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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 57  |  Issue : 2  |  Page : 155-157

A rare case report of congenital bilateral complete ankyloblepharon


Department of Oculoplasty, Regional Institute of Ophthalmology, Chennai, Tamil Nadu, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Savithiri Visvanathan
11/12, Zakkariah Colony, 3rd Street, Choolaimedu, Chennai - 600 044, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_34_19

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  Abstract 


Congenital ankyloblepharon describes an eyelid condition when the fused eyelid folds fail to separate and the child is born with completely or partially joined eyelids. Ankyloblepharon is categorized into three types: complete, partial, and interrupted. We report a case of congenital bilateral complete ankyloblepharon in a 1-day-old female neonate who presented with inability to open her eyes since birth. Congenital complete type of ankyloblepharon is extremely rare, and to our knowledge, this is the first case being reported in India. On examination, no other congenital anomalies were noted. The adhesions were incised, and the eyelids were separated. Early diagnosis and management of congenital ankyloblepharon prevent stimulus deprivation amblyopia, and the child should be screened for associated systemic abnormalities.

Keywords: Ankyloblepharon, congenital, eyelids


How to cite this article:
Mangalanathan H, Visvanathan S, Periasamy G, Ayubkhan FS. A rare case report of congenital bilateral complete ankyloblepharon. TNOA J Ophthalmic Sci Res 2019;57:155-7

How to cite this URL:
Mangalanathan H, Visvanathan S, Periasamy G, Ayubkhan FS. A rare case report of congenital bilateral complete ankyloblepharon. TNOA J Ophthalmic Sci Res [serial online] 2019 [cited 2019 Sep 17];57:155-7. Available from: http://www.tnoajosr.com/text.asp?2019/57/2/155/266389




  Introduction Top


Congenital ankyloblepharon describes an eyelid condition when the fused eyelid folds fail to separate and the child is born with completely or partially joined eyelids.[1] Congenital ankyloblepharon always remained a wonderment because of two reasons: (1) they are rare with an incidence of 4.4/1 lakh births, and to our knowledge, this is the first case of bilateral congenital complete ankyloblepharon being reported in India and (2) they are a deviation from normal embryological development that do not affect physiology of vision if corrected early. Another importance is that it is usually associated with systemic syndromes, for which the child should be screened and managed appropriately.


  Case Report Top


A 1-day-old female neonate was referred with complaints of inability to open her eyes since birth as noticed by her parents. She was the first born of a nonconsanguineous marriage, delivered at 37 weeks of gestation through lower segment cesarean section. The child weighed 3.2 kg at birth. At presentation, the Apgar score was 9/10. Ocular examination of both the eyes revealed adhesions throughout the margins of both the eyelids [Figure 1]. On thorough whole-body examination, no other congenital anomolies were noted. B-scan ultrasonography was done on both the eyes which showed the posterior segment to be normal. Under topical anesthesia using 2% lignocaine gel, with a pediatrician backup, the connecting tissue between the upper and lower eyelids was divided by one cut using Vannas scissors and Lims corneoscleral forceps. The excess tissue was trimmed. No bleeding occurred, and the child showed no signs of distress [Figure 2] and [Figure 3]. During the follow-up period after 2 weeks [Figure 4], the child was able to open and close her eyelids normally.
Figure 1: On examination, adhesions throughout the lid margins in both the eyes were present

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Figure 2: After incising the adhesions

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Figure 3: Immediate postoperative period

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Figure 4: Follow-up after 2 weeks

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  Discussion Top


Ankyloblepharon, the fusion of eyelid margins, is categorized into three types: complete, partial, and interrupted (ankyloblepharon filiforme adnatum wherein single or multiple strands of epithelial tissue are present between the upper and lower eyelids). The histology of these connecting strands has been shown to consist of vascularized central core of blood vessels surrounded by squamous epithelium.[2] The term was first proposed by Joseph Von Hasner in 1881.

Regarding embryological development, the upper eyelid develops from the frontonasal process while the lower eyelid develops from the maxillary process. The epithelial fusion of the eyelid margins occurs at around the 9th week of gestation. The margins usually remain fused until the 5th month. The separation begins to occur at the 7th month.[3] Rarely, the lid margins fail to separate leading to ankyloblepharon.

Clinical features noted are complete/partial adhesion of the upper and lower eyelids, reduced eyelid excursion, shortening of palpebral fissure, etc.

Congenital ankyloblepharon can be associated with syndromes such as Hay-Wells syndrome, CHAND syndrome, Edwards syndrome, and popliteal pterygium syndrome.[4] Other reported systemic associations include hydrocephalus, meningocele, imperforate anus, and bilateral syndactyly. Cardiac problems include ventricular septal defect and patent ductus arteriosus. Ocular association of infantile glaucoma and iridodysgenesis has also been reported.[5]

The differential diagnoses for ankyloblepharon are cryptophthalmos, microphthalmos, and blepharophimosis.

Central ankyloblepharon interferes with vision and causes amblyopia. Medial or lateral ankyloblepharon may restrict the visual fields.

The surgical management of congenital ankyloblepharon depends on its type. Fine filamentous adhesions can resolve spontaneously.[6] Fine bands of adhesions can be broken by forcibly separating the lids or with the use of muscle hook. More severe cases of congenital ankyloblepharon require surgery to separate the lid margins.[6]

The management is surgical by sectioning the lids along the normal division. Since development up to the usual point of eyelid separation has occurred, the eyelid structure and tarsus are usually otherwise normal. A No. 15 blade is used to separate the tissue.

In congenital anklyoblepharon, both visual prognosis and cosmetic appearance are usually very good.


  Conclusion Top


An early diagnosis and management of congenital ankyloblepharon can lead to a prevention of stimulus deprivation amblyopia which can have a devastating effect on the visual acuity of the neonate.

Although absent in our patient, it is important for pediatric ophthalmologists to be aware of the associated systemic abnormalities so that the child can be screened for these, followed up, and managed appropriately to improve the quality of life.

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.



 
  References Top

1.
Holds JB, Chang WJ, Durairaj VD, Foster JA, Gausas RE, Harrison AR, et al. Classification amd management of eyelid disorders. In: Robert B, editor. Orbit, Eyelids, and Lacrimal System: Basic and Clinical Science Course, 2014-2015. Sec. 7. San Francisco: American Academy of Ophthalmology; 2011. p. 155-96.  Back to cited text no. 1
    
2.
Judge HV, Mott WC, Gabriels J. Ankyloblepharon filiforme adnatum. Arch Ophthalmol 1929;2:702-8.  Back to cited text no. 2
    
3.
Akkermans CH, Stern LM. Ankyloblepharon filiforme adnatum. Br J Ophthalmol 1979;63:129-31.  Back to cited text no. 3
    
4.
Martínez-Ortiz A, Garrido-Contreras V, Pérez-Martínez A, Ardanaz-Aldave A, Zubieta-Garciandía A, Elso-Tartas J, et al. Ankyloblepharon filiforme adnatum: Presentation of a case. Pediatr Dermatol 2010;27:207-8.  Back to cited text no. 4
    
5.
Scott MH, Richard JM, Farris BK. Ankyloblepharon filiforme adnatum associated with infantile glaucoma and iridogoniodysgenesis. J Pediatr Ophthalmol Strabismus 1994;31:93-5.  Back to cited text no. 5
    
6.
MacFaul PA. The ocular adnexa. Part 2. Lacrimal orbital and paraorbital diseases. In: Duke-Elders S, editor. System of Ophthalmology. 8th ed. London: Henry Kimpton; 1975. p. 590-91.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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