|Year : 1962 | Volume
| Issue : 1 | Page : 19-21
Ankyloblepharon filiforme adnatum
SP Gupta, RC Saxena
King George's Medical College, Lucknow, India
|Date of Web Publication||18-Mar-2008|
S P Gupta
King George's Medical College, Lucknow
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta S P, Saxena R C. Ankyloblepharon filiforme adnatum. Indian J Ophthalmol 1962;10:19-21
Ankyloblepharon Filiforme Adnatum is a rare congenital anomaly wherein the lid margins are connected by fine bands of extensile tissue which reduce the palpebral fissure by interfering with the movements of the lids. This term was first proposed by V. Hasner (1882). Only few cases have been reported in the literature up till now with or without other associated congenital abnormalities.
| Case Report|| |
Baby R., Male, aged 12 days was admitted to the Ophthalmic section of Gandhi Memorial and Associated Hospitals, Lucknow on 9th September, 1960. This baby was unable to open his eyes as both the lids were adherent on both sides since birth.
Father :-aged 38 years had 'Hare-lip' on left side of upper lip, the plastic repair of which was done at the age of 10 years.
Mother:- aged 34 years is healthy and has no congenital abnormality.
Out of the seven children born before the case under report, four were males, all healthy with no congenital anomaly. Out of the three female children, the eldest one died when six months old but had no congenital abnormality, the second one 15 years old has no congenital abnormality either. The third female child (sixth in chronological order) is 6 years of age, had 'Hare-lip' and two symmetrical bands of Ankyloblepharon Filiforme Adnatum on both sides in the middle third of the lid. The bands were excised at the age of one month.
Baby 'R' is the eighth child of the parents and is a full term baby with normal delivery. The mother suffered from fever for 7 days in the fourth month of pregnancy.
| Examination|| |
A male baby of fairly good health, weighing 7 lbs. has 'Harelip' on left side of upper lip. [Figure - 1]. Systemic examination of the patient did not reveal any abnormality.
Right Eye:-Palpebral Aperture was extremely narrow and lid movements were limited. There were two fine extensile bands of skin attached to the centre of both lid margins anterior to the grey line. These bands were 1 mm. apart and measured 1 mm. in thickness and 3 mm. in length : [Figure - 1]. After excision of bands other structures of the eye were found normal.
Left Eye:-Palpebral aperture was extremely narrow and lid movements were limited. There were two fine extensile bands of skin attached to the middle third of both lid margins anterior to the grey line. These bands were 3 mm. apart and measured 1 mm. in thickness and 3 mm. in length [Figure - 2]. After excision of the bands other structures of the eye were normal.
| Operation|| |
The case was a aerated under general anaesthesia by open ether. The bands were cut near the lid margins and excised. There was slight bleeding which came under control easily.
| Histological Examinations|| |
The band was composed of central vascular connective tissue surrounded by squamous epithelium. The connective tissue was highly cellular. No muscle fibres or signs of inflammation could be found. [Figure - 4].
| Discussion|| |
This is the first case of its kind studied here in the last twenty years of this hospital. This rare condition is known in Ophthalmic literature since 1882 when V. Hasner proposed this term Ankyloblepharon Filiforme Adnatum for this defect. Single, multiple, unilateral and bilateral such bands have been described. Lohlein (1930) has demonstrated the occurrance of multiple hands which may be bilaterally symmetrical (Judge, Mott & Gabriels 1929, Mattsson 1950).
In the family of the case reported out of 8 children the sixth and the eighth children showed the abnormality. The sixth child was a female child and the case described is a male. In literature, so far no sex affinity has been discussed although in this family both the sexes have been affected. Since father had 'Hare-lip' and one of the sisters had 'Hare-lip' and ankyloblepharon these might be some hereditary element functioning in pathogenesis. Moreover the mother developed pyrexia which night have introduced some infection or enhanced the malnutrition.
Varying sizes of bands have been described by different authors, length varying from 1 to 10 mm. and breadth from 0.3 mm. to 6 mm. They were invariably extensile so as to almost double their length or more on stretching.
In the histological study of the bands in this case a highly cellular vascular connective tissue was seen surrounded by squamous epithelium, but no muscle fibres or any sign of inflammation could be seen, [Figure - 4]. In most histological studies of this condition, similar central vascular and cellular connective tissue strands surrounded by pavement epithelium are described. The connective tissue is highly cellular and embryonic in nature but Cordere found muscle fibres and numerous sub-epithelial glands. Bleeding on cutting was evident (Judge, Mott, Gabriels 1929). Wintersteiner could not find any sign of inflammation in the lid structures but a partial failure of Riolan's muscle was evident at the insertion of the bands.
The pathogenesis of this condition is still obscure. Several theories have been advanced till now. V. Hasner (1882) attributed the anomaly to a pathological growth of the skin of inflammatory origin. Wintersteiner on the other hand postulated an epithelial defect in fetal life through which connective tissue grows. This defect he considered due to trauma as by finger nails of the fetus in utero. It would seem much more probable that the condition is a pure aberrance of development due either to a temporary arrest of the growth of the epithelium or to an abnormally rapid proliferation of mesoderm allowing union at certain points of the mesenchyme of the lid folds without epithelial interposition. The present case studied has the following important features :
(i) Father had 'Hare-lip' defect.
(ii) The condition of Ankyloblepharon Filiforme Adnatum was associated with another defect, the 'Hare-lip'.
(iii) His elder sister had the similar defects.
(iv) The mother was not in good health due to multiple pregnancies and suffered from pyrexia of unknown etiology in the fourth month of eighth pregnancy for 7 days.
| Summary|| |
A case of Ankyloblepharon Filiforme Adnatum associated with 'Hare-lip' which had hereditary background is reported. The relevant literature is reviewed.
A possible importance of hereditary factor and malnutrition is being stressed in its etiology and pathogenesis.
| References|| |
Duke-Elder, S., (1952), "Test Book of Ophthalmology", Vol. V. Kimpton London, p. 4665.
Fine, (1933) Arch of Ophthal. 10, 821.
Ida Mann, (1957), "Development Abnormalities of the Eye", Sec. Ed. P. 383, B.M.A.
Judge, Mott and Gabriels, (1929), Arch, of Ophthal. 2, 702.
Mattsson, (1950), Acta. Ophthal. 28, 223.
Oppenheimer, (1924), Amer. J. of Ophthal. 7, 788.
Walsch, Frank B., (1957), "Clinical Neuro-ophthalmology" Sec. Ed. Williams & Wilkins, Baltimore, P. 383.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]