Purpose: We describe a case of a 68-year-old woman who had a biopsy-proven carcinoid of the lung and transient episodes of unilateral and bilateral vision loss presumed to be the result to intermittent serum elevations of vasoactive peptides secondary to an occult carcinoid. Observations: A 68-year-old female with history of carcinoid tumor of the lung presented with multiple recurrent and alternating unilateral and bilateral transient vision loss (TVL) which were increasing in frequency over a period of one month. Ophthalmologic examination was unremarkable. Further investigations were significant for elevated levels of serum chromogranin A. Positron emission tomography (PET) showed no recurrent or metastatic carcinoid tumor until after 11 months of persisting symptoms along with increased serum chromogranin A levels where three carcinoid tumorlets were detected in the left lower pulmonary lobe. Conclusions and importance: Clinicians should be aware that TVL can be a manifestation of the vasoactive secretory products in patients with history of carcinoid tumor.

Introduction This study investigated the effect of pars plana vitrectomy (VIT) versus pars plana vitrectomy combined with radial optic neurotomy (RON) on recent onset non-arteritic anterior ischemic optic neuropathy (NAION). Methods In this prospective interventional case series, individuals with recent-onset NAION, lower than one month and low vision (lower than 20/200) were recruited. Patients randomly underwent either VIT, or RON. Results 34 eyes of 34 patients were included in this study. 10, 9, and 15 eyes were randomly included in VIT, RON, and control groups, respectively. The BCVA of the VIT group improved significantly from 1.84 ± 0.5 logMAR at baseline to 1.29 ± 0.67, 0.93 ± 0.53, and 0.77 ± 0.47 logMAR at 1, 3, and 6 months, respectively (Ps < 0.05). The corresponding values for RON group were 1.73 ± 0.53 logMAR at baseline, which improved to 1.04 ± 0.65, 0.64 ± 0.28, and 0.61 ± 0.26 logMAR at the same follow-up visit times ( P < 0.05). The corresponding values for the control group were 1.6 ± 0.58 log MAR at baseline, which improved to 1.03 ± 0.29, 1.00 ± 0.32, and 0.32 ± 0.83 log MAR at the same follow-up visit times. There was no significant statistical difference in BCVA between the three groups. However, both interventions resulted in statistically significant improvement in mean deviation (MD) of visual field (VF) compared with the control group at the end of 6 months (VIT P = 0.006, RON P = 0.043). RNFLT decreased from baseline 235.3 ± 44.01 to 75.6 ± 17.68 at 1 month in the VIT group ( P < 0.001), from baseline 268.22 ± 65.9 to 76.67 ± 10.59 at 1 month in RON ( P < 0.001), while it decreased from baseline 179.48 ± 39.02 to 112.92 ± 44.51 at 1 month in the control group. Conclusion VIT and RON showed promising results in terms of MD of VF, and optic disc edema resolved faster in these groups compared to the control group in recent onset NAION. A larger sample size study is deemed necessary to generalize the results of this study.

Background Myelin oligodendrocyte glycoprotein-associated disease (MOGAD) has a wide phenotypic expression and should be considered in a differential diagnosis of patients with optic disc edema and increased intracranial pressure because MOGAD can mimic IIH and compressive optic neuropathy. Case presentation A 53-year-old woman with a history of presumed idiopathic intracranial hypertension (“IIH”) presented with new headache and visual loss. She had a BMI of 35.44 kg/m2 and a past medical history significant for depression, hepatitis C, hyperlipidemia, and uterine cancer post-hysterectomy. She had undergone multiple lumboperitoneal shunts for presumed IIH and had a prior pituitary adenoma resection. Her visual acuity was no light perception OD and counting fingers OS. After neuro-ophthalmic consultation, a repeat cranial MRI showed symmetric thin peripheral optic nerve sheath enhancement of the intra-orbital optic nerves OU. Serum MOG antibody was positive at 1:100 and she was treated with intravenous steroids followed by plasma exchange and rituximab. Conclusions This case highlights the importance of considering MOGAD in the differential diagnosis of optic neuropathy. Although likely multifactorial, we believe that the lack of improvement in our case from presumed IIH and despite adequate neurosurgical decompression of a pituitary adenoma with compression of the optic apparatus reflected underlying unrecognized MOGAD. Clinicians should consider repeat imaging of the orbit (in addition to the head) in cases of atypical IIH or compressive optic neuropathy especially when the clinical course or response to therapy is poor or progressive.

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PRÉCIS:Transscleral cyclophotocoagulation (TS-CPC) and endoscopic cyclophotocoagulation (ECP) were effective in reducing intraocular pressure (IOP) and glaucoma medications in childhood glaucoma. OBJECTIVE:To report the outcomes of continuous wave TS-CPC and ECP in childhood glaucoma. MATERIALS AND METHODS:We performed a systematic search of relevant databases. We collected data on age, follow-up duration, type of glaucoma, previous surgical interventions, preoperative and postoperative IOP, preoperative and postoperative number of glaucoma medications, adverse events, number of sessions, and success rates at different time points. The main outcome measures are the amount of IOP and glaucoma medication reduction. RESULTS:We included 17 studies studying 526 patients (658 eyes); 11 evaluated the effectiveness of TS-CPC (268 patients, 337 eyes), 5 evaluated ECP (159 patients, 197 eyes), and one study compared both techniques (56 patients, 72 eyes for TS-CPC vs 43 patients, 52 eyes for ECP). The median duration of follow-up was 28 months in the TS-CPC group and 34.4 months in the ECP group. The mean number of treatment sessions was 1.7 in the TS-CPC and 1.3 in the ECP. In the TS-CPC group, the mean IOP was significantly reduced from 31.2 ± 8 to 20.8 ± 8 mm Hg at the last follow-up ( P < 0.001). The mean number of glaucoma medications was reduced from 2.3 ± 1.3 to 2.2 ± 1.3 ( P = 0.37). In the ECP group, there was also a significant reduction in the mean IOP from 32.9 ± 8 mm Hg with a mean of 1.7 ± 0.7 glaucoma medications to 22.6 ± 9.8 mm Hg ( P < 0.0001) on 1.2 ± 1.1 medications ( P = 0.009) at the last follow-up. CONCLUSION:Both TS-CPC and ECP were effective in reducing the IOP and glaucoma medications in childhood glaucoma. Multiple treatment sessions were required.

