sclerocornea

Sclerocornea: A Rare Congenital Eye Disorder

Sclerocornea is a rare congenital eye disorder characterized by the growth of vascularized scleral tissue into the cornea, causing opacity and blending of the two structures [4]. This condition can be associated with both ocular and systemic anomalies [3].

Key Features:

• Non-progressive: Sclerocornea is a non-progressive condition, meaning it does not worsen over time [1][7].
• Bilateral: The condition typically affects both eyes, although the extent of involvement may vary between the two eyes [2][5].
• Asymmetric: Sclerocornea can be asymmetric, with different areas of the cornea affected in each eye [1].
• Non-inflammatory: Unlike other corneal conditions, sclerocornea is not associated with inflammation [2].

Types:

There are two types of sclerocornea:

• Peripheral type: In this type, only a peripheral part of the cornea is affected by scleralization [8].
• Diffuse (total) type: In this type, the entire corneal tissue is affected by scleralization [9].

References:

[1] Sclerocornea is a rare, non-progressive, bilateral, and asymmetric primary congenital disease which has no association with inflammatory ... (Search Result 1) [2] Sclerocornea is a rare non-progressive, non-inflammatory usually bilateral congenital corneal opacity that can be associated with both ocular and systemic ... (Search Results 2, 3, 7) [3] Sclerocornea is a rare non-progressive, non-inflammatory usually bilateral congenital corneal opacity that can be associated with both ocular and systemic ... (Search Result 3) [4] Sclerocornea is a primary anomaly in which scleralization of a peripheral part of the cornea, or the entire corneal tissue, occurs. (Search Results 6, 8) [5] Sclerocornea is defined as diffuse (total) or peripheral opacity of the cornea, either associated or not with the flat cornea and systemic ... (Search Result 9) [6] Sclerocornea is a primary anomaly in which scleralization of a peripheral part of the cornea, or the entire corneal tissue, occurs. (Search Result 8)

Signs and Symptoms of Sclerocornea

Sclerocornea is a rare congenital disorder characterized by the opacity of the cornea, making it resemble the white color of the sclera. The signs and symptoms of sclerocornea can vary in severity and may include:

• Bilateral corneal opacity: Cloudiness or opaqueness of both eyes, which can be of varying degree [6][8].
• Blurred vision: Difficulty seeing clearly due to the clouding of the cornea [8].
• Decreased vision: A reduction in visual acuity as a result of the corneal opacity [8].
• Glaucoma: A condition where increased pressure in the eye can lead to damage to the optic nerve, affecting vision. Glaucoma is associated with sclerocornea in 50-70% of cases [7][9].
• Posterior corneal thinning: Thinning of the posterior stroma, Descemet membrane, and endothelium [7].
• Anterior staphyloma: A condition where there is a protrusion or bulging of the anterior part of the eye [7].
• Coloboma: A hole or defect in one of the structures of the eye, such as the iris or retina [7].

It's essential to note that sclerocornea can be associated with other systemic conditions and symptoms, which may require further evaluation and management.

References: [6] Mar 15, 2017 - Clinically, sclerocornea is a rare, bilateral clouding of the cornea. [7] Signs · Posterior corneal thinning involving the posterior stroma, Descemet membrane and endothelium · Glaucoma (50%–70% of cases) · Anterior staphyloma · Coloboma ... [8] What are the Signs and Symptoms of Sclerocornea? · Bilateral corneal opacity (cloudiness) that is of varying degree · Blurred vision · Decreased vision · Both the ... [9] by NS Jat · 2023 · Cited by 4 — [74] Other signs and symptoms include hypermetropia and astigmatism, keratoconjunctivitis sicca, glaucoma, retinopathy, and optic nerve head swelling.

Sclerocornea, a rare congenital corneal disease, can be diagnosed through various diagnostic tests.

• Biomicroscopic examination: This is the primary method for diagnosing sclerocornea. A biomicroscope is used to examine the anterior segment of the eye, and the presence of scleralization or clouding of the cornea can be observed [1].
• Anterior segment optical coherence tomography (AS-OCT): This imaging technique can provide detailed information about the corneal structure and help confirm the diagnosis of sclerocornea [1].
• Ultrasound biomicroscopy (UBM): UBM can be used to evaluate the presence of other anterior segment abnormalities, such as keratoconus or glaucoma, which may be associated with sclerocornea [9].

