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Ankyloblepharon – Eyelid Fusion Explained

Ankyloblepharon refers to the partial or complete fusion of the upper and lower eyelids. This congenital or acquired condition can impair vision and often requires surgical treatment.

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Things worth knowing about "Ankyloblepharon"

Ankyloblepharon refers to the partial or complete fusion of the upper and lower eyelids. This congenital or acquired condition can impair vision and often requires surgical treatment.

Definition

Ankyloblepharon is a medical term describing the partial or complete adhesion of the upper and lower eyelid margins. The eyelids are joined by tissue bridges or scar tissue, resulting in a narrowed or completely closed palpebral fissure (the opening between the eyelids). The condition may be congenital (present at birth) or acquired later in life.

Causes

Ankyloblepharon can result from several different causes:

Congenital: Abnormal development during the embryonic phase, often associated with genetic syndromes such as Hay-Wells syndrome (AEC syndrome), popliteal pterygium syndrome, or CHAND syndrome.
Acquired: Scarring following severe eye injuries, burns, chemical injuries, or infections (e.g., Stevens-Johnson syndrome, ocular pemphigoid, or trachoma).
Iatrogenic: Rarely, as a complication following ocular surgery or medical procedures.

Symptoms

The symptoms depend on the extent of the fusion:

Reduced or absent opening of the palpebral fissure
Visual impairment or complete loss of vision in severe cases
Amblyopia (lazy eye) in children if the eye does not receive adequate visual stimulation
Cosmetic disfigurement
Dryness or irritation of the ocular surface

Diagnosis

Diagnosis is typically established through a thorough ophthalmological examination. The following methods are used:

Inspection of the palpebral fissure and assessment of the extent of fusion
Slit-lamp examination to evaluate the conjunctiva and cornea
Visual acuity testing
In children: screening for amblyopia
Genetic testing if an underlying syndrome is suspected

Treatment

Treatment of ankyloblepharon depends on the severity and underlying cause of the fusion:

Surgical Treatment

Surgical lysis (separation of the fused eyelid tissue) is the primary treatment. The goal is to restore a functional palpebral fissure. Depending on the extent of the condition, additional reconstructive procedures may be required, such as mucous membrane grafting or skin grafting.

Treatment of the Underlying Condition

In acquired forms, treating the underlying disease (e.g., immunosuppressive therapy for ocular pemphigoid) is essential to prevent recurrence of the fusion.

Prevention of Amblyopia

In children, visual development must be closely monitored after surgery. Amblyopia prevention strategies, such as patching of the unaffected eye, may be necessary to promote normal visual development.

Prognosis

The prognosis largely depends on the timing of diagnosis and treatment. When surgical correction is performed early in childhood, visual impairment can often be avoided or minimized. In acquired forms, the risk of recurrence is linked to the severity and control of the underlying disease.

References

Kanski, J. J. & Bowling, B. - Clinical Ophthalmology: A Systematic Approach, 8th Edition, Elsevier (2016).
Hay, R. J. et al. - AEC syndrome (ankyloblepharon-ectodermal defects-cleft lip/palate), Journal of Medical Genetics, PubMed (2020).
World Health Organization (WHO) - International Classification of Diseases, ICD-11, available at: https://icd.who.int

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