|Year : 2021 | Volume
| Issue : 1 | Page : 137-139
Bilateral non-arteritic anterior ischemic optic neuropathy with idiopathic intracranial hypertension secondary to attempted abortion related hemorrhage: A rare case report
Amit Kumar Deb, Gaurav Ghag, Pratik Gera, Nirupama Kasturi, Sandip Sarkar
Department of Ophthalmology, JIPMER Hospital, Puducherry, India
|Date of Submission||24-Feb-2020|
|Date of Acceptance||13-Jul-2020|
|Date of Web Publication||31-Dec-2020|
Dr. Sandip Sarkar
Department of Ophthalmology, JIPMER Hospital, Gorimedu, Puducherry - 605 006
Source of Support: None, Conflict of Interest: None
We report a unique case of bilateral non-arteritic anterior ischemic optic neuropathy with co-existing idiopathic intracranial hypertension (IIH) in a young female following severe blood loss from abortion. At presentation, she had bilateral gross diminution of vision (hand movements and light perception in right & left eyes respectively) and bilateral pallid disc edema. Lumbar puncture and brain imaging were suggestive of IIH. Temporal association of vision loss with acute blood loss as well as fluorescein angiography were suggestive of bilateral NAION. Patient was treated with intravenous (IV) mannitol, IV methylprednisolone followed by oral steroids, oral acetazolamide, repeat lumbar punctures, and blood transfusion. Vision in right eye improved to 20/40 & left eye 20/500 at sixth month. Multidisciplinary approach is, thus, needed in management of such a case.
Keywords: Abortion, blood loss, idiopathic intracranial hypertension, non-arteritic anterior Ischemic optic neuropathy
|How to cite this article:|
Deb AK, Ghag G, Gera P, Kasturi N, Sarkar S. Bilateral non-arteritic anterior ischemic optic neuropathy with idiopathic intracranial hypertension secondary to attempted abortion related hemorrhage: A rare case report. Indian J Ophthalmol Case Rep 2021;1:137-9
|How to cite this URL:|
Deb AK, Ghag G, Gera P, Kasturi N, Sarkar S. Bilateral non-arteritic anterior ischemic optic neuropathy with idiopathic intracranial hypertension secondary to attempted abortion related hemorrhage: A rare case report. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Feb 27];1:137-9. Available from: https://www.ijoreports.in/text.asp?2021/1/1/137/305527
Non-arteritic anterior ischemic optic neuropathy (NAION) is the second most common cause of optic neuropathy behind glaucoma. Systemic co-morbidities like diabetes, hypertension, and ocular factors like a small crowded disc; acute peri-operative blood loss are the common predisposing factors for NAION. However, literature on peripartum blood loss causing NAION is sparse. Bilateral NAION is rare and commonly seen in cases with severe hypotensive episodes following blood loss or surgery. Idiopathic intracranial hypertension (IIH) is characterized by increased cerebrospinal fluid (CSF) pressure in absence of an intracranial space-occupying lesion (ICSOL) or ventricular dilatation. IIH is typically seen in young obese women of the reproductive age group. Rarely, IIH can also be seen in young non-obese women (BMI <30) with history of recent weight gain of around 10%. Optic disc edema secondary to raised intracranial pressure (ICP) can rarely be a factor precipitating NAION in the setting of a reduced blood supply to the optic nerve head (ONH). We, hereby, report a unique case with features suggestive of IIH and bilateral simultaneous NAION in a young female following severe blood loss. To the best of our knowledge, this is the first case report describing bilateral NAION in IIH following acute blood loss.