Purpose:To compare the outcomes of nonpenetrating deep sclerectomy (NPDS) with and without an expanded polytetrafluoroethylene (e-PTFE) implant combined with phacoemulsification (PE). Design:Interventional case series with concurrent control group. Materials and methods:Patients with medically uncontrolled glaucoma underwent PE nonpenetrating deep sclerectomy (NPDS) and were consecutively divided into a study group receiving an e-PTFE implant and a control group undergoing PE-NPDS. Intraocular pressure (IOP), corrected distance visual acuity (CDVA), and the number of glaucoma medications at 1 day, 1 week, 1 month, 3 months, and 6 months were recorded. Results:A total of 22 eyes of 16 patients underwent PE-NPDS, including 11 eyes receiving an e-PTFE implant and another 11 eyes with no implant. NPDS with spacer achieved successful results in all patients, including eight (72.7%) complete and three (27.3%) qualified success, 6 months, postoperatively. The corresponding values in the control group were 10 (90.9%) and 1 (9.1%), respectively. In the spacer group, mean IOP was decreased from 19.3 ± 2.8 at baseline to 12.1 ± 2.0 mm Hg at month 6 (p < 0.001). Corresponding values for the control group were 18.6 ± 3.4 and 10.6 ± 1.5 mm Hg, respectively (p < 0.001). Mean IOPs were comparable between the study groups at all time points. Implant exposure occurred in one of the patients in the study group. While the implant was extruded, the IOP was medically controlled. Conclusion:Outcomes of PE-NPDS using an e-PTFE implant were comparable to the same surgery without a spacer in the short term. Larger studies with longer follow-ups are needed to determine the efficacy and safety of this new implant. How to cite this article:Hajizadeh M, Meshksar A, Hassanpour K, et al. Expanded Polytetrafluoroethylene Spacer for Nonpenetrating Deep Sclerectomy Combined with Cataract Surgery. J Curr Glaucoma Pract 2024;18(2):51-56.

| The occurrence of corneal ectasia after photorefractive keratectomy is a rare but serious complication of refractive surgery. Possible risk factors are not well assessed, but a probable reason is the failure to detect keratoconus pre-operatively. In this report, we describe a case of corneal ectasia after photorefractive keratectomy in a patient who presented a suspicious tomography pattern preoperatively but had no degenerative alterations associated with pathologic keratoconus, as revealed by in vivo corneal confocal microscopy. We also review eligible case reports of post-photorefractive keratectomy ectasia to find similar characteristics.

A 79-year-old female presented with subacute, painless, progressively worsening central vision in both eyes. Six years prior to presentation in 2016, the patient had acute unilateral loss of vision in the left eye and had a temporal artery biopsy consistent with giant cell arteritis (GCA). She was initially treated with intravenous corticosteroid therapy and subsequently was tapered off oral steroids. She received methotrexate and tocilizumab as steroid-sparing treatments for GCA. Her initial serum C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) were elevated but returned to normal after steroid and immunosuppressive treatment for GCA. Past medical, surgical, social, and family histories were otherwise non-contributory. The patient had stable vision at 20/40 in the right eye and 20/30 in the left eye and an inferior nerve fibre layer (NFL) visual field defect and optic atrophy in the left eye after presumed ischemic optic neuropathy from biopsy-proven GCA in 2016. Her global ocular coherence tomography (OCT) at this time showed a retinal NFL thickness of 114 μm in the right eye and 86 μm in the left eye with mild papillomacular bundle loss in the left eye (Fig. 1A). In 2022, the patient reported a gradual worsening of vision in both eyes over a 3-month follow-up interval. She also had 6 months of chronic diarrhea and was initially diagnosed with irritable bowel syndrome. The patient was referred to the neuro-ophthalmology service at Houston Methodist Hospital. On examination, her corrected visual acuity was now 20/80 in the right eye and 20/40 in the left eye compared with her baseline vision of 20/40 in the right eye and 20/30 in the left eye 4 months prior. The cup-to-disc ratio was 0.5 in both eyes with mild optic atrophy in the left eye and an otherwise normal fundus examination in both eyes. OCT of the peripapillary NFL showed a retinal NFL thickness of 100 μm in the right eye and 80 μm in the left eye with NFL dropout in the papillomacular bundle (temporal) quadrants in both eyes (Fig. 1B). Humphrey visual field (HVF) showed a mean deviation (MD) of –3.69 dB in the right eye and –3.01 dB in the left eye (down from an HVF MD of –3.25 dB in the right eye and –0.83 dB in the left eye 4 months prior). HVF 24-2 showed a new cecocentral scotoma in the right eye and a central scotoma in the left eye (Fig. 2). Serum ESR was 31 mm/h, and CRP was 39.9 mg/L (normal range, <8.0 mg/L). The patient denied headache, scalp tenderness, or temporal artery nodularity. There was no pain. The serum B12 (cobalamin) level was borderline low at 376 pg/mL, and the patient was treated with vitamin B12 supplementation. Serum folate and methylmalonic acid levels were normal, but the serum homocysteine level was slightly elevated at 10.7 μmol/L (normal, <10.4 μmol/L). The patient had no dietary restrictions, previous bariatric surgeries, or other gastrointestinal conditions associated with vitamin B12 deficiency. Gastroenterology work-up for the chronic diarrhea included negative colonoscopy and esophagogastroduodenoscopy with normal mucosa on biopsies. An initial diagnosis of irritable bowel syndrome was made. Abdominal magnetic resonance imaging and enterography revealed multiple targetoid lesions in the right hepatic lobe and a spiculated mesenteric mass with T2 hypo-intensity. A subsequent ultrasound-guided core liver mass biopsy confirmed a moderately differentiated (G2) tumour with positive synaptophysin and chromogranin staining consistent with a metastatic neuroendocrine tumour from a gastrointestinal site (carcinoid).1Cives M Strosberg JR Gastroenteropancreatic neuroendocrine tumors.CA Cancer J Clin. 2018; 68: 471-487Crossref PubMed Scopus (328) Google Scholar Subsequent positron emission tomography staging revealed multiple osseous, left psoas, and hepatic lesions with increased dotatate uptake, consistent with stage IV neuroendocrine cancer (carcinoid).1Cives M Strosberg JR Gastroenteropancreatic neuroendocrine tumors.CA Cancer J Clin. 2018; 68: 471-487Crossref PubMed Scopus (328) Google Scholar Carcinoid syndrome is a rare disorder that commonly arises from an underlying gastrointestinal neuroendocrine tumour (carcinoid). Liver metastasis is a common presentation of carcinoid syndrome because it allows humoral factors to circumvent first-pass metabolism, causing symptoms.2Daskalakis K Karakatsanis A Stalberg P Norlen O Hellman P Clinical signs of fibrosis in small intestinal neuroendocrine tumours.Br J Surg. 2017; 104: 69-75Crossref PubMed Scopus (56) Google Scholar The typical presentation of carcinoid syndrome is attributed to the hormonal effects of carcinoid-secreted serotonin. These symptoms include flushing, diarrhea, abdominal pain, and bronchospasm. Carcinoid syndrome also may cause mesenteric fibrosis from bowel ischemia, resulting in nutritional deficiencies and medically refractory diarrhea.2Daskalakis K Karakatsanis A Stalberg P Norlen O Hellman P Clinical signs of fibrosis in small intestinal neuroendocrine tumours.Br J Surg. 2017; 104: 69-75Crossref PubMed Scopus (56) Google Scholar Previous studies have associated midgut carcinoid with a greater than expected vitamin B12 deficiency in patients after small bowel resection, further supporting the malabsorptive effects of carcinoid.3Lind A Wangberg B Ellegard L Vitamin D and vitamin B12 deficiencies are common in patients with midgut carcinoid (SI-NET).Eur J Clin Nutr. 2016; 70: 990-994Crossref PubMed Scopus (21) Google Scholar In this case, we believe that the central visual loss, central scotoma in both eyes, and papillomacular bundle drop out on OCT in both eyes were due to malabsorption and chronic diarrhea from carcinoid syndrome causing vitamin B12 deficiency despite oral vitamin B12 supplementation and no history of small bowel resection. Three months after parenteral vitamin B12 supplementation, the patient's visual acuity improved to 20/25 in both eyes and the HVF MD was –2.95 dB in the right eye and –2.60 dB in the left eye. Her OCT global NFL thickness measured 103 μm in the right eye and 82 μm in the left eye with residual papillomacular bundle dropout in both eyes. Although our patient had biopsy-proven GCA previously, the patient had been stable after acute corticosteroid therapy and chronic immunosuppressive treatment. Serial serum acute-phase reactants (e.g., ESR and CRP) were normal, and the patient had no new or recurrent symptoms of GCA. GCA-related ischemic optic neuropathy more commonly presents with unilateral central and peripheral visual loss, optic disc swelling, cotton wool spots on the affected fundus, an associated headache, and jaw claudication.4Bajpai V Madan S Beri S Arteritic anterior ischaemic optic neuropathy: an update.Eur J Ophthalmol. 2021; 31 (2818–7)Crossref PubMed Scopus (5) Google Scholar The presentation of bilateral painless central visual loss with papillomacular NFL loss in both eyes was more consistent with toxic or nutritional optic neuropathy than GCA-related ischemic optic neuropathy.5Roda M di Geronimo N Pellegrini M Schiavi C Nutritional optic neuropathies: state of the art and emerging evidence.Nutrients. 2020; 12: 2653-2665Crossref PubMed Scopus (16) Google Scholar After parenteral B12 replacement, the patient had marked improvement in her central scotoma, which further supports her presenting visual field changes as a consequence of her carcinoid rather than a flare of GCA. She continues on somatostatin analogue treatment for metastatic carcinoid, which stabilized her chronic diarrhea, and serial imaging has been stable despite stage IV neuroendocrine cancer. Based on review of the English-language ophthalmic literature, to our knowledge, we believe that this case of carcinoid syndrome presenting with nutritional optic neuropathy from diarrhea is unique. Clinicians should be aware of the typical presentations of GCA-related optic neuropathy versus nutritional optic neuropathy. A normal endoscopic evaluation of the gastrointestinal system does not preclude neoplasm as the cause of diarrhea because carcinoid syndrome results from secretory hormonal effects (e.g., diarrhea) and not direct bowel involvement. The authors have no proprietary or commercial interest in any materials discussed in this article.

