Congenital Ptosis (Drooping Eyelid) Treatment & Management

Updated: May 21, 2019
  • Author: Donny W Suh, MD, FAAP; Chief Editor: Edsel B Ing, MD, MPH, FRCSC  more...
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Treatment

Medical Care

Mild cases of congenital ptosis can be observed if there are no amblyopia, strabismus, or abnormal head posture.

Depending on the severity of the congenital ptosis, patients should be monitored every 3-12 months for signs of amblyopia due to congenital ptosis.

External photographs can be helpful in monitoring patients and their head posture. If a child with previous chin-up head posture stops this behavior, amblyopia should be suspected.

To prevent amblyopia, children with severe ptosis should have their lid taped up for several hours of the waking day while awaiting surgical repair. Children with ptosis should also be checked for astigmatism.

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Surgical Care

Congenital ptosis has physical, functional, and psychological consequences. The method of repair depends on treatment goals, the underlying diagnosis, and the degree of levator function. Although the primary reason for the repair is functional, the lid height, contour, and eyelid crease should be symmetrized, when feasible. [7, 8, 9, 10, 11, 12]

Surgical correction of congenital ptosis can be undertaken at any age depending on the severity of the disease. Earlier intervention may be required if significant amblyopia or ocular torticollis is present. Severe cases of ocular torticollis may delay mobility in infants and toddlers because of the balance problems from extreme chin-up head posture. If intervention is not urgent, surgery is often delayed until age 3-4 years. Waiting until this age allows for more accurate measurements preoperatively. [13]

Surgery for ptosis in patients with a history of dry eyes, seventh cranial nerve palsy, or significant extraocular muscle abnormalities, such as severe Graves ophthalmopathy, double elevator palsy, or progressive external ophthalmoplegia, should be approached with great caution to avoid exposure keratopathy following the surgery.

Levator muscle resection

This procedure is the shortening of the levator-aponeurosis complex through a lid-crease incision. The skin incision is hidden either in the existing lid fold or in a new lid fold created to match that of the contralateral eyelid.

Moderate levator function must be present to offer a chance for correction with a levator resection. If the levator function is greater than 4 mm but less than 6 mm, a levator resection of greater than or equal to 22 mm is recommended. If the levator function is 6-8 mm, a levator resection of 16-18 mm is indicated. If the levator function is greater than 8 mm, a levator resection of 10-13 mm is indicated.

Contraindications: An external levator resection is not indicated when the levator function is less than 4 mm. In such cases, a long-term surgical outcome may result in undercorrection. Poor Bell phenomenon (limited elevation of the eye), reduced corneal sensitivity, or poor tear production can produce exposure keratopathy. Levator-tightening procedures accentuate jaw-winking and lagophthalmos upon downgaze.

Frontalis suspension procedure

This procedure is designed to augment the patient's lid elevation through brow elevation. Frontalis suspension procedures produce lagophthalmos in most cases. Some surgeons may perform a bilateral suspension procedure for severe unilateral congenital ptosis to obtain symmetry.

The procedure is indicated when the levator function is less than 4 mm.

Relative contraindications are poor Bell phenomenon (limited elevation of the eye), reduced corneal sensitivity, or poor tear production, which can produce exposure keratopathy. If surgery is still indicated, these patients need close postoperative follow-up care to avoid corneal exposure, infection, corneal ulcer and amblyopia.

Several materials are available to secure the lids to the frontalis muscles. [14, 15, 16, 17] These materials include the following:

  • Autogenous fascia lata: Autogenous fascia lata can be obtained from the leg of patients older than 3 years.

  • Preserved (tissue bank) fascia lata

  • Nonabsorbable suture material (eg, 2-0 Prolene, Nylon (Supramid) or Mersilene)

  • Silicone bands, silicone rods

  • ePTFE (expanded Poly Tetra Fluoro Ethylene), Gore-Tex

  • Autogenous materials used less frequently include palmaris longus tendon and temporalis fascia.

Patients may not be able to close their eyelids during sleep from a few weeks to several months following surgery. Families must be warned of this outcome before the operation. The problem of open lids during sleep improves with time; however, aggressive lubrication is needed to avoid exposure keratopathy.

Fasanella-Servat procedure

The upper lid is elevated by removing a block of tissue from the underside of the lid. This tissue includes the tarsus, conjunctiva, and Müller muscle. This procedure is not commonly performed for cases of congenital ptosis.

Mueller muscle–conjunctival resection

This surgery is chosen if the eyelid has had a good response to phenylephrine. The conjunctiva and the Müller muscle are marked off, clamped, and sutured. The tissues are resected. Then, the conjunctival layer is closed. This procedure is not commonly performed for cases of congenital ptosis.

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Consultations

Patients with congenital ptosis may have other conditions that need to be addressed. These conditions include amblyopia, strabismus, craniofacial abnormalities, and other neurologic findings. The following consultations may be appropriate depending on the associated findings:

  • Pediatric ophthalmologist

  • Pediatric oculoplastic service

  • Pediatric neurologist

  • Cardiologist (if mitochondrial disorder suspected)

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Diet

Normal

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Activity

As tolerated

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Complications

Complications associated with the frontalis suspension procedure for congenital ptosis repair include the following:

  • Granuloma: If suspension materials are not placed well beneath the skin, granuloma formation may occur. Granulomas should be treated conservatively because they tend to eventually resolve.

  • Lid asymmetry

  • Overcorrection with exposure keratopathy and dry eyes

  • Undercorrection: Suspension materials may dissolve or break. Suture material may tear through soft tissue. Undercorrected congenital ptosis repair may require repeat surgery.

  • Infection

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Long-Term Monitoring

Patients who underwent surgery for congenital ptosis are initially monitored every 2-4 weeks for signs of exposure keratopathy, infection, granuloma formation, and overcorrection and undercorrection. External photographic documentation can be helpful in monitoring patients.

Following the surgery, visual acuity, head posture, and refractive error should be carefully monitored. Any residual amblyopia should be treated aggressively.

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