Correspondence
Fig. 3 — Histology of skin biopsy with positive markers for melanoma. (A) Biopsy of left chest lesion showing a nodular proliferation in soft tissue composed of markedly atypical melanocytes arranged in nests (hematoxylin and eosin stain, original magnification 200 magnification; inset original magnification 20). (B) The tumour cells express MART-1 (melanomaassociated antigen recognized by T cells) by immunohistochemistry, confirming the diagnosis (original magnification 200).
REFERENCES 1. Rosenberg C, Finger PT. Cutaneous malignant melanoma metastatic to the eye, lids, and orbit. Surv Ophthalmol. 2008;53:187-202. 2. Zografos L, Ducrey N, Beati D, et al. Metastatic melanoma in the eye and orbit. Ophthalmology. 2003;110:2245-56. 3. Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology. 1997;104: 1265-76. 4. Keating GM. Vemurafenib: in unresectable or metastatic melanoma. BioDrugs. 2012;26:325-34.
Abducens nerve in a patient with Duane retraction syndrome An 8-year-old male was referred for left exotropia since early childhood. His visual acuities were 20/30 OU. He had 2 prism diopters of exophoria at distance and 20 prism diopters of left exotropia at near in the primary position. He had a left adduction deficit (Fig. 1A). Although he could fully abduct the left eye with maximal effort (Fig. 1A), there was a small abduction limitation most of the time, with limited abduction saccade. The left fissure narrowed on attempted adduction, and a left upshoot was observed. Anterior and posterior segment examinations were normal. High-resolution, T2-weighted magnetic resonance imaging (MRI; slice thickness, 0.7 mm) did not reveal a left abducens nerve in the cistern or orbit (Fig. 1B–D).
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5. Sosman JA, Kim KB, Schuchter L, et al. Survival in BRAF V600mutant advanced melanoma treated with vemurafenib. N Engl J Med. 2012;366:707-14. 6. Chapman PB, Hauschild A, Robert C, et al. Improved survival with vemurafenib in melanoma with BRAF V600E mutation. N Engl J Med. 2011;364:2507-16. Can J Ophthalmol 2014;49:e49–e52 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.12.012
Causes of adduction limitation include innervational causative factors such as internuclear ophthalmoplegia, partial third cranial nerve palsy, myasthenia gravis, orbital apex or cavernous sinus syndrome, type 2 and 3 Duane retraction syndrome (DRS), and Miller Fisher syndrome, as well as mechanical causes including orbit mass, orbital medial wall fracture, and orbital lateral wall fracture with an entrapment of the surrounding tissue. Inflammatory myositis, trauma to medial rectus, slipped medial rectus, and chronic progressive external ophthalmoplegia may also result in adduction limitation. Our patient did not show any ptosis, pupillary abnormalities, or orbital abnormalities. Neurologic examination was normal except for the limited adduction. He denied any history of trauma or eye surgery. In addition, the left abducens nerve was absent on MRI, whereas the right abducens nerve was clearly defined. There was no limitation of elevation or depression, or pupil abnormality that would
Correspondence
Fig. 1 — A, Ocular versions showing adduction deficit, upshoot, and fissure narrowing on attempting adduction in the left eye. B–D, Three axial magnetic resonance imaging slices showing the whole path of the cisternal segment of the right abducens nerves (arrows), as linear dark structures emerging from the pontomedullary sulcus (B), coursing in a superior oblique direction (C), and finally entering Dorello canal (D). The left abducens nerve was not observed.
