acute hydrops keratoconus

Acute Corneal Hydrops in Keratoconus: A Rare but Serious Complication

Acute corneal hydrops, also known as acute corneal edema, is a rare and serious complication of keratoconus, a condition that affects the shape of the cornea. This complication occurs when there is a tear in Descemet's membrane, a thin layer of tissue on the back of the cornea, allowing aqueous fluid to leak into the stroma, causing severe corneal edema.

Clinical Manifestations

The clinical manifestations of acute corneal hydrops include:

• Severe corneal edema
• Reduced visual acuity
• Sudden vision loss
• Light sensitivity
• Eye pain

These symptoms can occur suddenly and may be accompanied by a significant reduction in visual acuity. In some cases, the condition may lead to permanent vision loss if left untreated.

Prevalence

The prevalence of acute corneal hydrops in keratoconus patients is estimated to be between 0.2% to 2.8%. This complication can occur at any age but is most commonly seen between 20 and 40 years old.

References

• [1] Acute corneal hydrops presents most commonly between 20 and 40 years [3].
• [2] An estimated 0.2% to 2.8% of people who have keratoconus develop corneal hydrops [1].
• [3] The specific term “acute corneal hydrops” (hydrops corneae) was only popularized in 1940 following animal modeling and detailed clinical descriptions of focal disruption of Descemet membrane [13].

Symptoms of Acute Hydrops Keratoconus

Acute hydrops keratoconus, a complication of keratoconus, presents with several distinct signs and symptoms. These include:

• Markedly reduced visual acuity: The primary symptom of acute hydrops is a sudden decrease in vision, which can be severe [1].
• Intense photophobia: Patients often experience intense sensitivity to light, making it difficult for them to tolerate even dim lighting [2].
• Eye pain or irritation: Acute hydrops can cause significant discomfort and pain in the affected eye [3].
• Eye redness: The eye may appear red and inflamed due to the increased fluid pressure within the cornea [4].
• Tearing: Patients may experience excessive tearing, which can be a sign of the underlying condition [5].

Additional Signs

In some cases, acute hydrops keratoconus may also present with:

• Munson's sign: A V-shaped protrusion of the lower eyelid in downgaze, indicating advanced keratoconus [6].
• Breaks in Bowman's membrane: The cornea may develop breaks or thinning in its outer layer, leading to further complications [7].

Early Warning Signs

While acute hydrops is a sudden and severe manifestation of keratoconus, there are early warning signs that can indicate the onset of this condition. These include:

• Sudden decrease in visual acuity: A rapid decline in vision can be an early sign of acute hydrops [8].
• Corneal edema and pain: Increased fluid pressure within the cornea can cause discomfort, redness, and swelling [9].

References

[1] Sharma N, Maharana PK, Jhanji V, Vajpayee RB. Management of acute corneal hydrops in ectatic corneal disorders.

[2] Jul 8, 2024 — Epidemiology, pathophysiology, diagnosis and management of corneal hydrops.

[3] Aug 3, 2016 — Decreased vision · Eye pain or irritation · Eye redness · Tearing · Light sensitivity.

[4] by PK Maharana · 2013 · Cited by 90 — The patient presents with sudden onset decrease in vision, photophobia, and pain. Corneal thinning and ectasias combined with trivial trauma to the eye mostly ...

[5] This Clinical Management Guideline outlines the aetiology, signs, symptoms and evidence-based recommendations for diagnosing and treating corneal hydrops.

[6] KERATOCONUS SYMPTOMS AND SIGNS Symptoms. Symptoms of keratoconus generally begin in late teenage years or early twenties, but can start at any time. ...

[7] Read on as we dig deeper into keratoconus including its causes, symptoms, and treatment options. ... Some of the early signs of keratoconus include: ... Acute corneal hydrops is an uncommon ...

[8] Although keratoconus is a bilateral disease, symptoms may be more pronounced in one eye than the other.

[9] In severe cases, keratoconus may lead to the development of acute corneal hydrops. Progression typically ceases by the fourth decade of life.

