Papilledema revisiting after sinus angioplasty of ...

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to understand whether this is indeed rare or underreported. In addition, patients may also be warned to get ophthalmic consultation if they experience blurred vision or dark spots. 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 The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Acknowledgment We acknowledge the assistance by Dr. Sabyasachi Sengupta from Sengupta’s Research Academy in manuscript preparation. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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2. Thon OR, Gittinger JW Jr. Medication‑related pseudotumor cerebri syndrome. Semin Ophthalmol 2017;32:134‑43. 3. von Wolff A, Hölzel LP, Westphal A, Härter M, Kriston L. Selective serotonin reuptake inhibitors and tricyclic antidepressants in the acute treatment of chronic depression and dysthymia: A systematic review and meta‑analysis. J Affect Disord 2013;144:7‑15. 4. Ceylan ME, Evrensel A, Cömert G. Papilledema due to mirtazapine. Balkan Med J 2016;33:363‑5. 5. Hutcheon ML. An unexpected case of swollen optic nerves. Am J Ther 2011;18:e126‑9. 6. Owen RT. Controlled‑release fluvoxamine in obsessive‑compulsive disorder and social phobia. Drugs Today (Barc) 2008;44:887‑93. 7. Kelly SJ, O’Donnell T, Fleming JC, Einhaus S. Pseudotumor cerebri associated with lithium use in an 11‑year‑old boy. J AAPOS 2009;13:204‑6. 8. Renoux C, Vahey S, Dell’Aniello S, Boivin JF. Association of selective serotonin reuptake inhibitors with the risk for spontaneous intracranial hemorrhage. JAMA Neurol 2017;74:173‑80. 9. Grome JJ, Harper AM. The effects of serotonin on local cerebral blood flow. J Cereb Blood Flow Metab 1983;3:71‑7.

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10. Kao TY, Lin MT. Brain serotonin depletion attenuates heatstroke‑induced cerebral ischemia and cell death in rats. J Appl Physiol (1985) 1996;80:680‑4.

1. Phillips PH, Sheldon CA. Pediatric pseudotumor cerebri syndrome. J Neuroophthalmol 2017;37 Suppl 1:S33‑40.

11. Van Nueten JM, Janssens WJ, Vanhoutte PM. Serotonin and vascular reactivity. Pharmacol Res Commun 1985;17:585‑608.

Papilledema revisiting after sinus angioplasty of chronic cerebral venous sinus thrombosis

presented with blurring and transient obscuration of vision with papilledema, headache, tinnitus, and vomiting. On magnetic resonance venography and digital subtraction angiography, chronic transverse sinus thrombosis was diagnosed for which patients underwent endovascular intervention (stenting). After the resolution of CVST, patients developed the recurrence of symptoms which on digital subtraction angiography revealed dural arteriovenous malformation (DAVM). DAVM and papilledema resolved with endovascular embolization. This case series highlights a need of constant monitoring of CVST cases after the endovascular intervention.

Vaibhav Kumar Jain, Vivek Singh1, Vikas Kannaujia, Priyadarshini Mishra, Rajendra Vishnu Phadke1, Kumudini Sharma This case series aimed to review the significance of revisiting papilledema after successful cerebral venous sinus thrombosis (CVST) management with sinus angioplasty. Four patients Access this article online Quick Response Code:

Website: www.ijo.in DOI: 10.4103/ijo.IJO_930_17 PMID: ***

Departments of Ophthalmology and 1Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India Correspondence to: Prof. Kumudini Sharma, Department of Ophthalmology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow ‑ 226 014, Uttar Pradesh, India. E‑mail: [email protected] Manuscript received: 29.09.17; Revision accepted: 29.01.18

Key words: Cerebral venous sinus thrombosis, dural arteriovenous malformation, endovascular, glue embolization, stenting

Cerebral venous sinus thrombosis (CVST) may develop a rare and chronic complications such as dural arteriovenous malformation (DAVM). [1] Two‑fifths of the patients with DAVM had been found to be associated with CVST in an earlier study involving 69 patients.[2] DAVM may be completely

This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite this article as: Jain VK, Singh V, Kannaujia V, Mishra P, Phadke RV, Sharma K. Papilledema revisiting after sinus angioplasty of chronic cerebral venous sinus thrombosis. Indian J Ophthalmol 2018;66:714-7.


