Marcus Gunn jaw-winking syndrome is associated with strabismus in approximately 50%–60% of cases; usually superior rectus or double elevator palsy.
The incidence of anisometropia is reported to be 5%–25% (refractive difference between two eyes of ≥ 1.25 diopters of sphere or 1 diopter of cylinder).
Amblyopia occurs in 30%–50%; almost always secondary to strabismus or anisometropia, and only rarely due to occlusion by a ptotic eyelid.
Although it has been suggested that jaw winking improves over time, it is more likely that patients stop seeking care as they get older or learn to compensate for and mask the wink response.
Treat any amblyopia aggressively with occlusion therapy and/or correction of anisometropia prior to any consideration of ptosis surgery.
Consider eyelid surgery only when parents or patient and the surgeon agree about whether the most cosmetically objectionable condition is the ptosis or wink, or a combination of both.
If the jaw wink is small or cosmetically insignificant, it can be ignored in the treatment of the ptosis.
- Mild ptosis with good levator function: Müller muscle and conjunctival resection, Fasanella-Servat procedure, or standard external levator resection
- Moderate to severe ptosis with moderate to good levator function: External levator resection
- Severe ptosis with moderate to good levator function: Super maximum (> 30 mm) levator resection or frontalis suspension
- Severe ptosis with poor levator function: Frontalis suspension
If the jaw wink is significant, any attempt to repair the ptosis without addressing a significant jaw wink will result in an unacceptable exaggeration of the aberrant eyelid movement to a level above the superior corneal limbus.
- In these cases, a partial ptosis correction can be performed limiting the exaggeration of the wink.
- In most cases, elimination of levator function and resuspension of the eyelid to the brow is necessary.
- There are many techniques described for the correction of jaw-winking ptosis, reflecting an ongoing controversy regarding the surgical management of this condition:
- Many techniques for ablating levator function
- Excision of levator muscle via anterior approach
- Standard trans-eyelid anterior dissection
- Incise levator aponeurosis from anterior tarsal plate.
- Dissect superiorly and posteriorly to just below Whitnall's ligament:
- Blunt dissect from Mueller's muscle
- Release the medial and lateral horns
- Gentle bipolar cautery as needed
- Clamp just above Whitnall's ligament with a hemostat (approximately 20 mm above tarsal end of aponeurosis).
- Cauterize with bipolar cautery.
- Excise over cauterized region.
- The above approach can also be performed using a ptosis clamp to isolate and control the distal edge of the levator.
- Alternatively, a 1 cm section of levator can be excised ABOVE Whitnall's ligament:
- Use muscle hooks to isolate the levator above Whitnall's.
- Use hemostat and bipolar cautery to define and excise a 1 cm segment above Whitnall's ligament.
- Levator fixation to the superior arcus marginalis
- Proceed as above.
- Do not excise muscle superiorly.
- Anchor distal edge of levator to the superior arcus marginalis.
- Allows for reattachment if needed in the future (theoretically)
- Levator excision via posterior approach (Bowyer, OPRS 2004)
- 4-0 silk traction sutures through anterior lid margin
- Evert over Desmarres retractor.
- Incise conjunctiva and Mueller's muscle above the superior tarsal border.
- Blunt dissect Mueller's from the underside of the levator muscle.
- Incise levator aponeurosis posterior to anterior:
- Confirm in the preaponeurotic space by identifying preaponeurotic fat.
- Proceed with excision of levator muscle as above.
- Some advocate bilateral levator ablation and frontalis suspension for optimal symmetry (Beard, AJO 1965).
- Parents are rarely accepting of this approach.
- Others propose ablating levator on affected side only and then bilateral frontalis suspension (Callahan, AJO 1973).
- Parents are rarely agreeable to this approach either.
- Others advocate unilateral levator excision and frontalis suspension on affected side only (Kersten, OPRS 2005).
- Parents are more accepting of this approach.
- There have been a few small series advocating the use of an orbicularis oculi flap to correct the jaw wink (Tsai, Ann Plastic Surg 2002).
Other management considerations
- Superior rectus palsy
- Superior rectus muscle resection: only in absence of inferior rectus muscle restriction
- Because superior rectus muscle is loosely bound to overlying levator, the upper eyelid will be pulled inferiorly during resection, exacerbating any ptosis already present.
- Double-elevator palsy
- Deficit in elevation in all fields of gaze: result of superior rectus and inferior oblique palsy and/or inferior rectus restriction
- A combined superior rectus and inferior oblique palsy usually requires a Knapp procedure.
Common treatment responses, follow-up strategies
- Lower success rate than equivalent ptosis without jaw wink
- Success rates difficult to compare due to variety of surgical approaches, unilateral vs. bilateral, and so on
- 10% recurrence rate (or higher) of ptosis in some series (Demirci, Ophthalmol 2012)
- Standard post-op protocols and follow-up as indicated for cases of congenital ptosis
- Management of persistent jaw wink after surgery
- Many authors recommend waiting one year before readdressing.
- Basic management involves progression to next "degree" of jaw-wink surgery.
- For example, if levator was not initially excised, proceed to excision.
- If levator was excised, the patient may need more aggressive re-excision or release of scar adhesions.
- Management of under-corrected ptosis
- Defer re-operation for at least 3 months
- Except if there is a risk of occlusive amblyopia
- Under-corrected levator resection (if jaw wink not severe)
- Repeat levator resection
- Proceed to frontalis suspension (any approach)
- Under-corrected frontalis suspension with adequate frontalis use
- Revision of primary procedure
- Revision with different suspension material or different approach
- Frontalis or levator suspension
- Under-corrected frontalis suspension with poor frontalis use
- Proceed with contralateral frontalis suspension