have suggested a third cranial nerve palsy. Narrowing of the palpebral fissure OS in adduction was characteristic for DRS. Kim and Hwang1,2 reported the presence of the abducens nerve in all of their patients with type 2 DRS. Denis et al.3,4 did not observe the abducens nerve in the cistern of a patient with type 2 DRS using MRI, but did identify a severely hypoplastic abducens nerve in the orbit. Yonghong et al.5 reported a hypoplastic abducens nerve in a patient with DRS type 2. However, the image in their publication showed what appeared to be a normal-diameter abducens nerve compared with the contralateral side, before the nerve was abruptly cut off in the cistern (serial images were not shown). In our patient, full abduction could be attained with maximal effort. The almost normal abduction in DRS could be explained by the aberrant innervation of the lower branch of the oculomotor nerve.6 As previously
described in many pathologic studies,6 the lateral rectus may be innervated by the oculomotor branches even though the abducens nerve is absent. Scott and Wong7 have reported that the lateral rectus muscle may show healthy, well-formed muscle bundles in areas innervated by fibres from the oculomotor nerve, lending further support to this explanation. Further detailed orbital imaging or pathologic examination to identify the small orbital branches of the oculomotor and abducens nerve would be required to fully explain the abnormal eye movement in patients with DRS. In our case, 2 possible explanations should be considered. First, this case may be the first report of a complete absence of the abducens nerve in type 2 DRS. A second possibility is that this patient may represent an extreme case of type 3 DRS, showing a predominant limitation of adduction compared with abduction. Even though his abduction could be full with the greatest effort, but his saccade to the left was limited compared to the right eye. CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
e53
Correspondence Consequently, we questioned the diagnosis of type 2 DRS in this patient. In conclusion, along with a careful clinical examination, high-resolution MRI to clearly identify the path of the abducens may be helpful in the evaluation of DRS.
Supported by: This work was supported by Seoul R&BD Program (ST090841) and by a grant of Korea Health 21 R&D Project, Ministry of Health, Welfare and Family Affairs, Republic of Korea (A080299). Jae Hyoung Kim and Jeong-Min Hwang Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea Correspondence to: Jeong-Min Hwang, MD: [email protected]
Bilateral disc edema in a patient with Vogt–Koyanagi–Harada disease Vogt–Koyanagi–Harada disease (VKH) is characterized by bilateral diffuse choroiditis with exudative retinal detachment.1 In addition to visual loss, the presence of hearing loss, tinnitus, poliosis, vitiligo, or meningismus could be helpful for the diagnosis. However, in cases that show only marked bilateral optic disc edema without any signs or symptoms, it could be difficult to consider VKH, and other conditions such as elevated blood pressure/intracranial pressure and inflammation or infection may be considered in the differential diagnosis. We present a patient with remarkable optic disc edema as the only sign of VKH. A 40-year-old Korean female presented with bilaterally blurred vision a week ago. She had a mild headache and nausea, but did not have diplopia, vomiting, ocular pain, hearing loss, tinnitus, alopecia, poliosis, vitiligo, or meningismus, or previous penetrating ocular trauma or surgery. The best corrected visual acuities were 20/20 OU. Slitlamp examination showed no cells or flare in the anterior chamber or vitreous, as well as clear cornea and lenses without any precipitates or synechiae. Intraocular pressure was normal through all examinations. Hardy-Rand-Rittler color test was normal OU. On automated perimetry, blind spots were enlarged OU. Funduscopy revealed marked optic disc edema OU and splinter optic disc hemorrhage OS (Fig. 1A, 1B). Fluorescein angiography showed staining of the optic disc, multiple pinpoint leakages at the level of the retinal pigment epithelium, and late peripapillary dye pooling (Fig. 1C, 1D). Enhanced-depth imaging optical coherence
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CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
