Jaw ‑ winking phenomenon: Report of a case with review of ... · Kabuki syndrome has been reported.[14] Many oral‑ocular aberrations have been reported in the literature. One

Indian Journal of Dental Research, 26(3), 2015320

Jaw ‑ winking phenomenon: Report of a case with review of literature

Shobha Sundareswaran, Nipun CA, Vinay Kumar1

ABSTRACTJaw‑winking or the Marcus Gunn phenomenon (MGP), a congenital uncommon condition characterized by involuntary elevation of a drooping upper eyelid concomitant with various movements of the mandible, is well‑documented in medical literature, but very little information is available in dental literature. This is an interesting case report of a 17‑year‑old female with MGP, who reported for orthodontic treatment of her proclined upper anterior teeth, severe lower crowding, and mandibular micrognathia. Review of the literature revealed only two reported cases of dental anomalies in conjunction with MGP. The patient also gave a positive family history of MGP with a maternal cousin having a similar problem. Dentists and other oral health professionals can play a significant role in detection and diagnosis of this rare condition and should take protective measures during dental and oral surgical procedures.

Key words: Jaw winking, Marcus Gunn, ptosis

Department of Orthodontics, Government Dental College (Affiliated to Kerala University of Health Sciences), Calicut, Kerala, 1Unit of Orthodontics, Oral Health Sciences Center, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Address for correspondence: Dr. Shobha Sundareswaran E‑mail: [email protected]

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DOI: 10.4103/0970-9290.162895

males[2,5] with the left eyelid being more frequently involved. However, this has been refuted by Pratt et al., and Doucet and Crawford.[3,6] MGP is usually unilateral. Rarely, bilateral involvement has also been reported.[6] A rare case of bilateral MGP in an 18‑year‑old male patient with asymmetric bilateral ptosis and monocular elevation deficiency in the right eye was recently reported.[7] Ophthalmologic conditions accompanying the MGP have been extensively discussed in the literature. A high incidence of strabismus and amblyopia is reported to be associated with MGP. Associated anisotropia, superior rectus muscle weakness, and double elevator palsy may also be seen. However, reports of dental abnormalities are not well‑documented. Dental surgeons have every chance of being exposed to this condition during regular clinical practice. Hence, it is certainly within the domain of the dental practitioner to establish a tentative diagnosis of MGP. Occurrence of cleft lip and palate as well as supernumerary incisors has been reported in cases of MGP.[5,8]

CASE REPORT

A 17‑year‑old female patient reported to the Department of Orthodontics for the treatment of her severely proclined upper anterior teeth. Growth and development were apparently normal. Medical history did not reveal any systemic disorder. No other family member was reported to have a similar malocclusion.

Extraoral examination revealed a hyper leptoprosopic facial form with mild facial asymmetry, increased lower anterior

The jaw‑winking phenomenon is a congenital clinical entity characterized by involuntary elevation of a ptotic upper eyelid concomitant with various movements of the mandible. This was first described by Gunn in 1883[1] and is also called Marcus Gunn phenomenon (MGP). This unilateral synkinetic reflex retraction of the upper eyelid occurs during mouth opening or lateral excursions of the mandible to the contralateral side. Also called Pterygoid‑levator synkinesis, it is well‑documented in medical literature, but very little information is available in dental publications.

This condition is found in approximately 2–13% of all cases of congenital ptosis.[2,3] Recently, Carman et al.,[4] reported two cases of children who presented with ptosis and were diagnosed with MGP. It is evident at birth and often, parents are the first to detect the jaw‑winking as their infants feed. Earlier studies have reported a greater predilection for

Case RepoRt

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Received : 05‑01‑15Review completed : 09‑01‑15Accepted : 14‑05‑15

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Jaw ‑ winking phenomenon Sundareswaran, et al.

321Indian Journal of Dental Research, 26(3), 2015

face height, incompetent lips with excessive incisor display, convex profile, and steep mandibular plane. In rest position, there was blepharoptosis of the left upper eyelid [Figure 1]. However, when the mouth was opened, the left upper eyelid retracted suddenly, revealing sclera above the limbus [Figure 2]. The same phenomenon occurred when the mandible was moved to the contralateral side [Figure 3]. No retraction of the left upper eyelid was observed when the mandible was moved to the ipsilateral side [Figure 4]. The affected eyelid remained elevated while the mouth was held open or mandible moved to the opposite side and returned to its normal position only when the mouth was closed (the video file attached to this article shows ptosis and retraction of left upper eyelid with various mandibular movements – [Video File 1]). There was a full range of conjugate eye movements and visual acuity was 6/6 in both eyes.

