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Year : 2023  |  Volume : 3  |  Issue : 2  |  Page : 472-474

Anemia and idiopathic intracranial hypertension – A coincidence or a correlation

Department of Neuro-Ophthalmology, Aravind Eye Hospital, Thavalakuppam, Pondicherry, India

Date of Submission22-Oct-2022
Date of Acceptance16-Jan-2023
Date of Web Publication28-Apr-2023

Correspondence Address:
Arumugam Balraj
Aravind Eye Hospital, Thavalakuppam, Pondicherry 605 007
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2773_22

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Idiopathic intracranial hypertension (IIH) is commonly seen in females of reproductive age (15–45 years). Here, we report a unique case of a 22-year-old female presenting with complaints of headache and vomiting with a history of polycystic ovarian syndrome for which she was on treatment. The patient had bilateral optic disc edema on fundus examination. Hematological investigations showed severe iron deficiency anemia with hemoglobin 3.7 g/dl. Lumbar puncture showed raised cerebrospinal fluid opening pressure of 270 cm of H2O. Magnetic resonance imaging brain showed partially empty sella and T2 hyperintensity in optic nerve canal, suggestive of IIH. The patient was treated subsequently with blood transfusion followed by iron therapy. After treatment, she showed drastic improvement with the resolution of papilledema and normalization of visual field analysis. Our findings emphasize the importance of hematological investigations and prompt treatment in IIH, which ensure a better prognosis by shortening the disease course.

Keywords: Anemia, benign idiopathic intracranial hypertension, empty sella, headache, idiopathic intracranial hypertension

How to cite this article:
Balraj A, Hinge S. Anemia and idiopathic intracranial hypertension – A coincidence or a correlation. Indian J Ophthalmol Case Rep 2023;3:472-4

How to cite this URL:
Balraj A, Hinge S. Anemia and idiopathic intracranial hypertension – A coincidence or a correlation. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:472-4. Available from: https://www.ijoreports.in/text.asp?2023/3/2/472/374989

Idiopathic intracranial hypertension (IIH) is an entity with raised intracranial pressure in the absence of structural causes and normal cerebrospinal fluid (CSF) examination.[1] It commonly occurs in obese women of reproductive age group (15–35 years).[2] It is a diagnosis of exclusion, usually made only after ruling out secondary causes for raised intracranial pressure.[3] Association between anemia and IIH dates back to 100 years. There is no proven direct association between the two.[4] Evidence is limited to previous case series and case reports.[5]

  Case Report Top

A 22-year-old female presented with 1 month history of headache and vomiting without any diplopia. She also gave a history of polycystic ovarian syndrome (PCOS), for which she took oral contraceptive pills (OCPs) for 3 years and self-prescribed vitamin A supplements daily for 1 year.

On examination, her visual acuity was 6/6 in both eyes, and they had full range of extraocular movements. Posterior segment examination elicited bilateral grade III optic disc edema [Figure 1]a and [Figure 1]b. Visual field analysis using Humphrey Field Analyzer (HFA) 30-2 showed bilateral enlargement of blind spot. General examination showed a body mass index (BMI) of 20 kg/m2. Contrast-enhanced magnetic resonance imaging (MRI) brain showed no intracranial space-occupying lesion; T2 hyperintensity was noted in the intracanalicular portion of bilateral optic nerve [Figure 2]a and partial empty sella [Figure 2]b. Lumbar puncture showed raised opening pressure of 270 cm of H2O. The diagnosis of IIH was made in our case based on the criteria proposed by Friedman and Jacobson.[1]
Figure 1: (a and b) Fundus photographs showing grade III optic disc edema and peripapillary nerve fiber hemorrhages

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Figure 2: Magnetic resonance imaging brain with orbit showing pituitary gland appears to be compressed inferiorly suggestive of empty sella in the sagittal section (a) and significant fluid noted surrounding the bilateral optic nerve sheath in the axial section (b)

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OCPs and vitamin A supplements were stopped, and she was started on oral acetazolamide 250 mg thrice daily. The patient was reviewed after a month and no improvement in any symptoms or signs was found. Hematological investigations showed severe iron deficiency anemia (IDA) (hemoglobin [Hb]: 3.7 g/dl, mean corpuscular volume [MCV]: 66.4 fl, mean corpuscular hemoglobin [MCH]: 17.1 pg, mean corpuscular hemoglobin concentration [MCHC]: 25.7%, red cell distribution width [RDW]: 19.4%, serum ferritin: 1.5 ng/ml [13–150 ng/ml], serum iron: 18 μg/dl [50–170 μg/dl], total iron-binding capacity [TIBC]: 560 μg/dl [200–400 μg/dl], transferrin saturation: 7% [>16%]). Peripheral blood smear showed microcytic hypochromic red blood cells suggestive of IDA.

