From the Department of Ophthalmology, University of Florida, Gainesville, Florida.
The authors have no financial or proprietary interest in the materials presented herein.
Address correspondence to Matthew H. Oltmanns, MD, University of Florida, Department of Ophthalmology, 2000 SW Archer Road, 4th Floor—Eye Specialists, Gainesville, FL 32653. E-mail: email@example.com
Duane retraction syndrome (DRS) type I, the most common of the three forms of this condition, is characterized by unilateral limitation of abduction with normal adduction, as well as ipsilateral palpebral fissure narrowing on adduction and widening on attempted abduction. Marcus Gunn jaw-winking is a condition displaying unilateral ptosis, with retraction of the ptotic eyelid on stimulation of the ipsilateral pterygoid muscle. Inappropriate tearing that occurs when eating or sucking is referred to as paradoxical gustolacrimal tearing, or crocodile tears. In this article, we describe a boy who presented with DRS type I, Marcus Gunn jaw-winking, and crocodile tears in the same eye. To our knowledge, this is the first report in the literature of a patient with these three syndromes in the same eye.
A 17-month-old boy was referred to our clinic by his pediatrician for evaluation of a possible exotropia. The patient’s mother stated that she seemed to occasionally notice one eye drifting out. The patient was the product of an uncomplicated full-term pregnancy. No medical or developmental issues were noted, and social and family histories were unremarkable. The patient’s mother reported no exposure to toxins or potentially toxic medications during pregnancy.
Examination revealed central, steady, maintained fixation in both eyes. Hirschberg testing showed no tropia, and pupillary examination was normal. Extraocular motility testing was remarkable for limited abduction of the right eye (Fig. 1), with significant ipsilateral palpebral fissure narrowing of the same eye on adduction. Upshooting of the right eye was seen on adduction. Motilities were normal in the left eye. A 2- to 3-mm ptosis of the right upper eyelid was also noted (Fig. 2). However, when the patient drank from his bottle, the ptotic eyelid retracted (Fig. 3). In addition, tears were noted to form in the right eye as the patient was drinking from his bottle (Fig. 4). Age-appropriate hyperopia was found on retinoscopy performed after cycloplegia. Anterior and posterior segment health examination was unremarkable, and intraocular pressure was normal to palpation in both eyes.
Figure 1. Extraocular Motility Testing Showed Limited Abduction of the Right Eye.
Figure 2. Ptosis of 2 to 3 mm in the Right Upper Eyelid Was Noted.
Figure 3. The Ptosis Improved when the Patient Drank from His Bottle.
Figure 4. Tears Formed in the Right Eye of the Patient as He Drank from His Bottle.
Based on the external examination and motility findings, diagnoses of DRS type I, Marcus Gunn jaw-winking, and crocodile tears, all in the right eye, were made.
DRS type I is a congenital aberrant innervation syndrome characterized by unilateral limitation of abduction with normal adduction, as well as ipsilateral palpebral fissure narrowing on adduction and widening on attempted abduction.1 Electromyogram testing in these patients shows diminished electrical activity of the lateral rectus muscle on attempted abduction, but reveals simultaneous contraction of the lateral and medial recti on adduction.1 These electromyogram findings are consistent with anatomical dissections that have revealed innervation of the lateral rectus muscle by a branch of cranial nerve III in patients with DRS,2–4 as well as with magnetic resonance imaging evidence of absence of cranial nerve VI in patients with this condition.5,6
Marcus Gunn jaw-winking, also considered to be a syndrome of congenital aberrant innervation, is likely caused by connection of an anomalous branch of cranial nerve V to the branch of cranial nerve III subserving the levator muscle.7 Examination findings include unilateral ptosis, with retraction of the ptotic eyelid on stimulation of the ipsilateral pterygoid muscle. Stimulation can be produced by lateral jaw movement, chewing, sucking, or opening of the mouth.
Lacrimation evoked by chewing or sucking, known as crocodile tears, is a third syndrome of congenital aberrant innervation. Crocodile tears are commonly seen in patients with DRS.8
The most recent report in the literature of a patient with DRS and Marcus Gunn jaw-winking in the same eye was in this journal in 1983.9 Their patient’s findings were in the left eye, which has been noted to be the eye more commonly affected by DRS (59% in the left eye, 23% in the right eye, 18% in both eyes).10 As noted above, our patient’s findings were in the right eye, making our case somewhat more unusual. In addition, their patient did not display crocodile tears as in our patient.
