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Case Report

Ankyloblepharon Filiforme Adnatum: Report of Two Cases

Chandana Chakraborti, Krittika Pal Chaudhury, Jayanta Das, Arnab Biswas1

ABSTRACT Access this article online Website: Ankyloblepharon filiforme adnatum (AFA) is a rare congenital anomaly characterized by partial www.meajo.org or complete adhesion of upper and lower , usually seen as an isolated finding but often DOI: associated with other anomalies or a well‑defined syndrome. We report two cases of AFA who 10.4103/0974-9233.129780 presented at a tertiary eye care center of West Bengal. Family history of consanguinity was Quick Response Code: absent. One baby had abnormal tuft of hair over the small of the back. No other congenital abnormalities were detected in any of them. The adhesions of the eyelids were divided by a number 15 blade after crushing with mosquito forceps in both cases without any anesthesia. Subsequent ocular examinations following separation and during follow‑up revealed normal function, ocular motility, and fundus. In a case of AFA, timely separation of the eyelids is crucial to avoid the development of occlusion . Cases of AFA reported in the literature are reviewed.

Key words: Ankyloblepharon, Congenital Anomalies, Ectodermal Dysplasia

INTRODUCTION on July 15, 2006. He was unable to open his eyes at birth due to adherent upper and lower eyelids [Figure 1a]. The nkyloblepharon filiforme adnatum (AFA) is characterized baby weighing 2.7 kg was born at 40 weeks gestation by Aby partial or complete adhesion of the ciliary edges of spontaneous vaginal delivery with episiotomy to a 28‑year‑old the upper and lower eyelids at the gray line, by single or second gravida. The antenatal, intranatal, and postnatal periods multiple fine bands of extensible tissue, which reduces the were uneventful. The mother denied taking any drugs except palpebral fissure by interfering with the movement of the iron and vitamin supplements. There was no history of any lids. It should be differentiated from ankyloblepharon by the X‑ray exposure. The previous sibling, a 2‑year‑old female, was fact that in the latter, lid margins are directly fused together.1 healthy. There was no family history of congenital anomalies It is amblyogenic and its association with other congenital or consanguinity. abnormalities may account for high mortality and morbidity. Here, we present two newborns with AFA who were treated On examination the baby was noted to have bilateral partially successfully by separation of the eyelids. Case reports are rare fused eyelids. Apart from this, he was perfectly healthy without in the literature with or without other associated congenital any other congenital abnormalities identified after a thorough abnormalities.2‑9 pediatric assessment. A more detailed ophthalmic examination revealed two fine extensile bands of skin measuring about 1 mm CASE REPORTS in breadth and 3 mm in length between the upper and lower eyelid in the left eye. Similar bands, one centrally and one near Case 1 the lateral canthus were detected in the right eye. In both the A 4‑day‑old male infant was admitted to the ophthalmic eyes, the adhesions were divided by a number 15 blade after department of Calcutta National medical college and hospital being crushed with mosquito forceps. There was minimal

Department of , Calcutta National Medical College and Hospital, Kolkata, 1B B Eye Foundation, Kolkata, India Address for Correspondence: Dr. Chandana Chakraborti, A/1/1, Pearl Apartment, 50B, Kailas Bose Street, Kolkata - 700 006, India. E‑mail: [email protected]

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Chakraborti, et al.: Ankyloblepharon Filiforme Adnatum

bleeding [Figure 1b]. No sedation or local anesthetic was inheritance of varying degrees of penetrance. Sporadic cases required. Posterior surface of eyelids, ocular motility, anterior have, however, also been described.4 No sex affinity has been segment, and fundus were normal. discussed to date.2

