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 Table of Contents  
CASE SERIES
Year : 2021  |  Volume : 59  |  Issue : 4  |  Page : 372-375

The dancing droopy lids: Marcus gunn jaw winking phenomenon in congenital ptosis – A case series


1 Department of Squint, Neuro-Ophthalmolgy and Pediatric Ophthalmmology, Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Chennai, Tamil Nadu, India
2 Department of Ophthalmology, Madurai Medical College, Madurai, Tamil Nadu, India
3 Department of Ophthalmology, Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Chennai, Tamil Nadu, India

Date of Submission21-May-2021
Date of Decision05-Oct-2021
Date of Acceptance07-Oct-2021
Date of Web Publication21-Dec-2021

Correspondence Address:
Dr. Malarvizhi Raman
Department of Squint, Neuro-Ophthalmolgy and Pediatric Ophthalmmology, Regional Institute of Ophthalmology and Government Ophthalmic Hospital, Egmore, Chennai - 600 008, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/tjosr.tjosr_70_21

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  Abstract 


Marcus Gunn Jaw Winking Ptosis is a congenital ptosis associated with synkinetic movements of upper lid on masticating movements of the jaw. It is usually unilateral but rarely presents bilaterally. In this study, ten cases of unilateral ptosis since birth with moderate to severe degree movement of lids along movement of jaws (MGJW-Marcus Gunn Jaw Winking) presenting to Oculoplasty department were evaluated and managed according to the degree of jaw winking phenomenon and their outcomes were observed. The amount of jaw winking is the excursion of the upper lid in mm with the opening of the mouth. Ocular associations include strabismus (50-60%), anisometropia (5-25%) and amblyopia (30-60%). The cause for underlying amblyopia could be secondary to strabismus, anisometropia, or occlusion by the ptotic lid. It is graded into mild (maximum 2 mm), moderate (2 mm-5 mm) and severe (higher than 5 mm). Persistence of minimal jaw-winking phenomenon post-operatively after levator detachment. Frontalis sling is the most common procedure used to correct the ptosis following the obliteration of levator action. MGJWS mostly presented as moderate to severe jaw winking synkinesis of upper eyelid and accompanied by moderate to severe blepharoptosis. Eyelid excursion of jaw winking has direct correlation with ptosis and levator dysfunction. Unilateral levator aponeurosis excision with frontalis suspension is an effective surgical approach in the management of unilateral MGJWS, which achieved both satisfactory symmetrical outcome and resolution of jaw winking with eye movement.

Keywords: Jaw winking, Marcus Gunn, ptosis


How to cite this article:
Raman M, Anuradha A, Sheela S, Senthil Kumar NK. The dancing droopy lids: Marcus gunn jaw winking phenomenon in congenital ptosis – A case series. TNOA J Ophthalmic Sci Res 2021;59:372-5

How to cite this URL:
Raman M, Anuradha A, Sheela S, Senthil Kumar NK. The dancing droopy lids: Marcus gunn jaw winking phenomenon in congenital ptosis – A case series. TNOA J Ophthalmic Sci Res [serial online] 2021 [cited 2022 Aug 12];59:372-5. Available from: https://www.tnoajosr.com/text.asp?2021/59/4/372/333179




  Introduction Top


Marcus Gunn Jaw Winking (MGJW) Ptosis is congenital ptosis associated with synkinetic movements of the upper lid on masticating movements of the jaw. It is usually unilateral but rarely presents bilaterally. It was first described by Robert Marcus Gunn in 1883, is a disorder characterized by congenital ptosis accompanied by synkinetic elevation of the affected eyelid upon movement of the jaw.[1] It is known to affect males and females in equal proportion.[2] Here we present a case series of congenital ptosis with Marcus Gunn's jaw-winking synkinesis (MGJWS).


