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Spontaneous Cure of Congenital Brown's Syndrome
542
AMERICAN JOURNAL OF OPHTHALMOLOGY
Spontaneous Cure of Congenital Brown's Syndrome Charles R. Leone, Jr., M.D., and Randolph T. Leone, M.S. Department of Ophthalmology, University of Texas Health Science Center, San Antonio. Inquiries to Charles R. Leone, i«, M.D., 7950 Floyd Curl Drive, Suite 705 Medical Center Tower, San Antonio, TX 78229.
The superior oblique tendon sheath syndrome (Brown's) is characterized by a restriction of elevation in adduction with a lesser restriction in the primary and abducted positions.P The condition is usually congenital, but may be acquired. We encountered a case of spontaneous recovery of what has been otherwise thought to be a permanent condition. Shortly after birth congenital Brown's syndrome of the left eye had been diagnosed in a 21-year-old man. He had had several orthoptic examinations, which showed no change through age 15 years. At that time, his visual acuity was 20/20 in both eyes. In the primary position he had an 18-prism diopter right hypertropia and a 6-prism diopter exotropia. He had diplopia in the primary position, but with his head tilted backward, he was orthophoric and had stereopsis. With $aze to the left the deviation measured 9 prism diopters right hypotropia and 9 prism diopters exophoria. With gaze to the right, it measured 25 prism diopters right hypotropia and 4 prism diopters exophoria. The elevated chin position associated with this syndrome drew accusations of snobbish-
October, 1986
ness from his peers. Therefore, at approximately age 16 years, he consciously began to "exercise" his eye. He would forceably attempt to reduce his area of diplopia, particularly when he was reading or watching television. With almost daily exercise from age 16 to 18 years, better movement occurred in the affected eye until he noted no diplopia in the primary position. As he was able to elevate the eye an audible click was heard, and within a year, he had full movement without diplopia (Figure). When last examined three years later, he had full extraocular muscle rotation without an audible click. To our knowledge, there have not been any reported cases of spontaneous cure of congenital Brown's syndrome. Surgical treatment has not been associated with beneficial results, and should only be attempted if the vertical deviation in the primary position produces a cosrnetic defect, or if a cosmetically objectionable head position is assumed." The most plausible explanation for Brown's syndrome is an inability of the superior oblique tendon to pass freely through the trochlea, a function necessary to allow elevation of the eye in adduction;' During the time the patient was exercising his eye, he was not taking anti-inflammatory drugs, which could conceivably reduce swelling in the area of the trochlea. His persistent efforts forcibly to raise his left eye proved to be beneficial. The audible click was concomitant with his noticing improvement, and when he obtained full extraocular muscle rotation without diplopia, the click disappeared. This may have represented a loosening of a fibrous ob-
Figure (Leone and Leone). Top, The patient at age 7 years showing (from left to right) orthophoria in the primary position with the head tilted slightly backward, some restriction in the left eye in gaze upward and to the left, marked restriction in the left eye with gaze upward and to the right, and no restriction in downward gaze. Bottom, Appearance at age 21 years with full ocular rotations in all fields of gaze.
Vol. 102, No.4
Letters to the Journal
struction within the sheath or the trochlea that finally stretched or thinned out, allowing unimpeded passage of the tendon.
References 1. Brown, H. W.: Congenital structural anomalies of the muscles. In Allen, J. H. (ed.): Strabismus Ophthalmic Symposium II. St. Louis, C. V. Mosby, 1958, p. 391. 2. Von Noorden, G. K.: Binocular Vision and Ocular Motility, 2nd ed. St. Louis, C. V. Mosby, 1980, pp. 384-387. 3. Dale, R. T.: Fundamentals of Ocular Motility and Strabismus. New York, Grune and Stratton, 1982, pp. 315-318. 4. Helveston, E. M.: Atlas of Strabismus Surgery, 3rd ed. St. Louis, C. V. Mosby, 1985, p. 186.
Czarnecki's Sign as the Initial Finding in Acquired Oculomotor Synkinesis Terry A. Cox, M.D. Department of Ophthalmology, University of British Columbia, Vancouver. Inquiries to Terry A. Cox, M. D., Department of Ophthalmology, University of British Columbia, 2550 Willow St., Vancouver, British Columbia, Canada V5Z 3N9.
