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Show f. elin. Neuro-ophthdlmol. 3: 193-195, 1983. The Pupil in Congenital Third Nerve Misdirection Syndrome DAVID G. LOTUFO, M.D. J. LAWTON SMITH, M.D. GARY R. HOPEN, M.D. ZANE F. POLLARD, M.D. Abstract The involved pupil in the syndrome of aberrant regeneration of the oculomotor nerve usually remains larger than the uninvolved side, or approaches equality in size. A 4-year-old child was seen with congenital aberrant regeneration of the right third nerve. The right pupil was consistently smaller than the left, but particularly so in dim illumination. The question can be raised as to whether relative pupi\.lary miosis more commonly follows third nerve \ \ misdirection in children than adults. Aberrant regeneration of the oculomotor nerve produces a characteristic neuro-ophthalmologic picture. I - 6 With regards to the pupil in this syndrome, Walsh' presented four possibilities: 1) the pupil may return to normal; 2) it may remain widely dilated and fixed to light; 3) an Argyll Robertson like pupil may develop; or 4) the pupil may become smaller than normal and remain sluggish to light. The incidence with which each of these four pupillary patterns develops has not, to our knowledge, been determined. In adults with acquired third nerve palsy, however, the pupil usually either remains dilated or approaches normal size. In this paper, we present a child with congenital third nerve palsy who developed aberrant regeneration in whom the pupil became smaller on the affected side. The purpose of this report is to document that a constricted pupil may follow congenital oculomotor nerve regeneration, and to question whether miosis follows the misdirection syndrome more commonly in children than in adults. Case Report A 4-year-old boy presented to the Bascom Palmer Eye Institute on August 20, 1982, through the From the Department of Ophthalmology. Bascom Palmer Eye Institute, University of Miami School of Medicine, Miami, Florida. September 1983 courtesy of Dr. Carl Pfanstiel. The chief complaint was misalignment of the eyes. His birth was complicated by fetal bradycardia after a 16-hour labor. An occiput posterior presentation required forceps delivery during which he suffered a right frontoparietal orbital skull fracture. On the second day of life, there was marked ptosis on the right, the eye was positioned down and out, and the right pupil was recorded to be large and fixed. The diagnosis of a post-traumatic congenital right third nerve paralysis was made. When he was 1 month old, the right eye would come to the midline and he had recovered some degree of lid elevation on the right. Over the next few months, the head circumference began to enlarge and a right ventriculoperitoneal shunt was required for hydrocephalus. Examination at age 4 revealed normal general physical and neurological findings apart from the eyes. The corrected visual acuity was 20/40 in the right eye and 20/70 in the left eye. Notably, the child preferred to fix with the paretic right eye. As the patient fixed with the right eye, the right upper lid fell about 2 mm below the limbus (Fig. 1). There was a manifest left hypertropia in the primary position. The visual reduction in the left eye was attributed to suppression amblyopia. On downgaze, the right upper lid retracted-the so-called pseudo-Graefe sign (Fig. 2). Horizontal gaze-lid dyskinesis was evident-in that the right palpebral fissure was wider on adduction and narrower on abduction. These findings were classic for aberrant regeneration of the right oculomotor nerve, or the so-called misdirection-in-regeneration syndrome of the right third nerve. The ductions of the right eye were limited to 100 in upgaze, and 200 in downgaze, but abduction was intact and there was only minimal restriction of adduction. The rotations of the left eye were normal. Visual fields were full to confrontation and the fundi were normal. Pupillary examination in a well-lighted room showed little difference in pupillary size (Fig. 3). Both direct (Fig. 4) and consensual pupillary light reactions were absent in the right eye, but were 193 Congenital III Figure 1. In lighted room, note only minimal ptosis of right eye. Patient prefers to fix with this paretic right eye. The pupils appear essentially equal in size. A left hypertropia may be seen. Figure 2. On downgaze, marked retraction ot right upper lid occurs (the pseudo-Graefe sign of aberrant regeneration). Figure 3. In a lighted room, pupils are essentially equal in size. l ..11 Figure 4. Note that the right pupil does not react to light, but the left pupil does become smaller as a consensual response (compare with Fig. 3). Journal of Clinical Neuro-ophthalmology Lotufo et .11. Figure 5. In the semidarkened room, left pupil dilates promptly and the anisocoria is much more obvious. Note