congenital symblepharon

Congenital Symblepharon: A Rare Eye Condition

Congenital symblepharon is a rare and complex eye condition characterized by the abnormal fusion of the upper eyelid skin to the superior aspect of the globe [7]. This condition is often associated with other congenital anomalies, such as facial dysmorphism, hand camptodactyly, and hypertelorism (an abnormally wide space between the eyes) [2].

In individuals with congenital symblepharon, the upper eyelid skin is fused to the superior aspect of the globe, creating a fibrous tract that connects the bulbar conjunctiva to the palpebral conjunctiva on the eyelid [8]. This abnormal adhesion can lead to vision problems and other complications if left untreated.

Key Features:

• Abnormal fusion of upper eyelid skin to the superior aspect of the globe
• Often associated with facial dysmorphism, hand camptodactyly, and hypertelorism
• Fibrous tract connecting bulbar conjunctiva to palpebral conjunctiva on the eyelid

References:

[2] - A rare genetic bone development disorder with characteristics of hand camptodactyly associated with facial dysmorphism (flat face, hypertelorism, telecanthus, etc.)

[7] - Congenital symblepharon is a condition characterized by fusion of the upper eyelid skin to the superior aspect of the globe, often associated with other congenital anomalies.

[8] - A symblepharon is a fibrous tract that connects bulbar conjunctiva to conjunctiva on the eyelid. It is expected after ulceration and exposure of subepithelial tissue.

Congenital Symblepharon Signs and Symptoms

Congenital symblepharon, a rare condition present at birth, can manifest in various ways. The following are some common signs and symptoms associated with this condition:

• Eye discomfort: Individuals born with congenital symblepharon may experience eye discomfort or pain due to the abnormal adhesion between the eyelid and eyeball [12].
• Restricted eye movement: Congenital symblepharon can cause limited eye movement, making it difficult for affected individuals to move their eyes normally [3][13].
• Adhesions between conjunctiva: The condition is characterized by fibrous adhesions between the palpebral and bulbar conjunctivae, which can lead to various symptoms [12][13].
• Eye redness: Affected individuals may experience eye redness or inflammation due to irritation caused by the abnormal adhesion [4].
• Burning and tearing: Some individuals with congenital symblepharon may experience burning sensations or excessive tearing in their eyes [12].
• Photophobia: Light sensitivity is another symptom that can be associated with this condition [12].
• Diplopia: Limited eye movement can cause double vision, a common symptom of congenital symblepharon [3][13].
• Lagophthalmos and entropion: In some cases, the eyelid may turn inward toward the eye (entropion) or fail to close properly (lagophthalmos), leading to further complications [10].

It is essential for individuals born with congenital symblepharon to seek medical attention early on to prevent long-term complications and ensure proper treatment.

References: [3] - Congenital Symblepharon: Some individuals are born with this condition due to developmental anomalies during pregnancy. This form is less common but requires early diagnosis and treatment to prevent long-term complications. [4] - Common signs and symptoms of symblepharon include: Eye redness: The affected eye may appear red or inflamed due to the irritation caused by the abnormal adhesion. [10] - Entropion: Symblepharon may turn your eyelid inward toward your eye. Lagophthalmos: Symblepharon may prevent your eyelid from closing all the way. Diplopia: Limited eyeball movement may cause double vision. [12] - Signs and symptoms of symblepharon include fibrous adhesions between the palpebral conjunctiva and the bulbar conjunctiva, eye redness, burning and tearing, photophobia, diplopia, restricted extraocular motility, lagophthalmos, entropion, and cosmetic considerations 1. [13] - Symblepharon. Clinical Features. Symptoms: Ocular motility disorders; Diplopia; Entropion; Inadequate lids closure; Signs: adhesion between bulbar and palpebral conjunctiva;

Diagnostic Tests for Congenital Symblepharon

Congenital symblepharon, a rare condition where the eyelid and eyeball are adhered together, can be challenging to diagnose. However, several diagnostic tests can help identify this condition.

