Marcus Gunn Jaw-Winking Phenomenon : A Review

Delhi Journal of Ophthalmology
Major Review
Marcus Gunn Jaw-Winking Phenomenon :
A Review
Dewang Angmo, Mandeep S. Bajaj, Neelam Pushker, Supriyo Ghose
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi
Marcus Gunn jaw-winking phenomenon is the most common
form of congenital synkinetic neurogenic ptosis. In this
synkinetic phenomenon, the unilaterally ptotic eyelid elevates
with jaw movements. The movement that most commonly
causes elevation of the ptotic eyelid is lateral mandibular
movement to the contralateral side. This phenomenon is
usually first noticed by the mother when she is feeding or
nursing the baby. This article presents a review of Marcus
Gunn jaw-winking phenomenon including clinical features,
pathophysiology and treatment modalities.
In 1883 Robert Marcus Gunn described a 15yr girl with a
peculiar type of congenital ptosis that included an associated
winking motion of affected eyelid on the movement of jaw1.
This synkinetic jaw-winking phenomenon now bears its name.
Figure2: Patient with Marcus Gunn Jaw-Winking phenomenon. (A)
22 yrs female with unilateral upper eyelid ptosis as part of Marcus
Gunn phenomenon. (B) Left upper eyelid raises with the jaw
movement to opposite side.
Figure 1: Patient with Marcus Gunn Jaw-Winking phenomenon.
(A) 5year old child with unilateral upper eyelid ptosis as part of
Marcus Gunn phenomenon. (B) Elevation of concomitant upper
eyelid with mouth opening
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The Marcus Gunn phenomenon is known variously as jawwinking (a misnomer as eyelid rises rather than falls) and more
descriptively, as pterygoid-levator synkinesis2. The Marcus
Gunn phenomenon has been associated with congenital
blepharoptosis with an incidence of 4-6% [2,3,4]. Acquired
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forms have been described after eye surgery, trauma, post
Bells palsy and pontine tumors[2]. Spontaneous remission of
the acquired form may be expected, whereas the congenital
form persists (no improvement with age)[12]. Patients with
Marcus Gunn jaw-winking phenomenon have a variable
degree of blepharoptosis in the resting and primary position.
Although Marcus Gunn jaw-winking syndrome is usually
unilateral[12-13] it can present bilaterally in rare cases.
2) Functional Interference
1. Irritation of normally dormant connection
2. Disinhibition of pre-existing phylogenetically more
primitive mechanisms (Ascher): This is thought to
explain why individuals who are not affected will often
open their mouth while attempting to widely open their
eyes to place eye drops
3. Spread of impulses by irradiation
The characteristic feature of the phenomenon is that the
raising; and not winking of the affected eyelid is synchronous
with and proportionate to the opening of the mouth. The wink
reflex consists of a momentary upper eyelid retraction or
elevation to an equal or higher level than the normal fellow
eyelid upon stimulation of the ipsilateral pterygoid muscle
12-13.
3) Atavistic Reversion
1. In fish a strong associated movement of jaw opening and
eye opening i.e., deep muscle contracting and superficial
muscle relaxing. Thus a weak levator may only elevate
the lid when its antagonist, the orbicularis (superficial
muscle) is reflexly relaxed by jaw opening (external
pterygoid-deep muscle contraction )
2. EMG study suggested dysfunction in the midbrain and
brainstem
This response is followed by a rapid return to a lower position.
The amplitude of the wink tends to be worse in downgaze.
This rapid, abnormal motion of the eyelid can be the most
disturbing aspect of the jaw-winking syndrome.
MARCUS GUNN JAW-WINKING PHENOMENON
The wink phenomenon i.e., retraction of the ptotic lid occurs
in conjunction with stimulation of pterygoid muscle, which is
elicited by opening the mouth, thrusting the jaw to the contra
lateral side, jaw protrusion, chewing, smiling or sucking. This
wink phenomenon is often discovered early, as the infant is
bottle-feeding or breastfeeding[14].
Jaw-winking ptosis is almost always sporadic, but familial
cases with an irregular autosomal dominant inheritance
pattern have been reported[15].
Pathophysiology
A complete explanation has not yet been advanced to elucidate
the rationale of jaw-winking phenomenon. Various theories
have been hypothesized [15,16].