Apraxia of lid opening (ALO) is a nonparetic, nonrestrictive lid-opening abnormality that manifests as an inability to open the eyelid on command. The levator palpebra superioris muscle, the third cranial nerve, and the orbicularis oculi (OOc) are all normal in ALO. ALO is believed to be the result of supranuclear involuntary levator palpebrae inhibition versus stimulation (contraction) of the pretarsal OOc muscle. The underlying pathophysiology of ALO is still unknown, but it is associated with progressive supranuclear palsy, multiple system atrophy, parkinsonism, and other extrapyramidal-like syndromes.1Boghen D. Apraxia of lid opening: a review.Neurology. 1997; 48: 1491-1494Crossref PubMed Scopus (89) Google Scholar Multiple sclerosis (MS) is an autoimmune disorder characterized by demyelinating plaques in the CNS. We report a case of eyelid apraxia in a patient with MS described in the neurologic literature only once before.2Cartei M. A case of eyelid apraxia in a subject affected by multiple sclerosis: clinical-pathogenetic considerations.Riv Neurol. 1976; 46: 57-72PubMed Google Scholar To our knowledge, this is the first case of ALO in a patient with MS reported in the English-language ophthalmic literature. A 62-year-old white female presented with intermittent “ptosis.” She had a history of relapsing-remitting MS for 18 years that was treated with teriflunomide. She described her lid abnormality as transient difficulty in opening one or both eyes that would last minutes to days and then improve spontaneously. The passively closed eyelid would occur on awakening and could affect either the left or right eyelid. There was no blepharospasm or hemifacial spasm noted by history or on examination. Passive elevation of the lids manually would resolve the “ptosis” OU. Medications included teriflunomide, pramipexole, baclofen, atenolol, gabapentin, and omeprazole. Her last recorded MS exacerbation was 5 months prior to the neuro-ophthalmic visit. Electromyography and serologic testing for myasthenia gravis were negative. Repeat magnetic resonance imaging of the brain 4 months prior to the initial visit showed multifocal inactive and nonenhancing demyelination, and there were no white matter hyperintensities in the areas involving the brainstem motor nuclei, basal ganglia, or thalamus. Cervical spine magnetic resonance imaging showed multilevel cervical disc degenerative changes. During her neuro-ophthalmology visit, the patient was asymptomatic with a completely normal eye examination. External, lid, and extraocular motility examinations were normal OU. The patient reported that her “ptosis” OU at home would resolve after manually raising the affected eyelid or with active voluntary “lifting of her eyebrows.” The patient took photographs of her “ptosis” that were consistent with ALO (Figs. 1 and 2).Fig. 2Shows the manual eyelid elevation technique performed by the patient to improve the “ptosis.”View Large Image Figure ViewerDownload Hi-res image Download (PPT) ALO is a diagnosis of exclusion, and the levator palprebra superioris, third cranial nerve, and OOc should be normal. Normal lid opening is voluntary and requires supranuclear innervation to both levator muscles via the third nerve nucleus and single caudal subnucleus. Normal voluntary lid closure requires supranuclear input to the OOc. Patients with ALO, as in our case, often can open their eyes following reflex blinking or by certain other facial motor manoeuvres (e.g., massaging the eyelids or manual elevation of the lids). ALO differs from benign essential blepharospasm (BEB), which is also a focal dystonia involving the eyelid that, interestingly, is commonly associated with ALO. BEB is bilateral, and thus ALO in BEB is typically bilateral and supranuclear. Isolated ALO without BEB is much less common.1Boghen D. Apraxia of lid opening: a review.Neurology. 1997; 48: 1491-1494Crossref PubMed Scopus (89) Google Scholar,3Krack P Marion M. Apraxia of lid opening,” a focal eyelid dystonia: clinical study of 32 patients.Mov Disord. 1994; 9: 610-615Crossref PubMed Scopus (134) Google Scholar In some cases, diagnosis of ALO is unmasked with persistence of eye closure after therapeutic botulinum toxin injection into the OOc to treat BEB. Unlike BEB, however, ALO can be treated with botulinum toxin injection into the pretarsal OOc muscle, suggesting that pretarsal stimulation and not levator inhibition alone is the mechanism for ALO. Supranuclear inhibition of the levator in dorsal midbrain syndrome produces bilateral lid retraction (Collier lid retraction sign), and supranuclear stimulation of the unpaired central caudal nucleus of the levator in the third nerve nucleus also would be expected to be bilateral. Unilateral or alternating cases of ALO suggest that the focal dystonia mechanism is with OOc activation rather than central levator inhibition. Systemic extrapyramidal treatments, however, like levodopa have shown limited efficacy in ALO.1Boghen D. Apraxia of lid opening: a review.Neurology. 1997; 48: 1491-1494Crossref PubMed Scopus (89) Google Scholar To our knowledge, there is only 1 other case of apraxia of the eyelid in MS. This case appeared in the Italian neurologic literature—the patient had apraxia of eyelid closure concurrent with MS. That patient also had preserved reflexive blinking, a common finding in both apraxia of eyelid closure and ALO.2Cartei M. A case of eyelid apraxia in a subject affected by multiple sclerosis: clinical-pathogenetic considerations.Riv Neurol. 1976; 46: 57-72PubMed Google Scholar,4Almallouhi E Dale M. Bilateral apraxia of eyelid closure following bilateral subcortical frontal infarcts.J Neurol Sci. 2018; 387: 150-151Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar Some patients develop a form of “sensory trick” that can reverse or block the ALO. One commercial device (PressOp, Dystonia UK, London, UK) can help maintain an elevated eyelid in ALO patients. The mechanism for how manual elevation of the eyebrows or eyelids works to reduce ALO remains ill-defined. The PressOp applies pressure around the periorbit and is adjusted to the right position. Some patients with severe symptoms who fail botulinum toxin and other conservative measures may require surgical treatment (e.g., frontalis suspension surgery).5Dressler D Karapantzou C Rohrbach S Schneider S. Frontalis suspension surgery to treat patients with blepharospasm and eyelid opening apraxia: long-term results.J Neural Transm (Vienna). 2017; 124: 253-257Crossref PubMed Scopus (14) Google Scholar Oculoplastic consultation and information about PressOp were given to our patient, but currently no treatment modality has been pursued. In summary, clinicians should be aware that passive closure of the eyelids can mimic neurogenic ptosis. The inability to voluntarily open the eyelids in the absence of pathology in the eyelid, the levator muscle, the third cranial nerve, or the neuromuscular junction (e.g., myasthenia gravis) suggests ALO. Pretarsal OOc stimulation rather than levator inhibition is a more likely mechanism for ALO cases like ours that are unilateral or alternating because bilateral BEB and bilateral ALO are supranuclear and the levator subnucleus is unpaired (central caudal subnucleus in third nerve nucleus). Botulinum toxin can be used to treat ALO, but myasthenia gravis and true neurogenic ptosis should be excluded prior to administering any agents that could worsen neuromuscular blockade. MS is a very uncommon cause of ALO; the subtleness of ALO makes it often go undetected, presumably more so in the context of an uncharacteristic presentation in conjunction with a relapsing and remitting disease.1Boghen D. Apraxia of lid opening: a review.Neurology. 1997; 48: 1491-1494Crossref PubMed Scopus (89) Google Scholar,3Krack P Marion M. Apraxia of lid opening,” a focal eyelid dystonia: clinical study of 32 patients.Mov Disord. 1994; 9: 610-615Crossref PubMed Scopus (134) Google Scholar An alternating and unilateral presentation of ALO without BEB association may be typical of ALO in MS, and we believe that this case may be useful for further hypothesis generation on the mechanisms for ALO. Unilateral cases might support the hypothesis that stimulation of the pretarsal OOc rather than inhibition of the levator muscle as the mechanism for ALO in some cases. The authors have no proprietary or commercial interest in any materials discussed in this correspondence.

