ISSN: 2155-9570
Case Report - (2017) Volume 8, Issue 6
Ankyloblepharon is usually a sporadic congenital abnormality in which the upper and lower eyelids are joined together by single or multiple bands of skin tissue. Here we report a case of ankyloblepharon where the baby was having complete cleft palate, syndactyly of second and third toes of both the feet and congenital heart problem (Patent Foramen Ovale with left to right shunt).
Keywords: Ankyloblepharon; Eye lids
Ankyloblepharon is usually a sporadic congenital abnormality in which the upper and lower eyelids are joined together by single or multiple bands of skin tissue. There is partial and complete adhesion of the ciliary edges of superior and inferior eyelids. Strands of fine connective tissue join the upper and lower eyelids can be seen anywhere along the lid but never at lateral and medial canthus [1]. The tissue invariably arises from the grey line anterior to the meibomian gland orifices. Though, it is a rare condition but its presence should alert clinicians to the possibility of other important disorders.
Here we report a case of ankyloblepharon where the baby was having complete cleft palate, syndactyly of second and third toes of both the feet and congenital heart problem (Patent Foramen Ovale with left to right shunt).
A baby boy was born at 39 completed weeks gestational age and weighed 2.9 kg. Pregnancy and delivery was unremarkable. On neonatal examination complete cleft palate (Figure 1) was there and the baby had syndactyly (Figure 2) of second and third toes in both the feet and on eye examination a single band of tissue between left upper and lower eyelids (Figure 3) was seen.
This band was axial, covering the pupil and preventing full opening of the eyelid. There was no suggestive family history of this. There was no other significant family history of any congenital abnormalities. On investigation the patient had patent foramen ovale (PFO with left to right shunt). The band of skin tissue was divided with one cut with scissors under general anesthesia (Figure 4). No underlying ocular abnormalities were noted.
Ankyloblepharon filiforme adnatum (AFA) is a rare but potentially amblyogenic congenital abnormality of the eye lids. AFA was first described by Von Hasner in 1881, in which single or multiple bands of tissues join the upper and lower eyelids. Treatment is done to prevent stimulus deprivation amblyopia as well as to give comfort to the patient. Ozyazgan et al. [2] described treating AFA under intravenous sedation. But in our case we excised the tissue under general anaesthesia as the baby had congenital heart problem. While excising the tissue there was no bleeding and was done with one cut scissor technique.
AFA is important, as it can be associated with several disorders including trisomy 18 (Edward’s syndrome) and infantile glaucoma [3]. Systemic associations are seen [4], which includes, Hay-Wells Syndrome (ankyloblepharon-ectodermal dysplasia-clefting syndrome), poplitealpterygium syndrome (characterised by webbing of knee), curly hairankyloblepharon- nail dysplasia syndrome, and cleft lip and palate. Rosenman et al. [5] proposed a classification of AFA into four groups. Bacal et al [6] suggested a fifth group; AFA in association with chromosomal abnormalities.
Ankyloblepharon filiforme adnatum is a rare but can lead to stimulation amblyopia if not treated early. In our case the patient had ankyloblepharon filiforme adnatum with cardiac anomaly and cleft palate and syndactyly.