Case 2 The fusion of eyelids is a normal stage in human development. A 2‑day‑old male infant was admitted to the eye ward of The developing eyelid margins remain fused until the fifth our hospital on April 2, 2011 with similar signs as in gestational month but may take up to the seventh month of case 1 [Figure 2a and b]. Family history and antenatal history gestation to be completely separated.5 Fusion seen at birth as was unremarkable. On examination, fine extensible bands similar in AFA is abnormal. to as in case 1 were found in both eyes. Systemic examination did not reveal any abnormalities except a tuft of hair over the The length of the bands varies from 1 to 10 mm and breadth lumbosacral area proximal to the cleavage [Figure 2c]. The varies from 0.3 to 0.5 mm and are invariably extensible. patient was managed as in case 1. The bands always lie between the cilia and orifices of the tarsal gland. The band is always composed of a central vascular Both infants presented for follow up at one month and no connective tissue strand surrounded by pavement epithelium. abnormality was detected. The connective tissue is usually highly cellular and embryogenic in nature, but Cordero found muscle fibers and numerous DISCUSSION sub‑epithelial glands in the histological study of the band. When the bands are cut, bleeding may occur.1 First described by Von Hasner in 1881, AFA is a rare benign congenital anomaly, usually associated with multiple and Pathogenesis of the condition is disputed. Various theories include complex malformations, suggestive of an autosomal dominant a simple defect in separation, pathologic growth of the skin of inflammatory origin, an epithelial defect in fetal life through which connective tissue grows, most likely due to trauma, for example, fingernail trauma in utero. But the most accepted theory is that of pure aberrance of development, due either to a temporary arrest of the growth of epithelium or more probably, an abnormally rapid proliferation of mesoderm allowing union at certain points of the mesenchyme of the lid folds without 1 a epithelial interposition. It is usually a solitary malformation of sporadic occurrence, but can occur in an autosomal dominant pattern associated with cleft lip and palate in most familial cases. In some patients, it appears as a part of Edwards’ syndrome (Trisomy 18), Hay-Wells syndrome (a variant of ectodactyly-ectodermal dysplasia-cleft lip‑palate syndrome), popliteal b syndrome (characterized by intercrural webbing of the lower Figure 1: (a) Several extensible bands of tissue connecting the eyelid margins of the right and left eye in case 1 (b) Separated eyelids following surgical procedure limbs), and curly hair‑ankyloblepharon‑nail dysplasia (CHANDS). in case 1 It may also be reported in association with hydrocephalus,

a b c Figure 2: (a) Ankyloblepharon filiforme adnatum in case 2 (b) Extensible band of tissue connecting the eyelid margins in left eye in case 2 (c) Tuft of hair over the small of the back in case 2

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Chakraborti, et al.: Ankyloblepharon Filiforme Adnatum

meningocele, imperforate anus, bilateral syndactyly, infantile REFERENCES , and cardiac problems such as persistent ductus arteriosus and ventricular septal defect.6 An unusual report of 1. Duke-Elder S. System of Ophthalmology. Vol. 3, Part 2, London: a multiple malformation syndrome with AFA, with cleft lip and Henry Kimpton; 1964. p. 869. 2. Gupta SP, Saxena RC. Ankyloblepharon filiforme adnatum. palate, bilateral popliteal pterygia, bilateral complete syndactyly Indian J Ophthalmol 1962;10:19‑21. nd rd of 2 and 3 toes and hypoplastic nails, accessory nipple, and 3. Akkermans CH, Stern LM. Ankyloblepharon filiforme adnatum. partially descended testis has also been reported.4 Br J Ophthalmol 1979;63:129‑31. 4. Modi AJ, Adrianwalla SD. A multiple malformation syndrome In both the cases we could not find any causative factor for the with ankyloblepharon filiforme adnatum, with cleft lip and palate. Indian J Ophthalmol 1985;33:129‑31. defect as the perinatal and family histories were unremarkable. 5. Sharkey D, Marlow N, Strokes J. Ankyloblepharon filiforme In case 2, the tuft of hairs over the lumbosacral area may be adnatum. J Pediatr 2008;152:594. explained by ectodermal dysplasia reported in literature.6 6. Gruener AM, Mehat MS. A newborn with ankyloblepharon filiforme adnatum: A case report. Cases J 2009;2:8146. This report demonstrates the simplicity in treating the 7. Williams MA, G White ST, McGinnity. Ankyloblepharon filiforme adnatum. Arch Dis Child 2007;92:73‑4. condition and the importance of early intervention to avoid 8. Ioannides A, Georgakarakos ND. Management of the development of amblyopia. ankyloblepharon filiforme adnatum. Eye (Lond) 2011; 25:823. 9. Jain S, Atkinson AJ, Hopkisson B. Ankyloblepharon filiforme Moreover, considering the pattern of inheritance, genetic adnatum Br J Ophthalmol 1997;81:705.

counseling of the parents of the affected offspring may be Cite this article as: Chakraborti C, Chaudhury KP, Das J, Biswas A. suggested. However, the major practical importance of this Ankyloblepharon filiforme adnatum: Report of two cases. Middle East Afr J anomaly is, perhaps, to alert the physician as to the presence of Ophthalmol 2014;21:200-2. other associated congenital anomalies. Source of Support: Nil, Conflict of Interest: None declared.

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