Précis: The effectiveness of Ahmed glaucoma valve (AGV) and Baerveldt glaucoma implant (BGI) was comparable in the management of childhood glaucoma over the long term despite initial better success rate with BGI. There were higher tube block and retraction rates in the BGI group and higher tube exposure rates in the AGV group. Purpose: To evaluate the outcomes and safety of AGV and BGI in childhood glaucoma. Materials and Methods: We performed a systematic literature review of publications from 1990 to 2022 in PubMed, EMBASE, ClinicalTrials.gov, Ovid MEDLINE, Cochrane CENTRAL, and google scholar for studies evaluating AGV and BGI in childhood glaucoma. Primary outcome measures were intraocular pressure (IOP) reduction and glaucoma medication reduction. The secondary outcome measures were the success rates and incidence of postoperative complications. We conducted a meta-analysis using a random effects model. Results: Thirty-two studies met the inclusion criteria. A total of 1480 eyes were included. The mean IOP reduction was 15.08 mm Hg (P < 0.00001) for AGV and 14.62 (P < 0.00001) for the BGI group. The mean difference between pre and postoperative glaucoma medications was 1 (P < 0.00001) fewer medications in the AGV group and 0.95 (P < 0.0001) fewer medications in the BGI group. There was a lower success rate in the AGV versus BGI groups at 2 years [63% vs 83%, respectively (P < 0.0001) and 3 years (43% vs 79%, respectively (P < 0.0001)]; however, the success was higher for AGV at 5 years (63% vs 56% in the BGI group, P < 0.001). The incidence of postoperative complications was comparable in the AGV and BGI groups, with rates of 28% and 27%, respectively. Conclusions: The IOP and glaucoma medication reduction, success rates, and incidence of postoperative complications were comparable in Ahmed and Baerveldt groups. Most literature comes from retrospective low-quality studies on refractory childhood glaucoma. Further larger cohort studies are needed.

Carrabba, Nicole V. BS; Hummel, Lena A. MD; Pakravan, Mohammad MD, MBA; Charoenkijkajorn, Chaow MD; Lee, Andrew G. MDEditor(s): Avery, Robert DO; Golnik, Karl C. MD; Froment, Caroline MD, PhD; Wang, An-Guor MD Author Information

