Clinical Review & Education
JAMA Ophthalmology Clinical Challenge
Visual Disturbance After Bariatric Surgery Gregory Van Stavern, MD; Gokul Kumar, MD; Catherine Thuruthumaly, MD
A
B
Figure. A and B, Initial presentation of large intraretinal hemorrhages and disc edema in the left and right eye, respectively.
A woman in her 30s presented to the hospital with decreased vision in both eyes, confusion, and ataxia for 3 weeks. She had undergone gastric bypass surgery 3 months before presentation, which was complicated by a gastric ulcer and stricture. She had lost 34.5 kg since the procedure. Other medical history included hypothyroidism and thyroidectomy for thyroid cancer diagnosed 10 years ago. Her medication regimen inQuiz at jamaophthalmology.com cluded levothyroxine sodium and occasional bariatric chewable vitamins. She denied alcohol or tobacco use or drug abuse. Her best-corrected visual acuity was 20/200 OD and 20/400 OS. Her pupils were equally reactive, with no relative afferent pupillary defect. There was a mild abduction deficit in both eyes as well as prominent upbeating nystagmus. The amplitude of the nystagmus increased in upgaze and dampened with downgaze. She was unable to read the control plate of the Ishihara test. Findings from an anterior segment examination were unremarkable. Findings from a dilated fundus examination revealed disc edema in both eyes with peripapillary nerve fiber layer thickening and large intraretinal hemorrhages (Figure).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Lumbar puncture B. Magnetic resonance imaging and/or venography C. Intravenous vitamin therapy D. Intravenous corticosteroids
(Reprinted) JAMA Ophthalmology March 2015 Volume 133, Number 3
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Georgia User on 05/24/2015
345
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Wernicke encephalopathy with ocular manifestations
Answer C. Intravenous vitamin therapy Wernicke encephalopathy (WE) is best known by the classic triad of mental confusion, gait ataxia, and eye movement abnormalities (nystagmus and ophthalmoplegia). However, all 3 symptoms rarely occur in the same patient, leading to underdiagnosis of this condition. Confusion is the most common symptom, followed by ataxia and abnormal eye movement.1,2 The oculomotor abnormalities are well documented, but the fundus findings are underreported. Carl Wernicke’s original document from 1881 described a patient with bilateral optic neuritis with massive swelling and many streak hemorrhages. The intraretinal hemorrhages bear a resemblance to those seen in patients with Leber hereditary optic neuropathy, which suggests that mitochondrial dysfunction at the level of the retinal capillary endothelium might be responsible for the hemorrhages.3 However, to our knowledge, this finding and its proposed mechanism is not commonly discussed in the literature. Wernicke encephalopathy remains primarily a clinical diagnosis. Neuro-ophthalmic signs associated with WE consist of both horizontal and vertical gaze-evoked nystagmus, weakness or paralysis of the lateral recti muscles and weakness or paralysis of conjugate gaze, and optic neuropathy.4 Laboratory evaluations and neuroimaging can be helpful in unclear cases, but these should never delay prompt treatment with thiamine hydrochloride (vitamin B1) supplementation. Typical magnetic resonance imaging findings include high signal intensity on T2 sequences in the bilateral medial thalami and periventricular regions of the third ventricle.5 Vitamin B1 and α-transketolase serum levels are 2 laboratory tests that may indicate vitamin B1 deficiency, but there are no absolute levels that determine who is at risk for developing WE. The major barrier to proper diagnosis is a low index of suspicion, particularly in patients without alcoholism. Although most commonly seen in individuals with chronic alcoholism, WE has been found in a variety of other conditions, such as anorexia nervosa, bariatric surgery, forced starvation, hyperemesis gravidarum, and prolonged parenteral feeding. The human body is able to store between 30 and 50 mg of vitamin B1 at a time, and these reserves can ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri. Corresponding Author: Gregory Van Stavern, MD, Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, 660 S Euclid Ave, Campus Box 8096, St Louis, MO 63110 ([email protected]). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. REFERENCES 1. Torvik A, Lindboe CF, Rogde S. Brain lesions in alcoholics: a neuropathological study with clinical correlations. J Neurol Sci. 1982;56(2-3):233-248.
