Ankyloblepharon Filiforme Adnatum

Ankyloblepharon Filiforme Adnatum

Article
Eye & Vision
Kids' Zone
+2
Contributed byKrish Tangella MD, MBAApr 21, 2022

What are the other Names for this Condition? (Also known as/Synonyms)

  • AFA (Ankyloblepharon Filiforme Adnatum)
  • Ankyloblepharon Filiforme Congenitum
  • Interrupted Ankyloblepharon

What is Ankyloblepharon Filiforme Adnatum? (Definition/Background Information)

  • Ankyloblepharon is a medical condition involving the eyelids, in which both the lower and upper eyelids are either partially or completely fused to each other. But for this abnormality, the anatomical structure and functioning of the eyelids are normal
  • Ankyloblepharon Filiforme Adnatum (AFA) is a form of ankyloblepharon, in which the eyelids are attached at different points through multiple extensible tissue strands, along the lid margin. This form is also known as Interrupted Ankyloblepharon
  • It is a congenital condition (i.e., present at birth) that may occur in isolation, or form part of certain genetic disorders called Mendelian disorders. Ankyloblepharon Filiforme Adnatum may also form part of many other hereditary disorders such as cleft lip and cleft palate, Hay-Wells syndrome, and CHAND syndrome
  • A treatment of Ankyloblepharon Filiforme Adnatum involves treating any underlying causative condition; the eyelid abnormality may be resolved through a simple surgery. In a majority of cases, the prognosis of isolated cases is good with treatment

Nevertheless, it is particularly important to note that the presence of Ankyloblepharon Filiforme Adnatum should serve as a pointer for a complete evaluation (including physical health exams and family medical history assessment) to exclude any associated congenital abnormalities in the newborn child.

Who gets Ankyloblepharon Filiforme Adnatum? (Age and Sex Distribution)

  • Ankyloblepharon Filiforme Adnatum is typically a congenital condition that is diagnosed following the birth of the child
  • Both males and females may be affected
  • Worldwide, individuals of all races and ethnicity are prone to developing the condition

What are the Risk Factors for Ankyloblepharon Filiforme Adnatum? (Predisposing Factors)

Ankyloblepharon Filiforme Adnatum is seen along-with a host of syndromes and conditions such as:

  • Acro-dermato-ungual-lacrimal-tooth (ADULT) syndrome
  • Bilateral syndactyly
  • Curly Hair, Ankyloblepharon and Nail Dysplasia (CHAND) syndrome
  • Ectrodactyly ectodermal dysplasia and cleft lip/cleft palate (EEC) syndrome
  • Edwards syndrome (trisomy 18 syndrome)
  • Hay-Wells syndrome
  • Hydrocephalus
  • Imperforate anus
  • Limb-mammary syndrome (LMS)
  • Meningocele
  • Patent ductus arteriosus (PDA) 
  • Popliteal pterygium syndrome
  • Rapp-Hodgkin syndrome
  • Ventricular septal defects

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Ankyloblepharon Filiforme Adnatum? (Etiology)

Ankyloblepharon Filiforme Adnatum (AFA) is an eye condition in which the upper and lower eyelids are partially and irregularly fused along their margins.

  • Typically, the baby’s eyes remain closed until the fifth/sixth month of gestation, which is part of normal development
  • The eyelids separate around the seventh month, but sometimes may fail to separate along the eyelid margins fully or partially, due to some fetal development issue

Ankyloblepharon Filiforme Adnatum usually occurs in isolation (sporadic), but sometimes may form part of a syndrome. Thus, AFA may serve as an early indicator of other systemic abnormalities in the baby that calls for further investigation.

What are the Signs and Symptoms of Ankyloblepharon Filiforme Adnatum?

In Ankyloblepharon Filiforme Adnatum (AFA), no significant eye signs and symptoms are noted apart from compromised vision, unless the condition forms part of a syndrome. The signs and symptoms include:

  • Partial vision
  • Decreased eyelid movement
  • Pseudoexotropia - eye alignment appears to be turned outward, even though they are straight
  • A single eye or both the eyes may be involved

Additional signs and symptoms of the underlying/associated condition, if any, may be noted.

In AFA, the extreme corners of the eyelids are usually spared; the strands are not present on the periphery. The strands are seen to originate from behind the eyelashes on the upper and lower eyelids.

How is Ankyloblepharon Filiforme Adnatum Diagnosed?

Typically, Ankyloblepharon Filiforme Adnatum may indicate the presence of other systemic conditions, which may need to be investigated. The following observations and tests may be undertaken by the healthcare provider:

  • Physical examination and medical history evaluation
  • Assessment of the presenting signs and symptoms
  • General eye exam: A simple eye exam can help the ophthalmologist detect the condition. However, further testing may be necessary to diagnose the underlying cause (if any present)
  • Imaging studies, including CT and MRI scans of the body, may be undertaken based on the healthcare provider’s evaluation, in order to rule out other conditions
  • Other tests and procedures to diagnose the associated condition, if any present, is important

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Ankyloblepharon Filiforme Adnatum?

The lack of early treatment of Ankyloblepharon Filiforme Adnatum may result in complications due to atrophy from lack of usage of the eye. The complications may include:

  • Restricted field of vision
  • Amblyopia or lazy eye
  • Infantile glaucoma
  • Iridogoniodysgenesis
  • Cosmetic concerns
  • Decreased quality of life

Children with underlying/associated conditions may present additional complications.

How is Ankyloblepharon Filiforme Adnatum Treated?

  • The treatment of choice for Ankyloblepharon Filiforme Adnatum is surgery, which can help resolve the eye condition
  • However, treating the symptoms and managing any associated condition/disorder (if any present) is also important
  • Rarely, in some children, the connecting bands of the eyelid are known to release spontaneously

How can Ankyloblepharon Filiforme Adnatum be Prevented?

Currently, it is not possible to prevent Ankyloblepharon Filiforme Adnatum in many cases. However, the following factors may be considered to lower one’s risk for the same:

  • Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
  • If there is a family history of a systemic/syndromic condition, then genetic counseling will help assess risks, before planning for a child

What is the Prognosis of Ankyloblepharon Filiforme Adnatum? (Outcomes/Resolutions)

  • The overall prognosis of Ankyloblepharon Filiforme Adnatum (AFA) depends on the severity of the underlying condition and the treatment response
  • Sporadic cases generally have a good prognosis following proper (simple) surgical management of the condition. In some cases, AFA resolves on its own without any treatment

Additional and Relevant Useful Information for Ankyloblepharon Filiforme Adnatum:

Please visit our Eye & Vision Health Center for more physician-approved health information:

https://www.dovemed.com/health-topics/vision-center/

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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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