Ankyloblepharon is defined as the adhesion of the edges of the upper
eyelid
An eyelid is a thin fold of skin that covers and protects an eye. The levator palpebrae superioris muscle retracts the eyelid, exposing the cornea to the outside, giving vision. This can be either voluntarily or involuntarily. The human eye ...
with the lower eyelid. Ankyloblepharon must be differentiated from
blepharophimosis
Blepharophimosis is a congenital anomaly in which the eyelids are underdeveloped such that they cannot open as far as usual and permanently cover part of the eyes. Both the vertical and horizontal palpebral fissures (eyelid openings) are shortene ...
, in which palpebral aperture is reduced and there is telecanthus, but the eyelid margins are normal. Another condition similar to ankyloblepharon is
symblepharon, in which the palpebral
conjunctiva
The conjunctiva is a thin mucous membrane that lines the inside of the eyelids and covers the sclera (the white of the eye). It is composed of non-keratinized, stratified squamous epithelium with goblet cells, stratified columnar epithelium ...
is attached to the bulbar conjunctiva. Recognition of ankyloblepharon necessitates systemic examination to detect associated abnormalities such as genitourinary and cardiac abnormalities and syndactyly.
Presentation
Complication
The main complication of congenital ankyloblepharon is
amblyopia. Timely separation of the eyelids is very important to avoid the development of occlusion amblyopia.
Etiology
Ankyloblepharon may be congenital or acquired. The most common type is congenital.
Clinically, ankyloblepharon may be complete, partial, or interrupted. Complete ankyloblepharon is when the eyelids are fused throughout the lid margins. In the partial form, they are joined at one or more points. The interrupted form is also known as ankyloblepharon filiforme adnatum (AFA).
Congenital ankyloblepharon
During
fetal development
Prenatal development () includes the development of the embryo and of the fetus during a viviparous animal's gestation. Prenatal development starts with fertilization, in the germinal stage of embryonic development, and continues in fetal devel ...
, eyelid margins remain fused until the fifth gestational month, and may not be completely separated until the seventh month of gestation. Congenital ankyloblepharon occurs when the lid margins fail to separate at birth. The exact etiology of this condition is unknown. The currently accepted theory is that this condition is due to temporary epithelial arrest and rapid mesenchymal proliferation, allowing union of eyelids at abnormal positions.
Systemic associations
Congenital ankyloblepharon is seen in association with:
*
Hay–Wells syndrome
Hay–Wells syndrome (also known as AEC syndrome; see ''Naming'') is one of at least 150 known types of ectodermal dysplasia. These disorders affect tissues that arise from the ectodermal germ layer, such as skin, hair, and nails.
Genetics
Ha ...
: Ankyloblepharon-Ectodermal dysplasia-Clefting (AEC) syndrome also known as Hay-Wells syndrome is a rare autosomal dominant disorder characterized by ankyloblepharon, ectodermal dysplasia, and cleft palate and/or cleft lip.
* Curly hair–ankyloblepharon–nail disease (CHAND) syndrome: it is a clinical variant of AEC syndrome. It is also known as Baughman syndrome.
[
* ]Trisomy 18
A trisomy is a type of polysomy in which there are three instances of a particular chromosome, instead of the normal two. A trisomy is a type of aneuploidy (an abnormal number of chromosomes).
Description and causes
Most organisms that reprodu ...
(Edwards syndrome): AFA may be seen in association with trisomy 18
A trisomy is a type of polysomy in which there are three instances of a particular chromosome, instead of the normal two. A trisomy is a type of aneuploidy (an abnormal number of chromosomes).
Description and causes
Most organisms that reprodu ...
.
* Popliteal pterygium syndrome (PPS): Popliteal pterygium syndrome (PPS) is a rare autosomal dominant disorder, first described by Trelat in 1869. The clinical features of the syndrome are highly variable and show different combinations of anomalies like cleft palate, cleft lip, lower lip pits or sinuses, popliteal webbing, syndactyly, genitourinary anomalies, nail anomalies, syngnathia, ankyloblepharon, talipes, and digital reduction defects.
Acquired ankyloblepharon
Acquired ankyloblepharon may occur due to trauma
Trauma most often refers to:
* Major trauma, in physical medicine, severe physical injury caused by an external source
* Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event
*Traumatic i ...
or inflammatory conditions. It may be associated with symblepharon also. The following conditions cause ankyloblepharon:
* Chemical burn
A chemical burn occurs when living tissue is exposed to a corrosive substance (such as a strong acid, base or oxidizer) or a cytotoxic agent (such as mustard gas, lewisite or arsine). Chemical burns follow standard burn classification and may c ...
s, thermal burn
A thermal burn is a type of burn resulting from making contact with heated objects, such as boiling water, steam, hot cooking oil, fire, and hot objects. Scalds are the most common type of thermal burn suffered by children, but for adults thermal ...
s, or trauma
Trauma most often refers to:
* Major trauma, in physical medicine, severe physical injury caused by an external source
* Psychological trauma, a type of damage to the psyche that occurs as a result of a severely distressing event
*Traumatic i ...
to the eyes
* Cicatrising diseases such as Stevens–Johnson syndrome
Stevens–Johnson syndrome (SJS) is a type of severe skin reaction. Together with toxic epidermal necrolysis (TEN) and Stevens–Johnson/toxic epidermal necrolysis (SJS/TEN), it forms a spectrum of disease, with SJS being less severe. Erythema ...
or cicatricial ocular pemphigoid
* Inflammatory diseases such as herpes simplex infection
Herpes simplex is a viral disease, viral infection caused by the herpes simplex virus. Infections are categorized based on the part of the body infected.
Herpes labialis, Oral herpes involves the face or mouth. It may result in small bliste ...
or ulcerative blepharitis
* Trachoma
Trachoma is an infectious disease caused by bacterium '' Chlamydia trachomatis''. The infection causes a roughening of the inner surface of the eyelids. This roughening can lead to pain in the eyes, breakdown of the outer surface or cornea of ...
* Cicatricial conjunctivitis
* Mucous membrane pemphigoid: ocular form of mucous membrane pemphigoid may cause ankyloblepharon (4th stage of foster grading system).
Treatment
Lids should be separated by excision of adhesions between the lid margins and kept apart during the healing process. When adhesions extend to the angles, epithelial grafts should be given to prevent recurrences.
History
Ankyloblepharon was first described by von Anmmon in 1841. Ankyloblepharon filiforme adnatum (AFA), the interrupted form of Ankyloblepharon, was first described by Von Hasner in 1881.
Etymology
The word ankyloblepharon is derived from Greek ''ankylos''
ἀγκύλος
'bent, crooked, closed' and ''blepharon''
βλέφαρον
'eyelid'.
See also
* Symblepharon
* Blepharophimosis
Blepharophimosis is a congenital anomaly in which the eyelids are underdeveloped such that they cannot open as far as usual and permanently cover part of the eyes. Both the vertical and horizontal palpebral fissures (eyelid openings) are shortene ...
References
{{Eye pathology
Congenital disorders of eyes
Eye diseases