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An Unusual Case of Giant Cell Arteritis
Vol. 115, No.3
Letters to The Journal
from a similar mechanism of hyperperfusion or occurring as a complication of anticoagulation. Patients who undergo venoarterial extracorporeal membrane oxygenation should have complete ocular examinations so the diagnosis of extracorporeal membrane oxygenation retinopathy" or vitreous hemorrhage can be detected to avoid visual complications such as amblyopia.
References 1. Fuhrman, B. P., and Dalton, H. J.: Progress in pediatric extracorporeal membrane oxygenation. Crit. Care Clin. 8:191, 1992. 2. Young, T. L., Quinn, G. E., Baumgart, S., and Schaffer, D. B.: Extracorporeal membrane oxygenation causing unilateral retinal vasculopathy in neonates. Ophthalmology 99:146,1992. 3. Chevalier, J., Durandy Y., Batisse, A., Mathe, J., and Costil, J.: Preliminary report. Extracorporeal lung support for neonatal acute respiratory failure. Lancet 335:1364, 1990. 4. O'Rourke, P. P., Lillehei, C. W., Crone, R. K., and Vacanti, J. P.: The effect of extracorporeal membrane oxygenation on the survival of neonates with high-risk congenital diaphragmatic hernia. 45 cases from a single institution. J. Pediatr. Surg. 26:147, 1991.
393
ache. Previous medical problems consisted of long-standing migraine (without visual symptoms) and temporal lobe epilepsy. Examination disclosed a tender, nodular, cord-like left superficial temporal artery with reduced pulsation. Visual acuity was 20/20 in each eye, color vision (Ishihara plates) was normal, and there was no afferent pupillary defect. Results of the rest of the ocular examination were normal. Systemically there were no abnormal findings. All peripheral pulses were palpable and symmetrical. Her blood pressure was 140/80 mm Hg. Preliminary investigations showed a mild microcytic, hypochromic anemia with a hemoglobin level of 10.9 g/dl. The sedimentation rate was 10 mrn Zhr and the C-reactive protein concentration was < 5 mg/l. Results of a full biochemical profile were normal apart from an increased ",-glutamyltransferase level of 49 U/1 (range, 7 to 32 U/1). Despite the patient's age, a provisional diagnosis of giant cell arteritis was made on the basis of the clinical findings. A left superficial temporal artery biopsy was performed, and high-dose systemic corticosteroids were initiated. The biopsy specimen, which was sectioned at a number of levels, showed marked thickening of the vessel wall and narrowing of the lumen. There was subintimal fibrosis, disruption and multiplication of the internal elastic
An Unusual Case of Giant Cell Arteritis Peter Shah, F.C.Ophth., Philip I. Murray, F.C.Ophth., and John Harry, M.R.C.Path. Birmingham and Midland Eye Hospital.
Inquiries to Philip 1. Murray, F.C.Ophth., Academic Unit, Birmingham and Midland Eye Hospital, Church Street, Birmingham B3 2NS, United Kingdom.
A 41-year-old Pakistani woman was seen at the Accident and Emergency Department, Birmingham and Midland Eye Hospital, because of a two-week history of left-sided scalp and periorbital pain, which was not relieved by simple analgesics. She also complained of a constant dull ache in the region of her left temporomandibular joint and of a burning sensation in her tongue. She had had an episode of sudden onset of blurred vision in both eyes ten days earlier, which had lasted 30 minutes. The features were suggestive of a migrainous head-
Fig. 1 (Shah, Murray, and Harry). Transverse section of the superficial temporal artery biopsy showing thickening of the wall and narrowing of the lumen; there is fibrosis, calcification, and transmural inflammation (hematoxylin and eosin, x 50).
394
AMERICAN JOURNAL OF OPHTHALMOLOGY
March, 1993
2. Ellis, M. E., and Ralston, S.: ESR in the diagnosis and management of polymyalgia rheumaticaj giant cell arteritis syndrome. Ann. Rheum. Dis. 42:168,1983.
Orbital Metastasis From Medullary Carcinoma of the Thyroid Curtis E. Margo, M.D., and Marc H. Levy, M.D. Departments of Ophthalmology (C.E.M., M.H.L.) and (C.E.M.) Pathology, University of South Florida, and the Sarasota Retinal Institute.