| Case Report|| |
A 28-year-old female presented to us with history of sudden onset painless diminution of vision in both eyes associated with headache and vomiting for one day. She gave history of intake of over the counter medications (medical termination of pregnancy-MTP kit i.e., mifepristone 200 mg one tablet and misoprostol 200 microns each four tablets) for abortion a week prior to onset of visual symptoms. This was followed by profuse bleeding per vaginum for six days and subsequent visual loss. She had no history of headache, blackouts, tinnitus, or diplopia for any period prior to this episode. There was no history of intake of any chronic medications like steroids, tetracyclines, and oral contraceptive pills (OCPs). On examination, her weight was 70 kgs and height was 1.61 m with a body mass index (BMI) of 27 kg/m2. Her blood pressure was 110/70 mm Hg and pulse rate 90/min at presentation. Her best-corrected visual acuity (BCVA) was hand movements close to face (HMCF) in right eye (RE) and perception of light (PL) in left eye (LE). Bilateral mild restriction of abduction was noted. Pupils were 5 mm, fixed dilated in both eyes. Fundus examination showed bilateral diffuse pallid disc edema and peri-papillary superficial splinter hemorrhages [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Mid-peripheral and peripheral fundus examination was normal in both eyes. Fundus fluorescein angiography (FFA) showed bilateral optic disc filling delay in early phases [Figure 2]a and [Figure 2]b and disc staining in the late phases [Figure 2]c and [Figure 2]d––suggesting BL NAION. Complete hemogram showed severe anemia with haemoglobin (Hb) levels of 5.0 g%; reduced hematocrit––23% (normal 35%–45%); normal mean corpuscular volume (MCV)––88 fl (normal: 80–100 fl); normal serum ferritin––50 ng/mL (normal: 10–120 ng/mL); slightly elevated total count (11,900/μL); normal platelet count (3,48,000/μL) and a normal peripheral smear (normochromic normocytic anemia). Noncontrast computed tomography (NCCT) and CT Venogram (CTV) were normal [Figure 3]a, [Figure 3]b, [Figure 3]c, [Figure 3]d with no evidence of any intracranial space-occupying lesion (ICSOL), cerebral sinus venous thrombosis (CSVT) or dilatation of ventricles. Urine pregnancy test was positive and gynecological examination showed evidence of recent abortion. Lumbar puncture was done carefully and CSF examination showed an opening pressure of 84 cm H2O, normal protein (18 mg/dl), normal glucose (102 mg/dl) and chloride (112 meq/dl), microscopically acellular, and genexpert test for tuberculosis (TB) negative. Visual evoked potential (VEP) was non-recordable due to poor visual acuity. Diagnosis of bilateral NAION with co-existing IIH was made. Patient was treated with intravenous (IV) Mannitol, IV Methylprednisolone followed by tapering doses of oral steroids, Tab. acetazolamide, blood transfusion, oral hematinics and serial therapeutic lumbar punctures. CSF opening pressures on subsequent lumbar punctures were 40, 27, and 20 cm H2O on days 2, 4, and 7, respectively. Hb improved to 10.6 gm% after two weeks. Disc edema slowly subsided in both eyes [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. Pupillary reflex in RE recovered to ill sustained reaction while in LE pupil remained fixed, dilated at last visit. Her BCVA at last visit at sixth month was RE 20/40 and LE 20/500 LE.
|Figure 1: Fundus image at presentation showing pallid disc edema with peripapillary splinter hemorrhages (a and b), and OCT image at presentation showing increased thickness of peripapillary retinal nerve fiber layer (RNFL) with average RNFL thickness of 205 μm (OD) and 215 μm (OS) respectively (c and d)|
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|Figure 2: FFA showing delayed disc filling in both eyes in early phase (a and b) and disc staining in late phases (c and d)|
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|Figure 3: Computed tomograpgy with venogram (CTV) images showing normal cerebral ventricles (arrow) (a and c) and normal dural venous sinuses with no evidence of thrombosis (triangle) (b, c and d)|
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|Figure 4: Fundus image at follow up showing resolved disc edema (a and b) and OCT showing resolved disc edema (c and d) with average RNFL thickness of 146 μm (OD) and 141 μm (OS)|
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| Discussion|| |
Prelaminar region of the optic nerve head (ONH) consists of nerve fiber bundles, intervening glial septa, and fine vessels (peripapillary choroid and posterior ciliary arteries) enclosed by an unyielding Bruch membrane. The perfusion pressure in these fine vessels is difference between mean blood pressure (MBP) and the intraocular pressure (IOP) or ONH tissue pressure surrounding the prelaminar capillaries in case of a swollen optic disc. Therefore, in the event of a circulatory stasis in the prelaminar capillaries (as seen in disc edema), a critical balance develops between the blood pressure (BP) in the capillaries and the surrounding tissue pressure. Any factor that raises the IOP or ONH tissue pressure, or fall of the BP in the capillaries, will impair ONH perfusion.
