportive features of IIH.

  • MRI findings:

    • Flattened posterior globe contour ([Figure 1]).

    • Empty sella (CSF filling the sella turcica due to chronic ICP elevation).

    • Distension of perioptic subarachnoid spaces.

    • Optic nerve tortuosity.

  • MRV findings:

    • Bilateral transverse sinus stenosis without obstruction or thrombosis ([Figure 2]).

Figures


[Figure 1] Fundoscopy showing bilateral optic disk swelling with retinal hemorrhages.

[Figure 2] Coronal MRI and MRV demonstrating empty sella, flattened posterior globes, and transverse sinus stenosis.

6. Diagnostic Workup

  • Ophthalmological exam: Fundoscopy, optical coherence tomography (OCT), and visual field perimetry.

  • Neuroimaging: MRI/MRV to rule out mass or thrombosis.

  • Lumbar puncture:

    • Elevated opening pressure (>250 mmH₂O in adults).

    • Normal CSF composition.

  • Blood work: Rule out systemic/metabolic disorders.

7. Treatment

Goals

  • Relieve symptoms (headache, visual impairment).

  • Preserve vision.

  • Reduce intracranial pressure.

Lifestyle and Medical Therapy

  1. Weight loss: Most effective long-term management; even 5–10% weight reduction significantly improves outcomes.

  2. Pharmacologic:

    • Acetazolamide (carbonic anhydrase inhibitor): first-line, reduces CSF production.

    • Topiramate: an alternative, also aids weight loss.

    • Furosemide: adjunct therapy.

  3. Analgesics for headache.

Interventional and Surgical Therapy

  • Optic nerve sheath fenestration (ONSF) – for progressive vision loss.

  • CSF shunting (lumboperitoneal or ventriculoperitoneal) – for refractory cases.

  • Venous sinus stenting – in select patients with severe venous stenosis.

8. Prognosis

  • Most patients stabilize with medical and lifestyle management.

  • Vision loss occurs in ~10% if untreated or inadequately managed.

  • Prognosis improves significantly with early recognition and treatment.

  • Relapses may occur, especially if obesity persists.

Quiz

Q1. Which lumbar puncture finding is most characteristic of idiopathic intracranial hypertension (IIH)?

A) Elevated white blood cell count

B) Elevated opening pressure

C) Oligoclonal bands

D) Positive anti-aquaporin 4 antibody

E) Xanthochromia

Q2. Which MRI feature is NOT typically associated with idiopathic intracranial hypertension?

A) Flattened posterior globes

B) Empty sella

C) Transverse sinus stenosis

D) Large space-occupying mass 

E) Optic nerve sheath distension

Q3. Which patient group is at the highest risk for idiopathic intracranial hypertension?

A) Elderly men

B) Adolescent males

C) Women of childbearing age with obesity 

D) Children with hydrocephalus

E) Patients with Parkinson’s disease

Answer & Explanation:

1. Answer: B) Elevated opening pressure. Explanation: IIH typically presents with elevated CSF opening pressure and otherwise normal CSF constituents.

2. Answer: D) Large space-occupying mass.  Explanation: The hallmark of IIH is the absence of space-occupying lesions. MRI findings include flattened globes, empty sella, and sinus stenosis.

3. Answer: C) Women of childbearing age with obesity. Explanation: IIH is most common in young obese women, making option C correct.

Conclusion

Bilateral optic disk swelling with retinal hemorrhages is a clinical red flag. In the absence of mass lesions or thrombosis, IIH should be strongly suspected, particularly in obese women of childbearing age. Timely neuroimaging, lumbar puncture, and ophthalmological evaluation are crucial for diagnosis. Weight reduction and acetazolamide remain the cornerstone of therapy, with surgical options reserved for refractory cases.

References

[1] Biousse V, Newman NJ. Neuro-Ophthalmology Illustrated. 2nd ed. Stuttgart: Thieme; 2012.
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[7] Thambisetty M, Lavin PJ, Newman NJ, Biousse V. "Fulminant idiopathic intracranial hypertension." Neurology, vol. 68, no. 3, pp. 229–232, 2007.

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