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 In 1883 Robert Marcus Gunn described a 15yr girl with a form of congenital synkinetic neurogenic . In this peculiar type of congenital ptosis that included an associated synkinetic phenomenon, the unilaterally ptotic elevates winking motion of affected eyelid on the movement of jaw1. with jaw movements. The movement that most commonly This synkinetic jaw-winking phenomenon now bears its name. 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.

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.

The Marcus Gunn phenomenon is known variously as jaw- winking (a misnomer as eyelid rises rather than falls) and more Figure 1: Patient with Marcus Gunn Jaw-Winking phenomenon. descriptively, as pterygoid-levator synkinesis2. The Marcus (A) 5year old child with unilateral upper eyelid ptosis as part of Gunn phenomenon has been associated with congenital Marcus Gunn phenomenon. (B) Elevation of concomitant upper blepharoptosis with an incidence of 4-6% [2,3,4]. Acquired eyelid with mouth opening

Vol. 21, No. 3, January-March, 2011 DJO 19 Delhi Journal of Ophthalmology Marcus Gunn Jaw-Winking Phenomenon : A Review forms have been described after eye surgery, trauma, post 2) Functional Interference Bells palsy and pontine tumors[2]. Spontaneous remission of 1. Irritation of normally dormant connection the acquired form may be expected, whereas the congenital 2. Disinhibition of pre-existing phylogenetically more form persists (no improvement with age)[12]. Patients with primitive mechanisms (Ascher): This is thought to Marcus Gunn jaw-winking phenomenon have a variable explain why individuals who are not affected will often degree of blepharoptosis in the resting and primary position. open their mouth while attempting to widely open their Although Marcus Gunn jaw-winking syndrome is usually eyes to place eye drops unilateral[12-13] it can present bilaterally in rare cases. 3. Spread of impulses by irradiation

The characteristic feature of the phenomenon is that the 3) Atavistic Reversion raising; and not winking of the affected eyelid is synchronous 1. In fish a strong associated movement of jaw opening and with and proportionate to the opening of the mouth. The wink eye opening i.e., deep muscle contracting and superficial reflex consists of a momentary upper eyelid retraction or muscle relaxing. Thus a weak levator may only elevate elevation to an equal or higher level than the normal fellow the lid when its antagonist, the orbicularis (superficial eyelid upon stimulation of the ipsilateral pterygoid muscle muscle) is reflexly relaxed by jaw opening (external 12-13. pterygoid-deep muscle contraction ) 2. EMG study suggested dysfunction in the midbrain and This response is followed by a rapid return to a lower position. brainstem The amplitude of the wink tends to be worse in downgaze. This rapid, abnormal motion of the eyelid can be the most Measurement of Mgjwp disturbing aspect of the jaw-winking syndrome. The amount of jaw-winking is the excursion of the upper eyelid with synkinetic mouth movement. It is measured with MARCUS GUNN JAW-WINKING PHENOMENON a millimeter ruler. Jaw-winking is assessed as [2,10] : Mild < The wink phenomenon i.e., retraction of the ptotic lid occurs 2mm ; Moderate 2-5mm; Severe ≥ 6mm in conjunction with stimulation of pterygoid muscle, which is elicited by opening the mouth, thrusting the jaw to the contra Frequency lateral side, jaw protrusion, chewing, smiling or sucking. This Approximately 50% of blepharoptosis cases are congenital. wink phenomenon is often discovered early, as the infant is Incidence of Marcus Gunn jaw-winking syndrome among this bottle-feeding or breastfeeding[14]. population is approximately 4-5% [12,13].

Jaw-winking ptosis is almost always sporadic, but familial Associations cases with an irregular autosomal dominant inheritance 1. Ocular pattern have been reported[15]. 1. (50%-60%)[9] 1. Superior Rectus Palsy-25% Pathophysiology 2. Double Elevator Palsy-25% A complete explanation has not yet been advanced to elucidate 2. (5%-25%)[9] the rationale of jaw-winking phenomenon. Various theories Incidence of anisometropia among patients with Marcus have been hypothesized [15,16]. Gunn jaw-winking syndrome is reported to be 5-25%. 3. (30-60%)[9] 1) Aberrant connection Almost always secondary to strabismus or anisometropia, This hypothesis is favored by most authors, though they differ and only rarely, is due to occlusion by a ptotic eyelid. in opinion as to the location of the aberration 1. Cortical or sub cortical connections 2. Systemic 2. Internuclear connections or faulty distribution in the Systemic anomalies in association with Marcus Gunn posterior longitudinal bundle phenomenon are rare. 3. Infranuclear connection exists between motor branches 1. Cleft lip/ Cleft palate of the trigeminal nerve (CN V3) innervating the external 2. CHARGE Syndrome reported in association with pterygoid and the fibers of superior division of the bilateral cases. (CN III) that innervates the levator 3. Renal calculi (Awan 1976) muscle of the upper lid 4. Peripherally - some CN V fibers may reach the levator via Schultz and Burian (1960) reported a case of MGP associated the auriculo- temporal nerve with several systemic malformations. These included