The symptoms and signs of hydroxychloroquine (HCQ) retinopathy can mimic retinitis pigmentosa (RP) and both present with painless, progressive bilateral visual loss and clinical, optical coherence tomography (OCT), and electrophysiologic evidence for retinal dysfunction. We report a case of HCQ use unmasking an occult RP carrier of 2 known associated mutations. This unique presentation emphasizes the importance of consideration for genetic predisposition in atypical cases of HCQ toxicity. A 67-year-old black female had painless progressive bilateral visual loss and night blindness for 1 year. She had systemic lupus erythematosus (SLE), prediabetes, hypertension, hyperlipidemia, and osteoporosis. Past ocular history was significant for cataract extraction in both eyes. She had prior hand surgery. Her family history was negative for glaucoma or RP. She had been treated for SLE with HCQ (Plaquenil, Concordia Pharmaceuticals Inc. St. Michael, Barbados BB11005) at a dose of 400 mg daily (approximately 5.8 mg/kg daily) for 13 years, producing a cumulative dose of 1899 g. Serial outside ocular examinations including automated perimetry with Humphrey visual field (HVF) testing and OCT were normal, including testing 2 years prior to presentation to the neuro-ophthalmology service at Houston Methodist Hospital. At the time of the visual loss, the HCQ was discontinued, and the patient was switched to azathioprine for her SLE management. Her other medications included atenolol, gabapentin, losartan, potassium, and meloxicam. Neuro-ophthalmic examination 10 months after discontinuation of the HCQ showed visual acuity of 20/20 bilaterally, there was no relative afferent pupillary defect, colour plates were 14/14 bilaterally, and slit-lamp examination of the anterior chamber was normal bilaterally. Intraocular pressure was 14 mm Hg OD and 12 mm Hg OS, with cup-to-disc ratios of 0.5 OD and 0.4 OS. Fundus examination was significant for moderate arteriolar attenuation and bone spicule–like pigmentation of the peripheral retinas OU (Fig. 1A; see also Supplementary Fig. 1, available online). HVF testing showed markedly decreased peripheral visual fields to central islands OU (Fig. 1C; see also Supplementary Fig. 1, available online). OCT optic nerve global retinal nerve fibre layer thickness was 76 μm OD and 75 μm OS. Macular OCT showed ellipsoid zone disruption (not present on OCT performed 3 years prior) consistent with HCQ toxicity (Fig. 1B; see also Supplementary Fig. 1, available online). Laboratory studies including erythrocyte sedimentation rate, C-reactive protein, interferon gamma release assay for tuberculosis, paraneoplastic antibody, syphilis serology, and chest x-ray were negative. The patient's creatinine was elevated at 1.11 mg/dL with a low estimated glomerular filtration rate of 52 mL/min/1.73 m2. Computed tomography and magnetic resonance imaging of the brain were normal. Full-field electroretinogram (ERG) tracings showed essentially extinguished scotopic and photopic responses and revealed diffuse functional impairment of peripheral photoreceptors (rods and cones) and inner nuclear layers (bipolar cells and Mueller cells) OU (Fig. 1D; see also Supplementary Fig. 1, available online). Genetic testing panel for RP showed heterozygosity in the CRB1 gene for the sequence variant designated c.2506C>A. This gene has been reported in patients with autosomal recessive forms of RP.1Bujakowska K Audo I Mohand-Saïd S et al.CRB1 mutations in inherited retinal dystrophies.Hum Mutat. 2012; 33: 306-315Crossref PubMed Scopus (141) Google Scholar The patient also was found to be heterozygous at the RDH12 gene for sequence variant c.542G>A. Known pathogenic variants in RDH12 have been associated with autosomal recessive Leber congenital amaurosis and autosomal dominant and autosomal recessive RP.2Mackay DS Dev Borman A Moradi P et al.RDH12 retinopathy: novel mutations and phenotypic description.Mol Vis. 2011; 17: 2706-2716PubMed Google Scholar At the patient's last 2-year follow-up in the neuro-ophthalmology clinic, her vision and visual field were unchanged. RP includes a heterogeneous group of inherited retinal disorders (syndromic and nonsyndromic) that cause painless progressive visual loss due to degeneration of retinal photoreceptors. The disorder affects both rods and cones, but rod-predominant and cone-predominant forms of RP exist. Typical symptoms of RP include peripheral visual field loss and night blindness OU, and characteristic fundus findings develop over time (e.g., retinal arteriolar narrowing, bone spicule formation), but retinal abnormalities may be subtle in some cases (RP sine pigmento). Pericentral forms of RP can produce paracentral rather than peripheral visual field loss OU. Autosomal dominant, autosomal recessive, and X-linked inheritance patterns occur in RP. This patient had 2 predisposing genetic mutations associated with RP and was previously asymptomatic and had normal ocular examinations prior to and even during initial treatment with HCQ. She had several risk factors for HCQ toxicity, however, including duration (>5 years of use), daily dosing (>5 mg/kg daily dosing per absolute body weight), compromised renal function,3Marmor MF Kellner U Lai TY Melles RB Mieler WF. American Academy of Ophthalmology recommendations on screening for chloroquine and hydroxychloroquine retinopathy (2016 Revision).Ophthalmology. 2016; 123: 1386-1394Abstract Full Text Full Text PDF PubMed Scopus (748) Google Scholar and cumulative dose (threshold >1000 g). The typical ring scotoma of HVF loss in this case, however, was associated with retinal pigment epithelial change and bone spicule formation, which is not a feature of typical HCQ toxicity, which characteristically produces a “bull's eye” maculopathy. There are prior cases of severe HCQ toxicity mimicking RP with more typical diffuse retinal degeneration and reduced full-field ERG, suggesting that retinal dystrophy may predispose to HCQ toxicity.4Marmor M. Retinitis pigmentosa or severe hydroychloroquine retinopathy?.Invest Ophthalmol Vis Sci. 2016; 57: 157Google Scholar Both late-stage HCQ toxicity and late-stage RP may show diminished scotopic and photopic responses on ERG. ERG alone, however, will not differentiate between late HCQ toxicity and RP. In such cases, we recommend genetic testing for RP. Interestingly, ongoing clinical trials are investigating HCQ as a proposed treatment for an autosomal dominant form of RP.5Oral hydroxychloroquine (HCQ) for retinitis pigmentosa caused by P23H- rhodopsin (RHO) [Internet]. ClinicalTrials.gov identifier: NCT04120883. Updated February 15, 2022. Available at: https://clinicaltrials.gov/ct2/show/NCT04120883 (accessed February 24, 2022).Google Scholar We reviewed the PubMed and Google Scholar databases and identified 3 other cases of HCQ toxicity observed in patients with RP (Table 1; see also Supplementary Table 1, available online). Our patient's case of HCQ toxicity unmasking an occult autosomal recessive carrier state of RP is unique in the literature because of the presence of 2 known associated mutations that are presumed to be de novo given the patient's negative family history. Current guidelines for HCQ screening define prior macular disease as a risk factor for toxicity, and although RP is not explicitly listed as a risk factor, patients with unusual peripheral visual field loss or night blindness symptoms should be evaluated for underlying retinopathy.Table 1Reported cases of hydroxychloroquine toxicity in patients with retinitis pigmentosaCaseStudyYearPatient detailsDuration of HCQ use, yCumulative dose of HCQ, gOcular signs and symptoms at presentationClinical indication for HCQ useRP-associated genetic mutation(s) identified1Marmor et al.4Marmor M. Retinitis pigmentosa or severe hydroychloroquine retinopathy?.Invest Ophthalmol Vis Sci. 2016; 57: 157Google Scholar2016UnknownUnknownUnknownUnknownUnknownGenetic testing not performed2Katsman et al.6Katsman D Sanfilippo C Sarraf D. Panretinal degeneration associated with long-term hydroxychloroquine use and heterozygous USH2A mutation.Retin Cases Brief Rep. 2017; 11: S77-S80Crossref PubMed Scopus (7) Google Scholar201739-year-old female202774 (400 mg/kg/d)Decreased VA, nyctalopia, peripheral vision lossSLEUSH2A gene heterozygous mutation3Patel et al.7Patel P Jones K Friedman DI Birch DG Ufret-Vincenty RL. Unexpected etiology in a case of bilateral maculopathy.Case Rep Ophthalmol. 2021; 12: 622-628Crossref PubMed Scopus (2) Google Scholar202174-year-old female2.5132.5 (200 mg/d)Nyctalopia, pericentral vision lossRAHGSNAT gene homozygous missense variant c.1843G>A4This study202267-year-old female131899 (400 mg/kg/d)Nyctalopia, peripheral vision lossSLECRB1 gene heterozygous for sequence variant c.2506C>A;RDH12 gene heterozygous for sequence variant c.542G>AHCQ, hydroxychloroquine; RP, retinitis pigmentosa; VA, visual acuity; SLE, systemic lupus erythematosus; RA, rheumatoid arthritis. Open table in a new tab HCQ, hydroxychloroquine; RP, retinitis pigmentosa; VA, visual acuity; SLE, systemic lupus erythematosus; RA, rheumatoid arthritis. The authors have no proprietary or commercial interest in any materials discussed in this correspondence. Download .docx (.09 MB) Help with docx files Download .docx (.02 MB) Help with docx files