346
Visual Disturbance After Bariatric Surgery
be depleted in as little as 4 to 6 weeks,6 hence the recent surge of cases with the increasing popularity of bariatric procedures. Diagnostic evaluation to confirm the diagnosis should never take priority over actual thiamine supplementation. When untreated, WE can result in the most feared complication—Korsakoff syndrome. This disorder occurs in up to 85% of survivors and is characterized by severe retrograde amnesia with prominent confabulations. Without proper treatment, Korsakoff syndrome can lead to death in up to 20% of cases. With proper treatment, recovery of ocular manifestations can be rapid. Horizontal nystagmus can resolve within minutes, and gaze palsies often take 1 to 2 weeks for full recovery. However, central vestibular nystagmus may persist for several months.4 Once memory impairment occurs, only 20% of patients will experience full recovery. Lumbar puncture can be conducted after vitamin supplementation to rule out infectious processes, such as meningitis and increased intracranial pressure. Intravenous corticosteroids (choice D) would actually harm the patient because this treatment might elevate serum glucose levels; hyperglycemia can worsen WE without prior thiamine supplementation. With the recent increase in bariatric procedures, WE is likely to be seen with increasing frequency. Thus, the constellation of malnutrition from any cause with ocular motility dysfunction or nystagmus should prompt the physician to consider vitamin B1 deficiency as a cause. The presence of optic disc edema and intraretinal hemorrhages does not exclude WE as a diagnosis because they remain some of the more underreported features of the disease.7
Patient Outcome The patient started receiving intravenous thiamine on arrival. Three days after initiating therapy, her visual acuity had improved to 20/30 OU, her sensorium had cleared, and her abduction deficit had resolved. Her nystagmus, however, persisted and continued to be a source of frustration for the following 2 months. Findings from the dilated fundus examination 3 weeks after presentation revealed nearcomplete resolution of the intraretinal hemorrhages and disc edema, but her visual acuity had declined to 20/70 OU. This reduced visual acuity was likely caused by oscillopsia due to the nystagmus, with an element of impaired cognition and cooperation. She continues to take 100 mg of oral thiamine daily.
2. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff Syndrome and Related Neurological Disorders Due to Alcoholism and Malnutrition. 2nd ed. Philadelphia, PA: FA Davis; 1989:61-110. 3. Thomson AD, Cook CC, Guerrini I, Sheedy D, Harper C, Marshall EJ. Wernicke’s encephalopathy revisited: translation of the case history section of the original manuscript by Carl Wernicke ‘Lehrbuch der Gehirnkrankheiten fur Aerzte and Studirende’ (1881) with a commentary. Alcohol Alcohol. 2008; 43(2):174-179.
6. Thomson AD, Guerrini I, Marshall EJ. Nutrition issues in gastroenterology, series #75: Wernicke’s encephalopathy: role of thiamine. Pract Gastroenterol. 2009;33(6):21-30. 7. Bohnsack BL, Patel SS. Peripapillary nerve fiber layer thickening, telangiectasia, and retinal hemorrhages in Wernicke encephalopathy. J Neuroophthalmol. 2010;30(1):54-58.
4. Ropper AH. Adams and Victor’s Principles of Neurology. 9th ed. New York, NY: McGraw-Hill Medical; 2009:1109-1112. 5. Zuccoli G, Pipitone N. Neuroimaging findings in acute Wernicke’s encephalopathy: review of the literature. AJR Am J Roentgenol. 2009;192(2): 501-508.
JAMA Ophthalmology March 2015 Volume 133, Number 3 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Georgia User on 05/24/2015
jamaophthalmology.com
JAMA Ophthalmology Clinical Challenge
Visual Disturbance After Bariatric Surgery Gregory Van Stavern, MD; Gokul Kumar, MD; Catherine Thuruthumaly, MD
A
B
Figure. A and B, Initial presentation of large intraretinal hemorrhages and disc edema in the left and right eye, respectively.