DJO Vol. 21, No. 3, January-March, 2011 20 Marcus Gunn Jaw-Winking Phenomenon : A Review Delhi Journal of Ophthalmology ectrodactly, bilateral pes cavus with ankle varus, spina bifida procedure). occulta, bilateral undescended testis and supernumerary 3.Consider eyelid surgery only when the parents (or the incisors. patient) and the surgeon agree about whether the most cosmetically objectionable condition is the ptosis or the jaw- Race winking or whether it is a combination of both. No known racial predilection exists. 4. Many techniques are described for the correction of jaw- winking ptosis, reflecting the ongoing controversy regarding Sex the surgical management of this condition. Early reports showed jaw-winking ptosis to be more prevalent 5. If the jaw-winking is cosmetically insignificant, it can be in females than in males; however, larger case series have ignored in the treatment of the ptosis. shown an equal prevalence among males and females4, 9. If the ptosis is mild, the patient may elect not to proceed with surgery. If correction is desired, perform a Muller muscle and Age conjunctival resection (MMCR), a Fasanella-Servat procedure Marcus Gunn jaw-winking syndrome is usually evident at or a standard external levator resection[14,18]. birth. The winking phenomenon is often first noted by the If the ptosis is moderate to severe, a levator resection may be parents when the infant is feeding. indicated. Beard advocated performing more resection than normal to avoid undercorrection[13]. Treatment In severe ptosis, a super maximum (>30 mm) levator resection 1. Medical Care or frontalis suspension is necessary [19]. If amblyopia is encountered, treat aggressively with occlusion 6. Although the amount of ptosis and synkinetic eyelid therapy and/or correction of anisometropia prior to any movement is variable, those patients with more severe ptosis consideration of ptosis surgery. tend to have the worse aberrant upper eyelid movement. 2. Surgical Care 7. The jaw-wink is considered cosmetically significant if it is As with any patient who requires eyelid surgery, first address 2 mm or more [2]. associated strabismus. 8. Any attempt to repair the ptosis without addressing the jaw- winking would result in an exaggeration of the aberrant eyelid 1. Superior rectus palsy movement to a level well above the superior corneal limbus, Superior rectus palsy can be corrected by resecting the which would be unacceptable to the patient. superior rectus muscle but only in the absence of inferior 9. Several techniques have been suggested to obliterate rectus restriction. levator function, which effectively dampens the aberrant Since the superior rectus is loosely bound to the overlying eyelid movement. levator, the upper eyelid will be pulled inferiorly during Bullock advocated complete excision of the levator resection, exacerbating any ptosis already present. This can aponeurosis and muscle all the way to the orbital apex [18]. be addressed during the subsequent ptosis repair. Dillman and Anderson argued that removal of a portion of the 2. Double elevator palsy levator muscle above the Whitnall’s ligament (i.e., myectomy) Double elevator palsy manifests as a deficit in the elevation of is adequate to obliterate its function without extensive the in all fields of gaze. dissection and damage to eyelid structures [8,19]. It may be the result of superior rectus and inferior oblique Bowyer and Sullivan describe the removal of a portion palsy and/or inferior rectus restriction. of levator muscle above the Whitnall ligament through a Inferior rectus restriction may be suggested by the posterior conjunctival approach[16]. following Dryden et al proposed suturing the transected levator 1. Positive forced duction in elevation aponeurosis to the arcus marginalis of the superior orbital 2. Normal force generations in up gaze indicating rim[20]. This technique not only effectively deactivates an absence of superior rectus or inferior oblique the muscle but also allows the procedure to be reversed, if palsy necessary. 3. Poor or absent Bells phenomenon on the affected 10. Beard and others have advocated bilateral excision of side the levator muscle and bilateral frontalis suspension[12]. Inferior rectus restriction is treated by recession of the inferior While this approach almost completely eliminates the wink rectus muscle. and arguably results in better symmetry, it is often difficult to A combined superior rectus and inferior oblique (double persuade the parents and the patient to perform surgery on and elevator) palsy requires a transposition procedure to displace effectively damage the normal contralateral levator muscle. the medial and lateral recti muscles superiorly (Knapp’s 11. Satisfactory and predictable results also can be obtained

