General Dentistry

The Marcus Gunn phenomenon: Discussion and report of three cases Amit ChattopadhyayVK, Srinivas**/B. Sharatchandra**/N. Kantian**

The Marcus Gunn phenoinenon and Marin Ainat syndrome (inverted Marcus Gunn phe- notnenon) are discussed, along with their associated featttres. Two patients exhibiting the Marcus Gitnn phenomenon only and one patient exhibiting both the Marcus Gtmn phe- nomenon and Maiin Ainat syndrome are described. (Quintessence Int 1995:26:563-566.)

Introduction Characteristics, etiology, and treatment In 1883, Marcus Gutin' described for the first time a Marcus Gunn phenomenon is often first ohserved in sjTidrome consisting of unilateral, congenital the infant by the mother during breastfeeding, when and rapid, exaggerated elevation ofthe ptotic on the eye movements are noted to be associated with movement of the mandible to the contralateral side. suckitig activity, •* The elevation ofthe upper eyelid tnay This phenomenon is known variously as Marcus Gunn be stimulated by a variety of movements, including phenoinenon (MGP) or syndrotne. ¡aw winking syn- mandibular movements, tongue movements, chewing, droine. and pierygoid levator synkinesis.- A somewhat speaking, sucking, swallowing, smiling, mouth move- reverse phenomenon is seen in the Marin Amat ments, and whistling,'*"' The syndrome may also be syndrome, in which the eye closes automatically when stimulated by closing movement ofthe other eye,-' the patient opens his or her mouth forceiully and flilly, An excellent review-ofthe etiopathogenesis, chnical as during chewing. This may be associated with a fiow characteristics, and therapeutic jnanagement of MGP of tears.-' The inverse/inverted MGP (corneoman- has been presented by Del-Core et al,'" Marcus Gunn dibular reflex/pterygocomeal reflex) is manifested by phenomenon usually is of sporadic occurence,' Some a brisk movement ofthe mandible to the contralateral familial cases have been reported that involve up to two side when the is touched; the mandible may generations. Brooks,'^ however, believed most of also be thrust forward.^ the cases to be congenital. He reviewed cases of MGP The present report describes three patients exhib- that developed after eye surger>'. syphillis, trauma, and iting MGP, Although this disorder has been reported pontine tumors,^ The syndrome has also been reported often in the medical literature, few reports have to disappear temporarily,^ No sex predilection or side appeared in the dental literature. Furthermore, to the predilection (left/right) is apparent,* best of our knowledge, we are reporting only the third The phenomenon may be associated with other case of a patient with MGP on one side and Marin abnormalities, such as ambylopia, double elevator Amat syndrome on the other side. palsy, . and superior rectus muscle palsy,'''•'•^ as well as congenital atiomalies, such as ectrodactylia, bilateral pes cavus, spina bifida, forefoot ' Assistant Professor and Head, t3eparttnent of Oral Medicine and Radiology, SDM Coltege of Dentat Sciences and Hospital, Dtiarwad, adduction, bilateral undescended testis." and cleft lip.'' tndia. The etiology of MGP is obscure. It was originally •• Graduate Student, Department of Oral Medicine and Radiology, assumed that the syndrome was caused by aberrant SDM College of Dental Sciences and Hospital, Dtiarwad, India, innervation ofthe levator palpabrae superioris from Reprint requests: Dr Amit Chattopadhyay, 253E, DDA Fiats (MtG), Rajouri Garden, New Deltii 110027, tndia. the motor branch ofthe îrigeminal nerve because of

Quintessegflo Intomotipnol—Voluma gO,'W)jmber 8/1995 563 General Dentistry

Fig 1 Marcus Gunn phenomenon: The normally ptolJc right eyelid shows exaggerated opening on mandibular movement.

