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Acquired Brown's Syndrome Caused by Focal Metastasis to the Superior Oblique Muscle
598
April, 1987
AMERICAN JOURNAL OF OPHTHALMOLOGY
4. Norcia, A. M., Clarke, M., and Tyler, C. W.: Digital filtering and robust regression techniques for estimating sensory thresholds from the evoked po tential. IEEE Trans. Biomed. Eng. 2:26, 1985. 5. Orel-Bixler, D., and Norcia, A. M.: Subjective and VEP acuity for normal and abnormally sighted children. ARVO Abstracts. Supplement to Invest. Ophthalmol. Vis. Sci. Philadelphia, J. B. Lippincott, 1986, p. 152.
Acquired Brown's Syndrome Caused by Focal Metastasis to the Superior Oblique Muscle Michael L. Slavin, M . D . , and Seymour G o o d s t e i n , M . D . Division of Neuro-ophthalmology, Department of Ophthalmology, Long Island Jewish Medical Center, and Health Sciences Center, State University of New York at Stony Brook. Inquiries to Michael Slavin, M.D., Long Island Jewish Medical Center, New Hyde Park, NY 11042. Brown's syndrome 1 comprises congenital unilateral inability to raise an adducted eye, associated with a positive traction test. Most investigators believe that a lesion involving the superior oblique tendon, posterior to the trochlea (a taut superior oblique tendon, adhesions between the tendon and its sheath, and the like) would account for the ability of the superi or oblique muscle to contract normally but relax insufficiently. Cases of acquired Brown's syn drome 2 have been reported to occur after tuck ing of the superior oblique tendon, with in volvement of the tendon by nodules in rheumatoid arthritis, after surgery in the vicin ity of the trochlea, and with inflammatory con ditions of the superior oblique tendon. We encountered a case of acquired Brown's syn drome caused by isolated metastatic breast car cinoma of the posterior superior oblique mus cle. A 65-year-old woman with metastatic breast carcinoma had vertical diplopia on left upward gaze. There were no associated headaches, ataxia, weakness, numbness, or other neuro logic symptoms. Neuro-ophthalmic examina tion disclosed 1 mm of right proptosis and marked (20% of normal) limita'tion of elevation of the right eye on adduction. Elevation on abduction was normal and no overaction of the right superior oblique muscle was seen. Three prism diopters of left hypertropia were detect ed in primary gaze, which increased to 16
Figure (Slavin and Goodstein). Computed midorbital tomogram (coronal view) demonstrating ir regular mass lesion of the right superior oblique muscle (long arrow). Note the normal fellow superior oblique muscle (short arrow), and medial recti mus cles (arrowheads). prism diopters on left gaze. The patient was orthophoric on left downward gaze. Forced duction test disclosed mechanical restriction of the right globe on elevation in adduction. A mass lesion within the confines of the mid and posterior aspect of the right superior oblique muscle was demonstrated on computed tomog raphy (Figure). A presumptive diagnosis of metastatic breast carcinoma was made and ra diation therapy (3,000 rads) was subsequently administered. Subjective diplopia and objec tive ophthalmoplegia abated within three months. Discrete metastatic carcinoma involving an extraocular muscle 3 ' 4 with sparing of other or bital tissues is uncommon. The lesion in this case evidently prevented the relaxation of the superior oblique muscle, and posterior-anterior excursion of its tendon through the trochlea, which is necessary on upward gaze.
References 1. Brown, H. W.: Isolated inferior oblique paraly sis. An analysis of 97 cases. Trans. Am. Ophthalmol. Soc. 55:415, 1957.
Correspondence
Vol. 103, No. 4
2. : True and simulated superior oblique tendon sheath syndromes. Doc. Ophthalmol. 34:123, 1973. 3. Ashton, N., and Morgan, G.: Discrete carcinomatous metastases in the extraocular muscles. Br. J. Ophthalmol. 58:112, 1974. 4. Trokel, S. L., and Hilal, S. K.: Recognition and differential diagnosis of enlarged extraocular mus cles in computed tomography. Am. J. Ophthalmol. 87:503, 1979.
Correspondence Correspondence concerning recent articles or other mate rial published in THE JOUKNAL should be submitted within six weeks of publication. Correspondence must be typed double-spaced, on 8V4 x 11-inch bond paper with 1%-inch margins on allfoursides and should be no more than two typewritten pages in length. Every effort will be made to resolve controversies between the correspondents and the authors of the article before formal publication.