1) Aberrant connection
This hypothesis is favored by most authors, though they differ
in opinion as to the location of the aberration
1. Cortical or sub cortical connections
2. Internuclear connections or faulty distribution in the
posterior longitudinal bundle
3. Infranuclear connection exists between motor branches
of the trigeminal nerve (CN V3) innervating the external
pterygoid and the fibers of superior division of the
oculomotor nerve (CN III) that innervates the levator
muscle of the upper lid
4. Peripherally - some CN V fibers may reach the levator via
the auriculo- temporal nerve
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Marcus Gunn Jaw-Winking Phenomenon : A Review
Measurement of Mgjwp
The amount of jaw-winking is the excursion of the upper
eyelid with synkinetic mouth movement. It is measured with
a millimeter ruler. Jaw-winking is assessed as [2,10] : Mild <
2mm ; Moderate 2-5mm; Severe ≥ 6mm
Frequency
Approximately 50% of blepharoptosis cases are congenital.
Incidence of Marcus Gunn jaw-winking syndrome among this
population is approximately 4-5% [12,13].
Associations
1. Ocular
1. Strabismus (50%-60%)[9]
1. Superior Rectus Palsy-25%
2. Double Elevator Palsy-25%
2. Anisometropia (5%-25%)[9]
Incidence of anisometropia among patients with Marcus
Gunn jaw-winking syndrome is reported to be 5-25%.
3. Amblyopia (30-60%)[9]
Almost always secondary to strabismus or anisometropia,
and only rarely, is due to occlusion by a ptotic eyelid.
2. Systemic
Systemic anomalies in association with Marcus Gunn
phenomenon are rare.
1. Cleft lip/ Cleft palate
2. CHARGE Syndrome reported in association with bilateral cases.
3. Renal calculi (Awan 1976)
Schultz and Burian (1960) reported a case of MGP associated
with several systemic malformations. These included
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Marcus Gunn Jaw-Winking Phenomenon : A Review
ectrodactly, bilateral pes cavus with ankle varus, spina bifida
occulta, bilateral undescended testis and supernumerary
incisors.
Race
No known racial predilection exists.
Sex
Early reports showed jaw-winking ptosis to be more prevalent
in females than in males; however, larger case series have
shown an equal prevalence among males and females4, 9.
Age
Marcus Gunn jaw-winking syndrome is usually evident at
birth. The winking phenomenon is often first noted by the
parents when the infant is feeding.
Treatment
1. Medical Care
If amblyopia is encountered, treat aggressively with occlusion
therapy and/or correction of anisometropia prior to any
consideration of ptosis surgery.
2. Surgical Care
As with any patient who requires eyelid surgery, first address
associated strabismus.
1. 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.
2. 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
1. Positive forced duction in elevation
2. Normal force generations in up gaze indicating an absence of superior rectus or inferior oblique palsy
3. Poor or absent Bells 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’s
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Delhi Journal of Ophthalmology
procedure).
3.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 jawwinking or whether it is a combination of both.
4. Many techniques are described for the correction of jawwinking ptosis, reflecting the ongoing controversy regarding
the surgical management of this condition.
5. 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 Muller muscle and
conjunctival resection (MMCR), a Fasanella-Servat procedure
or a standard external levator resection[14,18].
If the ptosis is moderate to severe, a levator resection may be
indicated. Beard advocated performing more resection than
normal to avoid undercorrection[13].
In severe ptosis, a super maximum (>30 mm) levator resection
or frontalis suspension is necessary [19].
6. 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.
7. The jaw-wink is considered cosmetically significant if it is
2 mm or more [2].
8. Any attempt to repair the ptosis without addressing the jawwinking 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.
9. Several techniques have been suggested to obliterate
levator function, which effectively dampens the aberrant
eyelid movement.
Bullock advocated complete excision of the levator
aponeurosis and muscle all the way to the orbital apex [18].
Dillman and Anderson argued that removal of a portion of the
levator muscle above the Whitnall’s ligament (i.e., myectomy)
is adequate to obliterate its function without extensive
dissection and damage to eyelid structures [8,19].
Bowyer and Sullivan describe the removal of a portion
of levator muscle above the Whitnall ligament through a
posterior conjunctival approach[16].
Dryden et al proposed suturing the transected levator
aponeurosis to the arcus marginalis of the superior orbital
rim[20]. This technique not only effectively deactivates
the muscle but also allows the procedure to be reversed, if
necessary.
10. Beard and others have advocated bilateral excision of
the levator muscle and bilateral frontalis suspension[12].
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.
11. Satisfactory and predictable results also can be obtained
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after only unilateral levator excision on the affected side,
combined with bilateral frontalis suspension (Callahan) [24].
This leaves the normal functioning levator muscle to elevate
the nonptotic eyelid in primary position but produces a lag in
downgaze for improved symmetry.