Her mother reported that the condition had been present since birth and there was neither improvement nor worsening with age. She also reported that her nephew had

a similar problem. As they lived in another state, we were unable to verify this.

Intraoral examination revealed a constricted maxillary arch with severely proclined upper anteriors, missing upper right lateral incisor, crowding of lower arch, and exaggerated curve of Spee. Molars were in Class II relationship with an excessive overjet of 15 mm, overbite of 1.5 mm, crossbite of 14.15 and marked deviation of upper midline to right by 2.5 mm and lower midline to left by 2 mm. Cephalometric analysis revealed a Class II skeletal base, with dimensionally small, retrognathic mandible, vertical growth pattern, and deficient chin button.

DISCUSSION

The Marcus Gunn jaw‑winking syndrome is a rare problem varying in severity from a mild disorder to a significant cosmetic disability.

One of the characteristic signs of this phenomenon is blepharoptosis, which is present in the above case. There

Figure 1: Ptosis of the left upper eyelid in rest position Figure 2: Retraction of the left upper eyelid during mouth opening

Figure 3: Retraction of the left upper eyelid during movement of the mandible to the contralateral side (Revelation of sclera above the limbus)

Figure 4: Left upper eyelid remains ptotic during movement of the mandible to the ipsilateral side



Indian Journal of Dental Research, 26(3), 2015322

are reports in the literature indicating a tendency for improvement of the disorder with age. However, the patient described here did not report any such improvement.

Although most cases of MGP are said to be congenital, acquired forms of this condition are also known to exist. MGP is said to have developed after eye surgery, syphilis, trauma, pontine tumors, etc.[9,10] The congenital forms of this condition will usually be present throughout life unless corrective surgery is done.

Various theories have been proposed regarding the etiology of this disorder. Dual innervation of the levator palpebrae superioris muscle from both the occulomotor nucleus and external pterygoid portion of the trigeminal nucleus[1] is one such theory. Normally, levator muscle is innervated by the occulomotor nucleus.

The second theory on innervation states that there is a reflex arc arising from the motor division of the trigeminal nerve to the gasserian ganglion, propagated along intraneuronal connections to the occulomotor nucleus and finally to levator muscle.[2] This theory is based on embryologic evidence as the motor nucleus of the two cranial nerves appear at the same time and are located in close proximity.[11] Kinematic and MRI findings point to a brainstem structural abnormality in familial MGP patients thus supporting the hypothesis of a neural misdirection of trigeminal motor axons to the elevator palpebralis muscle.[12]

The third phylogenetic theory of atavism relates this phenomenon to lower animals and fish, where retraction of the upper eyelid is associated with opening of the mouth. In general, these related movements become inhibited through an evolutionary process.[11]

Systemic anomalies in association with MGP are rare. However, a case in which this disorder was associated with ectrodactylia, bilateral pes cavus with ankle varus, forefoot adduction, spina bifida, and bilateral undescended testes has been reported.[8] Association of MGP with renal calculi and cleft lip,[6] congenital left duplex kidneys and ureters,[13] and Kabuki syndrome has been reported.[14]

Many oral‑ocular aberrations have been reported in the literature. One such is synchronous closure of the eyelid with opening of the patient’s mouth. This is opposite to that described in the MGP and is known as “inverted Marcus Gunn” or “Marin Amat” syndrome. Pavone et al., in 1999 have reported the case of a 10‑year‑old boy with this phenomenon.[15] Another oral ocular aberration is the corneomandibular reflex or winking jaw phenomenon, where forceful pressure on the cornea results in sudden movement of the mandible to the contralateral side[16] “eye bobbing” is yet another reported disorder characterized by elevation and depression of the eye globes coinciding with

mandibular movements.[17] Simultaneous occurrence of MGP in one eye and inverted MGP in the other have also been reported (“see‑saw Marcus Gunn syndrome”).[18,19]

Family history of myasthenia gravis with ptosis, congenital nasal sinus tract, cleft lip and palate, congenital ptosis, and convergent strabismus in a case of MGP has been reported earlier.[11] This may be a variation of the MGP trait. Disabling of the involved levator muscle and unilateral or bilateral frontalis suspension are stated to be effective in the treatment of MGP. Eyelash ptosis and loss of eyelid crease were the most commonly reported complications, each occurring in 10% of the patients.[20]

CLINICAL IMPLICATIONS FOR THE DENTAL SURGEON

Any type of dental treatment for these patients would require the exercising of utmost care to prevent the potential risk of ocular damage, because mouth opening will lead to simultaneous lid retraction.