Pars Planitis and Autoimmune Endotheliopathy EDITOR: Regarding the article, "Pars planitis and au toimmune endotheliopathy" (Am. J. Ophthal mol. 102:633, November 1986) by AH A. Khodadoust, Yadolah Karnama, Kathleen M. Stoessel, and James E. Puklin, I agree that autoimmune endotheliopathy exists but dis agree with the interpretation in these cases of pars planitis. The authors interpreted inferior corneal endothelial changes as evidence of an endothelial rejection line analogous to that seen in graft rejection. I have seen similar patterns in patients with chronic iridocyclitis who did not have pars planitis. In 1981, Lucchese and I1 attributed the inferior opacification of the cornea to fibrous metaplasia of the endothelium secondary to keratic precipi tates. I have also observed a similar finding in patients with pars planitis. Do the authors believe that there is enough evidence to support their diagnosis of an au toimmune reaction against endothelium? Could gravity have caused the keratic precipi tates to collect inferiorly and the precipitates themselves (which often contain macrophages) have caused endothelial damage? If this was autoimmune why are all their rejec
599
tion lines inferior? The inferior cornea is not usually the only site of graft rejection. Final ly, why does not endothelial rejection pro gress to cover the whole cornea in untreated patients? Perhaps what the authors described is an epiphenomenon occurring from nonspecific sensitized cells precipitating out on the endo thelium and not actually a rejection. They may have described a mechanical or chemical irritation from the keratic precipitates. HOWARD H. TESSLER, M.D. Chicago, Illinois
Reference 1. Lucchese, N., and Tessler, H.: Keratitis associ ated with chronic iridocyclitis. Am. J. Ophthalmol. 92:717, 1981. Reply EDITOR: Dr. Tessler's question is a good one, and the answer is no. We have all seen numerous patients with long-standing hypopyon or uveitis in whom the entire corneal endothelium has been covered with a large number of mutton-fat-like keratic precipitates without any corneal edema, endothelial metaplasia, or other evidence of endothelial damage. Thus, the inflammatory cells in these patients do not have any mechanical, chemical, or irritat ing effects on endothelial cells, Keratic precip itates linearly arranged (endothelial rejection line) to our knowledge have been observed only in immune response. Fibrous metaplasia, although nonspecific, has been noted after endothelial allograft rejection (often referred to as retrocorneal membrane). The question on why the rejection line starts from the inferior portion of the cornea and does not progress to cover the entire en dothelium in untreated patients is legitimate. We do not know the answer. Gravity could be a factor (similar to distribution of the lesion in pars planitis) and in untreated cases the dis ease process might be a self-limited phenome non. In reference to Drs. Lucchese and Tessler's article, the corneal changes they describe might as well represent an autoimmune pro cess directed against the stroma or Descemet's membrane. There is an impairment of
April, 1987
AMERICAN JOURNAL OF OPHTHALMOLOGY
4. Norcia, A. M., Clarke, M., and Tyler, C. W.: Digital filtering and robust regression techniques for estimating sensory thresholds from the evoked po tential. IEEE Trans. Biomed. Eng. 2:26, 1985. 5. Orel-Bixler, D., and Norcia, A. M.: Subjective and VEP acuity for normal and abnormally sighted children. ARVO Abstracts. Supplement to Invest. Ophthalmol. Vis. Sci. Philadelphia, J. B. Lippincott, 1986, p. 152.
Acquired Brown's Syndrome Caused by Focal Metastasis to the Superior Oblique Muscle Michael L. Slavin, M . D . , and Seymour G o o d s t e i n , M . D . Division of Neuro-ophthalmology, Department of Ophthalmology, Long Island Jewish Medical Center, and Health Sciences Center, State University of New York at Stony Brook. Inquiries to Michael Slavin, M.D., Long Island Jewish Medical Center, New Hyde Park, NY 11042. Brown's syndrome 1 comprises congenital unilateral inability to raise an adducted eye, associated with a positive traction test. Most investigators believe that a lesion involving the superior oblique tendon, posterior to the trochlea (a taut superior oblique tendon, adhesions between the tendon and its sheath, and the like) would account for the ability of the superi or oblique muscle to contract normally but relax insufficiently. Cases of acquired Brown's syn drome 2 have been reported to occur after tuck ing of the superior oblique tendon, with in volvement of the tendon by nodules in rheumatoid arthritis, after surgery in the vicin ity of the trochlea, and with inflammatory con ditions of the superior oblique tendon. We encountered a case of acquired Brown's syn drome caused by isolated metastatic breast car cinoma of the posterior superior oblique mus cle. A 65-year-old woman with metastatic breast carcinoma had vertical diplopia on left upward gaze. There were no associated headaches, ataxia, weakness, numbness, or other neuro logic symptoms. Neuro-ophthalmic examina tion disclosed 1 mm of right proptosis and marked (20% of normal) limita'tion of elevation of the right eye on adduction. Elevation on abduction was normal and no overaction of the right superior oblique muscle was seen. Three prism diopters of left hypertropia were detect ed in primary gaze, which increased to 16
Figure (Slavin and Goodstein). Computed midorbital tomogram (coronal view) demonstrating ir regular mass lesion of the right superior oblique muscle (long arrow). Note the normal fellow superior oblique muscle (short arrow), and medial recti mus cles (arrowheads). prism diopters on left gaze. The patient was orthophoric on left downward gaze. Forced duction test disclosed mechanical restriction of the right globe on elevation in adduction. A mass lesion within the confines of the mid and posterior aspect of the right superior oblique muscle was demonstrated on computed tomog raphy (Figure). A presumptive diagnosis of metastatic breast carcinoma was made and ra diation therapy (3,000 rads) was subsequently administered. Subjective diplopia and objec tive ophthalmoplegia abated within three months. Discrete metastatic carcinoma involving an extraocular muscle 3 ' 4 with sparing of other or bital tissues is uncommon. The lesion in this case evidently prevented the relaxation of the superior oblique muscle, and posterior-anterior excursion of its tendon through the trochlea, which is necessary on upward gaze.
