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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 463-464

A rare case of Evans syndrome presenting as retrobulbar neuritis

Department of Neuro Ophthalmology, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Date of Submission06-Jun-2022
Date of Acceptance07-Dec-2022
Date of Web Publication28-Apr-2023

Correspondence Address:
Virna M Shah
Aravind Eye Hospital, Coimbatore, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_841_22

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Evans syndrome (ES) is a rare disease characterized by simultaneous development of autoimmune hemolytic anemia and immune thrombocytopenia with or without immune neutropenia. We report the first case of ES presenting as retrobulbar neuritis. Purpose of this report is to create awareness about ES. A 25-year-old female presented with sudden painful loss of vision. Neuroimaging confirmed retrobulbar neuritis. She had menorrhagia and iron-deficiency anemia. She had pallor, hepatosplenomegaly, and hemolytic anemia. She was treated with intravenous steroids. ES is a disease of high morbidity.

Keywords: Autoimmune anemia, Evans syndrome, optic neuritis, retrobulbar neuritis

How to cite this article:
Shah VM, Hema V K, Kumar K. A rare case of Evans syndrome presenting as retrobulbar neuritis. Indian J Ophthalmol Case Rep 2023;3:463-4

How to cite this URL:
Shah VM, Hema V K, Kumar K. A rare case of Evans syndrome presenting as retrobulbar neuritis. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 10];3:463-4. Available from: https://www.ijoreports.in/text.asp?2023/3/2/463/375050

Evans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia and immune thrombocytopenia with or without immune neutropenia. Robert Evans first described ES in 1951 as presence of anemia, reticulocytosis, increased blood bilirubin and fecal urobilinogen, no family history of hemolytic diseases, evidence of antibodies against erythrocytes, hemolysis of transfused erythrocytes, prolonged bleeding time, bone marrow aspiration with normal or increased number of megakaryocytes, and absence of exogenous toxic agents or a baseline disease. ES shows clinical variability at the onset with spontaneous remissions and exacerbations.[1],[2],[3] Its pathophysiology is postulated to be due to immune dysregulation with antibodies against erythrocytes, platelets, or granulocytes[4] and decreased CD4:CD8 ratio. Till today, no therapeutic regimen has been established. Steroids with and without intravenous immunoglobulin (IVIG) are recommended as the mainstay therapy.[5],[6] Red blood cell/platelet transfusion is indicated in cases of severe symptomatic patients due to the risk of exacerbations.[5] Outcomes with current therapeutic modalities are unsatisfactory, and in due course, devastating clinical events including death can occur. Optic neuritis is largely idiopathic in nature, but could be related to demyelinating lesions[4] or other less-frequent etiologies such as autoimmune disease, infectious and para-infectious causes (e.g., syphilis, tuberculosis), inflammatory and post-vaccination immunological responses, and vaccinations against measles and rubella. Ocular manifestations of ES are extremely rare (a few cases of vitreous hemorrhage, premacular hemorrhage, and retinal artery occlusion have been reported). ES presenting as optic neuritis has not been reported earlier. We report the first case of ES presenting as retrobulbar neuritis.

  Case Report Top

A 25-year-old female patient presented with sudden-onset defective vision in the right eye for 3 weeks. Defective vision was associated with pain on eye movements. She gave a past history of painless defective vision in both eyes 5 months back and was diagnosed as having nutritional optic neuropathy and was on oral supplements. She had been having menorrhagia for the past 6 years. She had consulted for the same elsewhere and was diagnosed to have iron-deficiency anemia. The patient also had multiple blood transfusions. Her general examination revealed pallor, clubbing, and hepatosplenomegaly. Ocular examination revealed a best corrected visual acuity of light perception in right eye (OD) and 20/200 in left eye (OS). Pupil showed relative afferent pupillary defect in the right eye. Anterior segment examination was normal. Dilated fundus evaluation showed disk pallor in both eyes [Figure 1]a and [Figure 1]b. Background retina and macula appeared normal.
Figure 1: (a) Right eye showing pale disk suggesting optic atrophy. (b) Left eye showing pale disk suggesting optic atrophy

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Color vision was defective in both eyes. Pattern visual-evoked potential showed prolonged P100 latency (134 ms) in the right eye. Magnetic resonance imaging (MRI) showed bilateral optic atrophy with inflammatory changes in the right optic nerve, suggesting optic neuritis. MRI spine was normal.

Systemic evaluation revealed hemolytic anemia. Direct coombs test was positive, and ultrasonography abdomen showed splenomegaly. Mantoux test, venereal disease laboratory test, and anti-neuromyelitis optica antibody were negative. She was diagnosed as retrobulbar neuritis (OD) and primary optic atrophy (OS). A systemic diagnosis was made of primary ES. She was treated with intravenous methylprednisolone 1 g for 3 days and was discharged on oral prednisolone (1 mg/kg body weight) in tapering doses for 11 days. At present, the patient is under our follow-up and also under treatment from a physician for ES with immunomodulatory agents.

  Discussion Top

ES is a rare and challenging autoimmune condition. Ocular involvement, in general, is rarely encountered in ES. Ababneh et al.[7] reported a case of vascular occlusive event in ES leading to severe loss of vision. Verma et al.[8] reported a case of premacular hemorrhage in a child with ES. Ohnishi et al.[9] reported a case of vitrectomy for vitreous hemorrhage in a case of ES. Our case is the first case report of retrobulbar neuritis in ES. Timely diagnosis in our case with the aid of expert opinion helped us to decrease further mortality and morbidity in the patient. Systemic steroids were used to help decrease the inflammatory response and further nerve damage. This proved to retard further worsening of vision in our case. We suggest more cases should be reported regarding ocular manifestations of ES for better understanding of presentations, pathophysiology, and treatment of ocular ES.

  Conclusion Top

In future, if more such cases are observed among patients who have ES, it might raise the necessity of adding ophthalmic examination as part of their initial evaluation. Patient may first present to an ophthalmologist, and a meticulous workup can help in early diagnosis of this rare disease.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Evans R, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired haemolytic anemia: Evidence for a common etiology. AMA Arch Intern Med 1951;87:48-65.  Back to cited text no. 1
Dhingra KK, Jain D, Mandal S, Khurana N, Singh T, Gupta N. Evans syndrome: a study of six cases with review of literature. Hematology 2008;13:356-60.  Back to cited text no. 2
Pui CH, Wilimas J, Wang W. Evans syndrome in childhood. J Pediatr 1980;97:754-8.  Back to cited text no. 3
Wang W, Herrod H, Pui CH, Presbury G, Wilimas J. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol 1983;15:381-90.  Back to cited text no. 4
Norton A, Roberts I. Management of Evans syndrome. Br J Haematol 2006;132:125-37.  Back to cited text no. 5
Miano M. How I manage Evans syndrome and AIHA cases in children. Br J Haematol 2016;172:524-34.  Back to cited text no. 6
Ababneh LT, Mahmoud IH, Al-Rimawi HS. A rare case of Evans syndrome associated with sudden loss of vision: A case report. Eur J Ophthalmol 2020;30:NP1-11.  Back to cited text no. 7
Verma G, Nayak L, Banerjee A, Nayak B. Premacular haemorrhage in a child with Evans syndrome BMJ Case Reports 2019;12:bcr-2019-229636. doi: 10.1136/bcr-2019-229636.  Back to cited text no. 8
Ohnishi A, Kaneko M, Hori S, Teramura M, Kato S. Vitrectomy for vitreous bleeding and tractional retinal detachment in a case of Evans syndrome. Jpn J Ophthalmol 2000;44:177-9.  Back to cited text no. 9


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