Epiblepharon

Epiblepharon is a congenital eyelid condition in which excess orbicularis muscle and horizontal skin near the margin of the eyelid override the upper or lower eyelid margin. In epiblepharon, a horizontal fold of the skin and pretarsal orbicularis override the lid margin, causing the eyelashes to turn inwards causing ocular irritation and corneal erosion ¹. Additionally, keratopathy may develop because of prolonged corneal contact by lashes or frequent rubbing of the eyes. Epiblepharon is a common eyelid anomaly in East Asian and Hispanic children, and often involves lower lids bilaterally ². The prevalence reported in oriental infants varies from 46 to 52.5 % in children younger than 1 year ³. In a study of 4449 Japanese children aged 3 months to 18 years, Noda et al. ⁴ reported that the prevalence of epiblepharon was 10%. This is similar to the prevalence of epiblepharon in a Singaporean study (9.5%) ⁵. Epiblepharon is much less prevalent in Non-East Asian populations, such as Indians and Malays ⁶. Epiblepharon tends to spontaneously disappear within the first 2 years of life in Asian children as the child undergoes facial bone growth, resulting in vertical skin and muscle extension and greater tension on the retractors ⁴.

The cause of this congenital eyelid anomaly is likely due to inadequate lower eyelid retractor development, where there is a lack of fenestrating fibers from the retractor aponeurosis which inserts under the skin. It can also be secondary to pretarsal orbicularis muscle that inserts abnormally close to the eyelid margin. Epiblepharon exhibits a familial tendency in an autosomal dominant pattern and can also present in patients with prominent cheeks.

In the evaluation of patients with epiblepharon, a history of tearing, photophobia, chronic ocular irritation, eye rubbing, and mucous discharge is commonly elicited. The patient may report tearing or a constantly wet and mattered eye. Symptoms may be exacerbated in downgaze, which maximizes ciliary-corneal contact. Decreased visual acuity with significant astigmatism may be present as a consequence of frequent eyelid squeezing or rubbing in response to foreign body sensation. The eyelid and margin exam will reveal a redundant fold of eyelid skin and underlying orbicularis present bilaterally, typically involving the medial one-third to one-half of the upper or lower eyelid. This prominent fold may result in the vertical misdirection of a few or many cilia towards the ocular surface, especially along the nasal half of the lower eyelid. A slit-lamp biomicroscopic examination is performed to determine if the eyelashes are rubbing against the conjunctiva or cornea. Signs of keratitis include conjunctival injection, epithelial defects that stain with fluorescein dye, corneal infiltrates, and corneal neovascularization.

It is well known that almost all epiblepharon cases are self-limited by the age of four or five years ⁷. Meanwhile, a conservative treatment with eye lubricants is indicated for preventing ocular surface damage. Surgery is only indicated when there is significant corneal injury caused by the rubbing of the eyelashes and usually involves the removal of the skin and some pre-tarsal orbicularis muscle in the affected eyelid. Although the surgery is almost always successful and complications are unusual, under correction or recurrence rates range from 4.9 to 23 % and the potential risk of the surgical and anesthetic procedures should be considered ⁸.

Epiblepharon causes

The cause of epiblepharon has not yet been established. There are various possible causes of epiblepharon. Some authors consider that the skin and pretarsal orbicularis muscle are weakly attached to the tarsus below, thus raising a skin fold near the lid margin and pushing the eyelashes toward the cornea ². Other authors have proposed that epiblepharon is due to a congenital absence of the insertion of lower-eyelid retractors to the skin and orbicularis muscle or an insertion very close to the lid margin ⁹. Others support the combination of orbicularis muscle hypertrophy with an extra skin fold ¹⁰. In addition, others have suggested failure of interdigitation of septae in the subcutaneous plane, failure of the lid retractor to gain access to the skin, and hypertrophy of the orbicularis muscle as possible causes ¹¹. However, no definite consensus has been reached on the cause of epiblepharon ¹. One of these hypotheses, hypertrophy of the orbicularis muscle, was not supported by a histological study ¹¹.

Epiblepharon symptoms

Children with congenital epiblepharon usually presents irritative signs and symptoms due to the ocular surface eyelash contact. Symptomatic treatment with ocular lubricants is usually indicated. When the symptoms persist or corneal complications occur despite the use of lubricants, surgical treatment is indicated.

Epiblepharon treatment

Congenital epiblepharon is self-limited in most cases at around two years of age. As the prevalence of epiblepharon decreases with age, epiblepharon that is mild or present at an early age may be conservatively treated with eye lubricants to prevent ocular surface damage ¹². Other options include waiting for the child to grow older and hoping for spontaneous resolution, or waiting for the epiblepharon to worsen to warrant surgical correction.

Naik et al ¹³ treated a 4-month-old infant with epiblepharon with medial eyelashes touching with an injection of 0.2 ml of hyaluronic acid gel (Juvederm Ultra) in the sub-orbicular plane in the valley above the abnormal skin fold. He observed an immediate out-turning of the eyelid, which persisted until the fourth month.

Chen et al ⁹ injected a single 12.5 IU of botulinum toxin A (Dysport ®) into the medial orbicularis muscle portion in the lower eyelid epiblepharon patients successfully improves the clinical signs and symptoms. Chen et al ⁹ demonstrated in their epiblepharon patients that botulinum toxin A improves the eyelid skin fold, the area of corneal touch by cilia and corneal erosion area. These changes were seen since the first week of application and remained during the whole study period (24 weeks).

Deka et al ¹⁴ used botulinum toxin A for the treatment of lower lid entropion. In his study, three children with congenital entropion were included. The author stated that botulinum toxin is a safe and effective procedure for correction of senile entropion and some cases of congenital entropion, and no complications or side effects ofthis treatment were noticed.

Indications for epiblepharon surgical repair include persistent epiblepharon with symptoms, keratopathy and astigmatism attributed to epiblepharon ¹⁵. Although the surgery is almost always successful and complications are unusual, under correction or recurrence rates range from 4.9 to 23 % and the potential risk of the surgical and anesthetic procedures should be considered ⁸.

Epiblepharon surgery

The underlying principle of surgical correction is to effect an external rotation of the cilia away from the eye. Various techniques have been used to treat epiblepharon and surgical approaches include the lid crease and capsulopalpebral fascia repair ¹⁶, modified Hotz procedure ¹⁷, Quickert everting suture technique ¹⁸ and cilia rotational suture with minimal skin excision. However, regardless of the technique that is adopted, the medial most portion of the lower eyelids needs to be addressed to ensure successful surgery and prevent recurrence ¹⁹. Combined epicanthoplasty is suitable for this purpose, but the resultant unsightly scar might be problematic for growing children. For medial correction of epiblepharon, great care should be taken to excise the proper amount of excessive tissue that is dragged by the epicanthal fold, avoiding canalicular damage.

Recurrence of epiblepharon after surgical repair is also troublesome, especially at the medial portion of the lower eyelids ²⁰. Woo et al ²¹ previously reported a 7.7% recurrence rate after epiblepharon surgery using a rotating suture technique. Chang et al. ²⁰ reported a recurrence rate of 15.9% and an under-correction rate of 9.1% after a modified Hotz procedure.

References

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