REFERENCES 1. Kim JH, Hwang JM. Presence of the abducens nerve according to the type of Duane’s retraction syndrome. Ophthalmology. 2005;112:109-13. 2. Kim JH, Hwang JM. Abducens nerve is present in patients with type 2 Duane’s retraction syndrome. Ophthalmology. 2012;119:403-6. 3. Denis D, Dauletbekov D, Alessi G, Chapon F, Girard N. Duane retraction syndrome: MRI features in two cases. J Neuroradiol. 2007;34:137-40. 4. Denis D, Dauletbekov D, Girard N. Duane retraction syndrome: type II with severe abducens nerve hypoplasia on magnetic resonance imaging. J AAPOS. 2008;12:91-3. 5. Yonghong J, Kanxing Z, Zhenchang W, et al. Detailed magnetic resonance imaging findings of the ocular motor nerves in Duane’s retraction syndrome. J Pediatr Ophthalmol Strabismus. 2009;46:278-85. 6. Hotchkiss MG, Miller NR, Clark AW, Green WR. Bilateral Duane’s retraction syndrome. A clinical-pathologic case report. Arch Ophthalmol. 1980;98:870-4. 7. Scott AB, Wong GY. Duane’s syndrome. An electromyographic study. Arch Ophthalmol. 1972;87:140-7. Can J Ophthalmol 2014;49:e52–e54 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2014.01.008
tomography (OCT) revealed marked choroidal thickening in the posterior pole (Fig. 2, thin white arrows) and an irregular, undulated retinal pigment epithelial line due to choroidal folds and edema (thick white arrows). The optic disc edema was composed of circumpapillary serous retinal detachments (open arrow) with true retinal nerve fibre layer edema (grey arrow) (Fig. 2). Systemic blood pressure measured 120/80 mm Hg. Neurologic examination was nonrevealing. All hematologic and serologic laboratory investigations revealed normal results. Cerebrospinal fluid pressure was 23 mm Hg with no component abnormalities. Thin-section brain magnetic resonance (MR) imaging and MR venography revealed normal findings. Optic disc edema rapidly decreased with oral prednisolone 60 mg/day (Fig. 1E, 1F, 3), and there was no evidence of recurrence after steroid tapering for up to 4 months. Bilateral optic disc swelling can develop as a result of different causative factors, such as elevated intracranial pressure, inflammation, infection, demyelination, ischemia, or infiltration. However, VKH presenting as bilateral optic disc swelling without exudative retinal detachment has seldom been reported.2 In our case, marked choroidal thickening in the posterior pole and focal areas of leakage at the level of the retinal pigment epithelium suggested diffuse choroiditis, which appeared as only marked bilateral disc edema on fundus photographs. In cases showing diffuse bilateral choroiditis of the posterior pole and no additional neurologic signs, a diagnosis of probable VKH should be considered. Enhanced-depth imaging OCT
Fig. 3 — Histology of skin biopsy with positive markers for melanoma. (A) Biopsy of left chest lesion showing a nodular proliferation in soft tissue composed of markedly atypical melanocytes arranged in nests (hematoxylin and eosin stain, original magnification 200 magnification; inset original magnification 20). (B) The tumour cells express MART-1 (melanomaassociated antigen recognized by T cells) by immunohistochemistry, confirming the diagnosis (original magnification 200).
REFERENCES 1. Rosenberg C, Finger PT. Cutaneous malignant melanoma metastatic to the eye, lids, and orbit. Surv Ophthalmol. 2008;53:187-202. 2. Zografos L, Ducrey N, Beati D, et al. Metastatic melanoma in the eye and orbit. Ophthalmology. 2003;110:2245-56. 3. Shields CL, Shields JA, Gross NE, Schwartz GP, Lally SE. Survey of 520 eyes with uveal metastases. Ophthalmology. 1997;104: 1265-76. 4. Keating GM. Vemurafenib: in unresectable or metastatic melanoma. BioDrugs. 2012;26:325-34.
Abducens nerve in a patient with Duane retraction syndrome An 8-year-old male was referred for left exotropia since early childhood. His visual acuities were 20/30 OU. He had 2 prism diopters of exophoria at distance and 20 prism diopters of left exotropia at near in the primary position. He had a left adduction deficit (Fig. 1A). Although he could fully abduct the left eye with maximal effort (Fig. 1A), there was a small abduction limitation most of the time, with limited abduction saccade. The left fissure narrowed on attempted adduction, and a left upshoot was observed. Anterior and posterior segment examinations were normal. High-resolution, T2-weighted magnetic resonance imaging (MRI; slice thickness, 0.7 mm) did not reveal a left abducens nerve in the cistern or orbit (Fig. 1B–D).