Vol. 21, No. 3, January-March, 2011 DJO 21 Delhi Journal of Ophthalmology Marcus Gunn Jaw-Winking Phenomenon : A Review after only unilateral levator excision on the affected side, 10. Doucet TW; Crawford JS. The quantification, natural course combined with bilateral frontalis suspension (Callahan) [24]. and surgical results in 57eyes with Marcus Gunn (Jaw-Winking) This leaves the normal functioning levator muscle to elevate Syndrome. Am J Ophthal 1981; 92: 702-707. the nonptotic eyelid in primary position but produces a lag in 11. Mauriello JA, Wagner RS, Caputo AR et al. Treatment of downgaze for improved symmetry. congenital ptosis by maximum levator resection. Ophthalmol; 12. Kersten et al advocate unilateral levator muscle excision 1986:93; 466-9. and frontalis sling only on the affected side[21]. If the 12. Doucet TW; Crawford JS. The quantification, natural course postoperative result is judged to be unsatisfactory, the parents and surgical results in 57eyes with Marcus Gunn (Jaw-Winking) or the patient can opt for further surgery to the contralateral Syndrome. Am J Ophthal 1981; 92: 702-707. side. Any amblyopia and strabismus should first be addressed, 13. Beard C. Ptosis, 3rd ed. St. Louis, CV Mosby; 1981: Pg 76- as there may be insufficient drive to lift the disinserted eyelid. 143,150-74,184 207. 13. Islam et al described a technique of dissecting a frontalis 14. Pratt SG, Beyer CK, Johnson CC. The Marcus Gunn flap hinged superiorly through a suprabrow incision that is then phenomenon. A review of 71 cases. Ophthalmology. brought down into an eyelid crease incision[22]. The frontalis 1984;91(1):27-30 flap is used to suspend the ptotic eyelid after extirpation of the 15. Duke Elder S: Normal and abnormal development; congenital levator muscle. deformities. In: System of Ophthalmology. Vol 3, pt 2. St. 14. Lemagne and Neuhaus described techniques that involve Louis: CV Mosby; 1963:900-5. transection of the involved levator followed by transposition 16. Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw of the distal segment to the brow, which effectively suspends winking synkinesis. Ophthal Plast Reconstr Surg. 2004; the eyelid to the frontalis muscle[7,23]. Their techniques 20(2):92-8. maintain normal eyelid contour, as the levator aponeurotic 17. Putterman AM. Jaw-winking blepharoptosis treated by attachments are left undisturbed. the Fasanella-Servat procedure. Am J Ophthalmol. 1973; 75(6):1016-22. References 18. Bullock JD. Marcus-Gunn jaw-winking ptosis: classification 1. Gunn RM. Congenital ptosis with peculiar associated and surgical management. J Pediatr Ophthalmol Strabismus. movements of the affected lid. Trans Ophthal Soc UK. 1883; 1980;17(6):3759 3:283-7. 19. Epstein GA, Putterman AM. Super-maximum levator resection 2. Demirci H, Frueh BR, NelsonCC: Marcus Gunn Jaw-Winking for severe unilateral congenital ptosis. Ophthalmic Surg. 1984; Synkinesis: Clinical Features and Management: Ophthalmology 15(12):971-9. ,Feb2010. 20. Dryden RM, Fleming JC, Quickert MH. Levator transposition 3. Park DH, Choi WS, Yoon SH. Treatment of jaw winking and frontalis sling procedure in severe unilateral ptosis and syndrome. Ann Plast Surg 2008; 60(4): 404-9. the paradoxically innervated levator. Arch Ophthalmol. 1982; 4. Khwarg SF, Tarbet KJ, Dortzbach RK, Lucarelli MJ. 100(3):462-4. Management of moderate to severe Marcus Gunn jaw-winking 21. Kersten RC, Bernardini FP, Khouri L, et al. Unilateral frontalis ptosis. Ophthalmology 1999; 106(6): 1191-6. sling for the surgical correction of unilateral poor-function 5. Barthowski SB, Zapata J, Wyszynska. Management of MG ptosis. Ophthal Plast Reconstr Surg. 2005; 21(6):412-6; ptosis in 19 patients. J Craniomaxillofac Surg 1999; 27(1): 25- discussion 416-7. 9. 22. Islam ZU, Rehman HU, Khan MD. Frontalis muscle flap 6. Morax S, Mimoun G. Surgical treatment of MG syndrome. advancement for jaw-winking ptosis. Ophthal Plast Reconstr Ophthalmologie 1989; 3(2): 160-3. Surg. 2002; 18(5):365-9. 7. Neuhaus RW. Eyelid suspension with transposed LPS muscle. 23. Lemagne JM. Transposition of the levator muscle and its Am J Ophthalmol 1985; 100(2): 308-11. reinnervation. Eye. 1988; 2 (Pt 2):189-92. 8. Dillman DB, Anderson RL. Levator myomectomy in synkinetic 24. Callahan A. Correction of unilateral blepharoptosis with ptosis. Arch Ophthalmol 1984; 102(3): 422-3. bilateral eyelid suspension. Am J Ophthal 1972; Vol-74; Pg 9. Pratt SG, Beyer ,CK Johnson CC. The Marcus Gunn phenomenon 321-326. : A retrospective review of 71 cases. Ophthalmology 1984;90:27- 30.

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