Fig. 2 (Shah, Murray, and Harry). Inflammatory cells including giant cells infiltrating the vessel wall (hematoxylin and eosin, x 375).
lamina, and conspicuous calcification. A transmural inflammatory reaction was present, and giant cells were seen. The histopathologic features were consistent with those of arteriosclerosis in association with giant cell arteritis (Figs. 1 and 2). Over the next few weeks the patient's symptoms improved and the corticosteroid dose was tapered accordingly. Other investigations showed a mild polyclonal increase in serum IgM of 2.35 gil (range, 0.5 to 2.0 gil). Tests for syphilis, anticardiolipin antibodies, and an autoantibody screen were all negative, and results of chest x-ray and electrocardiogram were both normal. In order to exclude Takayasu's disease, digital subtraction angiography was performed. This showed the aorta, its main branches in the cephalic and iliofemoral vessels, and the pulmonary arteries to be normal. Although the clinical features were typical of giant cell arteritis, this condition is rare in patients under 60 years of age and it almost exclusively affects white populations.' Also, a normal sedimentation rate does not exclude the diagnosis."
References 1. Bengtsson, B.-A.: Epidemiology of giant cell arteritis. Clin. Rheumatol. 5:379, 1991.
Inquiries to Curtis E. Margo, M.D., University of South Florida, 12901 Bruce Downs Blvd., MDC Box 21, Tampa, FL 33612.
Medullary carcinoma of the thyroid is a neuroendocrine tumor that originates from parafollicular C-cells, the source of calcitonin. I We treated a patient with medullary carcinoma of the thyroid metastatic to the orbit nine years after the primary tumor was resected. Clinically, the tumor simulated Graves' orbitopathy. A 67-year-old man with a history of thyroid disease was referred for segmental right conjunctival vascular injection localized to the inferior bulbar surface. There was 2 mm of right proptosis and double vision in upgaze caused by limitation of the right inferior rectus muscle (positive forced duction). He had been on a trial of oral corticosteroids for several weeks, but showed no signs of improvement. A magnetic
Fig. 1 (Margo and Levy). Magnetic resonance imaging scan discloses an irregularly shaped orbital mass (arrows) immediately below the right inferior rectus muscle.
Letters to The Journal
from a similar mechanism of hyperperfusion or occurring as a complication of anticoagulation. Patients who undergo venoarterial extracorporeal membrane oxygenation should have complete ocular examinations so the diagnosis of extracorporeal membrane oxygenation retinopathy" or vitreous hemorrhage can be detected to avoid visual complications such as amblyopia.
References 1. Fuhrman, B. P., and Dalton, H. J.: Progress in pediatric extracorporeal membrane oxygenation. Crit. Care Clin. 8:191, 1992. 2. Young, T. L., Quinn, G. E., Baumgart, S., and Schaffer, D. B.: Extracorporeal membrane oxygenation causing unilateral retinal vasculopathy in neonates. Ophthalmology 99:146,1992. 3. Chevalier, J., Durandy Y., Batisse, A., Mathe, J., and Costil, J.: Preliminary report. Extracorporeal lung support for neonatal acute respiratory failure. Lancet 335:1364, 1990. 4. O'Rourke, P. P., Lillehei, C. W., Crone, R. K., and Vacanti, J. P.: The effect of extracorporeal membrane oxygenation on the survival of neonates with high-risk congenital diaphragmatic hernia. 45 cases from a single institution. J. Pediatr. Surg. 26:147, 1991.
393
ache. Previous medical problems consisted of long-standing migraine (without visual symptoms) and temporal lobe epilepsy. Examination disclosed a tender, nodular, cord-like left superficial temporal artery with reduced pulsation. Visual acuity was 20/20 in each eye, color vision (Ishihara plates) was normal, and there was no afferent pupillary defect. Results of the rest of the ocular examination were normal. Systemically there were no abnormal findings. All peripheral pulses were palpable and symmetrical. Her blood pressure was 140/80 mm Hg. Preliminary investigations showed a mild microcytic, hypochromic anemia with a hemoglobin level of 10.9 g/dl. The sedimentation rate was 10 mrn Zhr and the C-reactive protein concentration was < 5 mg/l. Results of a full biochemical profile were normal apart from an increased ",-glutamyltransferase level of 49 U/1 (range, 7 to 32 U/1). Despite the patient's age, a provisional diagnosis of giant cell arteritis was made on the basis of the clinical findings. A left superficial temporal artery biopsy was performed, and high-dose systemic corticosteroids were initiated. The biopsy specimen, which was sectioned at a number of levels, showed marked thickening of the vessel wall and narrowing of the lumen. There was subintimal fibrosis, disruption and multiplication of the internal elastic
An Unusual Case of Giant Cell Arteritis Peter Shah, F.C.Ophth., Philip I. Murray, F.C.Ophth., and John Harry, M.R.C.Path. Birmingham and Midland Eye Hospital.