Various theories as to why these syndromes occur have been offered. Developing mesenchyme and muscle masses attract embryonal nerves. Delayed development of primordial ocular muscles may remove the stimulus necessary for the ventral abducens nerve fibers to innervate the lateral rectus.2 Because the oculomotor nerve develops later than the abducens nerve, branches from the third cranial nerve may then be erroneously attracted to the earlier-developing lateral rectus muscle.11 The result of such misdirected innervation is seen clinically as DRS. Alternative theories suggest that brainstem changes may be responsible for DRS. This hypothesis is supported by the fact that brainstem functions, such as auditory evoked responses, are abnormal in patients with this condition.12
Intraneural association fibers have been postulated to be the cause of Marcus Gunn jaw-winking.9 These fibers could account for the anomalous interaction between motor branches of the trigeminal and oculomotor nerves that produces this syndrome.
The most common explanation for crocodile tears suggests a disturbance in the development of the sixth and seventh nerve nuclei and the superior lacrimal nucleus in the brainstem.8 Ramsay and Taylor suggested nuclear damage or dysgenesis near the abducens nucleus, with anomalous lacrimation caused by innervation of the lacrimal gland by fibers subserving salivation.13
As noted above, our case is unique in that it is the first report in the literature of these three syndromes of anomalous innervation occurring in the same eye. What makes this case even more interesting is that there was no known toxin exposure during the pregnancy. Thalidomide has been shown to cause clusters of aberrant innervation syndromes. However, there was no intrauterine exposure to this medication during pregnancy. Fortunately, our patient’s ocular growth and development was not severely affected. We will continue to monitor him closely.
- Alexandrakis G, Saunders RA. Duane retraction syndrome. Ophthalmol Clin North Am. 2001;14:407–417. doi:10.1016/S0896-1549(05)70238-8 [CrossRef]
- Bremer JL. Recurrent branches of the abducens nerve in human embryos. Am J Anat. 1920;28:371–397. doi:10.1002/aja.1000280205 [CrossRef]
- Hoyt WF, Nichtigaller H. Anomalies of ocular motor nerves: Neuroanatomic correlates of paradoxical innervation in Duane’s syndrome and related congenital ocular motor disorders. Am J Ophthalmol. 1965;60:443–448.
- Tillack TW, Winer JA. Anomaly of the abducens nerve. Yale J Biol Med. 1961–62;34:620–624.
- Kim JH, Hwang JM. Presence of the abducens nerve according to the type of Duane’s retraction syndrome. Ophthalmology. 2005;112:109–113. doi:10.1016/j.ophtha.2004.06.040 [CrossRef]
- Parsa CF, Grant E, Dillon WP Jr, du Lac S, Hoyt WF. Absence of the abducens nerve in Duane syndrome verified by magnetic resonance imaging. Am J Ophthalmol. 1998;125:399–401. doi:10.1016/S0002-9394(99)80158-5 [CrossRef]
- Miller NR. Walsh and Hoyt’s Clinical Neuro-Ophthalmology, 4th ed. Vol 2. Baltimore: Williams and Wilkins; 1985:949–951.
- Miller MT, Stromland K, Ventura L. Congenital aberrant tearing: a re-look. Trans Am Ophthalmol Soc. 2008;106:100–116.
- Isenberg S, Blechman B. Marcus Gunn jaw winking and Duane’s retraction syndrome. J Pediatr Ophthalmol Strabismus. 1983;20:235–237.
- DeRespinis PA, Caputo AR, Wagner RS, Guo S. Duane’s retraction syndrome. Surv Ophthalmol. 1993;38:257–288. doi:10.1016/0039-6257(93)90077-K [CrossRef]
- Freedman HL, Kushner BJ. Congenital ocular aberrant innervation: new concepts. J Pediatr Ophthalmol Strabismus. 1997;34:10–16.
- Jay W, Hoyt CS. Abnormal brainstem auditory evoked potentials in Stilling-Turk-Duane retraction syndrome. Am J Ophthalmol. 1980;89:814–818.
- Ramsay J, Taylor D. Congenital crocodile tears: a key to the aetiology of Duane’s syndrome. Br J Ophthalmol. 1980;64:518–522. doi:10.1136/bjo.64.7.518 [CrossRef]