the close approximation of the nuclei of the occulo- Case reports motorand trigeminal nerves. However, a supranuclear, or at least a combined supranuciear-nuclear, involve- Three patients with MGP were observed in the ment has been suggested." Other theories, such as Department of Oral Medicine and Radiology, SDM genetic origin and phylogenetic atavism, have also College of Dental Sciences, Dharwad, India, betweeti been suggested to explain the etiology of MGP, These January and September 1993. All the patients had theories and other aspects of MGP have been excel- come to the hospitai for complaints other than MGP. lently reviewed by Brooks,•* Del-Core et al,'" Pratt et None of them reported any significant family or al,'^ and Simpson," medical history. Management of MGP has been quite varied and mainly revolves around correction of the ptosis. Case I Although claims of spontaneous correction of MGP have been made, it has not been documented properly. A 22-year-old man reported for restoration of his One long-term follow-up study could not fmd such a teeth. The right eyelid was congenitally ptotic and correction." Many cases remain untreated, and the exhibited MGP on mandibular movements (Fig 1), patients soon learn to mask the condition by perform- Thorough opthalmologic and neurologic evaluatioti ing certain other movements or at times just do not failed to reveal any other abnormalities. A computer- care. Simpson" mentioned that many adults learn to ized tomographic (CT) scan revealed pneumatized hide the lid retraction by slightly bending their heads anterior clinoid processes ofthe sphenoid bone. downward and looking up; this action results in a raised hd position, minimizing the "jaw-winking" and Case 2 ptosis. For this reason, mild cases of MGP may be successfijlly managed without surgical intervention.'' A 65-year-old man reported for treatment of Ms Surgical procedures used to alleviate MGP inciude chronic periodontal problems. This patient was not ievator transposition and bilateral frontal sling, '^ aware of his jaw-winking movements (Fig 2 ), The right bilateral Ievator muscle excision and autogenous fascia upper eyelid was ptotic and opened widely when the lata brow suspension,'^-" the Fasanella-Servat proce- mandible was moved to the contralateral side, Opthal- dure,-' the Beard procedure,-- brow suspension using mologic and neurologic evaluations and a CT scan did nonabsorbable suture,-^ and the modified Berke-Neu- not reveal any other abnormality haus procedure,^"" Most of the surgically managed cases have been reported to have good results. However, many patients may require two or more Case 3 operations to achieve acceptable ñinctional and esthet- ic results.-^ A 14-year-old boy reported because he was experi- encing pain in his carious teeth. The right upper eyelid

564 Quintessence International Volume 26, Numher 8/199^ General Dentistry

Fig 2 Marcus Gunn phenomenon: The ptotic nght eyelid shows exag- gerated opening on mandibular movement.

was found to be ptotic and moved wide open on mandibular movements to the contralateral side. However, the left upper eyelid was wide open at normal gaze and drooped down when the mandible was moved to the opposite side. Tbis patient therefore exhibited MGP on the right eyelid and Marin Amat syndrome on tbe left side. The patient had no histor>' of trauma or facial paralysis. This was specifically enquir- ed about many times because Marin Amat syndrome is often associated with trauma or faciai paralysis.-*'' Comprehensive opthalmologic and neurologic work- ups and a CT scan revealed no other abnormalities. This is only tbe third report of both MGP and Marin Amat syndrome in the same patient.-*

Discussion Establishing a diagnosis of MGP is within the domain of the dental surgeon.'* Marcus Gunn phenomenon may be associated with a variety of other opthalmolo- gic, neurologic, and other congenital disorders, which, at times, are not obvious. Therefore, a comprehensive medical evaluation ofthe patient and a detailed history are mandatory. When possible, a family history may be established if MGP appears in the family. Dental practitioners must take special care with patients who exhibit MGP because the eye remains wide open when the various dental procedures are being performed, thereby increasing the risk of eye Fig 3 Marcus Gunn phenomenon: The plodc right eyelid injuries to the patient. The patient must therefore be opens wide on mandibular movement. Mann Amat syn- drome: The left eyelid, which is normally wide open, droops required to wear eye shields at all times during dental down during mandibular movement to the contralateral procedures. side

