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Case of the week 54 ( May 2012 )

Jaw-winking phenpmenon




The mother of this 6m old infant complains of the disfiguring eye lid movements of her baby which occurs only durin suckling.
She asks for treatment of this disorder


 See video of this patient
 

 

Management of

jaw-winking Syndrome

  • 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.
    • 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, or a standard external levator resection
    • If the ptosis is moderate to severe, a levator resection may be indicated. Beard advocated performing more resection than normal to avoid undercorrection.
    • In severe ptosis, a supermaximum (>30 mm) levator resection or frontalis suspension is necessary.
  • 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.
  • Beard and others have advocated bilateral excision of the levator muscle and bilateral frontalis suspension. 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. 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. 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. Their techniques maintain normal eyelid contour, as the levator aponeurotic attachments are left undisturbed.

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