In 1978, Czarnecki and Thompson! described segmental contractions of the iris sphincter associated with eye movements in seven of 14 patients with acquired oculomotor synkinesis (aberrant regeneration of the third nerve). I recently saw a man with a traumatic third nerve palsy who demonstrated Czarnecki's sign before he developed other signs of oculomotor synkinesis. A 23-year-old man was involved in a motor vehicle accident. In addition to other injuries, he sustained craniocerebral trauma with extensive basal skull fracture. In the neurosurgical intensive care unit, he was found to have a complete left third nerve palsy. He gradually recovered from his injuries and left the hospital three weeks after admission. Fifty days after the initial injury, he still had a complete left third nerve palsy. The pupil in that eye did not react to light, but examination at the slit lamp showed that a small segment of the pupillary sphincter, extending from the 1 to 2 o'clock meridians (that is, superotemporal), constrict-
543
ed briskly with attempted downgaze. A followup examination 141J2 weeks after the injury showed the classic findings of acquired oculomotor synkinesis: the upper eyelid raised on downgaze (pseudo-von Graefe's sign) and the eye adducted on attempted upgaze. Adduction and depression were both 50% limited, upgaze was absent, and the upper eyelid drooped 2 mm. The iris sphincter contracted segmentally with downgaze in an area extending from the 3 to 4 o'clock meridians as well as in the previously noted area. No segment of the pupil reacted to light. Czarnecki and Thompson! believed that these segmental contractions of the iris provided evidence that axons originally innervating extraocular muscles were now innervating the iris sphincter. If this theory is correct, Czarnecki's sign should occur late in the development of the acquired oculomotor synkinesis syndrome, because the iris is much further from the superior orbital fissure than are the motor endplates of the extraocular muscles. The findings in my patient implied that regenerating axons reinnervate the ciliary ganglion, not the iris. This seems logical because both extraocular muscle fibers and the ciliary ganglion contain nicotinic cholinergic receptors. An alternative explanation is that acquired oculomotor synkinesis is caused by ephaptic transmission'': subscribers to this hypothesis are spared the task of explaining how an axon originally innervating a muscle can innervate a ganglion cell.
References 1. Czarnecki, J. S. c., and Thompson, H. S.: The iris sphincter in abberant regeneration of the third nerve. Arch. Ophthalmol. 96:1606, 1978. 2. Lepore, F. E., and Glaser, J. S.: Misdirection revisited. A critical appraisal of acquired oculomotor nerve synkinesis. Arch. Ophthalmol. 98:2206, 1980.
Spontaneous Contractions of the Pupillary Sphincter in Traumatic Ophthalmoplegia Terry A. Cox, M.D. Department of Ophthalmology, University of British Columbia. Inquiries to Terry A. Cox, M.D., Department of Ophthalmology, University of British Columbia, 2550 Willow St., Vancouver, British Columbia V5Z 3N9.
AMERICAN JOURNAL OF OPHTHALMOLOGY
Spontaneous Cure of Congenital Brown's Syndrome Charles R. Leone, Jr., M.D., and Randolph T. Leone, M.S. Department of Ophthalmology, University of Texas Health Science Center, San Antonio. Inquiries to Charles R. Leone, i«, M.D., 7950 Floyd Curl Drive, Suite 705 Medical Center Tower, San Antonio, TX 78229.
The superior oblique tendon sheath syndrome (Brown's) is characterized by a restriction of elevation in adduction with a lesser restriction in the primary and abducted positions.P The condition is usually congenital, but may be acquired. We encountered a case of spontaneous recovery of what has been otherwise thought to be a permanent condition. Shortly after birth congenital Brown's syndrome of the left eye had been diagnosed in a 21-year-old man. He had had several orthoptic examinations, which showed no change through age 15 years. At that time, his visual acuity was 20/20 in both eyes. In the primary position he had an 18-prism diopter right hypertropia and a 6-prism diopter exotropia. He had diplopia in the primary position, but with his head tilted backward, he was orthophoric and had stereopsis. With $aze to the left the deviation measured 9 prism diopters right hypotropia and 9 prism diopters exophoria. With gaze to the right, it measured 25 prism diopters right hypotropia and 4 prism diopters exophoria. The elevated chin position associated with this syndrome drew accusations of snobbish-
October, 1986
ness from his peers. Therefore, at approximately age 16 years, he consciously began to "exercise" his eye. He would forceably attempt to reduce his area of diplopia, particularly when he was reading or watching television. With almost daily exercise from age 16 to 18 years, better movement occurred in the affected eye until he noted no diplopia in the primary position. As he was able to elevate the eye an audible click was heard, and within a year, he had full movement without diplopia (Figure). When last examined three years later, he had full extraocular muscle rotation without an audible click. To our knowledge, there have not been any reported cases of spontaneous cure of congenital Brown's syndrome. Surgical treatment has not been associated with beneficial results, and should only be attempted if the vertical deviation in the primary position produces a cosrnetic defect, or if a cosmetically objectionable head position is assumed." The most plausible explanation for Brown's syndrome is an inability of the superior oblique tendon to pass freely through the trochlea, a function necessary to allow elevation of the eye in adduction;' During the time the patient was exercising his eye, he was not taking anti-inflammatory drugs, which could conceivably reduce swelling in the area of the trochlea. His persistent efforts forcibly to raise his left eye proved to be beneficial. The audible click was concomitant with his noticing improvement, and when he obtained full extraocular muscle rotation without diplopia, the click disappeared. This may have represented a loosening of a fibrous ob-
Figure (Leone and Leone). Top, The patient at age 7 years showing (from left to right) orthophoria in the primary position with the head tilted slightly backward, some restriction in the left eye in gaze upward and to the left, marked restriction in the left eye with gaze upward and to the right, and no restriction in downward gaze. Bottom, Appearance at age 21 years with full ocular rotations in all fields of gaze.