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CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
5. Sosman JA, Kim KB, Schuchter L, et al. Survival in BRAF V600mutant advanced melanoma treated with vemurafenib. N Engl J Med. 2012;366:707-14. 6. Chapman PB, Hauschild A, Robert C, et al. Improved survival with vemurafenib in melanoma with BRAF V600E mutation. N Engl J Med. 2011;364:2507-16. Can J Ophthalmol 2014;49:e49–e52 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2013.12.012
Causes of adduction limitation include innervational causative factors such as internuclear ophthalmoplegia, partial third cranial nerve palsy, myasthenia gravis, orbital apex or cavernous sinus syndrome, type 2 and 3 Duane retraction syndrome (DRS), and Miller Fisher syndrome, as well as mechanical causes including orbit mass, orbital medial wall fracture, and orbital lateral wall fracture with an entrapment of the surrounding tissue. Inflammatory myositis, trauma to medial rectus, slipped medial rectus, and chronic progressive external ophthalmoplegia may also result in adduction limitation. Our patient did not show any ptosis, pupillary abnormalities, or orbital abnormalities. Neurologic examination was normal except for the limited adduction. He denied any history of trauma or eye surgery. In addition, the left abducens nerve was absent on MRI, whereas the right abducens nerve was clearly defined. There was no limitation of elevation or depression, or pupil abnormality that would
Correspondence
Fig. 1 — A, Ocular versions showing adduction deficit, upshoot, and fissure narrowing on attempting adduction in the left eye. B–D, Three axial magnetic resonance imaging slices showing the whole path of the cisternal segment of the right abducens nerves (arrows), as linear dark structures emerging from the pontomedullary sulcus (B), coursing in a superior oblique direction (C), and finally entering Dorello canal (D). The left abducens nerve was not observed.
have suggested a third cranial nerve palsy. Narrowing of the palpebral fissure OS in adduction was characteristic for DRS. Kim and Hwang1,2 reported the presence of the abducens nerve in all of their patients with type 2 DRS. Denis et al.3,4 did not observe the abducens nerve in the cistern of a patient with type 2 DRS using MRI, but did identify a severely hypoplastic abducens nerve in the orbit. Yonghong et al.5 reported a hypoplastic abducens nerve in a patient with DRS type 2. However, the image in their publication showed what appeared to be a normal-diameter abducens nerve compared with the contralateral side, before the nerve was abruptly cut off in the cistern (serial images were not shown). In our patient, full abduction could be attained with maximal effort. The almost normal abduction in DRS could be explained by the aberrant innervation of the lower branch of the oculomotor nerve.6 As previously
described in many pathologic studies,6 the lateral rectus may be innervated by the oculomotor branches even though the abducens nerve is absent. Scott and Wong7 have reported that the lateral rectus muscle may show healthy, well-formed muscle bundles in areas innervated by fibres from the oculomotor nerve, lending further support to this explanation. Further detailed orbital imaging or pathologic examination to identify the small orbital branches of the oculomotor and abducens nerve would be required to fully explain the abnormal eye movement in patients with DRS. In our case, 2 possible explanations should be considered. First, this case may be the first report of a complete absence of the abducens nerve in type 2 DRS. A second possibility is that this patient may represent an extreme case of type 3 DRS, showing a predominant limitation of adduction compared with abduction. Even though his abduction could be full with the greatest effort, but his saccade to the left was limited compared to the right eye. CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
e53
Correspondence Consequently, we questioned the diagnosis of type 2 DRS in this patient. In conclusion, along with a careful clinical examination, high-resolution MRI to clearly identify the path of the abducens may be helpful in the evaluation of DRS.