that the right pupil is notably smaller than the left. normal in the left eye. In dim illumination, however, the right pupil measured 3 mm in diameter and was consistently smaller than the left pupil which measured 5 mm (Fig. 5). The pupils had been found previously to dilate equally to cocaine drops by one of us (ZRP). Discussion The clinical attributes of the oculomotor nerve aberrant regeneration syndrome are listed in Table l.l-~ This syndrome is most commonly understood to result from extensive and haphazard growth that characterizes the regeneration of injured nerve fibers9 - 10 In this report, we direct attention to the pupil. As noted in Table 1, the pseudo-Argyll Robertson pupil is a commonly recognized pupillary consequence of aberrant regeneration.:l . -I. ~ This situation is similar to the true Argyll Robertson pupil which will react to the near response, but not at all to light. Unlike the true Argyll Robertson pupil, however, the pseudo-Argyll Robertson pupil will also constrict on adduction as well as to the near sykinesis. With regard to the patient presented in this paper, we believe it is uncommon for the affected pupil to be smaller than the other pupil following third nerve paralysis. The fact that this does occur, however, is exemplified by two adult patients with smaller affected pupils reported by Ford et al. H In discussing the patient reported, one observer raised the question as to whether a miotic pupil TABLE l. The Third Nerve Misdirection Syndrome I. Retraction of the globe on attempted vertical gaze 2. Adduction of the globe on attempted vertical gaze 3. Upper lid retraction on attempted down gaze (the pseudoGraefe sign) 4. Narrowing of the fissure on abduction, and widening of the fissure on attempted adduction (the horizontal gaze-lid dyskinesis) 5. Pseudo-Argyll Robertson pupil 6. Relative monocular vertical optokinetic responses September 1983 might not more commonly follow third nerve regeneration in children than adults. Millerl2 found that the majority of oculomotor nerve palsies in the 30 children he reviewed were congenital and were often followed by aberrant regeneration. Perhaps the aberrant regeneration of children with congenital third nerve palsy is more extensive than in adults with acquired third nerve palsy, resulting in a smaller or constricted pupil. To answer this question, the incidence of pupillary change (whether larger, equal, or smaller) must be determined in this syndrome. We are not aware that such a study has been reported. Our patient is presented to raise this question and to stimulate others to help answer it. Summary A 4-year-old boy is documented who developed a smaller pupil following a congenital third nerve palsy. The incidence of relative pupillary constriction following third nerve regeneration in both children and adults warrants further investigation. The question as to whether miosis follows the misdirection syndrome more commonly in children than in adults has been raised. References 1. Cowers, W.R.: The movements of the eyelids. Med. Chir. Tr. 62: 429-440, 1979. 2. Bielschowsky, A.: Lectures on motor .Jnomalies of the eyes: II. Paralysis of individual eye muscles. Arch.Ophthalmol. 13: 33-59, 1935. 3. Bender, M.B.: Synkinetic pupillary phenomenon and the Argyll-Robertson pupil. Arch. Neuflli. Psychiatry 53: 4 18-422, 1945. 4. Bender, M.B., and Alpert, S.: Abnormal ocular and pupillary mivements following oculomotor paralysis. Arch. Ophth.llmo/. 18: 411-414, 1937. 5. Sturm, R.I., and Smith, j.L.: Aberrant regeneration of the oculomotor nerve: Monocular optokinetic response. Trans. Am. Acad. Ophth.llmol. Oto/aryngo/. 69: 1054- lObO, 1%5. b. Forster, R.K., Schatz, N.)., and Smith, j.L.: A subtle eyelid sign in aberrant regeneration of the third nerve. Am. ]. Ophthalmol. 67: 69b-698, 1969. 7. Walsh, F.B.: Third nerve regeneration: A clinical evaluation. Br.]. Ophthalmol. 41: 577-598, 1957. 195 Congenitallll 8. Ford, F.R., Walsh, F.B., and King, A.: Clinical observations on the pupil phenomena resulting from regeneration of the third nerve with special reference to the Argyll-Robertson pupil. Bull. Johns Hopkins Hosp. 68: 309-318, 1941. 9. Ramon y Cajal, S.: Degeneration and Regeneration of the Nervous System. Oxford University Press, New York, 1928. 10. Bender, M.B., and Fulton, ].F.: Functional recovery in ocular muscles of a chimpanzee after section of oculomotor nerve. f. Neurophysiol. 2: 285-292,1939. 11. Ford, F.R., and Woodhall, B.: Phenomena due to misdirection of regenerating fibers of cranial, spinal and autonomic nerves: Clinical observations. Arch. Surg. 36: 480-496, 1938. 12. Miller, N.R.: Solitary oculomotor nerve palsy in children. Am. f. Ophthalmol. 83: 106-111, 1977. Write for reprints to: ]. L. Smith, MD., P.O. Box 016880, Miami, Florida 33101. Journal of Clinical Neuro-ophthalmology |
