Congenital Ptosis In Babies: Causes, Symptoms And Treatment

Ptosis in babies, also known as congenital ptosis or congenital blepharoptosis, causes droopy eyelids since birth. It can occur as a result of weakening in the levator palpebrae superioris muscle, which typically elevates the eyelids. Most babies do not show this symptom immediately after birth, and it is usually detected after a few weeks or months.

Congenital ptosis is a multifactorial condition and can be one-sided (unilateral) or both-sided (bilateral). It may also affect both eyelids with/without symmetry. Surgical correction is necessary if your baby’s normal vision is being hindered by droopy eyelids.

Read on to know about the causes, symptoms, complications, diagnosis and treatment for congenital ptosis.

Possible Causes Of Droopy Eyelids In Babies

Congenital ptosis can be idiopathic (unknown cause) in many babies. Some may have inherited the condition in the family, suggesting chromosomal or genetic abnormalities. In babies with congenital ptosis, the muscle tissue in the levator muscles is often replaced by fat or fibrous tissue. These histological findings indicate that congenital ptosis can be due to developmental defects in the muscle structure (1).

Other possible causes of congenital ptosis may include (2):

• Congenital fibrosis of the extraocular muscles (CFEOM) type 1, 2, and 3 can be associated with ptosis and other eye abnormalities.

• Neuromuscular junction failure or a neurological dysfunction of the levator muscle can cause congenital ptosis in some babies.

• Duane retraction syndrome is a congenital eye movement disorder due to the underdevelopment of cranial nerves. Although this does not affect the nerves of the eyelid, ptosis may occur due to an attempt to abduct the eyes.

• Marcus Gunn jaw-winking syndrome leads to the winking of eyelids on the movement of the jaw. This may occur due to an abnormal link between the oculomotor nerve of the eyelid to trigeminal nerve motor branches supplying the jaw.

• BPES (blepharophimosis, ptosis, and epicanthus inversussyndrome) is an inherited autosomal dominant disorder with blepharoptosis (loosening of the eyelid), blepharophimosis (narrowing of the eyelid), epicanthus inversus (fold in the inner lower eyelids), and telecanthus (widely set eyes).

• Horner syndrome is an oculosympathetic palsy that causes dysfunction of the sympathetic nerves of the eyes and often shows other eye problems with ptosis.

Babies can also have ptosis due to neurological conditions, such as third cranial nerve palsy, birth trauma, myasthenia gravis, and eyelid masses (periorbital tumors), since they can affect nerve and muscle functions of the eyes. This is called acquired ptosis and is often associated with double vision and other abnormalities.

Signs And Symptoms Of Ptosis In Babies

The drooping of the eyelid is the hallmark sign of congenital ptosis in babies. One or both eyelids can be involved. In addition, some babies may have increased tearing (watery eyes). Severe ptosis can interfere with a baby’s vision; the major part of the eye will be covered. It is also common for babies to raise their eyebrows while attempting to lift eyelids (3).

Babies may have abnormal head positions, such as lifting their head and chin up to see underneath the eyelids. If your baby has any signs of ptosis, seek a pediatrician consultation for a detailed diagnosis.

Complications Of Congenital Ptosis

Droopy eyelids may result in the following problems in some babies (4).

• Astigmatism: Pressure on the front of the eyes can distort the eyeball and cause a refractive error.

• Chin-up position: Severe ptosis can cause a baby to keep the chin up to see beneath the eyelids. This may lead to neck issues and developmental delays, which may require surgical correction.

• Muscle contraction in the forehead: Contraction of muscles elevating the upper eyelid (frontalis muscle) is seen in some babies. It may need surgical correction.

• Amblyopia (lazy eye): Babies with droopy eyelids for a long time can develop amblyopia. This can be due to astigmatism or other associated refractive errors known as refractive amblyopia. Some babies may have deprivation amblyopia due to severe ptosis covering the entire eyes.

If left untreated, abnormal head positions to see beneath the droopy eyelids can cause deformations of the head and neck in some babies over the years. Timely interventions can prevent these complications.

Diagnosis Of Ptosis

A complete eye examination can help identify ptosis and its complications in babies. History, vision assessment, refraction, head position, and eyelid position are assessed. Radiographic examinations such as CT scans or X-rays are not usually ordered unless some mass is suspected (4).

Doctors may do follow-up examinations to look for complications and rule out neurological causes of ptosis. Babies may often get referred to various specialists, such as neurologists, depending on the underlying reasons.

Treatment For Ptosis In Babies

Mild ptosis that is not interfering with normal vision development may not require treatment. This should be evaluated and confirmed by a pediatric ophthalmologist. Babies with ptosis require regular monitoring to look for complications, such as deprivation amblyopia, since these cannot be reversed after age seven to ten years.

Ptosis treatment is warranted if the vision is impaired. Early medical therapies and surgeries may help to preserve vision (3). Some may opt for surgeries in late childhood or teenage even if the vision is normal since abnormal eyelid position can have negative psychosocial effects. Decreased field of vision and frontal headache can also be a reason for choosing eyelid correction surgeries later in life.

Surgical Correction Of Congenital Ptosis

Congenital ptosis can be repaired using various surgical methods depending on the degree of eye muscle (levator) function and underlying problems. Functional restore is the primary goal of these surgeries. But surgeons may also consider contour, eyelid height, creases, and symmetry for cosmetic reasons.

Surgical procedures to correct ptosis may include the following (1).

• Levator muscle resection is done to reduce droopy eyelid if the levator muscles have a moderate function.

• Frontalis suspension procedure achieves lid elevation through brow elevation. This procedure is usually recommended if the levator function is low. The lid is secured to the frontalis muscle with silicone bands or rods or through autogenous materials, such as tendons.

• Fasanella-Servat procedure is a method of elevating the upper eyelid by removing tissues from the underside of the lid. It is not commonly done for congenital ptosis correction.

• Muller muscle-conjunctival resection is the suturing of multiple muscles and conjunctiva. This is less often done for congenital ptosis since it is done if the lid has a good response to phenylephrine.

Surgeons may often do procedures on both eyelids even though the baby has one-sided ptosis, for symmetric appearance. Eyelid correction can be delayed if the baby has no vision impairment. Babies may require follow-ups after procedures to look for complications and improvements. Inflammation may occur in some cases due to implanted materials.

References

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Reviewer

Dr. Garima Garg SethMD

     

Dr. Garima Garg Seth is a well-known pediatrician in Gurgaon, having more than 19 years of experience in this field. She has completed her education from topmost organizations in India, including AIIMS, New Delhi; Sir Ganga Ram Hospital, New Delhi; and CMC Vellore.

Read full bio of Dr. Garima Garg Seth