Cilioretinal Artery Occlusion Following Diagnostic Lumbar Puncture in a Patient of Idiopathic Intracranial Hypertension

Clinical Correspondence Section Editors: Robert Avery, DO Karl C. Golnik, MD Caroline Froment, MD, PhD An-Guor Wang, MD Cilioretinal Artery Occlusion Following Diagnostic Lumbar Puncture in a Patient of Idiopathic Intracranial Hypertension Srikanta Kumar Padhy, MD, Suman Sahu, MD I diopathic intracranial hypertension is a heterogenous syndrome characterized by an inexplicable increase in intracranial pressure, predominantly affecting obese women of childbearing age. The mechanism of IIH is unclear; however, the possible pathogenesis is the imbalance between cerebrospinal fluid (CSF) formation and absorption. Diagnostic or therapeutic repeated lumbar puncture that drains CSF is an accepted treatment of this condition. One of the common side effects of lumbar puncture (LP) is postprocedure headache for which epidural blood patch is the treatment of choice when a conservative management with analgesics fail. The rebound increase in intracranial hypertension following the epidural patch (which closes the CSF leak) could be either due to increased CSF production in response to CSF depletion or due to disturbed resorption secondary to prolonged leak. Nevertheless, this phase is transient, the choroid plexus ultimately adjusts leading to abatement of hypertension (1). Cilioretinal artery occlusion (CLRAO) can occur in isolation or in conjunction with other vascular comprise in retina such as retinal venous occlusion. We report an extremely rare case of CLRAO following diagnostic lumbar puncture in a patient of idiopathic intracranial hypertension never reported in the literature. A 31-year-old man presented with the chief complaints of severe headache with nausea and pulsatile tinnitus for 1 month. There was history of transient episode of blurring of vision in both eyes 3 months ago, which recovered on its own. Medical history pertaining to any systemic illness was essentially absent except for recent unusual gain of weight (22 kg) in recent 6 months (body mass index, 32.5 kg/m2). The unaided visual acuity at presentation was 20/20 in both eyes with normal intraocular pressures. Dilated fundus evalLV Prasad Eye Institute, MTC Campus, Bhubaneswar, India. The authors report no conflicts of interest. Ethics approval: All procedures performed in the study were in accordance with the ethical standards of the Institutional ethics committee, and with the 1964 Helsinki declaration and its later amendments. Informed consent was obtained from the participant included in the study for publication of clinical data and photograph. Address correspondence to Srikanta Kumar Padhy, MD, Consultant, Department of Ophthalmology, Retina, Uvea and ROP Services, LV Prasad Eye Institute, MTC Campus, Bhubaneswar, India; E-mail: [e-mail] Padhy and Sahu: J Neuro-Ophthalmol 2023; 43: e175-e176 uation revealed bilateral disc edema with ill-defined margins (Fig. 1A, B). Humphrey visual field showed an enlarged blind spot in the right eye; the left eye was normal. MRI and magnetic resonance venography brain with contrast revealed partially empty sella, but no evidence of any focal or space occupying lesion or cerebral venous thrombosis was found. A provisional diagnosis of idiopathic intracranial hypertension was made, and the patient was referred to neurologist for diagnostic LP. Lumbar puncture of the patient revealed an opening pressure of 52 cm of water with normal CSF content (normal protein, sugar, and electrolyte levels). Following LP, an epidural blood patch was performed to relieve headache. Diagnostic lumbar puncture immediately relieved his headache, and he was started on oral acetazolamide (500 mg twice a day). Six hours after LP, FIG. 1. Color fundus photograph of right (A) and left (B) eyes, respectively, shows disc edema (Frisen Grade 2) with blurry margins at presentation. Following Lumbar puncture, the disc edema worsened in both eyes with appearance of peripapillary hemorrhage inferior to disc, left eye (C) in addition reveals an area of stark retinal whitening along the inferotemporal arcade. The whitening appeared to follow a retinal arteriole emanating from the optic nerve head constituting a cilioretinal artery occlusion. Following lumboperitoneal shunt, at first and third months, both eyes show resolving disc edema and retinal infarction. A narrowed cilioretinal artery can be observed at 3 months following incident in the left eye (D). e175 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Clinical Correspondence patient again presented to the ophthalmology emergency department with sudden onset of blurring of vision in his left eye. He was maintaining a visual acuity of 20/20 in both eyes. Fundus examination revealed persistent