References 1. Brown, H. W.: Isolated inferior oblique paraly sis. An analysis of 97 cases. Trans. Am. Ophthalmol. Soc. 55:415, 1957.
Correspondence
Vol. 103, No. 4
2. : True and simulated superior oblique tendon sheath syndromes. Doc. Ophthalmol. 34:123, 1973. 3. Ashton, N., and Morgan, G.: Discrete carcinomatous metastases in the extraocular muscles. Br. J. Ophthalmol. 58:112, 1974. 4. Trokel, S. L., and Hilal, S. K.: Recognition and differential diagnosis of enlarged extraocular mus cles in computed tomography. Am. J. Ophthalmol. 87:503, 1979.
Correspondence Correspondence concerning recent articles or other mate rial published in THE JOUKNAL should be submitted within six weeks of publication. Correspondence must be typed double-spaced, on 8V4 x 11-inch bond paper with 1%-inch margins on allfoursides and should be no more than two typewritten pages in length. Every effort will be made to resolve controversies between the correspondents and the authors of the article before formal publication.
Pars Planitis and Autoimmune Endotheliopathy EDITOR: Regarding the article, "Pars planitis and au toimmune endotheliopathy" (Am. J. Ophthal mol. 102:633, November 1986) by AH A. Khodadoust, Yadolah Karnama, Kathleen M. Stoessel, and James E. Puklin, I agree that autoimmune endotheliopathy exists but dis agree with the interpretation in these cases of pars planitis. The authors interpreted inferior corneal endothelial changes as evidence of an endothelial rejection line analogous to that seen in graft rejection. I have seen similar patterns in patients with chronic iridocyclitis who did not have pars planitis. In 1981, Lucchese and I1 attributed the inferior opacification of the cornea to fibrous metaplasia of the endothelium secondary to keratic precipi tates. I have also observed a similar finding in patients with pars planitis. Do the authors believe that there is enough evidence to support their diagnosis of an au toimmune reaction against endothelium? Could gravity have caused the keratic precipi tates to collect inferiorly and the precipitates themselves (which often contain macrophages) have caused endothelial damage? If this was autoimmune why are all their rejec
599
tion lines inferior? The inferior cornea is not usually the only site of graft rejection. Final ly, why does not endothelial rejection pro gress to cover the whole cornea in untreated patients? Perhaps what the authors described is an epiphenomenon occurring from nonspecific sensitized cells precipitating out on the endo thelium and not actually a rejection. They may have described a mechanical or chemical irritation from the keratic precipitates. HOWARD H. TESSLER, M.D. Chicago, Illinois
Reference 1. Lucchese, N., and Tessler, H.: Keratitis associ ated with chronic iridocyclitis. Am. J. Ophthalmol. 92:717, 1981. Reply EDITOR: Dr. Tessler's question is a good one, and the answer is no. We have all seen numerous patients with long-standing hypopyon or uveitis in whom the entire corneal endothelium has been covered with a large number of mutton-fat-like keratic precipitates without any corneal edema, endothelial metaplasia, or other evidence of endothelial damage. Thus, the inflammatory cells in these patients do not have any mechanical, chemical, or irritat ing effects on endothelial cells, Keratic precip itates linearly arranged (endothelial rejection line) to our knowledge have been observed only in immune response. Fibrous metaplasia, although nonspecific, has been noted after endothelial allograft rejection (often referred to as retrocorneal membrane). The question on why the rejection line starts from the inferior portion of the cornea and does not progress to cover the entire en dothelium in untreated patients is legitimate. We do not know the answer. Gravity could be a factor (similar to distribution of the lesion in pars planitis) and in untreated cases the dis ease process might be a self-limited phenome non. In reference to Drs. Lucchese and Tessler's article, the corneal changes they describe might as well represent an autoimmune pro cess directed against the stroma or Descemet's membrane. There is an impairment of