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asymptomatic or may present with mild symptoms to fatal cerebral hemorrhage.[3] In the earlier case reports, DAVM after CVST presented with varied symptoms such as a headache, tinnitus, aphasia, seizure, irritability, hemiparesis, and depending on the location.[4‑6] Cases presenting with ophthalmic symptoms leading to the diagnosis of DAVM have been reported rarely.[7] This is a first case series reporting the cases of the transverse sinus (TS) thrombosis who after successful endovascular stenting showed the recurrence of papilledema and visual loss which on further evaluation led to the diagnosis of DAVM.

Case Reports Case 1 A 48‑year‑old male patient presented with the blurring of vision, with a headache, and tinnitus for the past 1 month. Best‑corrected visual acuity (BCVA) was 6/6 both eyes. Fundus showed florid papilledema both eyes [Fig. 1a and b]. Visual fields showed the enlargement of a blind spot. Optical coherence tomography (OCT) showed marked thickening of retinal nerve fiber layer (RNFL). Magnetic resonance venography (MRV) revealed thrombosis of the right TS with partial thrombosis of the superior sagittal sinus. The patient was positive for antiphospholipid antibodies. After starting medical treatment (acetazolamide, furosemide, and glycerine) for raised intracranial pressure with anticoagulant, the papilledema improved minimally. After 2 months, vision deteriorated to 6/60 and 6/9 in the right and left eye, respectively, with persistent papilledema. Digital subtraction angiography (DSA) revealed chronic right TS thrombosis with narrowing of lumen [Fig. 2a] with >10 mmHg pressure gradient at the site of narrowing. He underwent balloon dilatation and stenting of right TS [Fig. 2b] with patent stent and normal flow in poststent angiogram [Fig. 2c]. Papilledema resolved with stable vision and [Fig. 1c and d] secondary optic

atrophy in the right eye [Fig. 1c]. OCT showed the resolution of thickened RNFL. On the recurrence of a headache, diminished vision and papilledema after 3 months, DSA showed the patent stent with DAVM near right TS [Fig. 2d]. On glue embolization of DAVM [Fig. 2e], papilledema resolved in both eyes. On the last follow‑up of 5 years, patient is asymptomatic. Case 2 A 26‑year‑old male patient presented with transient obscuration of vision with tinnitus for 1 year and diminution of vision for 2 months. BCVA was 6/60 and 6/9 in the right and left eye, respectively. Examination revealed relative afferent pupillary defect, secondary optic atrophy in the right eye and full‑blown papilledema left eye. Bruit was heard behind the right ear. MRV revealed chronic thrombosis with narrowing of the lumen of right TS. He was advised endovascular angioplasty of the sinus, but patient deferred that treatment. After 6 months, the patient presented with further decrease of vision with papilledema, DSA demonstrated right TS thrombosis with DAVM. Angioplasty and stenting of stenosed TS was performed, but no significant improvement in fundus was noticed. Hence, DAVM was embolized with coils and onyx. Postembolization of DAVM symptoms and papilledema improved with no further deterioration of vision. On follow‑up

a

b

c a

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Figure 1: Fundus photographs of a patient (Case I) with cerebral venous sinus thrombosis showing florid papilledema both eyes at the time of presentation (a and b), and the resolution of papilledema with secondary optic atrophy right eye after stenting of venous sinus (c and d)

e

Figure 2: Digital subtraction angiograms of a patient (Case I) with cerebral venous sinus thrombosis showing chronic right transverse sinus thrombosis (blue arrow) (a); balloon dilatation and stenting of right transverse sinus thrombosis (blue arrow) (b); poststenting angiogram showing patent right transverse sinus stent with normal flow (blue arrow) (c); repeat Digital subtraction angiogram after 3 months of stenting procedure showing dural arteriovenous malformation (blue arrow) in relation to right transverse sinus with retrograde and antegrade flow (d); postglue embolization of dural arteriovenous malformation (e)


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leading to the release of angiogenic factors such as vascular endothelial growth factor with subsequent formation of abnormal connections between arteries and venous sinuses.[9]

a

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c

d

Figure 3: Digital subtraction angiogram of a patient (Case IV) depicting dural arteriovenous malformation (blue arrow) postcerebral venous sinus thrombosis (a); enlarged cortical veins (hanging veins) with partially thrombosed venous sinuses (blue arrow) (b); glue injection in one of the dural feeders (blue arrow) (c); resolved dural arteriovenous malformation postembolization (d)