Quintessengg-i Ml imhpr B/1995 565 General Dentistry

Dental surgeons can also play a very useful role in 12, KirkliamTH. Fatnilial Marcus Gunn phenomenon. Hr J Opthalmol convincing and preparing patients psychologically to 1969:53:282-283. procure surgical treatment for their ptosis. n. Kuder GG, Laws HW. Heriditary Miircus Gunn phenomenon. Can J Opthalmol 1968:3:97-98". 14, DoucEt TW, Crawford JS, The qualification, natural course, and surgial results in 57 eyes wilh Marcus Gunn ijaw-winkinj] Acknowledgmeuts syndrome. Am J Opthalmol 1981:92:702-707. 15, Prall SG, et al. The Marcus Gunn phenomenon: A review of 71 The iiiiihors e.\press iheir llianks to Dr A, V. Kulkarni, Professor and eases, Oplhalmology 1984:9h27-30, Head, Departmcni of Anatomy, for kindly referring cnse I to their clinic. 16, Schultz RO, Burian HM, Bilaleral jaw-winking reflex in associaliun wuh multiple eongenital anomalies. Arch Opihalmol 196O;64;946- 949. 17, Simpson DG. Marcus Gunn phenomenon following squint jnú plosis surgery, definition and review. AMA Arch Oplhalmol i956;56:743-748. References Í8, Dryden RM, et al, Lsvator transposition and frontalis sling 1, Gunn RM, Congenital plosis with peculiar associated muvements of procedure in severe unilalerdi piosis and the paradoxically inner- the affected lid, Trans Opthalmol Soc UK 1883;3:2S3-187. vate Ü le vator. Arch Opthalmol 1982; 100:461-464. 2, Shafer WG, Leiy HM, Hine MK. A Tcxibook of Oral Pathology, ed Beard C, A new treatment for severe unilateral congenital ptosis wilh 4. Philadelphia: Sajrders, [9Hi. jaw-winking. Am J Opthalmol I965;59.252-258, 3, Gorlin RJ, Cohen MM Jr, Levin LS, Syndromes of the Head and Oesleric CS, et al Eye bobbing associated wilh jaw movements Neck, ed 3. New York Oxford University Press, 1990. Oplhülmology 1982:89,63-67, 4, Brooks JK. The Marcus Gunn phenomenon. Discussion and report Putlerman AAM. Jaw-winking blepharoptosis treated by Ilie ofa case. Oral Surg Oral Med Oral Palho! l9S7;64:687-692. Fasanella-Servat procedure. Am J Opthalmol 1973:75: iOI6-1022 5, Creel D, Problems of ocular miswiring in albinism. Duane's Bullock JD, Marcus Gunn Jaw winking ptosis: Classificalion and syndrome and Marcus Gunn phenomenon. Int Opthalmol Clin surgieal management, J Pediatr Opthalmol 1980,17:375- 1984;24:I65-176, 379, 6, Kirkham TH, Paradoxical elevation of eyelid on smiling. Am J Betharia SM, Frontalis sling: A modiljed simple technic. Br J Oplhalraoi I971;72:207-20S. Opthalmol 1985:69:443-445. 7, Parry R, An unusual case of the Marcus Gunn syndrome. Trans Bartkowski SB, et al. Personai experience and the results of surgLcal Opihalmol Soc UK, 1957;77:18I-185, treatmenl of bieph aro ptosis, Klin Oczna I99h93:164-I65. S, Spaeth EB. The Marcus Gunn phenomenon; Discussion, presenta- StasiorOG, Ptosis-Prcoperative examination. J Pediatr Opthalmol tion of four instances and consideration of its surgial correction. Strabismus i982:l9:246-247 Am J Opthalmol 1947;30:14J-i58. Rana PVS, Wadia RS. The Mann Amat syndrome; an unusual facial 9, Wanenberg R. Winking-jaw phenomenon. Arch Neurol Psyehiat synkinesis. J Neurol Neurosurg Psychiatry, 1985:48:939-941. 194S:59'734-753. Freuh BR. Associated facial contractions afler seventh nerve palsy 10, Del Core G, et al. Marcus Gunn phenomenon. 'Jaw winking." Arch mimicking jaw winking. Oplhalmology 19SJ:9O; 1105-1109, Stomatol Napoli l989;3O;389-397. Wartenberg R "Inverted Marcus Gunn phenomenon" (so called 11, Halls HF, et al. Three cases of Marcus Gunn phenomenon in Iwo Marin Amat syndrome). Arch Neurol Psychiatr 1948;60:584- generations. Am J Opthalmol 1949;32:53-59. 596, a

Moving Soon? Please notify us promptly of any change of address to assure an uninterrupted subscription. Complete the form below and send to: 0/ Subscription Department, Quintessence Publishing Co. Inc, 551 North Kimberly Drive, Carol Stream, IL 60188

NAME MOVING FROM:

NEW ADDRESS, {OLD ADDRESS)

CITY, STATE, ZiP. (CITY, STATE, ZIP)

NOTE: To expedite delivery, send the change of address as soon as you know il, and allow 6 weeks tor processing.

566 Quintessence International Volume 26, Numtier