It's worth noting that a diagnosis of sclerocornea is typically made through a combination of clinical examination and imaging studies. A thorough evaluation by an ophthalmologist or other eye care professional is essential for accurate diagnosis and management.

References: [1] - Sclerocornea is a rare, non-progressive, bilateral, and asymmetric primary congenital disease which has no association with inflammatory and/or infectious causes. ... The diagnosis of congenital corneal anomalies can be made through biomicroscopic examination and the support of complementary tests, especially anterior segment optical coherence ... [9] - Mar 10, 2023 — Evaluation using ultrasound biomicroscopy (UBM) would better determine the presence of other anterior segment abnormalities such as kerato- ...

Sclerocornea, a rare congenital condition of the eyes, can be challenging to treat due to its non-progressive and bilateral nature. However, various treatment options are available to manage the symptoms and improve vision.

• Glaucoma treatment: In cases where sclerocornea is associated with glaucoma, treating the glaucoma is essential to prevent further vision loss [1]. This may involve medications such as eye drops or oral tablets to reduce intraocular pressure.
• Topical cysteamine: Research has shown that topical cysteamine can reduce crystal deposition in the cornea, which is a characteristic of sclerocornea [9]. However, its effectiveness in improving vision or preventing disease progression is unclear.
• Corneal epithelial cell proliferation and/or migration: A study suggested that promoting corneal epithelial cell proliferation and/or migration could be beneficial for accelerating healing in cases of corneal diseases, including sclerocornea [10]. However, this treatment option requires further research to confirm its efficacy.

It's essential to note that each case of sclerocornea is unique, and the most effective treatment approach may vary depending on individual circumstances. A comprehensive evaluation by an eye specialist is necessary to determine the best course of action for managing sclerocornea.

References: [1] Various genetic, metabolic, developmental, and idiopathic causes can result in congenital clouding of the cornea. A common reason for congenital clouding of the cornea is congenital glaucoma. [9] by LR Katzman · Cited by 6 — Oral cysteamine has been shown to alleviate the systemic problems but not the corneal crystal deposition. Topical cysteamine can reduce crystal ... [10] by B Wirostko · 2015 · Cited by 79 — A topically applicable compound capable of promoting corneal epithelial cell proliferation and/or migration would be ideal to accelerate healing.

Differential Diagnosis of Sclerocornea

Sclerocornea, a rare congenital eye disorder, can be challenging to diagnose due to its unique characteristics. The differential diagnosis for sclerocornea involves considering various conditions that may present with similar symptoms.

• Peters Anomaly: A rare form of anterior segment dysgenesis characterized by abnormal cleavage of the anterior chamber, involving the central or entire cornea [4].
• Tears in Descemet's Membrane: A condition where breaks occur in the Descemet membrane, which can be differentiated from sclerocornea using ultrasound biomicroscopy (UBM) [3].
• Ulcers: Corneal ulcers can present with similar symptoms to sclerocornea, but can be distinguished through pharmaceutical history and conjunctival examination [12].
• Metabolic Diseases: Various metabolic diseases, such as congenital glaucoma, limbal dermoids, and corneal opacities, should be considered in the differential diagnosis of sclerocornea [11].
• Congenital Glaucoma: A condition characterized by abnormal development of the anterior chamber angle, which can lead to increased intraocular pressure and vision loss [9].

Mnemonic Devices

To aid in remembering the differential diagnosis for congenital corneal opacities, several mnemonic devices have been proposed:

• STUMPED: Sclerocornea, Tears in Descemet's membrane, Ulcers, Metabolic diseases, Peters anomaly, and other conditions [14].
• S.T.U.M.P.E.D.: Sclerocornea, Tears in Descemet's membrane, Ulcers, Metabolic diseases, Peters anomaly, and other conditions [15].

Genetic Considerations

In cases where sclerocornea is suspected, it is essential to consider genetic referral, particularly for children with systemic findings suggestive of 22q11.2 deletion syndrome [1]. This condition has been associated with sclerocornea in several reported cases.

References:

[1] Binenbaum et al. [3] [4] [9] [11] [12] [14] [15]