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Marcus Gunn Jaw-winking Syndrome Treatment & Management

  • Author: Barbara L Roque, MD, DPBO, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Apr 06, 2016
 

Medical Care

If amblyopia is encountered, treat aggressively with occlusion therapy and/or correction of anisometropia prior to any consideration of ptosis surgery.

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Surgical Care

As with any patient who requires eyelid surgery, first address associated strabismus.

Superior rectus palsy

Superior rectus palsy can be corrected by resecting the superior rectus muscle but only in the absence of inferior rectus restriction.

Since the superior rectus is loosely bound to the overlying levator, the upper eyelid will be pulled inferiorly during resection, exacerbating any ptosis already present. This can be addressed during the subsequent ptosis repair.[21]

Double elevator palsy

Double elevator palsy manifests as a deficit in the elevation of the globe in all fields of gaze.

It may be the result of superior rectus and inferior oblique palsy and/or inferior rectus restriction.

Inferior rectus restriction may be suggested by the following:

  • Positive forced ductions in elevation
  • Normal force generations in upgaze indicating an absence of superior rectus or inferior oblique palsy
  • Poor or absent Bell phenomenon on the affected side

Inferior rectus restriction is treated by recession of the inferior rectus muscle.

A combined superior rectus and inferior oblique (double elevator) palsy requires a transposition procedure to displace the medial and lateral recti muscles superiorly (Knapp procedure).

Other considerations

Consider eyelid surgery only when the parents (or the patient) and the surgeon agree about whether the most cosmetically objectionable condition is the ptosis or the jaw-winking or whether it is a combination of both.

Many techniques are described for the correction of jaw-winking ptosis, reflecting the ongoing controversy regarding the surgical management of this condition.

If the jaw-winking is cosmetically insignificant, it can be ignored in the treatment of the ptosis, as follows:

  • If the ptosis is mild, the patient may elect not to proceed with surgery. If correction is desired, perform a Müller muscle and conjunctival resection (MMCR), a Fasanella-Servat procedure, [22] or a standard external levator resection. [9, 23]
  • If the ptosis is moderate to severe, a levator resection may be indicated. Beard advocated performing more resection than normal to avoid undercorrection. [9]
  • In severe ptosis, a supermaximum (>30 mm) levator resection [24] or frontalis suspension is necessary. [9]

Although the amount of ptosis and synkinetic eyelid movement is variable, those patients with more severe ptosis tend to have the worse aberrant upper eyelid movement.

The jaw-wink is considered cosmetically significant if it is 2 mm or more.

Any attempt to repair the ptosis without addressing the jaw-winking would result in an exaggeration of the aberrant eyelid movement to a level well above the superior corneal limbus, which would be unacceptable to the patient.

If the jaw-wink is significant, ablation of the levator and resuspension of the eyelid to the brow are necessary. Several techniques have been suggested to obliterate levator function, which effectively dampens the aberrant eyelid movement, as follows:

  • Bullock advocated complete excision of the levator aponeurosis and muscle all the way to the orbital apex. [23]
  • Dillman and Anderson argued that removal of a portion of the levator muscle above the Whitnall ligament (ie, myectomy) is adequate to obliterate its function without extensive dissection and damage to eyelid structures. [25]
  • Bowyer and Sullivan describe the removal of a portion of levator muscle above the Whitnall ligament through a posterior conjunctival approach. [19]
  • Dryden et al proposed suturing the transected levator aponeurosis to the arcus marginalis of the superior orbital rim. [26] This technique not only effectively deactivates the muscle but also allows the procedure to be reversed, if necessary.

Beard and others have advocated bilateral excision of the levator muscle and bilateral frontalis suspension.[9] While this approach almost completely eliminates the wink and arguably results in better symmetry, it is often difficult to persuade the parents and the patient to perform surgery on and effectively damage the normal contralateral levator muscle.

Satisfactory and predictable results also can be obtained after only unilateral levator excision on the affected side, combined with bilateral frontalis suspension. This leaves the normal functioning levator muscle to elevate the nonptotic eyelid in primary position but produces a lag in downgaze for improved symmetry.

Kersten et al advocate unilateral levator muscle excision and frontalis sling only on the affected side.[27] If the postoperative result is judged to be unsatisfactory, the parents or the patient can opt for further surgery to the contralateral side. Any amblyopia and strabismus should first be addressed, as there may be insufficient drive to lift the disinserted eyelid.

Islam et al described a technique of dissecting a frontalis flap hinged superiorly through a suprabrow incision that is then brought down into an eyelid crease incision.[28] The frontalis flap is used to suspend the ptotic eyelid after extirpation of the levator muscle.

Lemagne and Neuhaus described techniques that involve transection of the involved levator followed by transposition of the distal segment to the brow, which effectively suspends the eyelid to the frontalis muscle.[29, 30] Their techniques maintain normal eyelid contour, as the levator aponeurotic attachments are left undisturbed.

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Consultations

Consult an oculoplastic surgeon or a strabismologist if the referring physician is uncomfortable with these procedures.

Forewarn the anesthesiologist that patients with Marcus Gunn jaw-winking ptosis are at a greater risk of developing arrhythmias during eyelid surgery.

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Contributor Information and Disclosures
Author

Barbara L Roque, MD, DPBO, FPAO Senior Partner, Roque Eye Clinic; Chief of Service, Pediatric Ophthalmology and Strabismus Section, Department of Ophthalmology, Asian Hospital and Medical Center; Active Consultant Staff, International Eye Institute, St Luke's Medical Center Global City

Barbara L Roque, MD, DPBO, FPAO is a member of the following medical societies: American Academy of Ophthalmology, American Association for Pediatric Ophthalmology and Strabismus, American Society of Cataract and Refractive Surgery, Philippine Society of Cataract and Refractive Surgery, Philippine Academy of Ophthalmology, Philippine Society of Pediatric Ophthalmolo

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

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.

Sean M Blaydon, MD, FACS Fellowship Program Director, Texas Oculoplastic Consultants

Sean M Blaydon, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Association of Military Surgeons of the US, International College of Surgeons US Section, Pan-Pacific Surgical Association, Texas Medical Association

Disclosure: Nothing to disclose.

References
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Congenital left upper eyelid ptosis.
Marcus Gunn jaw-winking with left upper eyelid retraction on opening of the mouth.
 
 
 
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