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Show Raven Press, New York Neurb-'"anatornicalFeature Photo Pterygoid---Levator Synkinesis The Mafcus Gunn}aw-WinkirtgPhenomerion F.RodrteyEve, M.D., F.R.C.S. TABLE 1. MRrC/IS Gunn Jaw-Winking Phenomenon Unilateral congenital ptosis with elevation of the ptotic lid on opening the mouth or deviating the jaw laterally was originally described by Marcus Gunn as maxillo-palpebral synkinesis in 1883 (1). Synonymous. terms include "pterygoid"levator synkinesis", "trigemino-oculomotor. synkinesis," or, more simply, "the Marcus Gunn jaw"winking phenomenon" (2,3). This unusual entity seems to represent. anomalous innervation between. the motor division of the fifth cranial nerve and the ipsilateral levator palpebrae superioris .•• Typical and atypical variants have been described (Table 1), Poor ipsilateral superior rectus function and/or amblyopia may be associated findings. Theconditionmay improve spontaneously with time, but can be a problem of major cosmetic significance. A 5-year"0Id boy with a typical variant of this syndrome is shown before and after surgical cor" rection. Figure 1 demonstrates 3 mm of left ptosis in primary gaze. Lateral jaw movement to the right resulted in maximal elevation of the ptotic lid which did not occur with jaw movement left (Figs. 2 and 3). A bilateral. disinsertion of the levator muscles (at least 8=10 mm belly of muscle excised) via the posterior approach, was followed by bilateral. autogenous fascia-lata. brow suspension en" suring that the lid height was placed 2 mm higher than on the normal side. Marked postoperative improvement of the jaw-winking is shown in Figs. 4 and 5. The treatment of •this condition de-serves comment (3,4), When there is minimal jawwinking, the ptosis is treated by levator resection. It is recommended that the resection be greater than in uncomplicated congenital ptosis because FroIl1the Department of Ophthalmology, University of Texas Medical Branch, Galveston, Texas. Address correspondence and reprint requests to Dr. F. Rodney Eve, 807 Craig Street, Corpus Christi, Texas. 78404, U.S.A. Typical clinical features Lid elevation on mouth opening (digastric) Lid elevation on jaw movement to opposite side (external pterygoid) Lid elevation movement to same side (internal pterygoid) Atypical Clinical Features Lid elevation on protrusion of jaw Lid elevation on protrusion of tongue Lid elevation on clenching teeth Lid elevation on swallowing Lid elevation on inspiration Lid elevation on smiling Lid elevation with voluntary nystagmus Adapted from Ref. 3. undercorrection is the rule. If there is minimal ptosis (2 mm or less) and minimal jaw-winking (2-3 mmorless) then a Fasanella=Servat procedure. can. be· done. In cases with severe •jawwinking, excision of the levator is performed. One then has. the choice. of correcting the resulting FiG. f. tefl pmsl's I'npnmary gaze. 62 F. RODNEY EVE FIG. 2. Jaw lert~pr,eope,ralir....e photograph. FIG. 3. Elevation of ptotic lid on moving jaw laterally to the right-preoperative photograph. complete ptosis by suspending the lid from the superior rectus or from the brow. However, unilateral brow suspension is cosmetically unacceptable. Lid suspension from the superior rectus muscle, especially if weak, would probably have a shortlived effect. This has led to the recommendation of bilateral levator excisions and bilateral frontalis suspensions in severe cases. The two surgical "pearls" for a successful operation are that the affected muscle must have at least 8-10 mm removed from the belly of the muscle to prevent recurrence of the jaw-winking and that there should FIG. 4. Jaw right-postoperative photograph. FIG. 5. Jaw left-postoperative photograph. be about 2 mm overcorrection with the brow suspension compared to the normal side. REFERENCES 1. Gunn RM. Congenital ptosis with peculiar associated movements of the affected lid. Trans. Ophthalmol. Soc. U.K. 1883;3:283-7. 2. Sano K. Trigemino-oculomotor synkineses. Neurologia 1959;1:29-51. 3. Miller NR. Walsh and Hoyt's Clinical Neuro-ophthalmology. 4th Edition. Baltimore: Williams and Wilkins, 1985;2:94951. 4. Pratt SG, Beyer CK, Johnson Cc. The Marcus Gunn phenomenon: a review of 71 cases. Ophthalmology 1984;90:2730. |