  Case Details Top


Ten cases of unilateral ptosis since birth with moderate-to-severe degree movement of lids along the movement of jaws (MGJW) presented to the Oculoplasty department. Demographics and complaints of the patients are enlisted in [Table 1]. The details of ptosis evaluation are as documented in [Table 2]. [Table 3] elaborates on the surgical outcomes of the cases. The age of presentation varied from 1.5 years to 17 years. No positive family history. No other significant history.
Table 1: Demographics and complaints of the patients

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Table 2: Detailed ptosis evaluation of patients

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Table 3: Surgical management and outcomes of the cases (all cases were taken up for surgery under general anesthesia)

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Figure 1: (a) Preoperative image (case 2). (b) Postoperative image (case 2)

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Figure 2: (a) Preoperative image (case 3). (b) Postoperative image (case 3)

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Amblyopia was treated aggressively with occlusion therapy and correction of anisometropia before ptosis surgery was carried out.

Unilateral levator excision with frontalis sling procedure: The surgical procedure performed is as follows. Lid crease incision was made, orbicularis muscle incised using radiofrequency cautery, tarsal plate was identified. Levator aponeurosis is freed from the superior border of the tarsal plate and carefully dissected, taking care not to incise the conjunctiva by using subconjunctival local anesthetic ballooning technique. Traction was applied on the levator using 6-0 silk when dissection is carried out above the preaponeurotic fat pad. Once 12–15 mm of levator aponeurosis has been separated up to Whitnall's ligament, it is cut and the orbicularis and skin is closed with interrupted 6'o vicryl sutures. Aponeurosis excision was followed by frontalis suspension using silicon sling on the affected side. This technique effectively releases the elevator function of Lipopolysaccharide (LPS) and simultaneously protects the deep orbital tissues like the superior rectus under the LPS.


  Discussion Top


This case series of MGJWS has certain significant features including a broad age range and varied presentation. All cases had unilateral ptosis on presentation. The margin reflex distance and levator function were ranging from −1 to +1 and 2 mm to 7 mm respectively. Bell's phenomenon was intact in all cases, hence enabling us to proceed with the procedure as mentioned above.

Jaw-winking phenomenon is reported in 2%–13% of congenital blepharoptosis patients by Park et al.[3] The abnormal synkinetic movement associated with MGJWS results from a congenital aberrant connection between motor branches of trigeminal nerve controlling muscles of mastication and superior division of oculomotor nerve controlling the levator palpebrae superioris. Although most cases of Marcus Gunn phenomena are said to be congenital, acquired forms of this condition are also known to exist. The eyelid wink is triggered by chewing, suction, lateral mandible movement, or sternocleidomastoid contraction, causing the tongue to protrude, even when smiling.[4] The amount of jaw winking is the excursion of the upper lid in mm with the opening of the mouth.[2] Ocular associations include strabismus (50%–60%), anisometropia (5%–25%), and amblyopia (30%–60%). The cause for underlying amblyopia could be secondary to strabismus, anisometropia, or occlusion by the ptotic lid. It is graded into mild (maximum 2 mm), moderate (2 mm–5 mm), and severe (higher than 5 mm). If the jaw-winking is cosmetically insignificant or if the ptosis is mild, it is advisable not to proceed with surgery. Relative contraindications for surgical intervention of MGJWS are poor Bell phenomena, reduced corneal sensations and dry eye. All these clinical conditions can ultimately result in vision-threatening exposure keratitis.

Medical management includes aggressive management of amblyopia, when detected, with occlusion and correction of anisometropia. Regular follow-up once in 6 months is essential to check for astigmatism due to compression by the ptotic lid. Offering a combination of two procedures in a single sitting avoids the necessity of general/local anesthesia-related complications and defers the need for another hospital stay for the patient. Several approaches have been suggested for the essential initial step of the release of the lid from levator action. Challenges in doing the same include levator aponeurosis having numerous attachments below Whitnall's ligament (making separation of LPS aponeurosis from lacrimal gland lobes difficult), recurrence of fibrous connections between levator muscle and eyelid.[5] Persistence of minimal jaw-winking phenomenon postoperatively after levator detachment, similar to four cases, has been reported by Manners et al.[6] Frontalis sling is the most common procedure used to correct the ptosis following the obliteration of levator action according to several studies by Bowyer et al.,[7] Bartkowski et al.[8] A variety of surgical techniques were reported, such as the levator muscle excision followed by frontalis suspension,[5],[7],[9],[10] Fasanella–Servat procedure,[11] levator sling,[12] and modified levator resection or plication.[12] Of the above procedures, levator excision with frontalis suspension was a widely used technique for good results in synkinesis elimination.


