- Email: [email protected]
Solitary neurofibroma in the absence of neurofiromatosis
Correspondence
A clinical diagnosis of iridocorneal endothelial (ICE) syndrome with secondary glaucoma in the left eye and sensorineural deafness in the left ear was made. Interestingly, the unilateral visual and aural symptoms had occurred almost at the same time. We speculate that there may be some aetiological association between them. Without any sign of pulling from some membrane, such as in corectopia, the ICE syndrome in our case seems to support the neural crest hypothesis better.1 Activated by some subclinical inflammatory event caused by trauma or infection, relatively undifferentiated “nests” of neural-crestderived mesenchymal cells in the corneal endothelium and iris may proliferate abnormally and produce localized iris atrophy. The little white keratic precipitates in the left eye may indicate some kind of inflammation. Abnormal proliferation or degeneration of neural-crest-derived cells in the superior cervical ganglion may also lead to disorder in the microcirculation of the internal ear and to hearing loss, although the specific mechanism has not been fully made clear.2–4 As a result, the symptoms of the 2 parts (the eye and the ear) appeared similar. In addition, failure in evoked oto-acoustic emission indicates the damage of outer hair cells in the left internal ear. It is also possible that some kind
of viral infection degraded both the eye5 and the internal ear. Another possibility is that the association is just coincidental, as it has never been reported previously. REFERENCES 1. Bahn CF, Falls HF, Varley GA, Meyer RF, Edelhauser HF, Bourne WM. Classification of corneal endothelial disorders based on neural crest origin. Ophthalmology 1984;91:558–63. 2. Gil-Loyzaga P, Vicente-Torres MA, Arce A, Cardinali DP, Esquifino A. Effect of superior cervical ganglionectomy on catecholamine concentration in rat cochlea. Brain Res 1998;779:53–7. 3. Watanabe K, Sugimoto T, Tsunoda A. Effect of sympathectomy on capillaries of the cochlear lateral wall. ORL J Otorhinolaryngol Relat Spec 1990;52:205–10. 4. Shen Y, Qiu J, Qiao L, Liu S. Sympathetic nerve-innervated cochlear blood vessels in guinea pig: retrograde tracing combined with immunohistochemical double-labeled technique. J Fourth MilMed Univ 2006;27:1097–9. 5. Alvarado JA, Underwood JL, Green WR, et al. Detection of herpes simplex viral DNA in the iridocorneal endothelial syndrome. Arch Ophthalmol 1994;112:1601–9.
Rongrong Hu, Yangshun Gu, Xiuyi Li Department of Ophthalmology, First Affiliated Hospital College of Medicine, Zhejiang University Hangzhou, P.R.China [email protected] Can J Ophthalmol 2007;42:627–8 doi: 10.3129/can.j.ophthalmol.i07-089
Solitary neurofibroma in the absence of neurofiromatosis
Fig.1—Slit-lamp photograph of the left eye shows localized atrophy of the iris at the 3–4 o’clock position, within which a temporal hole appeared.
Fig. 2—In vivo confocal microscopic image shows large, epithelioid-like cells with blurring boundaries and hyper-reflective, irregularly shaped nuclei in the left corneal endothelium.
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CAN J OPHTHALMOL—VOL. 42, NO. 4, 2007
A
25-year-old gentleman presented with a 1-year history of progressive right upper lid swelling and recent onset of discomfort. External examination revealed a hard, mobile superomedial orbital mass, causing 2 mm inferior and 1 mm lateral globe displacement. The Hertel exophthalmometer measured 17 mm on the right and 15 mm on the left. The extraocular motility was full. Computed tomography (CT) scan of the orbits showed a 1 × 0.7 cm soft tissue density mass in the right anteromedial orbit with capsular enhancement and variable density (Fig. 1). The lesion was excised and appeared to have arisen from the superior orbital nerve with posterior extension deeper into the orbit. Histopathologic results showed a well-circumscribed lesion arising from a nerve and surrounded by thin perineurium. Several myelinated axons, bundles of Schwann cells, and collagen fibers were distributed within a myxoid stroma (Fig. 2), which was positive for Alcian blue. Scattered and perivascular lymphocytes were also seen. The tumor was diagnosed as soli-
Correspondence
extending to 23 years. These authors described postoperative sensory deficit in 72% of patients with neurofibroma. Shields et al.5 reported a patient who had no manifestations of neurofibromatosis with 3 separate right orbital lesions. It is difficult to determine in our patient whether the posterior mass represents a tumor extension or a separate second lesion. He had sensory deficit over the right forehead, no recurrence of his tumor anteriorly, no change in the posterior mass, and no significant exophthalmos after 1 year. Fig. 1—Coronal computed tomography scan of the right orbit with anterior mass.