disc edema in both eyes with peripapillary hemorrhage. In addition, the left eye demonstrated an area of stark retinal whitening along the inferotemporal arcade. The whitening appeared to follow a retinal arteriole emerging from the optic nerve head (Fig. 1C). HVF displayed an enlargement of blind spot in the right eye, with additional superior visual field defect in left eye (Fig. 2A–D). The clinical picture was concerning for inner retinal infarction, which was established with swept-source optical coherence tomography. The patient was referred to the neurologist for urgent management in view of a new-onset CLRAO in the left eye. Fundus fluorescein angiography could not be done in the view of fluorescein allergy. Systemic workups, including coagulation profile, blood pressure, carotid artery doppler, negative antinuclear and anticytoplasmic antibodies and extractable nuclear antigens, echocardiogram, and electrocardiogram, failed to detect any vasculitic or thromboembolic cause for his cilioretinal artery infarction. The patient subsequently underwent lumbar thecoperitoneal shunt surgery 1 week later. At 1- and 3-month follow-up, a marked resolution of disc edema was noted with subsequent disappearance of retinal whitening at the final visit. A narrowed cilioretinal artery could be observed at this visit (Fig. 1D). The proposed mechanism of CLRAO in the setting central retinal venous occlusion (CRVO) is attributable to hemodynamic block, where an escalation in retinal venous pressure above that of choroidal vascular bed causes the cilioretinal artery to enter a state of relative stasis (2,3). Other mechanism is the phenomenon of choroidal steal, which states that during CRVO, the retinal arterial blood takes the path of least resistance in to choroid bypassing the cilioretinal artery (4). Our patient developed cilioretinal retinal artery occlusion in the left eye with worsening of disc edema in both the eyes following lumbar puncture, which could be attributed to a paradoxical increase in intracranial pressure following LP. However, he did not have any signs of CRVO. We hypothesized 2 mechanisms for the possible clinical findings in our case. First, perhaps the marked disc edema elevated the proximal pressure in cilioretinal circulation either through direct compression or through increasing venous pressure, such that blood flow in the posterior ciliary artery was preferentially diverted to the low-pressure choroidal circulation supporting the theory by Mcleod et al. (4) Second, we also cannot discount the possibility that the severe disc edema occluded the cilioretinal artery through extrinsic pressure (5). Cilioretinal artery infarction in the setting of isolated papilledema is an extremely rare presentation. Our patient underwent lumbar thecoperitoneal shunt surgery 1 week later resulting in a fall in intracranial pressure and subsequent decrease in papilledema and relief of symptoms. STATEMENT OF AUTHORSHIP Conception and design: S. K. Padhy; Acquisition of data: S. Sahu; Analysis and interpretation of data: S. K. Padhy, S. Sahu. Drafting the manuscript: S. K. Padhy; Revising it for intellectual content: S. Sahu. Final approval of the completed manuscript: S. K. Padhy. ACKNOWLEDGMENTS The authors acknowledge Dr Soumyava Basu (Consultant, Retina, uvea and ROP services, LV Prasad Eye Institute, MTC Campus, Bhubaneswar) for his guidance during manuscript preparation. REFERENCES FIG. 2. Humphrey visual field 30-2 SITA standard reveals a normal report in left eye (A) and an enlarged blind spot in the right eye (B). Post lumbar puncture, the left eye had a superior visual field defect corresponding to the area of ischemia seen in the fundus picture (C) and a further increase in size of blind spot noted in right eye (D). e176 1. Panikkath R, Welker J, Johnston R, Lado-Abeal J. Intracranial hypertension and intracranial hypotension causing headache in the same patient. Proc (Bayl Univ Med Cent). 2014;27:217– 218. 2. Hayreh SS, Fraterrigo L, Jonas J. Central retinal vein occlusion associated with cilioretinal artery occlusion. Retina 2008;28:581–594. 3. McCannel CA. Choroidal infarction or cilioretinal artery occlusion in the setting of elevated intracranial pressure due to fulminant idiopathic intracranial hypertension? J Neuroophthalmol 2011;31:194–195. 4. McLeod D. Central retinal vein occlusion with cilioretinal infarction from branch flow exclusion and choroidal arterial steal. Retina 2009;29:1381–1395. 5. Mahroo OA, Mohamed MD, Graham EM, Mann SS, Plant GT, Afridi SK, Hammond CJ. Cilioretinal artery territory infarction associated with papilledema in a patient with neurofibromatosis type 2. J Neuroophthalmol 2016;36:58–60. Padhy and Sahu: J Neuro-Ophthalmol 2023; 43: e175-e176 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.