of 3 years, stable vision with no evidence of papilledema was noticed. Case 3 A 48‑year‑old female patient presented with a headache, vomiting for 1 month, and diminution of vision for 1 year which deteriorated recently. On examination, BCVA of 6/36 right eye and 6/24 left eye with chronic papilledema was noted. MRV showed chronic right TS thrombosis with partial recanalization. DSA confirmed the MRV findings. She underwent balloon dilatation with stenting of right TS. Following that, vision improved (6/18 both eyes) with the resolution of papilledema. After 6 months, she had the recurrence of symptoms and papilledema. DSA showed a DAVM near right TS which resolved on glue embolization. On follow‑up of 4 years, the patient is asymptomatic with the vision of 6/18 both eyes. Case 4 A 35‑year old female patient who had undergone stenting for right TS thrombosis outside presented with right‑sided headache, transient obscuration of vision and tinnitus for 4 months. On examination, BCVA of 6/18 both eyes with chronic papilledema was noticed. DSA indicated DAVM near left TS [Fig. 3a] with enlarged cortical veins with partially thrombosed sinuses [Fig. 3b] which was embolized with coils and glue [Fig. 3c]. After embolization, DAVM resolved [Fig. 3d]. The patient is asymptomatic with stable vision on follow‑up of 5 years.

Discussion After a successful endovascular intervention of CVST, occasionally patient may develop DAVM. Houser et al. first reported the development of DAVM in patients with CVST.[8] DAVMs are acquired vascular malformations formed between meningeal arteries and dural veins. The exact mechanism for the genesis of DAVM in patients with CVST is not known but likely to involve multiple factors. It has been proposed that DAVM arises from venous hypertension leading to low perfusion pressure and subsequent cerebral ischemia further

Patients with DAVM may present with ophthalmic features such as the blurring of vision, transient obscuration of vision, rapid fall of vision, and papilledema. In an earlier case report, Houser et al. reported a patient of DAVM presenting with blurred vision, headache, low back pain, and slurred speech after 3 years of TS thrombosis.[10] Another case of DAVM presenting with ophthalmic features developing 1 year after treatment of CVST reported by Micieli et al. recently.[7] Similarly, our cases of CVST presented with various ophthalmic features with symptoms of raised intracranial pressure which resolved after endovascular management. We reported the reappearance of papilledema associated with a headache and blurring of vision associated with raised intracranial pressure which pointed toward the new appearance of vascular thrombosis or a dural malformation such as DAVM which was confirmed on DSA. OCT was an effective tool to document the RNFL thickening in papilledema and monitor the treatment response in the follow‑up period similarly used by Micieli et al.[7]

Conclusion After successful management of CVST, continuous monitoring including ophthalmic evaluation is highly required as these patients may develop rare complications such as DAVM which may further lead to a visual and neurological deficit. 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. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

References 1. Siddiqui FM, Kamal AK. Complications associated with cerebral venous thrombosis. J Pak Med Assoc 2006;56:547‑51. 2. Tsai LK, Jeng JS, Liu HM, Wang HJ, Yip PK. Intracranial dural arteriovenous fistulas with or without cerebral sinus thrombosis: Analysis of 69 patients. J Neurol Neurosurg Psychiatry 2004;75:1639‑41. 3. Gupta A, Periakaruppan A. Intracranial dural arteriovenous fistulas: A Review. Indian J Radiol Imaging 2009;19:43‑8. 4. Nishio A, Ohata K, Tsuchida K, Tsuyuguchi N, Hara M, Komiyama M, et al. Dural arteriovenous fistula involving the superior sagittal sinus following sinus thrombosis – Case report. Neurol Med Chir (Tokyo) 2002;42:217‑20. 5. Chaudhary MY, Sachdev VP, Cho SH, Weitzner I Jr., Puljic S, Huang YP, et al. Dural arteriovenous malformation of the major venous sinuses: An acquired lesion. AJNR Am J Neuroradiol 1982;3:13‑9. 6. Pierot L, Chiras J, Duyckaerts C, Jason M, Martin N. Intracranial dural arteriovenous fistulas and sinus thrombosis. Report of five

[Downloaded free from http://www.ijo.in on Friday, April 20, 2018, IP: 92.1.228.82] Case Reports May 2018 cases. J Neuroradiol 1993;20:9‑18. 7. Micieli JA, Derkatch S, Pereira VM, Margolin EA. Development of dural arteriovenous fistulas after cerebral venous sinus thrombosis. J Neuroophthalmol 2016;36:53‑7.

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9. Wang SS, Li CH, Zhang XJ, Wang RM. Investigation of the mechanism of dural arteriovenous fistula formation induced by high intracranial venous pressure in a rabbit model. BMC Neurosci 2014;15:101.