A woman in her 30s presented to the hospital with decreased vision in both eyes, confusion, and ataxia for 3 weeks. She had undergone gastric bypass surgery 3 months before presentation, which was complicated by a gastric ulcer and stricture. She had lost 34.5 kg since the procedure. Other medical history included hypothyroidism and thyroidectomy for thyroid cancer diagnosed 10 years ago. Her medication regimen inQuiz at jamaophthalmology.com cluded levothyroxine sodium and occasional bariatric chewable vitamins. She denied alcohol or tobacco use or drug abuse. Her best-corrected visual acuity was 20/200 OD and 20/400 OS. Her pupils were equally reactive, with no relative afferent pupillary defect. There was a mild abduction deficit in both eyes as well as prominent upbeating nystagmus. The amplitude of the nystagmus increased in upgaze and dampened with downgaze. She was unable to read the control plate of the Ishihara test. Findings from an anterior segment examination were unremarkable. Findings from a dilated fundus examination revealed disc edema in both eyes with peripapillary nerve fiber layer thickening and large intraretinal hemorrhages (Figure).
jamaophthalmology.com
WHAT WOULD YOU DO NEXT?
A. Lumbar puncture B. Magnetic resonance imaging and/or venography C. Intravenous vitamin therapy D. Intravenous corticosteroids
(Reprinted) JAMA Ophthalmology March 2015 Volume 133, Number 3
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Georgia User on 05/24/2015
345
Clinical Review & Education JAMA Ophthalmology Clinical Challenge
Diagnosis Wernicke encephalopathy with ocular manifestations
Answer C. Intravenous vitamin therapy Wernicke encephalopathy (WE) is best known by the classic triad of mental confusion, gait ataxia, and eye movement abnormalities (nystagmus and ophthalmoplegia). However, all 3 symptoms rarely occur in the same patient, leading to underdiagnosis of this condition. Confusion is the most common symptom, followed by ataxia and abnormal eye movement.1,2 The oculomotor abnormalities are well documented, but the fundus findings are underreported. Carl Wernicke’s original document from 1881 described a patient with bilateral optic neuritis with massive swelling and many streak hemorrhages. The intraretinal hemorrhages bear a resemblance to those seen in patients with Leber hereditary optic neuropathy, which suggests that mitochondrial dysfunction at the level of the retinal capillary endothelium might be responsible for the hemorrhages.3 However, to our knowledge, this finding and its proposed mechanism is not commonly discussed in the literature. Wernicke encephalopathy remains primarily a clinical diagnosis. Neuro-ophthalmic signs associated with WE consist of both horizontal and vertical gaze-evoked nystagmus, weakness or paralysis of the lateral recti muscles and weakness or paralysis of conjugate gaze, and optic neuropathy.4 Laboratory evaluations and neuroimaging can be helpful in unclear cases, but these should never delay prompt treatment with thiamine hydrochloride (vitamin B1) supplementation. Typical magnetic resonance imaging findings include high signal intensity on T2 sequences in the bilateral medial thalami and periventricular regions of the third ventricle.5 Vitamin B1 and α-transketolase serum levels are 2 laboratory tests that may indicate vitamin B1 deficiency, but there are no absolute levels that determine who is at risk for developing WE. The major barrier to proper diagnosis is a low index of suspicion, particularly in patients without alcoholism. Although most commonly seen in individuals with chronic alcoholism, WE has been found in a variety of other conditions, such as anorexia nervosa, bariatric surgery, forced starvation, hyperemesis gravidarum, and prolonged parenteral feeding. The human body is able to store between 30 and 50 mg of vitamin B1 at a time, and these reserves can ARTICLE INFORMATION Author Affiliations: Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, St Louis, Missouri. Corresponding Author: Gregory Van Stavern, MD, Department of Ophthalmology and Visual Sciences, Washington University School of Medicine, 660 S Euclid Ave, Campus Box 8096, St Louis, MO 63110 ([email protected]). Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported. REFERENCES 1. Torvik A, Lindboe CF, Rogde S. Brain lesions in alcoholics: a neuropathological study with clinical correlations. J Neurol Sci. 1982;56(2-3):233-248.