This review discusses the physical examination and diagnostic tests necessary to diagnose optic neuritis (ON) and provides an update on the approach and management of acute ON. A comprehensive search of the PubMed database was conducted, limited to English-language journals and recent publications. A total of 160 articles were initially screened by title, of which 73 articles were included in the narrative synthesis. ON is an inflammation of the optic nerve that can be caused by different systemic and neurological disorders. It is commonly presented as a subacute unilateral painful vision loss, and based on its clinical manifestation, it can be classified as typical or atypical. Atypical ON is bilateral with visual acuity of worse than 20/200 or has an atypical demographic presentation for demyelination, such as a non-Caucasian male with optic disc swelling, for which neuromyelitis optica spectrum disorder (NMOSD), myelin-oligodendrocyte glycoprotein antibody-associated disease (MOGAD), or other etiologies should be considered. Steroids and immunosuppressants are the main treatment options for ON, and timely treatment initiation is critical to preventing irreversible vision loss, especially in atypical cases.

Optic nerve sheath meningioma (ONSM) is typically a slowly growing benign tumour originating from the arachnoid layer surrounding the optic nerve. ONSM often grows circumferentially around the optic nerve within the sheath, producing a gradual, progressive, ipsilateral optic neuropathy.1Patel BC, De Jesus O, Margolin E. Optic nerve sheath meningioma [Internet]. National Institutes of Health Optic Nerve Sheath Meningioma Web Site [2022 may 24]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK430868/(accessed October 14, 2022).Google Scholar ONSM occurs most often in middle-aged females and can have tumour hormonal receptors that may account for the female predilection and tumour growth during pregnancy.1Patel BC, De Jesus O, Margolin E. Optic nerve sheath meningioma [Internet]. National Institutes of Health Optic Nerve Sheath Meningioma Web Site [2022 may 24]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK430868/(accessed October 14, 2022).Google Scholar We describe an ONSM that presented with acute, rapidly progressive optic neuropathy following hormonal fertility therapy. Based on our review of the English-language, ophthalmic literature, we believe that our case is novel and unique. A 39-year-old gravida 5 para 4 woman presented with a 1-month history of acute, painless, and progressive loss of vision in the right eye. Visual acuity was 20/25 bilaterally with a pale optic nerve in the right eye. Magnetic resonance imaging (MRI) of the head and orbit without contrast material reportedly disclosed no abnormalities. The patient was diagnosed with optic neuritis and treated with corticosteroids without improvement. Past medical and surgical histories were significant for ulcerative colitis treated with budesonide and a remote right-sided oophorectomy due to ovarian cyst rupture. The patient had 1 year of in vitro fertilization (IVF) 5 years prior to symptom onset. Five months after the onset of the initial gradual vision loss, the patient underwent additional rounds of IVF. Each round of IVF involved exogenous hormone intake during ovarian stimulation and embryo transfer cycles. Ovarian stimulation cycles followed the long gonadotropin-releasing hormone agonist protocol, which includes multiple days of leuprorelin, follicle-stimulating hormone, human menopausal gonadotropin, and a single trigger shot of human chorionic gonadotropin. Embryo transfer cycles required daily exogenous estradiol and progesterone. Nine months after starting IVF, the patient complained of subacute visual loss in the right eye, and she was referred to an outside neuro-ophthalmologist. Repeat open MRI of the head was unremarkable. One week later, the patient underwent closed MRI with gadolinium, which revealed circumferential enhancement along the right optic nerve and adjacent dura. Given her young age, the rapid progression of symptoms, and her history of ulcerative colitis, this was deemed as likely an inflammatory optic perineuritis, and she was treated empirically with intravenous followed by oral corticosteroids. Initially, she had a steroid-responsive and steroid-dependent course, but the patient's symptoms continued to progress. Because of her autoimmune history, initial response to steroids, acute change in vision, and prior negative MRIs, it was not clear whether this was a case of optic perineuritis or optic nerve sheath meningioma without a biopsy. A right-sided exploratory craniotomy confirmed a tumour wrapping around the ophthalmic artery and optic nerve in the suprachiasmatic region of the right eye. The pathology showed World Health Organization (WHO) grade I meningioma. Tumour receptor testing was positive for progesterone receptors. The vision 6 days postoperatively worsened to light perception in the right eye despite high-dose corticosteroid therapy. The patient also received a left-sided oophorectomy a few weeks later for ovarian cysts. Two months after craniotomy, the patient was referred to the Houston Methodist Hospital Neuro-Ophthalmology Department. On examination, her visual acuity was light perception in the right eye with a relative afferent pupillary defect in the right eye. The optic nerve was pale with residual disc edema in the right eye. The eye examination was unremarkable in the left eye. MRI of the brain and orbits with contrast material and fat suppression at this time revealed diffuse posterior orbital optic nerve sheath enhancement consistent with an ONSM (Fig. 1). The patient was treated with external-beam stereotactic fractionated conformal radiotherapy, but her vision remained light perception in the right eye and 20/20 in the left eye. Because the patient was status post bilateral sequential oophorectomy and there was concern for hormonal involvement in ONSM growth, she chose not to pursue further pregnancy. ONSM is typically characterized by slow, painless, and progressive vision loss of the affected eye. Many authors believe that the classic neuroimaging features of ONSM obviate the need for histopathologic confirmation. Observation is an acceptable initial management option for patients with stable visual function, but symptomatic and progressive ONSM is typically treated with stereotactic external-beam fractionated conformal radiotherapy. Surgical resection of the tumour is generally not recommended because damage to the optic nerve and (or) blood supply leads to worsening visual loss.1Patel BC, De Jesus O, Margolin E. Optic nerve sheath meningioma [Internet]. National Institutes of Health Optic Nerve Sheath Meningioma Web Site [2022 may 24]. Available at: https://www.ncbi.nlm.nih.gov/books/NBK430868/(accessed October 14, 2022).Google Scholar In this case, an exploratory craniotomy was done at an outside hospital because of the patient's conflicting presentation. The prior ulcerative colitis, acute change in vision, prior negative MRIs, and partial steroid-responsive and steroid-dependent course led to the initial misdiagnosis of inflammatory optic perineuritis. Although rapid progression of meningioma can occur in higher WHO grade (II or III) tumours (e.g., atypical, invasive, anaplastic, or aggressive pathologic subtypes), our case was WHO grade I. Meningioma has a higher prevalence in females, and these tumours may grow and regress with pregnancy, childbirth, and hormonal therapy.2Bickerstaff ER Small JM Guest IVA The relapsing course of certain meningiomas in relation to pregnancy and menstruation.J Neurol Neurosurg Psychiatry. 1958; 21: 89-91Crossref PubMed Scopus (208) Google Scholar3Benson VS Pirie K Green J et al.Hormone replacement therapy and incidence of central nervous system tumours in the Million Women Study.Int J Cancer. 2010; 127: 1692-1698Crossref PubMed Scopus (56) Google Scholar An increased relative risk of meningioma has been described after hormone-replacement therapy.4Wigertz A Lönn S Mathiesen T Ahlbom A Hall P Feychting M Risk of brain tumors associated with exposure to exogenous female sex hormones.Am J Epidemiol. 2006; 164: 629-636Crossref PubMed Scopus (125) Google Scholar These aforementioned characteristics suggest that meningiomas may be directly influenced by estrogen and (or) progesterone, particularly when considering that about two thirds of meningiomas have progesterone receptors.4Wigertz A Lönn S Mathiesen T Ahlbom A Hall P Feychting M Risk of brain tumors associated with exposure to exogenous female sex hormones.Am J Epidemiol. 2006; 164: 629-636Crossref PubMed Scopus (125) Google Scholar Our patient received two rounds of IVF treatments prior to her acute worsening of symptoms and 2 additional rounds of IVF after diagnosis. We hypothesize that the rapid growth of the ONSM in this case may have been related to IVF treatment given the progesterone receptors on the tumour.5Claus EB Calvocoressi L Bondy ML Wrensch M Wiemels JL Schildkraut JM. Exogenous hormone use, reproductive factors, and risk of intracranial meningioma in females.J Neurosurg. 2013; 118: 649-656Crossref PubMed Scopus (91) Google Scholar Although antiprogesterone agents have been explored as a treatment option for ONSM, studies have been small with nondefinitive results.6Ji Y Rankin C Grunberg S et al.Double-blind phase III randomized trial of the antiprogestin agent mifepristone in the treatment of unresectable meningioma: SWOG S9005.J Clinl Oncol. 2015; 33: 4093-4098Crossref PubMed Scopus (106) Google Scholar We elected to proceed with conventional fractionated radiation therapy, which has shown effective and tolerable outcomes.7Turbin RE Thompson CR Kennerdell JS Cockerham KP Kupersmith MJ A long-term visual outcome comparison in patients with optic nerve sheath meningioma managed with observation, surgery, radiotherapy, or surgery and radiotherapy.Ophthalmology. 2002; 109: 890-899Abstract Full Text Full Text PDF PubMed Scopus (192) Google Scholar Clinicians should be aware of the clinical and radiographic features of typical and atypical ONSM as well as the high risk of vision loss after surgical intervention. The risk of exogenous and endogenous hormones in ONSM (especially in progesterone- or estrogen-receptor-positive meningiomas) remains ill-defined. The authors have no proprietary or commercial interest in any materials discussed in this correspondence.