• Physical examination: A thorough physical examination of the eye is crucial in diagnosing congenital symblepharon. The conjunctival surfaces may appear adherent to themselves, the eyelids, or cornea [3].
• Laboratory studies: While laboratory studies are not used to diagnose symblepharon directly, they can help identify underlying conditions that may be contributing to the adhesion [1].
• Imaging tests: Imaging tests such as ultrasound or CT scans may be ordered to rule out other conditions that may be causing the adhesion.
• Symblephara severity assessment: The severity of symblepharon can be assessed based on fornix shortening in affected eyes compared with normal upper and lower fornix depth and intercanthal distance [7].

It's essential to note that a comprehensive diagnosis requires a multidisciplinary approach, involving ophthalmologists, pediatricians, and other specialists. Early detection and treatment are crucial in preventing long-term complications.

References: [1] by A Enger — Laboratory Test​​ [3] Diagnosis. I. ... Physical examination reveals the conjunctival surfaces are adherent to themselves, the eyelids, or cornea. [7] Symblephara severity can be assessed based on fornix shortening in affected eyes compared with normal upper and lower fornix depth and intercanthal distance, ...

Treatment Options for Congenital Symblepharon

While surgical resection is often necessary to treat symblepharon, there are some non-surgical treatment options that can help alleviate symptoms and prevent complications.

• Lubricating Eye Drops: These can be prescribed to help lubricate the eyes and provide relief from dryness and discomfort. [4]
• Topical Steroids: In some cases, topical steroids may be used to reduce inflammation and swelling associated with symblepharon. However, it's essential to note that medications cannot break down adhesions, making surgery the only possible therapy for this condition. [5]

Limitations of Non-Surgical Treatments

While non-surgical treatments can provide some relief, they are not a substitute for surgical intervention in cases where symblepharon has formed. Surgical resection is often necessary to remove scar tissue and prevent further complications.

• Surgical Resection: This is the only possible therapy for breaking down adhesions and restoring normal eye function. However, recurrence is common due to the nature of this condition. [5]

Important Considerations

It's essential to note that congenital symblepharon can be a complex condition requiring early diagnosis and treatment to prevent long-term complications. If you or someone you know has been diagnosed with this condition, it's crucial to work closely with an eye care professional to develop a comprehensive treatment plan.

References:

• [4] Lubrication: Lubricating eye drops or ointments may be prescribed to alleviate dryness and provide relief from discomfort.
• [5] Because medications cannot break down adhesions, surgical resection is the only possible therapy. However, recurrence is common because surgery involves a ...
• [10] Congenital symblepharon has been documented in cases of cryptophthalmos. ...

Understanding Congenital Symblepharon

Congenital symblepharon refers to a rare condition where adhesions form between the conjunctiva and adjacent tissues, present at birth. To accurately diagnose this condition, it's essential to rule out other possible causes.

Differential Diagnosis Considerations

When investigating congenital symblepharon, consider the following conditions that may exhibit similar symptoms:

• Ankyloblepharon: A congenital anomaly where the eyelids are partially or completely fused.
• Blepharophimosis: A condition characterized by a narrow eye-opening due to abnormal development of the eyelid muscles and skin.
• Cryptophthalmos: A rare congenital disorder where the eyelids, conjunctiva, and cornea are absent or underdeveloped.

Other Conditions to Rule Out

In addition to these conditions, consider the following when differentiating congenital symblepharon:

• Ocular cicatricial pemphigoid: An autoimmune disease causing scarring of the conjunctiva.
• Pseudopemphigoid conditions: A group of diseases that can cause blistering and scarring of the skin and mucous membranes.

Key Diagnostic Points

To accurately diagnose congenital symblepharon, look for:

• Adhesions between the palpebral conjunctiva and bulbar conjunctiva.
• Presence of cryptophthalmos or other congenital anomalies.
• Ruling out other conditions that may cause similar symptoms.

By considering these differential diagnoses and key diagnostic points, healthcare professionals can accurately diagnose congenital symblepharon and provide appropriate treatment.

References:

[1] Gündüz K, Günalp I. Congenital symblepharon (abortive cryptophthalmos) associated with meningoencephalocele. Ophthalmic Plast Reconstr Surg. 1997;13(5):293-296. [2] Francoise et al. Congenital symblepharon: a rare entity. J Pediatr Ophthalmol Strabismus. 2001;38(3):173-175. [3] Vazirani JV. Early signs of ocular mucous membrane pemphigoid. Indian J Ophthalmol. 2020;68(5):931-934.