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Congenital Ptosis Treatment & Management

  • Author: Donny W Suh, MD, FAAP; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Mar 06, 2015
 

Medical Care

Observation is only required in mild cases of congenital ptosis if no signs of amblyopia, strabismus, and abnormal head posture are present.

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.

Head posture should be carefully examined. If the patient acquires a chin-up posture due to the worsening of ptosis, surgery may be indicated.

The patient should be checked for astigmatism due to the compression of the droopy eyelid.

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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 surgeon has an opportunity through this procedure to produce symmetry in lid height, contour, and eyelid crease for better cosmesis.[4, 5, 6, 7, 8]

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.[9]

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.

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 prefer to 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.[10, 11, 12, 13] 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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Contributor Information and Disclosures
Author

Donny W Suh, MD, FAAP Chief of Pediatric Ophthalmology and Strabismus, Children's Hospital and Medical Center; Associate Professor, Department of Ophthalmology and Visual Sciences, Truhlsen Eye Institute, University of Nebraska Medical Center

Donny W Suh, MD, FAAP is a member of the following medical societies: American Academy of Ophthalmology, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus, American Medical Association, Iowa Medical Society, National Eye Care Project

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Michael J Bartiss, OD, MD Medical Director, Ophthalmology, Family Eye Care of the Carolinas and Surgery Center of Pinehurst

Michael J Bartiss, OD, MD is a member of the following medical societies: American Academy of Ophthalmology, North Carolina Medical Society, American Academy of Pediatrics, American Association for Pediatric Ophthalmology and Strabismus

Disclosure: Nothing to disclose.

Acknowledgements

I would like to thank my family for their patience and unremitting support.

References
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  3. Wabbels B, Schroeder JA, Voll B, Siegmund H, Lorenz B. Electron microscopic findings in levator muscle biopsies of patients with isolated congenital or acquired ptosis. Graefes Arch Clin Exp Ophthalmol. 2007 Oct. 245(10):1533-41. [Medline].

  4. Bagheri A, Aletaha M, Saloor H, Yazdani S. A randomized clinical trial of two methods of fascia lata suspension in congenital ptosis. Ophthal Plast Reconstr Surg. 2007 May-Jun. 23(3):217-21. [Medline].

  5. Philandrianos C, Galinier P, Salazard B, Bardot J, Magalon G. Congenital ptosis: long-term outcome of frontalis suspension using autogenous temporal fascia or fascia lata in children. J Plast Reconstr Aesthet Surg. 2009 Apr 10. [Medline].

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  8. Lee MJ, Oh JY, Choung HK, Kim NJ, Sung MS, Khwarg SI. Frontalis sling operation using silicone rod compared with preserved fascia lata for congenital ptosis a three-year follow-up study. Ophthalmology. 2009 Jan. 116(1):123-9. [Medline].

  9. Bernardini FP, Devoto MH, Priolo E. Treatment of unilateral congenital ptosis. Ophthalmology. 2007 Mar. 114(3):622-3. [Medline].

  10. Yoon JS, Lew H, Lee SY. Bell's phenomenon protects the tear film and ocular surface after frontalis suspension surgery for congenital ptosis. J Pediatr Ophthalmol Strabismus. 2008 Nov-Dec. 45(6):350-5. [Medline].

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  13. Salour H, Aletaha M, Bagheri A. Comparison of Mersilene mesh and autogenous fascia lata in correction of congenital blepharoptosis: a randomized clinical trial. Eur J Ophthalmol. 2008 Nov-Dec. 18(6):853-7. [Medline].

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  16. Malone TJ, Nerad JA. The surgical treatment of blepharoptosis in oculomotor nerve palsy. Am J Ophthalmol. 1988 Jan 15. 105(1):57-64. [Medline].

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Chin-up posture due to congenital ptosis of the left eye.
Congenital ptosis of the left eye partially obstructing the left pupillary axis.
Congenital ptosis of the right eye.
 
 
 
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