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Figure (Rush). Left, Left eye, central 30-degree computerized perimetry results showed narrow, dense upper and lower arcuate defects. Right, Right eye, central 30-degree computerized perimetry results showed peripheral constriction, worse superonasally, paracentral scotomas, and mild generalized depression.
been described." Like our patient, he experienced shimmering lights, which were more bothersome in dark surroundings. His abnormal electroretinograms showed results similar to those of congenital stationary night blindness. This patient had similar results: extinguished rod responses and slightly diminished b-wave cone responses. Like the patient of Berson and Lessell," my patient received no prednisone and had no histologic confirmation of the retinal locus of disease. Unlike that patient, who was not tested, his serum displayed antibodies to human retina and optic nerve in high titers. None were directed at the cancer-associated retinopathy, 23-kd retinal
antigen.'
References 1. Sawyer, R. A., Selhorst, J. B., Zimmerman, L. E., and Hoyt, W. F.: Blindness caused by photore-
ceptor degeneration as a remote effect of cancer. Am.
J. Ophthalmol. 81:606, 1976. 2. Rizzo, J. F. III, and Gittinger, J. W., [r.: Selective
immunohistochemical staining in the paraneoplastic retinopathy syndrome. Ophthalmology 99:1286, 1992. 3. Berson, E. L., and Lessell, S.: Paraneoplastic nightbIindness with malignant melanoma. Am. J. Ophthalmol. 106:307, 1988. 4. ThirkilL C. E., Fitzgerald, P., Sergott, R. c.. Roth, A. M., Tyler, N. K., and Keltner, J. L.: Cancer-
associated retinopathy (CAR syndrome) with antibodies reacting with retinal, optic-nerve, and cancer cells. N. Engl. J. Med. 321:1589, 1989.
Vitreous Hemorrhage and Extracorporeal Membrane Oxygenation Marcia D. Carney, M.D., Edwin Wortham, M.D., and K. Bakeer Al-Mateen, M.D. Departments of Ophthalmology (M.D.C., E.W.) and Pediatrics (K.B.A.), Medical College of Virginia, Virginia Commonwealth University. Supported in part by a grant from Research to Prevent Blindness, Inc., New York, New York. Inquiries to Marcia D. Carney, M.D., Department of Ophthalmology, Box 262, MCV Station, Medical College of Virginia, Richmond, VA 23298. Neonatal venoarterial extra corporeal membrane oxygenation is an intensive care unit application of cardiopulmonary bypass that provides complete circulatory and pulmonary support in potentially reversible life threatening conditions. These conditions include meconium aspiration syndrome, persistent pulmonary hypertension, and congenital diaphragmatic hernia. In venoarterial extra corporeal membrane oxygenation, blood is drained from the right atrium via a right internal jugular venous can-
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AMERICAN JOURNAL OF OPHTHALMOLOGY
nula and propelled with a roller or centrifugal pump through a silicone membrane oxygenator for gas exchange. Fully saturated oxygenated blood is then returned to the aortic arch through a common carotid artery cannula. Use of extracorporeal membrane oxygenation in older children and adults with septic shock, pneumonia, postcardiotomy myocardial dysfunction, and acute respiratory distress syndrome is not as well defined as its use in neonatal respiratory failure. 1 There is a significant risk of bleeding at the cannulation site and intracranial hemorrhage with extracorporeal membrane oxygenation because of the systemic anticoagulation with heparin that is necessary to prevent clotting in the bypass circuit. Despite improved cannulation techniques and cautious anticoagulation, bleeding remains the primary reason for premature discontinuation of extracorporeal life support in neonates. Other sites of hemorrhage may also be associated with extracorporeal membrane oxygenation. A 3,670-g male infant was the product of a term gestation. The infant was delivered on Sept. 9, 1991. Delivery was complicated by severe meconium aspiration. Despite maximal therapy with surfactant, pressors, and high frequency jet ventilation, the infant's condition continued to deteriorate. An echocardiogram was normal. Venoarterial extracorporeal membrane oxygenation was begun. Just before extracorporeal membrane oxygenation cannulations, the patient's course was complicated by cardiac arrest requiring chest compressions and medications. The patient underwent cannulation of the right carotid artery and right internal jugular vein for extracorporeal membrane oxygenation on Sept. 10. Extracorporeal membrane oxygenation was continued for five days. Decannulation was required secondary to hemorrhage at the cannulation site despite normal platelet count. Because of the patient's poor respiratory status, corticosteroids were started. On Sept. 20, the patient was seen because of seizure activity. At the time of neurologic examination a decreased red reflex was noted in the left eye. B-scan ultrasonography disclosed a vitreous hemorrhage in the left eye (Figure). Ophthalmoscopy of the right eye disclosed that the retina was normal. The patient was examined by a pediatric ophthalmologist who believed amblyopia might occur if the dense vitreous hemorrhage did not resolve. However, with the patient's poor respiratory status and development of a large (6.5 ern) pneumatocele in the
March, 1993
Figure (Carney, Wortham, and AI-Mateen). The left eye with dense, vitreous ultrasound echoes in the presence of dense vitreous hemorrhage.
left lung field, the parents refused any surgery until the child was stable. Without clearing of the vitreous hemorrhage at 5 months, he underwent combined pars plana lensectomy and vitrectomy, which disclosed dense vitreous hemorrhage, some remaining temporal choroidal hemorrhage, and old subretinal hemorrhage. During his postoperative period, the infant was fitted with a soft contact lens. The innovative advantages of venoarterial extracorporeal membrane oxygenation include dissociation of lung function and gas exchange, the mechanical support of the cardiac and respiratory systems, blood oxygenation, and the reliability of oxygen delivery.' Extracorporeal membrane oxygenation can be used in lifethreatening pulmonary disease in neonates and for temporary cardiac rescue. Young and associates' described retinal hemorrhages and exudates, venous tortuosity, and asymmetric vascular development in six of 27 patients undergoing extra corporeal membrane oxygenation. They proposed that the mechanism for this unilateral vasculopathy be a hyperperfusion or reperfusion injury, or both." Bleeding complications associated with extracorporeal membrane oxygenation are believed to be secondary to the systemic heparinization and thrombocytopenia. Although central nervous system hemorrhage is thought to be a risk and direct complication of extracorporeal membrane oxygenation.!" this cardiopulmonary bypass method appears to be successful in improving the survival of neonates whose predicted mortality approaches 100% with conventional therapy. Our patient had a dense vitreous hemorrhage that required vitrectomy possibly resulting
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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.
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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).