Vol. 102, No.4
Letters to the Journal
struction within the sheath or the trochlea that finally stretched or thinned out, allowing unimpeded passage of the tendon.
References 1. Brown, H. W.: Congenital structural anomalies of the muscles. In Allen, J. H. (ed.): Strabismus Ophthalmic Symposium II. St. Louis, C. V. Mosby, 1958, p. 391. 2. Von Noorden, G. K.: Binocular Vision and Ocular Motility, 2nd ed. St. Louis, C. V. Mosby, 1980, pp. 384-387. 3. Dale, R. T.: Fundamentals of Ocular Motility and Strabismus. New York, Grune and Stratton, 1982, pp. 315-318. 4. Helveston, E. M.: Atlas of Strabismus Surgery, 3rd ed. St. Louis, C. V. Mosby, 1985, p. 186.
Czarnecki's Sign as the Initial Finding in Acquired Oculomotor Synkinesis Terry A. Cox, M.D. Department of Ophthalmology, University of British Columbia, Vancouver. Inquiries to Terry A. Cox, M. D., Department of Ophthalmology, University of British Columbia, 2550 Willow St., Vancouver, British Columbia, Canada V5Z 3N9.
In 1978, Czarnecki and Thompson! described segmental contractions of the iris sphincter associated with eye movements in seven of 14 patients with acquired oculomotor synkinesis (aberrant regeneration of the third nerve). I recently saw a man with a traumatic third nerve palsy who demonstrated Czarnecki's sign before he developed other signs of oculomotor synkinesis. A 23-year-old man was involved in a motor vehicle accident. In addition to other injuries, he sustained craniocerebral trauma with extensive basal skull fracture. In the neurosurgical intensive care unit, he was found to have a complete left third nerve palsy. He gradually recovered from his injuries and left the hospital three weeks after admission. Fifty days after the initial injury, he still had a complete left third nerve palsy. The pupil in that eye did not react to light, but examination at the slit lamp showed that a small segment of the pupillary sphincter, extending from the 1 to 2 o'clock meridians (that is, superotemporal), constrict-
543
ed briskly with attempted downgaze. A followup examination 141J2 weeks after the injury showed the classic findings of acquired oculomotor synkinesis: the upper eyelid raised on downgaze (pseudo-von Graefe's sign) and the eye adducted on attempted upgaze. Adduction and depression were both 50% limited, upgaze was absent, and the upper eyelid drooped 2 mm. The iris sphincter contracted segmentally with downgaze in an area extending from the 3 to 4 o'clock meridians as well as in the previously noted area. No segment of the pupil reacted to light. Czarnecki and Thompson! believed that these segmental contractions of the iris provided evidence that axons originally innervating extraocular muscles were now innervating the iris sphincter. If this theory is correct, Czarnecki's sign should occur late in the development of the acquired oculomotor synkinesis syndrome, because the iris is much further from the superior orbital fissure than are the motor endplates of the extraocular muscles. The findings in my patient implied that regenerating axons reinnervate the ciliary ganglion, not the iris. This seems logical because both extraocular muscle fibers and the ciliary ganglion contain nicotinic cholinergic receptors. An alternative explanation is that acquired oculomotor synkinesis is caused by ephaptic transmission'': subscribers to this hypothesis are spared the task of explaining how an axon originally innervating a muscle can innervate a ganglion cell.
References 1. Czarnecki, J. S. c., and Thompson, H. S.: The iris sphincter in abberant regeneration of the third nerve. Arch. Ophthalmol. 96:1606, 1978. 2. Lepore, F. E., and Glaser, J. S.: Misdirection revisited. A critical appraisal of acquired oculomotor nerve synkinesis. Arch. Ophthalmol. 98:2206, 1980.
Spontaneous Contractions of the Pupillary Sphincter in Traumatic Ophthalmoplegia Terry A. Cox, M.D. Department of Ophthalmology, University of British Columbia. Inquiries to Terry A. Cox, M.D., Department of Ophthalmology, University of British Columbia, 2550 Willow St., Vancouver, British Columbia V5Z 3N9.