Protective eye shield wear is mandatory during routine dental procedures like cavity preparation, use of prophylactic paste, amalgam removal, etc. Special care and precaution must be exercised during orthodontic procedures using sharp instruments, while cutting wires, using fine ligatures, brackets, pins, and halogen/LED curing light as protective reflex closure of the eyelid is not possible for these patients. Furthermore, short appointments are preferable to reduce the problems due to prolonged exposure to the halogen/LED lights of the dental unit.

CONCLUSION

Patients with MGP may be more vulnerable to eye injuries during simultaneous lid retraction and opening of the mouth. Dental surgeons will need to handle such cases also while dealing with their oral health related problems. It is important for the dental practitioners to establish a tentative diagnosis, take adequate precautions, and refer suspected patients for ophthalmologic examination.

REFERENCES

1. Gunn RM. Congenital ptosis with peculiar associated movements of the affected lid. Trans Ophthalmol Soc U K 1883;3:283‑7.

2. Spaeth EB. The Marcus Gunn phenomenon; discussion, presentation of four instances and consideration of its surgical correction. Am J Ophthalmol 1947;30:143‑58.

3. Pratt SG, Beyer CK, Johnson CC. The Marcus Gunn phenomenon. A review of 71 cases. Ophthalmology 1984;91:27‑30.

4. Carman KB, Ozkan S, Yakut A, Ekici A. Marcus Gunn jaw winking synkinesis: Report of two cases. BMJ Case Rep 2013;2013: pii: Bcr2012008210.

5. Awan KJ. Marcus gunn (jaw‑winking) syndrome. Am J Ophthalmol 1976;82:503‑4.

6. Doucet TW, Crawford JS. The quantification, natural course, and surgical



323Indian Journal of Dental Research, 26(3), 2015

results in 57 eyes with Marcus Gunn (jaw‑winking) syndrome. Am J Ophthalmol 1981;92:702‑7.

7. Shah AD, Kumar AB, Kothari K. Bilateral Marcus Gunn jaw winking synkinesis with monocular elevation deficiency: A case report and literature review. Int Ophthalmol 2012;32:199‑201.

8. Schultz RO, Burian HM. Bilateral jaw winking reflex in association with multiple congenital anomalies. Arch Ophthalmol 1960;64:946‑9.

9. Simpson DG. Marcus Gunn phenomenon following squint and ptosis surgery; definition and review. AMA Arch Ophthalmol 1956;56:743‑8.

10. Malik SR, Mohan H, Sood GC. Blascovicz operation in a case of Marcus Gunn phenomenon. Br J Ophthalmol 1964;48:173‑5.

11. Brooks JK. The Marcus Gunn phenomenon. Discussion and report of a case. Oral Surg Oral Med Oral Pathol 1987;64:687‑92.

12. Conte A, Brancati F, Garaci F, Toschi N, Bologna M, Fabbrini G, et al. Kinematic and diffusion tensor imaging definition of familial Marcus Gunn jaw‑winking synkinesis. PLoS One 2012;7:e51749.

13. Altman K. The Marcus Gunn (jaw‑winking) phenomenon: A case report. Br J Oral Maxillofac Surg 1990;28:53‑4.

14. Emmert‑Buck LT, Preslan MW, Kathuria SS. Jaw‑winking ptosis in a patient with Kabuki syndrome. J Pediatr Ophthalmol Strabismus 2004;41:369‑72.

15. Pavone P, Garozzo R, Trifiletti RR, Parano E. Marin‑Amat syndrome: Case report and review of the literature. J Child Neurol 1999;14:266‑8.

16. Wartenberg R. Winking‑jaw phenomenon. Arch Neurol Psychiatry 1948;59:734‑53.

17. Oesterle CS, Faulkner WJ, Clay R, Folk ER, Lucchese N, Putterman AM. Eye bobbing associated with jaw movement. Ophthalmology 1982;89:63‑7.

18. Nagpaul PN, Charan H. Marcus Gunn phenomenon. Br J Ophthalmol 1968;52:484‑5.

19. Kumar V, Goel N, Raina UK, Ghosh B. “See‑saw” Marcus Gunn syndrome. Ophthal Plast Reconstr Surg 2011;27:e144‑5.

20. Demirci H, Frueh BR, Nelson CC. Marcus Gunn jaw‑winking synkinesis: Clinical features and management. Ophthalmology 2010;117:1447‑52.

How to cite this article: Sundareswaran S, Nipun CA, Kumar V. Jaw - winking phenomenon: Report of a case with review of literature. Indian J Dent Res 2015;26:320-3.Source of Support: Nil, Conflict of Interest: The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


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Jaw ‑ winking phenomenon: Report of a case with review of ... · Kabuki syndrome has been reported.[14] Many oral‑ocular aberrations have been reported in the literature. One