|Year : 1970 | Volume
| Issue : 2 | Page : 64-68
Epicanthus inversus - a review of two cases
BN Consul, DP Sharma, HN Chhabra, HM Sarin
Department of Ophthalmology, Sawai Man Singh Medical College and Hospital, Jaipur, Rajasthan, India
B N Consul
Department of Ophthalmology, Sawai Man Singh Medical College and Hospital, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Consul B N, Sharma D P, Chhabra H N, Sarin H M. Epicanthus inversus - a review of two cases. Indian J Ophthalmol 1970;18:64-8
Epicanthus inversus is a rare condition which was noted first by Axenfeld and Bronsl and later described in detail by Braum  , Callahan  , and Johnson  . It is characterised by a small skin fold that arises in the lower lid, and extends upward and inwards in a curve leading to more or less lateral displacement of the inner canthus (referred to as "Telecanthus" by Mustarde  ). The condition is always associated with ptosis, usually resulting from weak but nonparalysed levator muscle and a certain degree of blepharophymosis. The palpebral fissure may slant obliquely downwards from the medial canthal angle.
In this paper we are presenting two cases of epicanthus inversus because of extreme rarity of the condition and to comment on the surgical techniques used by us.
CASE NO. 1
The first of the two cases was a 7 year old female child brought with the complaints of (1) inability to open both the eyes since birth. She used to turn her head backwards while looking forward, and (2) narrow palpebral fissure.
She was a full term normally delivered child. No such abnormality could be detected in her other family members. The patient was well developed with average intelligence. General and systemic examination revealed no other abnormality or mal-development.
Ocular examination - [Figure - 1] showed the following features:- The upper lids were flat and without the normal skin creases. The lower lid skin was also tight. There was ptosis of both the lids with minimum demonstrable levator action. Both the palpebral fissures were 19 x 3 mm. (blepharophymosis), instead of average 28 x 9 mm. The distance between the two medial canthi was 35 mm (telecanthus), instead of average 30 mm. The puncta were laterally displaced and were almost touching the corneal margin. The visual acuity and fundi were normal in both the eyes.
Bilateral correction of epicanthus inversus was done under general anaesthesia by Mustarde's rectangular skin flap technique as described below: -
The incisions were given as shown in [Figure - 2] (top) A rectangular flap (A) was outlined and elevated in the lower lid. A similar flap (B) was outlined in the upper lid, and the nasal flaps (C) and (D) were outlined and cut ([Figure - 2] bottom). The skin flaps were undermined. To prevent the possible folding back of the flaps on each side, sutures were placed through each flap, to which a semifine clamp was then fixed..
A supramid suture was inserted through the medial canthal ligament at its point of junction with the tarsal plate and was sutured to the fascia and periosteum of the nasal bone. The medial shifting of the canthal angle permitted the transposition of the flaps (A) and (C), and (B) and (D) to lie flat ([Figure - 2] bottom), even around the multiple curves of the intercanthal area (D). After transposition, all the four skin flaps were sutured by inter-rupted 6-0 black silk sutures ([Figure - 2] bottom). The operation was completed with a pressure dressing over the medial canthal region. This dressing and all the sutures were removed on the sixth postoperative day and a very gratifying improvement was achieved [Figure - 3]. The child was only 7 years old and since we had taken about two hours in this procedure, the correction of ptosis was left to a later stage.
CASE NO. 2
The second patient was a five year old female child, admitted to the same hospital with the complaints of a small palpebral fissure and inability to open her eyes since birth. She was a full term normally delivered child. No such abnormality could be detected in her other family members. The child was well built with average intelligence. There was no other maldevelopment or defect in the body. Ocular examination - Showed the following features:- The distance between the two medial canthi was 34 mm. Length of the palpebral fissure was 16 mm while its width was 4 mm. There was ptosis of both the lids with little amount of levator action. The visual acuity and fundi were normal.
Under general anaesthesia the epicanthus in both the eyes was corrected by Spaeths  technique. The postoperative period was uneventful and the functional results were satisfactory. The patient was advised to come four months later for the correction of ptosis, but she did not turn up.
| Comments|| |
Epicanthus inversus affects mostly the male children and has dominant transmission. Both of our cases were females and without hereditary background. Braum considered it to be due to pressure from the amniotic bands.
Unlike the other types, the epicanthus inversus tends to improve very little with age. For its correction a major reconstruction of the lids is imperative and it is difficult to do this properly in very small children (Johnson). It is very rare for amblyopia to develop from the blepharophymosis alone, nor any spinal abnormalities in these children. The older the child, the better the results. Ofcourse, we almost always have to operate before the child goes to school because of psychologic problems which may arise from the taunts of his associates. Therefore mostly operations are done between the age of 3 and 5 years. We got very good results by operating the cases at 5 and 7 years of age.
Several operative techniques have been tried to correct this deformity (Spaeth's operation, Johnson's modification of the Blair procedure, and Mustarde's rectangular skin flap procedure. We got very good results by Mustarde's technique in our first case and satisfactory results by Spaeth's procedure in the second case. This is because Mustarde's  rectangular flaps transpose more skin than triangular flaps from areas where it is not needed to areas where it is needed, and secondly because the only vertical scars crossing the intercanthal line are those that are close to the lid margins, so that a secondary fold developing at a later stage will occur much less than with other techniques. The latter may eventually be supplanted by Mustarde's  method.
One cannot correct the ptosis properly until the epicanthus has been repaired and the vertical pull of the epicanthal fold relieved. Further-more the skin of these children is quite inelastic (Waardenburg, Franceschetti and Klein  , called it `stiff ptosis'). Therefore, ptosis is generally corrected three to four months after the correction of blepharophymosis by resection of the levator muscle or by means of autogenous fascia lata slings.
Regardless of the procedure used, one cannot of course make the unfortunate patient with the syndrome of epicanthus inversus, blepharophymosis and ptosis appear completely normal, but improvement of a considerable degree can be expected.
| Summary|| |
Two cases of the syndrome of epicanthus inversus, blepharophymosis and ptosis are reported with special reference to their surgical management, one by Mustarde's method and the other by that suggested by Spaeth.
| References|| |
Axenfeld and Brons.: Joseph's Hb.d. kosmetik, Leipzig, 518, (1912).
Braum.: Klin. Mbl. Augenheilk, 68, 110, (1922).
Callahan, Alston.: `Correction of Blepharophymosis". Plastic and reconstrictive surgery of the eye and adnexa. Edited by Troutman, R. C., p. 169-184. Butterworths, Washington (1962).
Johnson, C. C.: Surgical repair of the syndrome of epicanthus inversus, blepharophymosis and ptosis. Arch. Ophth. 71,
Johnson, C. C.: Epicanthus. Amer. J. Ophth. 66,
Mustarde, J. C. (1966). Quoted by Johnson, C. C., Amer. J. Ophth. 66, 939, (1968).
Spaeth, B.: Principles and practice of Ophthalmic surgery Ed. IV,
p. 400. Philadelphia, Lea and Febiger (1948).
Waardenburg, P. J., Franceschetti, A. C., Klein, D.: Genatics and Ophthalmology, p. 415. Assen, Netherlands, Royal Van. Gorcum (1961).
[Figure - 1], [Figure - 2], [Figure - 3]