Anemia and idiopathic intracranial hypertension – A coincidence or a correlation

Arumugam Balraj; Shreeya Hinge

doi:10.4103/IJO.IJO_2773_22

CASE REPORT

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

Anemia and idiopathic intracranial hypertension – A coincidence or a correlation

Arumugam Balraj, Shreeya Hinge
Department of Neuro-Ophthalmology, Aravind Eye Hospital, Thavalakuppam, Pondicherry, India

Date of Submission	22-Oct-2022
Date of Acceptance	16-Jan-2023
Date of Web Publication	28-Apr-2023

Correspondence Address:
Arumugam Balraj
Aravind Eye Hospital, Thavalakuppam, Pondicherry 605 007
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJO.IJO_2773_22

Abstract

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

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

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

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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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