Definition

Epicanthus is a condition characterized by a vertical fold of skin at the base of the nose, which causes the sclera or the white part of the eye near the nose to be obscured. This is the most common congenital eyelid condition. Epicanthal folds are a typical feature of East Asian and Central Asian eye shapes and can also be found in some Native American and European populations, such as Scandinavians.

Epicanthus is often associated with various conditions, including congenital ptosis (drooping eyelid), Down syndrome, and blepharophimosis (a condition where the eyelid tissue is shortened both vertically and horizontally).

Causes

Epicanthus is frequently observed in individuals of the Mongoloid race. It can occur independently or in conjunction with other conditions, including:

• Telecanthus (An increased distance between the inner corners of the eyes beyond normal limits).
• Ptosis (drooping of the upper eyelid).
• Injuries to the eyes.
• Caused by congenital disorders, such as:
• Down syndrome: A genetic disorder leading to mental retardation and developmental abnormalities. Children with Down syndrome typically have a Mongoloid facial appearance, short necks, and abnormal ears.
• Fetal alcohol syndrome: A condition caused by maternal alcohol consumption during pregnancy, leading to growth impairments, facial abnormalities, low birth weight, and epicanthal folds. Affected children may also have vision and hearing impairments.
• Turner syndrome: A genetic disorder in females resulting from abnormalities in the X chromosome, often leading to infertility, short stature, and epicanthal folds.
• Noonan Syndrome: A genetic disorder that affects normal development and growth, often accompanied by heart abnormalities and chest deformities.
• Williams syndrome: A genetic disorder characterized by kidney and heart abnormalities, developmental delays, and learning disabilities, with clinical features including short stature, a wide mouth, swollen eyes, and epicanthal folds.
• Age: Some fetuses lose their epicanthal folds between 3-6 months of gestation. Epicanthal folds are commonly seen in young children across various ethnicities, particularly before the nose fully develops.

Risk Factor

Several risk factors may increase the likelihood of epicanthus, including:

• High-risk populations come from Southeast Asia, East Asia, North Asia, Central Asia, and Native American communities. Meanwhile, the low-risk populations come from certain European populations, such as those from England, Russia, and Ireland.
• Advanced maternal age
• History of having a previous pregnancy with congenital defects.
• Alcohol consumption

Symptoms

Parents may notice and seek medical advice for the following symptoms in their children:

• Unusual appearance due to eye folds (aesthetic concerns).
• Eyes appearing crossed-eyes.
• Narrowed eye shape.
• Blurred vision.
• Drooping eyelids (ptosis).
• Disrupted tear flow, potentially leading to eye swelling.
• Lagophthalmos (inability to completely close the eyes).

Diagnosis

Diagnosing epicanthus is a straightforward process. A doctor can establish the diagnosis through physical examination and the use of a slit lamp for detailed inspection.

Epicanthus can be classified according to the type of fold present:

1. Supraciliary Epicanthus (The fold extends from under the eyebrow towards the tear duct near the nose).
2. Palpebral Epicanthus (The fold is equally prominent on both the lower and upper eyelids, similar in appearance to supraciliary epicanthus).
3. Tarsal Epicanthus (The fold runs along the upper eyelid, a type commonly seen in Asians).
4. Inversus Epicanthus (The fold appears on the lower eyelid and runs upwards).

In addition to diagnosing epicanthus, the doctor will investigate the underlying causes to determine the appropriate treatment plan.

Management

Management of epicanthus should be conducted under medical supervision. Most cases do not require treatment and tend to resolve as the child grows. However, persistent epicanthus that does not resolve with age can be corrected surgically. Surgery is usually considered after the child reaches the age of 5 or during puberty. In Asians, special surgical techniques may be needed to preserve ethnic characteristics. The anesthesia for surgery can be general or local, depending on the patient's age. Below are some common surgical procedures for epicanthus:

1. Speath Inverted "V" Method: Suitable for mild cases of epicanthus due to its simplicity and effectiveness.
2. Roveda Procedure: Provides good results in patients with extensive epicanthal folds.
3. Double Z Plasty: Recommended if the abnormality involves both eyelids without telecanthus.
4. Verwey Y-V Plasty: Used for patients with moderate epicanthus.
5. Pang's Procedure: Involves cutting the eyelid, and is also useful in correcting asymmetrical folds resulting from ptosis surgery.

Complications

Although epicanthus itself is not a disease, it can lead to several complications, including:

• Blurred vision.
• Crossed eyes.
• Disruption of tear flow.
• Dry eyes, especially if accompanied by lagophthalmos (inability to close the eyes completely).
• In some children, it may affect appearance, leading to lower self-confidence due to a "mongoloid" facial appearance.

Prevention

There is no specific prevention for epicanthus. Prevention efforts are more focused on managing related conditions, such as congenital diseases associated with epicanthus. Preventive measures include routine prenatal screening tests in pregnancy, avoid alcohol consumption during pregnancy, and consult a doctor if planning a pregnancy, particularly for those with a history of congenital disorders in previous pregnancies or within the family.

When to See a Doctor?

Epicanthal folds are commonly found in children, especially those of Asian descent, and often disappear during or after puberty. However, if the epicanthus persists and interferes with your child's daily activities, you should consult an eye specialist. The doctor may recommend surgical treatment to correct the eyelid shape. Surgical techniques have been successful in treating most cases of epicanthus.

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