The patient was given blood transfusion with two units of packed red blood cells on the same day and intravenous iron therapy for the next 3 days. Following this, oral iron supplements were prescribed for 6 months. After 1 month, her hemoglobin improved to 7.8 g/dl. The patient was symptomatically better. Hemoglobin was increased to 10 g/dl at 6 months. Fundus examination showed resolution of papilledema [Figure 3]a and [Figure 3]b. Visual field analysis was also normal.
Figure 3: (a and b) Fundus photographs showing resolution of disc edema in both eyes

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  Discussion Top

IIH is more commonly seen in obese females of reproductive age group (15–45 years).[1] Pal et al.[3] found that among IIH patients, only 3.3% were obese and 6% were overweight. But in our case, the patient had a normal BMI. They concluded that obesity is not a dominant risk factor for developing IIH in the Indian population and absence of obesity is a good prognostic factor. Our patient had PCOS and was taking OC pills for the same. Incidence of IIH in PCOS is five to eight times more compared to the general population.[4] High endogenous and exogenous estrogen levels as seen in PCOS and obesity are considered to be thrombophilic and can form microthrombi in arachnoid villi, thus reducing CSF absorption and causing IIH, especially in patients with preexisting coagulation disorders.[4]

Vitamin A and its compounds are considered as an etiological factor causing IIH.[1] Libien et al.[5] concluded that vitamin A or its metabolites are not implicated in development of IIH, in contrast to several previous reports. Our case presented with severe IDA (hemoglobin <5.0). Various mechanisms are postulated by which IDA can lead to IIH. One of them is that iron regulates thrombopoiesis by checking erythropoietin levels. In IDA, iron deficiency causes thrombocytosis due to loss of inhibition and increased erythropoietin, thus producing a hyperviscous and hypercoagulable state, and another theory attributes the hyperviscosity to impaired deformability of the red blood cells in IDA.[6] There is a strong association between IDA and cerebral venous sinus thrombosis (CVST). IDA is a hypercoagulable state owing to reduced deformability of red blood cells and an increased concentration of Factor VIII, increasing the risk of thrombosis.[7] Our patient did not show any signs or symptoms of CVST or any evidence of thrombosis on MRI brain, probably due to timely intervention.

The increased prevalence of PCOS in women with IIH may reflect PCOS-driven morbid obesity. The mechanism of IIH in PCOS suggested that increased intra-abdominal and cardiac filling pressures, associated with obesity, may result in a retrograde increase in CSF pressure. But PCOS can occur in nonobese females also. The mechanism of causing IIH in our patient may be due to exogenous estrogens (OCPs), vitamin A, and severe anemia, which may have reduced CSF reabsorption in the brain by promoting microthrombi of the arachnoid villi, leading to IIH.[7],[8]

Treatment of IIH includes lifestyle modifications, medical treatment, and CSF fluid diversion procedures if required. Lifestyle modifications include low-salt, low-calorie diet and regular exercise for weight reduction.[9] Since our patient had normal BMI, these measures were not advocated. Raoof and Hoffmann[10] found significant difference in improvement of perimetric mean deviation in patients treated with acetazolamide and those treated with a placebo. Acetazolamide alone did not show any improvement in the symptoms or signs in our patient. Ma et al.[6] documented that IIH patients with anemia show significant improvement in papilledema with anemia treatment, as seen in our case.

  Conclusion Top

This case report highlights the importance of proper evaluation of IIH patients. It warrants routine anemia evaluation in reproductive age group females, followed by prompt and aggressive treatment of underlying causes to reverse papilledema and avoid sequelae like permanent visual loss or CVST.

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 initial s 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

Friedman DI, Jacobson DM. Diagnostic criteria for idiopathic intracranial hypertension. Neurology 2002;59:1492-5.  Back to cited text no. 1
Biousse V, Rucker JC, Vignal C, Crassard I, Katz BJ, Newman NJ. Anemia and papilledema. Am J Ophthalmol 2003;135:437-46.  Back to cited text no. 2
Pal A, Sengupta P, Biswas D, Sen C, Mukherjee A, Pal S. Pattern of idiopathic intracranial hypertension in Indian population. Ann Indian Acad Neurol 2019;22:47-51.  Back to cited text no. 3
[PUBMED]  [Full text]  
Glueck CJ, Aregawi D, Goldenberg N, Golnik KC, Sieve L, Wang P. Idiopathic intracranial hypertension, polycystic-ovary syndrome, and thrombophilia. J Lab Clin Med 2005;145:72-82.  Back to cited text no. 4
Libien J, Kupersmith MJ, Blaner W, McDermott MP, Gao S, Liu Y, et al. Role of vitamin A metabolism in IIH: Results from the idiopathic intracranial hypertension treatment trial. J Neurol Sci 2017;372:78-84.  Back to cited text no. 5
Ma Z, Jiang H, Meng C, Cui S, Peng J, Wang J. Idiopathic intracranial hypertension in patients with anemia: A retrospective observational study. PLoS One 2020;15:e0236828.  Back to cited text no. 6
Coutinho JM, Zuurbier SM, Gaartman AE, Dikstaal AA, Stam J, Middeldorp S, et al. Association between anemia and cerebral venous thrombosis: Case-control study. Stroke 2015;46:2735-40.  Back to cited text no. 7
Ramanand SJ, Ghongane BB, Ramanand JB, Patwardhan MH, Ghanghas RR, Jain SS. Clinical characteristics of polycystic ovary syndrome in Indian women. Indian J Endocrinol Metab 2013;17:138-45.  Back to cited text no. 8
Sinclair AJ, Burdon MA, Nightingale PG, Ball AK, Good P, Matthews TD, et al. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension: Prospective cohort study. BMJ 2010;341:c2701. doi: 10.1136/bmj.c2701.  Back to cited text no. 9
Raoof N, Hoffmann J. Diagnosis and treatment of idiopathic intracranial hypertension. Cephalalgia Int J Headache 2021;41:472-8.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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