Purpose: To investigate the efficacy of capsulectomy shunt revision (CSR) compared with the implantation of a second Ahmed glaucoma valve (re-AGV) in glaucoma patients with failed shunts.Design: Quasi-experimental study.Subjects: Forty-six eyes with failed Ahmed glaucoma valves (AGVs) were included in the study; 25 underwent CSR, whereas 21 underwent re-AGV.Methods: Patients were scheduled for CSR or re-AGV based on the appearance and accessibility of the existing AGV versus the feasibility for re-AGV in other quadrants. The CSR involved incision and dissection down to the thick fibrous capsule around the AGV plate, which was excised extensively. For re-AGV, the second shunt was implanted in the supranasal or infranasal quadrants.Main Outcome Measures: Surgical success, defined as intraocular pressure (IOP) > 5 mmHg, < 21 mmHg, IOP reduction > 20% from baseline, and no reoperation for glaucoma. Secondary outcome measures were IOP, number of glaucoma medications, and complications during a 12-month follow-up period.Results: Mean IOP was significantly lower than preoperative values at all time points in both study groups (P < 0.001). Intraocular pressure decreased significantly from 28.3 & PLUSMN; 5.04 mmHg at baseline to 16.4 & PLUSMN; 2.4 mmHg at final follow-up in the capsulectomy group (P = 0.002). Corresponding IOP values for re-AGV were 30.99 & PLUSMN; 6.2 and 13.6 & PLUSMN; 3.8 mmHg, respectively (P = 0.001). Intraocular pressure in the CSR group was higher than re-AGV during the study (P = 0.003). The cumulative probability of success at 12 months was significantly higher in the re-AGV group (87.5% vs 53.3%, P = 0.002). There was no significant difference in the number of glaucoma medications and overall complications rate between the study groups. Wound leakage was the only complication more common in the CSR group (P = 0.012). Conclusion: In the eyes with a failed AGV, re-AGV and CSR are both effective. Implantation of a second shunt seems more effective than the surgical revision of an existing device; however, the latter procedure may be a viable option in selected cases.Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article. Ophthalmology Glaucoma 2023;6:325-331 2022 by the American Academy of Ophthalmology

Noubani, Lema H. BS; Hummel, Lena A. MD; Pakravan, Mohammad MD, MBA; Charoenkijkajorn, Chaow MD; Mortensen, Peter W. MD; Lee, Andrew G. MDEditor(s): Avery, Robert DO; Golnik, Karl C. MD; Froment, Caroline MD, PhD; Wang, An-Guor MD Author Information

Gupta, Akash BA; Pakravan, Mohammad MD, MBA; Charoenkijkajorn, Chaow MD; Lee, Andrew G. MDEditor(s): Avery, Robert DO; Golnik, Karl C. MD; Froment, Caroline MD, PhD; Wang, An-Guor MD Author Information