346
Visual Disturbance After Bariatric Surgery
be depleted in as little as 4 to 6 weeks,6 hence the recent surge of cases with the increasing popularity of bariatric procedures. Diagnostic evaluation to confirm the diagnosis should never take priority over actual thiamine supplementation. When untreated, WE can result in the most feared complication—Korsakoff syndrome. This disorder occurs in up to 85% of survivors and is characterized by severe retrograde amnesia with prominent confabulations. Without proper treatment, Korsakoff syndrome can lead to death in up to 20% of cases. With proper treatment, recovery of ocular manifestations can be rapid. Horizontal nystagmus can resolve within minutes, and gaze palsies often take 1 to 2 weeks for full recovery. However, central vestibular nystagmus may persist for several months.4 Once memory impairment occurs, only 20% of patients will experience full recovery. Lumbar puncture can be conducted after vitamin supplementation to rule out infectious processes, such as meningitis and increased intracranial pressure. Intravenous corticosteroids (choice D) would actually harm the patient because this treatment might elevate serum glucose levels; hyperglycemia can worsen WE without prior thiamine supplementation. With the recent increase in bariatric procedures, WE is likely to be seen with increasing frequency. Thus, the constellation of malnutrition from any cause with ocular motility dysfunction or nystagmus should prompt the physician to consider vitamin B1 deficiency as a cause. The presence of optic disc edema and intraretinal hemorrhages does not exclude WE as a diagnosis because they remain some of the more underreported features of the disease.7
Patient Outcome The patient started receiving intravenous thiamine on arrival. Three days after initiating therapy, her visual acuity had improved to 20/30 OU, her sensorium had cleared, and her abduction deficit had resolved. Her nystagmus, however, persisted and continued to be a source of frustration for the following 2 months. Findings from the dilated fundus examination 3 weeks after presentation revealed nearcomplete resolution of the intraretinal hemorrhages and disc edema, but her visual acuity had declined to 20/70 OU. This reduced visual acuity was likely caused by oscillopsia due to the nystagmus, with an element of impaired cognition and cooperation. She continues to take 100 mg of oral thiamine daily.
2. Victor M, Adams RD, Collins GH. The Wernicke-Korsakoff Syndrome and Related Neurological Disorders Due to Alcoholism and Malnutrition. 2nd ed. Philadelphia, PA: FA Davis; 1989:61-110. 3. Thomson AD, Cook CC, Guerrini I, Sheedy D, Harper C, Marshall EJ. Wernicke’s encephalopathy revisited: translation of the case history section of the original manuscript by Carl Wernicke ‘Lehrbuch der Gehirnkrankheiten fur Aerzte and Studirende’ (1881) with a commentary. Alcohol Alcohol. 2008; 43(2):174-179.
6. Thomson AD, Guerrini I, Marshall EJ. Nutrition issues in gastroenterology, series #75: Wernicke’s encephalopathy: role of thiamine. Pract Gastroenterol. 2009;33(6):21-30. 7. Bohnsack BL, Patel SS. Peripapillary nerve fiber layer thickening, telangiectasia, and retinal hemorrhages in Wernicke encephalopathy. J Neuroophthalmol. 2010;30(1):54-58.
4. Ropper AH. Adams and Victor’s Principles of Neurology. 9th ed. New York, NY: McGraw-Hill Medical; 2009:1109-1112. 5. Zuccoli G, Pipitone N. Neuroimaging findings in acute Wernicke’s encephalopathy: review of the literature. AJR Am J Roentgenol. 2009;192(2): 501-508.
JAMA Ophthalmology March 2015 Volume 133, Number 3 (Reprinted)
Copyright 2015 American Medical Association. All rights reserved.
Downloaded From: http://archopht.jamanetwork.com/ by a University of Georgia User on 05/24/2015
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