Diagnostic Tests for Acute Hydrops Keratoconus

Acute corneal hydrops, a complication of keratoconus, requires prompt and accurate diagnosis to initiate appropriate treatment. Several diagnostic tests can be employed to diagnose this condition.

• Corneal Tomography: This test provides additional parameters for evaluating the anterior and posterior corneal surfaces, which is essential in diagnosing acute corneal hydrops [2]. It may also be useful in patients without a known history of ectasia [1].
• Anterior Segment Optical Coherence Tomography (AS-OCT): AS-OCT is an extremely useful tool for diagnosis, surgical planning, and monitoring the response to treatment [4][7]. This imaging technique allows improved ability to monitor the response to treatment [5].
• Ultrasound Biomicroscopy: Modern imaging modalities such as ultrasound biomicroscopy have enlightened us about the corneal structure and can be used in conjunction with other tests for accurate diagnosis.
• In Vivo Confocal Microscopy: This test provides high-resolution images of the cornea, which is essential in diagnosing acute corneal hydrops.

Other Diagnostic Tests

A Seidel test may also be performed to detect an aqueous leak or vitreous exposure [8]. However, this test is not directly related to the diagnosis of acute corneal hydrops.

It's worth noting that there is no standard-of-care treatment for corneal hydrops, and a comprehensive evaluation by an ophthalmologist is necessary to determine the best course of action [10].

References: [1] Context 2 [2] Context 3 [4] Context 5 [7] Context 6 [8] Context 8

Based on the available information, it appears that there are limited treatment options for acute corneal hydrops (ACH) associated with keratoconus.

Topical Antibiotics and Cycloplegics

Conventional treatment of ACH includes topical antibiotics, cycloplegics, and hypertonic saline as well as patching and bandage soft contact lens [2]. This approach is aimed at reducing inflammation, preventing infection, and providing comfort to the patient. However, it may not address the underlying cause of the condition.

Hypertonic Sodium Chloride

Conservative management for ACH includes the use of hypertonic sodium chloride to reduce epithelial edema [6]. This treatment option may help alleviate symptoms but is unlikely to resolve the condition on its own.

Intracameral Gas

There have been reports of using intracameral perfluoropropane gas in the treatment of ACH, although this approach is not widely adopted [3]. Another study reported the use

Understanding Differential Diagnosis in Acute Hydrops Keratoconus

The differential diagnosis for acute corneal hydrops, a condition where a break in Descemet's membrane allows aqueous to enter the stroma causing severe corneal thickening, decreased vision, light sensitivity, tearing, and pain, includes several corneal ectatic disorders that require careful differentiation.

Key Conditions to Consider

• Keratoconus: A progressive eye disease characterized by thinning of the cornea, leading to a cone-shaped deformity. It is often associated with acute hydrops.
• Forme fruste keratoconus: An early, asymptomatic form of keratoconus that can be diagnosed only through analysis of corneal morphology.
• Pellucid marginal degeneration: A rare condition characterized by thinning and ectasia of the peripheral cornea.
• Keratoglobus: A rare, congenital ectatic disorder affecting the entire cornea.

Diagnostic Considerations

When diagnosing acute hydrops keratoconus, it is essential to consider these conditions and rule out other potential causes, such as infectious keratitis, autoimmune keratitis, traumatic posterior annular keratopathy, or post-traumatic complications. A thorough examination of the patient's medical history, clinical presentation, and diagnostic tests (such as anterior segment optical coherence tomography) can help differentiate between these conditions.

References

• [1] Forme fruste keratoconus is an early, asymptomatic form of the disease with no apparent clinical signs; it can be diagnosed only through analysis of corneal morphology. [3]
• The differential diagnosis for acute corneal hydrops includes previously undiagnosed or known corneal ectasia including keratoconus (most commonly). [2]
• Acute corneal hydrops is the development of stromal and epithelial oedema of the cornea caused by the rupture of Descemet's membrane. It occurs in conditions such as keratoconus, pellucid marginal degeneration, and keratoglobus. [8]