Marcus Gunn Jaw-winking Syndrome Treatment & Management

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

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. ^[22]

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, ^[23] or a standard external levator resection. ^{[10, 24]}

• If the ptosis is moderate to severe, a levator resection may be indicated. Beard advocated performing more resection than normal to avoid undercorrection. ^[10]

• In severe ptosis, a supermaximum (>30 mm) levator resection ^[25] or frontalis suspension is necessary. ^[10]

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. ^[24]

• 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. ^[26]

• Bowyer and Sullivan describe the removal of a portion of levator muscle above the Whitnall ligament through a posterior conjunctival approach. ^[20]

• Dryden et al proposed suturing the transected levator aponeurosis to the arcus marginalis of the superior orbital rim. ^[27] 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. ^[10] 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. ^[28] 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. ^[29] 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. ^{[30, 31]} Their techniques maintain normal eyelid contour, as the levator aponeurotic attachments are left undisturbed.

A retrospective review of cases showed that both tarsofrontalis sling with or without levator excision is equally effective in dampening the eyelid excursion in Marcus Gunn Jaw-winking Syndrome. ^[32]

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.

Complications of surgery to repair jaw-winking ptosis include the following:

• Unacceptable eyelid height or contour

• Exaggerated postoperative wink

• Asymmetry

• Poor wound healing

• Wound infection

• Bleeding or hematoma formation

• Inflammation and granuloma formation around frontalis sling material

While the patient is awake, apply cold compresses to the eyelids for 20 minutes every 1-2 hours for 2-3 days to decrease swelling and bruising.

See the patient postoperatively in 5-7 days for suture removal.

Schedule regular follow-up appointments with a general or pediatric ophthalmologist for any child with preexisting amblyopia.

Marcus Gunn jaw-winking ptosis usually is treated on an outpatient basis.

Antibiotic ophthalmic ointment, such as erythromycin, is prescribed postoperatively, and it should be applied 2-4 times a day along sutures and in the eye for 1 week.