Inquiries to Philip 1. Murray, F.C.Ophth., Academic Unit, Birmingham and Midland Eye Hospital, Church Street, Birmingham B3 2NS, United Kingdom.
A 41-year-old Pakistani woman was seen at the Accident and Emergency Department, Birmingham and Midland Eye Hospital, because of a two-week history of left-sided scalp and periorbital pain, which was not relieved by simple analgesics. She also complained of a constant dull ache in the region of her left temporomandibular joint and of a burning sensation in her tongue. She had had an episode of sudden onset of blurred vision in both eyes ten days earlier, which had lasted 30 minutes. The features were suggestive of a migrainous head-
Fig. 1 (Shah, Murray, and Harry). Transverse section of the superficial temporal artery biopsy showing thickening of the wall and narrowing of the lumen; there is fibrosis, calcification, and transmural inflammation (hematoxylin and eosin, x 50).
394
AMERICAN JOURNAL OF OPHTHALMOLOGY
March, 1993
2. Ellis, M. E., and Ralston, S.: ESR in the diagnosis and management of polymyalgia rheumaticaj giant cell arteritis syndrome. Ann. Rheum. Dis. 42:168,1983.
Orbital Metastasis From Medullary Carcinoma of the Thyroid Curtis E. Margo, M.D., and Marc H. Levy, M.D. Departments of Ophthalmology (C.E.M., M.H.L.) and (C.E.M.) Pathology, University of South Florida, and the Sarasota Retinal Institute.
Fig. 2 (Shah, Murray, and Harry). Inflammatory cells including giant cells infiltrating the vessel wall (hematoxylin and eosin, x 375).
lamina, and conspicuous calcification. A transmural inflammatory reaction was present, and giant cells were seen. The histopathologic features were consistent with those of arteriosclerosis in association with giant cell arteritis (Figs. 1 and 2). Over the next few weeks the patient's symptoms improved and the corticosteroid dose was tapered accordingly. Other investigations showed a mild polyclonal increase in serum IgM of 2.35 gil (range, 0.5 to 2.0 gil). Tests for syphilis, anticardiolipin antibodies, and an autoantibody screen were all negative, and results of chest x-ray and electrocardiogram were both normal. In order to exclude Takayasu's disease, digital subtraction angiography was performed. This showed the aorta, its main branches in the cephalic and iliofemoral vessels, and the pulmonary arteries to be normal. Although the clinical features were typical of giant cell arteritis, this condition is rare in patients under 60 years of age and it almost exclusively affects white populations.' Also, a normal sedimentation rate does not exclude the diagnosis."
References 1. Bengtsson, B.-A.: Epidemiology of giant cell arteritis. Clin. Rheumatol. 5:379, 1991.
Inquiries to Curtis E. Margo, M.D., University of South Florida, 12901 Bruce Downs Blvd., MDC Box 21, Tampa, FL 33612.
Medullary carcinoma of the thyroid is a neuroendocrine tumor that originates from parafollicular C-cells, the source of calcitonin. I We treated a patient with medullary carcinoma of the thyroid metastatic to the orbit nine years after the primary tumor was resected. Clinically, the tumor simulated Graves' orbitopathy. A 67-year-old man with a history of thyroid disease was referred for segmental right conjunctival vascular injection localized to the inferior bulbar surface. There was 2 mm of right proptosis and double vision in upgaze caused by limitation of the right inferior rectus muscle (positive forced duction). He had been on a trial of oral corticosteroids for several weeks, but showed no signs of improvement. A magnetic
Fig. 1 (Margo and Levy). Magnetic resonance imaging scan discloses an irregularly shaped orbital mass (arrows) immediately below the right inferior rectus muscle.