The author thanks Drs. Valerie White and Jack Rootman from the University of British Columbia and Vancouver General Hospital for their assistance in the confirmation of the diagnosis of this case.
REFERENCES
Fig. 2—Histopathology showing myelinated axons and Schwann cells in a myxoid stroma (hematoxylin–eosin; original magnification ×100).
tary neurofibroma in the absence of neurofibromatosis. The exact incidence of true solitary or isolated neurofibroma in the orbit is difficult to obtain because of its relation to neurofibromatosis; however, it is relatively uncommon. Rootman1 encountered only 3 cases of solitary neurofibroma in his series of orbital tumors, occurring in middle-aged persons as a slow-growing, upper quadrant solid mass. Garrity and Henderson2 had 9 cases, arising usually from the supraorbital frontal branch of the trigeminal nerve. They noted multiple tumors in the same patient involving other nerves transversing the orbit. Krohel et al.3 reported 8 cases with histopathology features identical to those of our case. They pointed out the occurrence of anesthesia in the distribution of the involved sensory nerve and recommended surgical excision as the treatment of choice. Rose and Wright4 showed a 93% incidence of benign neurilemmoma or neurofibroma among orbital peripheral nerve sheath tumors. They defined a family history of systemic neurofibromatosis in one-quarter of patients with solitary neurofibroma. The tumors typically had a smooth contour on the CT scan with orbital wall expansion and possible extension through the superior orbital fissure in 41% of the cases, which can limit the surgical resection (as in our case). Despite incomplete resection, no recurrences requiring further surgery occurred during a follow-up
1. Rootman J. Neoplasia. In: Diseases of the Orbit: a Multidisciplinary Approach, 2nd ed. Philadelphia: Lippincott Williams and Wilkins; 2003. 2. Garrity JA, Henderson JW. Tumors of peripheral nerve sheath origin. In: Henderson’s Orbital Tumors, 4th ed. Philadelphia: Lippincott Williams and Wilkins; 2007. 3. Krohel GB, Rosenberg PN, Wright JE, Smith RS. Localized orbital nurofibromas. Am J Ophthalmol 1985;100:458–64. 4. Rose GE, Wright JE. Isolated peripheral nerve sheath tumors of the orbit. Eye 1991;5:668–73. 5. Shields JA, Shields CL, Lieb WE, Eagle RC. Multiple orbital neurofibromas unassociated with von Recklinghausen’s disease. Arch Ophthalmol 1990;108:80–3.