NAION is the most common acute optic neuropathy seen in patients above 50 years of age. It occurs due to impaired blood supply in the short posterior ciliary arteries. Acute severe blood loss can lead to reduced perfusion pressure in these vessels supplying the ONH. The resultant ONH non-perfusion or hypo-perfusion in susceptible persons can in turn lead to NAION.
Patients with optic disc edema secondary to raised ICP usually present with transient visual obscuration, headache, and diplopia. In such susceptible eyes, this transient visual loss may progress to gross dimunition of vision because of development of NAION by any factor that lowers the perfusion pressure in the ONH including acute blood loss, change of posture etc. Our patient, however, had no preceeding symptoms suggestive of raised ICP and IIH was merely an incidental diagnosis. Pre-existing IIH was diagnosed in our patient based on a very high cerebro-spinal fluid (CSF) opening pressure, normal brain imaging, and normal CSF cytology reports. CSVT was ruled out by a normal CT venogram. MRI and MRV are, however, the imaging modalities of choice to rule out CSVT and diagnose IIH. Sudden vision loss due to IIH alone without any preceeding symptoms is unlikely in our case and there was also no discernible cause for acute rise in ICP. Acute blood loss due to abortion was the critical event impairing ONH perfusion, thereby, resulting in bilateral simultaneous NAION and subsequent vision loss. Pre-existing optic disc edema due to IIH was, therefore, a mere predisposing factor for NAION in our case. Pupils were fixed dilated in both eyes at presentation which is an unlikely finding in papilledema alone and hints towards an additional ischemic insult to the bilateral optic discs. FFA findings were also suggestive of bilateral NAION rather than IIH/papilledema in our case. FFA showed delayed optic disc filling in early phases and late leakage in both eyes. FFA in NAION typically shows delayed optic disc filling and circular or localized peripapillary choroid filling delays in contrast to early phase disc leakage seen in papilledema., The temporal association between abortion-related acute severe blood loss and dimunition of vision; absence of any discernible cause of acute rise in ICP; fixed dilated pupils, FFA findings; normal brain imaging with elevated CSF opening pressure and normal CSF cytology reports all imply towards the diagnosis of coexistent bilateral NAION and IIH in our case.
Another important clinical finding in our case that warrants discussion is co-existent severe anemia at presentation. Hemoglobin was 5.9 gm % at presentation. However, there was no past history suggestive of chronic anemia or iron deficiency anemia e.g., weakness, fatigue, shortness of breath, and palpitations. Peripheral blood smear showed normochromic normocytic anemia; hematocrit was reduced; MCV and serum ferritin levels were normal, thereby, ruling out chronic anemia or iron deficiency anemia. Also retinal examination in both eyes in our patient had no other signs of anemic retinopathy like Roth's spots, and peripheral retinal hemorrhages. BP at presentation was low along with a higher pulse rate. Anemia in this case was, therefore, due to acute blood loss. Chronic anemia can rarely present as bilateral disc edema which is unlikely in this case. There are few case reports of bilateral NAION with severe anemia following acute systemic hypotension., In our case, we had described an unique presentation of bilateral NAION with severe anemia and systemic hypotension following abortion-related acute blood loss in the setting of pre-existing disc edema due to IIH.
Treatment of blood loss associated NAION primarily includes correction of anemia and hypovolemia as done in our case. In addition, she received a course of IV methylprednisolone and oral steroids. Use of systemic steroids during very early stages of NAION may reduce capillary permeability, leading to quicker resolution of disc edema. IIH in our patient was managed with intravenous mannitol, oral acetazolamide, and serial therapeutic lumbar punctures.
| Conclusion|| |
Blood loss related hypotension is an important risk factor for the development of NAION that may be bilateral at onset. Associated headache in a case of NAION related disc edema warrants lumbar puncture and brain imaging to rule out IIH or any ICSOL. Successful management and favorable visual outcome in such a case needs a multidisciplinary approach involving neurologist, radiologist, and ophthalmologist.
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.
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