8. Houser OW, Baker HL Jr., Rhoton AL Jr., Okazaki H. Intracranial dural arteriovenous malformations. Radiology 1972;105:55‑64.

10. Houser OW, Campbell JK, Campbell RJ, Sundt TM Jr. Arteriovenous malformation affecting the transverse dural venous sinus – An acquired lesion. Mayo Clin Proc 1979;54:651‑61.

Optic nerve aplasia: A case series

occasional deviation of the right eye since birth. Examination revealed absent perception of light, pseudoptosis, and total afferent pupillary defect (TAPD) in the right eye. The left eye had vision 20/20 with normal clinical findings.

K Samyukta Sadasivan, Neelam Pawar, Meenakshi Ravindran, Ramakrishnan Rengappa1 Optic nerve aplasia (ONA) is a congenital optic nerve anomaly characterized by the absence of optic nerve head, retinal blood vessels, retinal ganglion cells, and optic nerve fibers in a malformed eye. Clinically, the condition presents with the absence of perception of light, afferent pupillary defect and a fundus appearance of absent optic nerve head, and retinal vessels with associated ocular and nonocular abnormalities. Systemic anomalies have been reported with bilateral ONA, whereas unilateral ONA is seen in otherwise healthy individuals. We report three cases of ONA with varied clinical presentations. Key words: Aplasia of optic nerve, central nervous system anomaly, congenital optic nerve anomaly

Optic nerve aplasia (ONA) is a rare, nonhereditary developmental anomaly characterized by congenital absence of the optic nerve, retinal blood vessels, retinal ganglion cells, and optic nerve fibers in a blind eye causing lifelong visual handicap. Unilateral ONA is generally associated with otherwise normal brain development, whereas bilateral ONA is accompanied by central nervous system (CNS) malformations[1] though variations may exist. Ocular or nonocular abnormalities may coexist. Herein, we report three cases of ONA with varied clinical presentations.

Case Reports Case 1 A 6‑year‑old girl, born to a nonconsanguineous marriage, was brought in with the complaints of apparent small size with Access this article online Quick Response Code:

Website: www.ijo.in DOI: 10.4103/ijo.IJO_1108_17 PMID: ***

Department of Paediatric Ophthalmology and Squint, 1Chief Medical Officer, Aravind Eye Hospital, Tirunelveli, Tamil Nadu, India Correspondence to: Dr. K Samyukta Sadasivan, Fellow in Pediatric and Squint Services, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Tirunelveli ‑ 627 001, Tamil Nadu, India. E‑mail: [email protected] Manuscript received: 20.11.17; Revision accepted: 03.02.18

The right eye had 50 prism diopters exotropia with modified Krimsky. Exophthalmometry by Leudde’s measured 8 mm (right eye), 10 mm (left eye) suggestive of enophthalmos of the right eye [Fig. 1]. Fundus examination of the right eye absence of the optic nerve and retinal chorioretinal atrophic patches and a background retina. The left eye fundus limits.

showed a complete blood vessels with highly tessellated was within normal

Ultrasound B‑scan showed the absence of the optic nerve shadow in the right eye. Computerized tomography imaging of the brain and orbit at the level of the optic nerve showed enophthalmos of the right eye. The anterior segment of the right optic nerve in its intraorbital course was absent, posterior segment appeared as a thin cord. No significant anomaly of the brain was noted. T1 weighted, coronal magnetic resonance imaging (MRI) revealed complete absence of the optic nerve in the right eye in the expected location in the intraconal fat, thus confirming the absence of the orbital part of the optic nerve [Fig. 2]. Case 2 A 5‑year‑old child with unremarkable birth history was brought in with the complaints of having no vision in the left eye which appeared small in size since birth. There was a past history of trauma with stone and ocular surgery in the right eye. On examination, best‑corrected visual acuity in the right eye was 20/40 with Cardiff cards and no perception of light in the left eye. Right eye showed linear macular corneal scar at 11 o’ clock limbus. Iris tissue was deficient from 9 to 11 o’clock hours and the pupil was irregular. Lens was clear and fundus examination was within normal limits. Left eye corneal diameter was 12 mm vertically and 9 mm horizontally. There was partial aniridia from 7 to 2 o’ clock hours [Fig. 3]. Fundus examination revealed the complete absence of the optic nerve and retinal vessels and areas of chorioretinal atrophy in the macula and mid‑peripheral retina. This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: [email protected] Cite this article as: Sadasivan KS, Pawar N, Ravindran M, Rengappa R. Optic nerve aplasia: A case series. Indian J Ophthalmol 2018;66:717-9.