Hind M. Alkatan, MD Department of Pathology King Khaled Eye Specialist Hospital Riyadh, Kingdom of Saudi Arabia [email protected] Can J Ophthalmol 2007;42:628–9 doi: 10.3129/can.j.ophthalmol.i07-087
The new face of age-related macular degeneration
I
n the past, we have managed wet age-related macular degeneration with many different treatments, without achieving desirable results. The Macular Photocoagulation Study, demonstrated a decrease in the rate of severe vision loss. There was, however, a 50% chance of recurrence within 2 years of treatment.1 The focus then shifted to photodynamic therapy with verteporfin, but because of recurrence and the lack of improvement in visual acuity (VA), its use in clinical practice has diminished. Then, the vascular-endothelial-growth factor (VEGF), a protein that regulates normal and abnormal angiogenesis, became the primary focus. Treatments so far have been divided into selective blockade or pan-blockade of this protein. The first anti-VEGF treatment used was pegap-
CAN J OPHTHALMOL—VOL. 42, NO. 4, 2007
629
A clinical diagnosis of iridocorneal endothelial (ICE) syndrome with secondary glaucoma in the left eye and sensorineural deafness in the left ear was made. Interestingly, the unilateral visual and aural symptoms had occurred almost at the same time. We speculate that there may be some aetiological association between them. Without any sign of pulling from some membrane, such as in corectopia, the ICE syndrome in our case seems to support the neural crest hypothesis better.1 Activated by some subclinical inflammatory event caused by trauma or infection, relatively undifferentiated “nests” of neural-crestderived mesenchymal cells in the corneal endothelium and iris may proliferate abnormally and produce localized iris atrophy. The little white keratic precipitates in the left eye may indicate some kind of inflammation. Abnormal proliferation or degeneration of neural-crest-derived cells in the superior cervical ganglion may also lead to disorder in the microcirculation of the internal ear and to hearing loss, although the specific mechanism has not been fully made clear.2–4 As a result, the symptoms of the 2 parts (the eye and the ear) appeared similar. In addition, failure in evoked oto-acoustic emission indicates the damage of outer hair cells in the left internal ear. It is also possible that some kind
of viral infection degraded both the eye5 and the internal ear. Another possibility is that the association is just coincidental, as it has never been reported previously. REFERENCES 1. Bahn CF, Falls HF, Varley GA, Meyer RF, Edelhauser HF, Bourne WM. Classification of corneal endothelial disorders based on neural crest origin. Ophthalmology 1984;91:558–63. 2. Gil-Loyzaga P, Vicente-Torres MA, Arce A, Cardinali DP, Esquifino A. Effect of superior cervical ganglionectomy on catecholamine concentration in rat cochlea. Brain Res 1998;779:53–7. 3. Watanabe K, Sugimoto T, Tsunoda A. Effect of sympathectomy on capillaries of the cochlear lateral wall. ORL J Otorhinolaryngol Relat Spec 1990;52:205–10. 4. Shen Y, Qiu J, Qiao L, Liu S. Sympathetic nerve-innervated cochlear blood vessels in guinea pig: retrograde tracing combined with immunohistochemical double-labeled technique. J Fourth MilMed Univ 2006;27:1097–9. 5. Alvarado JA, Underwood JL, Green WR, et al. Detection of herpes simplex viral DNA in the iridocorneal endothelial syndrome. Arch Ophthalmol 1994;112:1601–9.
Rongrong Hu, Yangshun Gu, Xiuyi Li Department of Ophthalmology, First Affiliated Hospital College of Medicine, Zhejiang University Hangzhou, P.R.China [email protected] Can J Ophthalmol 2007;42:627–8 doi: 10.3129/can.j.ophthalmol.i07-089
Solitary neurofibroma in the absence of neurofiromatosis
Fig.1—Slit-lamp photograph of the left eye shows localized atrophy of the iris at the 3–4 o’clock position, within which a temporal hole appeared.
Fig. 2—In vivo confocal microscopic image shows large, epithelioid-like cells with blurring boundaries and hyper-reflective, irregularly shaped nuclei in the left corneal endothelium.
628
CAN J OPHTHALMOL—VOL. 42, NO. 4, 2007
A
25-year-old gentleman presented with a 1-year history of progressive right upper lid swelling and recent onset of discomfort. External examination revealed a hard, mobile superomedial orbital mass, causing 2 mm inferior and 1 mm lateral globe displacement. The Hertel exophthalmometer measured 17 mm on the right and 15 mm on the left. The extraocular motility was full. Computed tomography (CT) scan of the orbits showed a 1 × 0.7 cm soft tissue density mass in the right anteromedial orbit with capsular enhancement and variable density (Fig. 1). The lesion was excised and appeared to have arisen from the superior orbital nerve with posterior extension deeper into the orbit. Histopathologic results showed a well-circumscribed lesion arising from a nerve and surrounded by thin perineurium. Several myelinated axons, bundles of Schwann cells, and collagen fibers were distributed within a myxoid stroma (Fig. 2), which was positive for Alcian blue. Scattered and perivascular lymphocytes were also seen. The tumor was diagnosed as soli-
Correspondence
extending to 23 years. These authors described postoperative sensory deficit in 72% of patients with neurofibroma. Shields et al.5 reported a patient who had no manifestations of neurofibromatosis with 3 separate right orbital lesions. It is difficult to determine in our patient whether the posterior mass represents a tumor extension or a separate second lesion. He had sensory deficit over the right forehead, no recurrence of his tumor anteriorly, no change in the posterior mass, and no significant exophthalmos after 1 year. Fig. 1—Coronal computed tomography scan of the right orbit with anterior mass.