  Conclusion Top


MGJWS mostly presented as moderate-to-severe jaw winking synkinesis of the upper eyelid and accompanied by moderate–to-severe blepharoptosis. Eyelid excursion of jaw winking has direct correlation with ptosis and levator dysfunction. Unilateral levator aponeurosis excision with frontalis suspension is an effective surgical approach in the management of unilateral MGJWS, which achieved both satisfactory symmetrical outcome and resolution of jaw winking with eye movement. However, severe jaw winking is a risk factor for residual eyelid synkinesis after surgery. With present surgical procedures, it is difficult to completely eliminate the synkinetic eyelid movement in severe MGJWS.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bair H, Garcia GA, Erickson BP. Surgical management of jaw-winking synkinesis and ptosis in Marcus Gunn syndrome: A systematic outcomes analysis. Plast Aesthet Res 2020;7:68 Pg 2/12. Available from: https://parjournal.net/article/view/. [Last accessed on 2021 Feb 28].  Back to cited text no. 1
    
2.
Demirci H, Frueh BR, Nelson CC. Marcus Gunn jaw-winking synkinesis: Clinical features and management. Ophthalmology 2010;117:1447-52.  Back to cited text no. 2
    
3.
Park DH, Choi WS, Yoon SH. Treatment of the jaw-winking syndrome. Ann Plast Surg 2008;60:404-9.  Back to cited text no. 3
    
4.
Torres MR, Calixto N Jr., Oliveira LR, Steiner SA, Iscold AM. Marcus Gunn phenomenon: Differential diagnosis of palpebral ptoses in children. J Pediatr (Rio J) 2004;80:249-52.  Back to cited text no. 4
    
5.
Khwarg SI, Tarbet KJ, Dortzbach RK, Lucarelli MJ. Management of moderate-to-severe Marcus-Gunn jaw-winking ptosis. Ophthalmology 1999;106:1191-6.  Back to cited text no. 5
    
6.
Manners RM, Rosser P, Collin JR. Levator transposition procedure: A review of 35 cases. Eye (Lond) 1996;10(Pt 5):539-44.  Back to cited text no. 6
    
7.
Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw winking synkinesis. Ophthalmic Plast Reconstr Surg 2004;20:92-8.  Back to cited text no. 7
    
8.
Bartkowski SB, Zapala J, Wyszyńska-Pawelec G, Krzystkowa KM. Marcus Gunn jaw-winking phenomenon: Management and results of treatment in 19 patients. J Craniomaxillofac Surg 1999;27:25-9.  Back to cited text no. 8
    
9.
Davis G, Chen C, Selva D. Marcus Gunn syndrome. Eye (Lond) 2004;18:88-90.  Back to cited text no. 9
    
10.
Koelsch E, Harrington JW. Marcus Gunn jaw-winking synkinesis in a neonate. Mov Disord 2007;22:871-3.  Back to cited text no. 10
    
11.
Putterman AM. Jaw-winking blepharoptosis treated by the Fasanella-Servat procedure. Am J Ophthalmol 1973;75:1016-22.  Back to cited text no. 11
    
12.
Ning Q, Cao J, Xie J, Gao Q, Wang C, Ye J. Unilateral Levator aponeurosis excision for Marcus Gunn Syndrome and risk factors of residual jaw winking. Journal of ophthalmology. 2019 Nov 4;2019. ARticle ID 2058047: ePages 1- 9.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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