“Eye-opener moment” – Bilateral neonatal broad-based ankyloblepharon filiforme adnatum

A term male neonate with uneventful antenatal history was referred with the complaint of inability to open both eyes. Systemic assessment of the neonate did not reveal any abnormality. Ocular examination showed partial fusion of bilateral upper and lower eyelids by multiple broad bands, arising from the gray lines [Fig. 1a and c]. These bands interfered with lid opening, resulting in an interpalpebral aperture of only 1.5 mm. Fifteen days later, under sedation, the bands were retracted anteriorly with an iris repositor [Fig. 1b and d] and excised with Vannas scissors at the level of each eyelid margin. As the bands are relatively avascular and devoid of active nerve endings, excision is painless and does not result in active bleeding. Subsequent thorough eye examination did not reveal any other abnormality [Fig. 1e].Figure 1: Ankyloblepharon filiforme adnatum: (a) preoperative image of the right eye; (b) intraoperative image of the right eye; (c) preoperative image of the left eye; (d) intraoperative image of the left eye; (e) postoperative image of both eyesDiscussion Ankyloblepharon filiforme adnatum (AFA), a congenital anomaly of the lid,[1-3] is not visually impairing, but if it is multiple and broad based as in our case, it needs an early excision to prevent amblyopia. Ankyloblepharon may be an isolated sporadic malformation or may be associated with other inherited conditions. It may be associated with central nervous system and/or cardiac anomalies, ectodermal syndromes, cleft lip and/or palate, popliteal pterygium syndrome, and gastrointestinal abnormalities. AFA has been reported to be associated with infantile glaucoma and iridodysgenesis.[4-6] The systemic associations of congenital ankyloblepharon have been reported in following conditions[4-6]: Hay–Wells syndrome: A rare autosomal dominant disorder characterized by ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC syndrome). Curly hair, ankyloblepharon, and nail dysplasia syndrome: An autosomal recessive disorder characterized by ankyloblepharon, ectodermal dysplasia, curly hair, and hypoplastic nails, with absence of cleft lip or palate. It is also known as Baughman syndrome. Edwards’ syndrome or trisomy 18: Edwards’ syndrome or trisomy 18 patients may also have AFA. Rapp–Hodgkin syndrome: An autosomal dominant syndrome characterized by ectodermal dysplasia, cleft lip and/or palate, and mid-facial hypoplasia. Popliteal pterygium syndrome: It is characterized by intercrural webbing of the lower limbs. Conclusion Though we report an isolated AFA, multisyndromic AFA is not uncommon,[4] warranting a thorough systemic evaluation in every infant presenting with AFA. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.

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Journal
Indian Journal of Ophthalmology - Case Reports
Published
2024-10-01
DOI
https://doi.org/10.4103/ijo.ijo_572_24
Primary Topic
Hedgehog Signaling Pathway Studies
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article
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article

“Eye-opener moment” – Bilateral neonatal broad-based ankyloblepharon filiforme adnatum

R Krishnaprasad, Deepti Joshi, Nilima Khochikar, Tejas Atnoor
Indian Journal of Ophthalmology - Case Reports
Hedgehog Signaling Pathway Studies
article

“Eye-opener moment” – Bilateral neonatal broad-based ankyloblepharon filiforme adnatum

R Krishnaprasad, Deepti Joshi, Nilima Khochikar, Tejas Atnoor
article en

Abstract

A term male neonate with uneventful antenatal history was referred with the complaint of inability to open both eyes. Systemic assessment of the neonate did not reveal any abnormality. Ocular examination showed partial fusion of bilateral upper and lower eyelids by multiple broad bands, arising from the gray lines [Fig. 1a and c]. These bands interfered with lid opening, resulting in an interpalpebral aperture of only 1.5 mm. Fifteen days later, under sedation, the bands were retracted anteriorly with an iris repositor [Fig. 1b and d] and excised with Vannas scissors at the level of each eyelid margin. As the bands are relatively avascular and devoid of active nerve endings, excision is painless and does not result in active bleeding. Subsequent thorough eye examination did not reveal any other abnormality [Fig. 1e].Figure 1: Ankyloblepharon filiforme adnatum: (a) preoperative image of the right eye; (b) intraoperative image of the right eye; (c) preoperative image of the left eye; (d) intraoperative image of the left eye; (e) postoperative image of both eyesDiscussion Ankyloblepharon filiforme adnatum (AFA), a congenital anomaly of the lid,[1-3] is not visually impairing, but if it is multiple and broad based as in our case, it needs an early excision to prevent amblyopia. Ankyloblepharon may be an isolated sporadic malformation or may be associated with other inherited conditions. It may be associated with central nervous system and/or cardiac anomalies, ectodermal syndromes, cleft lip and/or palate, popliteal pterygium syndrome, and gastrointestinal abnormalities. AFA has been reported to be associated with infantile glaucoma and iridodysgenesis.[4-6] The systemic associations of congenital ankyloblepharon have been reported in following conditions[4-6]: Hay–Wells syndrome: A rare autosomal dominant disorder characterized by ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC syndrome). Curly hair, ankyloblepharon, and nail dysplasia syndrome: An autosomal recessive disorder characterized by ankyloblepharon, ectodermal dysplasia, curly hair, and hypoplastic nails, with absence of cleft lip or palate. It is also known as Baughman syndrome. Edwards’ syndrome or trisomy 18: Edwards’ syndrome or trisomy 18 patients may also have AFA. Rapp–Hodgkin syndrome: An autosomal dominant syndrome characterized by ectodermal dysplasia, cleft lip and/or palate, and mid-facial hypoplasia. Popliteal pterygium syndrome: It is characterized by intercrural webbing of the lower limbs. Conclusion Though we report an isolated AFA, multisyndromic AFA is not uncommon,[4] warranting a thorough systemic evaluation in every infant presenting with AFA. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest.

Indian Journal of Ophthalmology - Case ReportsVol. 4(4)
Joshi Hospital (IN)
Openalex Percentile: Top 83%
Hedgehog Signaling Pathway Studies
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