12. Kersten et al advocate unilateral levator muscle excision
and frontalis sling only on the affected side[21]. 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.
13. 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[22]. The frontalis
flap is used to suspend the ptotic eyelid after extirpation of the
levator muscle.
14. 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[7,23]. Their techniques
maintain normal eyelid contour, as the levator aponeurotic
attachments are left undisturbed.
References
1. Gunn RM. Congenital ptosis with peculiar associated
movements of the affected lid. Trans Ophthal Soc UK. 1883;
3:283-7.
2. Demirci H, Frueh BR, NelsonCC: Marcus Gunn Jaw-Winking
Synkinesis: Clinical Features and Management: Ophthalmology
,Feb2010.
3. Park DH, Choi WS, Yoon SH. Treatment of jaw winking
syndrome. Ann Plast Surg 2008; 60(4): 404-9.
4. Khwarg SF, Tarbet KJ, Dortzbach RK, Lucarelli MJ.
Management of moderate to severe Marcus Gunn jaw-winking
ptosis. Ophthalmology 1999; 106(6): 1191-6.
5. Barthowski SB, Zapata J, Wyszynska. Management of MG
ptosis in 19 patients. J Craniomaxillofac Surg 1999; 27(1): 259.
6. Morax S, Mimoun G. Surgical treatment of MG syndrome.
Ophthalmologie 1989; 3(2): 160-3.
7. Neuhaus RW. Eyelid suspension with transposed LPS muscle.
Am J Ophthalmol 1985; 100(2): 308-11.
8. Dillman DB, Anderson RL. Levator myomectomy in synkinetic
ptosis. Arch Ophthalmol 1984; 102(3): 422-3.
9. Pratt SG, Beyer ,CK Johnson CC. The Marcus Gunn phenomenon
: A retrospective review of 71 cases. Ophthalmology 1984;90:2730.
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10. Doucet TW; Crawford JS. The quantification, natural course
and surgical results in 57eyes with Marcus Gunn (Jaw-Winking)
Syndrome. Am J Ophthal 1981; 92: 702-707.
11. Mauriello JA, Wagner RS, Caputo AR et al. Treatment of
congenital ptosis by maximum levator resection. Ophthalmol;
1986:93; 466-9.
12. Doucet TW; Crawford JS. The quantification, natural course
and surgical results in 57eyes with Marcus Gunn (Jaw-Winking)
Syndrome. Am J Ophthal 1981; 92: 702-707.
13. Beard C. Ptosis, 3rd ed. St. Louis, CV Mosby; 1981: Pg 76143,150-74,184 207.
14.Pratt SG, Beyer CK, Johnson CC. The Marcus Gunn
phenomenon. A review of 71 cases. Ophthalmology.
1984;91(1):27-30
15. Duke Elder S: Normal and abnormal development; congenital
deformities. In: System of Ophthalmology. Vol 3, pt 2. St.
Louis: CV Mosby; 1963:900-5.
16. Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw
winking synkinesis. Ophthal Plast Reconstr Surg. 2004;
20(2):92-8.
17. Putterman AM. Jaw-winking blepharoptosis treated by
the Fasanella-Servat procedure. Am J Ophthalmol. 1973;
75(6):1016-22.
18. Bullock JD. Marcus-Gunn jaw-winking ptosis: classification
and surgical management. J Pediatr Ophthalmol Strabismus.
1980;17(6):3759
19. Epstein GA, Putterman AM. Super-maximum levator resection
for severe unilateral congenital ptosis. Ophthalmic Surg. 1984;
15(12):971-9.
20. Dryden RM, Fleming JC, Quickert MH. Levator transposition
and frontalis sling procedure in severe unilateral ptosis and
the paradoxically innervated levator. Arch Ophthalmol. 1982;
100(3):462-4.
21. Kersten RC, Bernardini FP, Khouri L, et al. Unilateral frontalis
sling for the surgical correction of unilateral poor-function
ptosis. Ophthal Plast Reconstr Surg. 2005; 21(6):412-6;
discussion 416-7.
22. Islam ZU, Rehman HU, Khan MD. Frontalis muscle flap
advancement for jaw-winking ptosis. Ophthal Plast Reconstr
Surg. 2002; 18(5):365-9.
23. Lemagne JM. Transposition of the levator muscle and its
reinnervation. Eye. 1988; 2 (Pt 2):189-92.
24.Callahan A. Correction of unilateral blepharoptosis with
bilateral eyelid suspension. Am J Ophthal 1972; Vol-74; Pg
321-326.
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