Supported by: This work was supported by Seoul R&BD Program (ST090841) and by a grant of Korea Health 21 R&D Project, Ministry of Health, Welfare and Family Affairs, Republic of Korea (A080299). Jae Hyoung Kim and Jeong-Min Hwang Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea Correspondence to: Jeong-Min Hwang, MD: [email protected]
Bilateral disc edema in a patient with Vogt–Koyanagi–Harada disease Vogt–Koyanagi–Harada disease (VKH) is characterized by bilateral diffuse choroiditis with exudative retinal detachment.1 In addition to visual loss, the presence of hearing loss, tinnitus, poliosis, vitiligo, or meningismus could be helpful for the diagnosis. However, in cases that show only marked bilateral optic disc edema without any signs or symptoms, it could be difficult to consider VKH, and other conditions such as elevated blood pressure/intracranial pressure and inflammation or infection may be considered in the differential diagnosis. We present a patient with remarkable optic disc edema as the only sign of VKH. A 40-year-old Korean female presented with bilaterally blurred vision a week ago. She had a mild headache and nausea, but did not have diplopia, vomiting, ocular pain, hearing loss, tinnitus, alopecia, poliosis, vitiligo, or meningismus, or previous penetrating ocular trauma or surgery. The best corrected visual acuities were 20/20 OU. Slitlamp examination showed no cells or flare in the anterior chamber or vitreous, as well as clear cornea and lenses without any precipitates or synechiae. Intraocular pressure was normal through all examinations. Hardy-Rand-Rittler color test was normal OU. On automated perimetry, blind spots were enlarged OU. Funduscopy revealed marked optic disc edema OU and splinter optic disc hemorrhage OS (Fig. 1A, 1B). Fluorescein angiography showed staining of the optic disc, multiple pinpoint leakages at the level of the retinal pigment epithelium, and late peripapillary dye pooling (Fig. 1C, 1D). Enhanced-depth imaging optical coherence
e54
CAN J OPHTHALMOL — VOL. 49, NO. 2, APRIL 2014
REFERENCES 1. Kim JH, Hwang JM. Presence of the abducens nerve according to the type of Duane’s retraction syndrome. Ophthalmology. 2005;112:109-13. 2. Kim JH, Hwang JM. Abducens nerve is present in patients with type 2 Duane’s retraction syndrome. Ophthalmology. 2012;119:403-6. 3. Denis D, Dauletbekov D, Alessi G, Chapon F, Girard N. Duane retraction syndrome: MRI features in two cases. J Neuroradiol. 2007;34:137-40. 4. Denis D, Dauletbekov D, Girard N. Duane retraction syndrome: type II with severe abducens nerve hypoplasia on magnetic resonance imaging. J AAPOS. 2008;12:91-3. 5. Yonghong J, Kanxing Z, Zhenchang W, et al. Detailed magnetic resonance imaging findings of the ocular motor nerves in Duane’s retraction syndrome. J Pediatr Ophthalmol Strabismus. 2009;46:278-85. 6. Hotchkiss MG, Miller NR, Clark AW, Green WR. Bilateral Duane’s retraction syndrome. A clinical-pathologic case report. Arch Ophthalmol. 1980;98:870-4. 7. Scott AB, Wong GY. Duane’s syndrome. An electromyographic study. Arch Ophthalmol. 1972;87:140-7. Can J Ophthalmol 2014;49:e52–e54 0008-4182/14/$-see front matter & 2014 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jcjo.2014.01.008
tomography (OCT) revealed marked choroidal thickening in the posterior pole (Fig. 2, thin white arrows) and an irregular, undulated retinal pigment epithelial line due to choroidal folds and edema (thick white arrows). The optic disc edema was composed of circumpapillary serous retinal detachments (open arrow) with true retinal nerve fibre layer edema (grey arrow) (Fig. 2). Systemic blood pressure measured 120/80 mm Hg. Neurologic examination was nonrevealing. All hematologic and serologic laboratory investigations revealed normal results. Cerebrospinal fluid pressure was 23 mm Hg with no component abnormalities. Thin-section brain magnetic resonance (MR) imaging and MR venography revealed normal findings. Optic disc edema rapidly decreased with oral prednisolone 60 mg/day (Fig. 1E, 1F, 3), and there was no evidence of recurrence after steroid tapering for up to 4 months. Bilateral optic disc swelling can develop as a result of different causative factors, such as elevated intracranial pressure, inflammation, infection, demyelination, ischemia, or infiltration. However, VKH presenting as bilateral optic disc swelling without exudative retinal detachment has seldom been reported.2 In our case, marked choroidal thickening in the posterior pole and focal areas of leakage at the level of the retinal pigment epithelium suggested diffuse choroiditis, which appeared as only marked bilateral disc edema on fundus photographs. In cases showing diffuse bilateral choroiditis of the posterior pole and no additional neurologic signs, a diagnosis of probable VKH should be considered. Enhanced-depth imaging OCT