A carotid-cavernous fistula (CCF) is an abnormal arteriovenous communication between the carotid artery system and the cavernous sinus. Common ocular manifestations of CCFs include pulsating exophthalmos, conjunctival chemosis, periorbital bruit, diplopia, and ophthalmoplegia.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar We report a patient with atypical CCF who presented with audible blinks and describe the possible pathogenesis of this phenomenon. A 54-year-old white female presented with intermittent double vision, eye redness, facial pain, and lid swelling OS with an unusual complaint of audible blinks OS. Her medical history was significant for pituitary hyperplasia status post transsphenoidal resection, stage 0 breast cancer, and a remote history of Bartonella henselae neuroretinitis. Medication, family, and social histories were noncontributory. The patient presented initially with headache and progressive bitemporal visual field loss. Cranial magnetic resonance imaging confirmed an enlarged pituitary lesion compressing the optic chiasm. Transsphenoidal resection and biopsy showed focal fibrosis of the adenohypophysis. Postoperative imaging was negative for recurrence, and her symptoms and signs resolved. Four months later, the patient presented with an unusual audible clicking sound with blinking, intermittent diplopia, dry eye, and lid swelling OS that would decrease during the day and after sleeping upright. Visual acuity was 20/25 OD and 20/40 OS. Motility was full OU without nystagmus. Her monocular horizontal diplopia OS worsened with extreme left gaze only with difficulty realigning eyes returning from the gaze consistent with a preexisting left cranial nerve VI palsy. External examination showed moderate lid edema, mild ptosis, mild lacrimal gland enlargement, and conjunctival chemosis OS. Slit-lamp biomicroscopy showed a mild nuclear sclerotic cataract consistent with 20/40 vision OS. Because the clicking sounds resolved by the appointment, the patient showed a video recording of her blinking that confirmed the symptom of audible blinks that presented on upward and downward excursions of the eyelid OS without a visible air bubble (Video 1, available online). Laboratory studies including tuberculosis, syphilis, IgG subclass levels, pituitary hormones, vitamin B12, and thyroid function studies were unremarkable. Magnetic resonance imaging of the brain and orbits revealed asymmetric prominence of the left cavernous sinus (Fig. 1). Cerebral angiogram revealed a left-sided Cognard type IV dural CCF and right-sided Cognard type I dural CCF (Fig. 2). The patient underwent coil embolization and complete obliteration of the left CCF, which resolved the lid edema and audible blinks OS. At the follow-up visit, her vision remained stable, but she had a postoperative left abduction deficit with worsened diplopia consistent with left cranial nerve VI palsy.Fig. 2Lateral left (A) and frontal (B) cerebral angiograms reveal a left-sided Cognard type IV dural carotid-cavernous fistula.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Direct CCFs have a direct connection between the internal carotid artery and the cavernous sinus, whereas dural CCFs have an indirect connection involving the cavernous sinus and cavernous arterial branches.2Barrow DL Spector RH Braun IF Landman JA Tindall SC Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas.J Neurosurg. 1985; 62: 248-256Crossref PubMed Scopus (845) Google Scholar Cognard classification distinguishes between benign and aggressive types of dural CCFs by cortical venous drainage (CVD), dural sinus drainage, and venous outflow architecture.3Gandhi D Chen J Pearl M Huang J Gemmete JJ Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33: 1007-1013Crossref PubMed Scopus (220) Google Scholar Ocular manifestations of CCFs include pulsatile exophthalmos, chemosis, periorbital bruit, ophthalmoplegia, elevated intraocular pressure, and exposure keratopathy.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar If there is a suspicion for CCF, angiography is the accurate diagnostic method to identify the location of a fistula, draining pattern, flow rate, and reflux.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar,3Gandhi D Chen J Pearl M Huang J Gemmete JJ Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33: 1007-1013Crossref PubMed Scopus (220) Google Scholar Endovascular intervention is recommended for CCFs with high-flow fistulas or CVD because of possible vascular complications.3Gandhi D Chen J Pearl M Huang J Gemmete JJ Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33: 1007-1013Crossref PubMed Scopus (220) Google Scholar Low-risk cases are managed conservatively because some resolve spontaneously.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar,3Gandhi D Chen J Pearl M Huang J Gemmete JJ Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33: 1007-1013Crossref PubMed Scopus (220) Google Scholar However, secondary glaucoma, diplopia, intolerable bruit or headache, exposure keratopathy, or debilitating visual symptoms could suggest an indication for intervention.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar,2Barrow DL Spector RH Braun IF Landman JA Tindall SC Tindall GT. Classification and treatment of spontaneous carotid-cavernous sinus fistulas.J Neurosurg. 1985; 62: 248-256Crossref PubMed Scopus (845) Google Scholar Our patient had a left-sided Cognard type IV dural CCF, which consisted of CVD and venous ectasia; she also had a right-sided Cognard type I dural CCF, which was benign and showed no signs of CVD.3Gandhi D Chen J Pearl M Huang J Gemmete JJ Kathuria S. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment.AJNR Am J Neuroradiol. 2012; 33: 1007-1013Crossref PubMed Scopus (220) Google Scholar We hypothesize that the CCF produced intermittent lid swelling OS. The audible blinks that improved with her upright posture suggest a gravity-dependent phenomenon. Her ocular symptoms and audible blinks could be the result of increased resistance from the retrograde venous drainage into the ophthalmic and facial veins through retrograde flow from the type IV dural CCF, leading to the intraorbital fluid accumulation.1Chaudhry IA Elkhamry SM Al-Rashed W Bosley TM. Carotid cavernous fistula: ophthalmological implications.Middle East Afr J Ophthalmol. 2009; 16: 57-63Crossref PubMed Google Scholar Fluid collection in the dependent supine position and nocturnal lagophthalmos also might be factors in the morning that led to audible blinks and resolved as vascular congestion improved throughout the day. Postoperatively, our patient's audible blinks and lid edema resolved along with resolution of the left CCF. It is not clear why audible blinks are not heard more often, however, in patients with thyroid eye disease or CCF. Interestingly, the only previously reported cases of audible blinks in the literature were described as a side effect of prostaglandin analogue use and the silent sinus syndrome.4Abedi F Chappell A Craig JE. Audible clicking on blinking: an adverse effect of topical prostaglandin analogue medication.Clin Exp Ophthalmol. 2017; 45: 304-306Crossref PubMed Scopus (2) Google Scholar,5Dailey RA Cohen JI. Surgical repair of the silent sinus syndrome.Ophthalmic Plast Reconstr Surg. 1995; 11: 261-268Crossref PubMed Scopus (27) Google Scholar We speculate that enophthalmos could lead to a negative pressure and air entrapment after eyelid closure, producing a clicking sound on eyelid opening and air bubble “bursting.”4Abedi F Chappell A Craig JE. Audible clicking on blinking: an adverse effect of topical prostaglandin analogue medication.Clin Exp Ophthalmol. 2017; 45: 304-306Crossref PubMed Scopus (2) Google Scholar,5Dailey RA Cohen JI. Surgical repair of the silent sinus syndrome.Ophthalmic Plast Reconstr Surg. 1995; 11: 261-268Crossref PubMed Scopus (27) Google Scholar Alternatively, intraorbital fluid rather than air accumulation could have produced the audible blink. To the best of our knowledge, this is the first case of CCF presenting with audible blinks to be described in the English-language literature. Online-only material: This article includes online-only material. Video 1 can be found on the CJO web site at http://pubs.nrc-cnrc.gc.ca/cjo/cjo.html. It is linked to this article in the online contents of the xxx 2022 issue. The authors have no proprietary or commercial interest in any materials discussed in this correspondence. https://www.canadianjournalofophthalmology.ca/cms/asset/061d087e-0fc4-40f5-9b63-adfafbc87b70/mmc1.mp4Loading ... Download .mp4 (1.3 MB) Help with .mp4 files