The author thanks Drs. Valerie White and Jack Rootman from the University of British Columbia and Vancouver General Hospital for their assistance in the confirmation of the diagnosis of this case.
REFERENCES
Fig. 2—Histopathology showing myelinated axons and Schwann cells in a myxoid stroma (hematoxylin–eosin; original magnification ×100).
tary neurofibroma in the absence of neurofibromatosis. The exact incidence of true solitary or isolated neurofibroma in the orbit is difficult to obtain because of its relation to neurofibromatosis; however, it is relatively uncommon. Rootman1 encountered only 3 cases of solitary neurofibroma in his series of orbital tumors, occurring in middle-aged persons as a slow-growing, upper quadrant solid mass. Garrity and Henderson2 had 9 cases, arising usually from the supraorbital frontal branch of the trigeminal nerve. They noted multiple tumors in the same patient involving other nerves transversing the orbit. Krohel et al.3 reported 8 cases with histopathology features identical to those of our case. They pointed out the occurrence of anesthesia in the distribution of the involved sensory nerve and recommended surgical excision as the treatment of choice. Rose and Wright4 showed a 93% incidence of benign neurilemmoma or neurofibroma among orbital peripheral nerve sheath tumors. They defined a family history of systemic neurofibromatosis in one-quarter of patients with solitary neurofibroma. The tumors typically had a smooth contour on the CT scan with orbital wall expansion and possible extension through the superior orbital fissure in 41% of the cases, which can limit the surgical resection (as in our case). Despite incomplete resection, no recurrences requiring further surgery occurred during a follow-up
1. Rootman J. Neoplasia. In: Diseases of the Orbit: a Multidisciplinary Approach, 2nd ed. Philadelphia: Lippincott Williams and Wilkins; 2003. 2. Garrity JA, Henderson JW. Tumors of peripheral nerve sheath origin. In: Henderson’s Orbital Tumors, 4th ed. Philadelphia: Lippincott Williams and Wilkins; 2007. 3. Krohel GB, Rosenberg PN, Wright JE, Smith RS. Localized orbital nurofibromas. Am J Ophthalmol 1985;100:458–64. 4. Rose GE, Wright JE. Isolated peripheral nerve sheath tumors of the orbit. Eye 1991;5:668–73. 5. Shields JA, Shields CL, Lieb WE, Eagle RC. Multiple orbital neurofibromas unassociated with von Recklinghausen’s disease. Arch Ophthalmol 1990;108:80–3.
Hind M. Alkatan, MD Department of Pathology King Khaled Eye Specialist Hospital Riyadh, Kingdom of Saudi Arabia [email protected] Can J Ophthalmol 2007;42:628–9 doi: 10.3129/can.j.ophthalmol.i07-087
The new face of age-related macular degeneration
I
n the past, we have managed wet age-related macular degeneration with many different treatments, without achieving desirable results. The Macular Photocoagulation Study, demonstrated a decrease in the rate of severe vision loss. There was, however, a 50% chance of recurrence within 2 years of treatment.1 The focus then shifted to photodynamic therapy with verteporfin, but because of recurrence and the lack of improvement in visual acuity (VA), its use in clinical practice has diminished. Then, the vascular-endothelial-growth factor (VEGF), a protein that regulates normal and abnormal angiogenesis, became the primary focus. Treatments so far have been divided into selective blockade or pan-blockade of this protein. The first anti-VEGF treatment used was pegap-
CAN J OPHTHALMOL—VOL. 42, NO. 4, 2007
629