The Role of Lumbar Drains in the Perioperative Management of Primary Spontaneous Temporal Lobe Encephaloceles and Cerebrospinal Fluid Leaks.

OBJECTIVE: To examine the role of lumbar drains (LDs) in the success of spontaneous temporal cerebrospinal fluid (CSF) leak and encephalocele repair. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary academic health system. PATIENTS: Patients undergoing repair of spontaneous temporal lobe encephaloceles or middle fossa CSF leaks during years 2017 to 2023. INTERVENTIONS: Transmastoid, middle fossa craniotomy, or combination approaches to CSF leak repair. OUTCOME MEASURES: Failure rate, complication rate, length of stay (LOS), readmission. RESULTS: Sixty-nine patients were included, with a combination approach performed in 78.3%, transmastoid in 17.4%, and isolated middle fossa craniotomy in 4.3%. Mean body mass index was 33.2, mean bony defect size width was 6.51 mm, and defect locations included the epitympanum, antrum, mastoid, and petrous apex. Multilayer closure with three or more layers was performed in 87.0%. LD was used in 73.9% of cases for a mean duration 2.27 days and was associated with longer LOS (3.27 vs. 1.56 d, p = 0.006) but not with failure rate, complications, discharge destination, or readmission. Only one major complication occurred as a result of the drain, but low-pressure headache was anecdotally common. CONCLUSIONS: Use of LD in the repair of spontaneous CSF leaks and temporal lobe encephaloceles is associated with longer LOS but not failure rates or other admission-level outcomes.

Use of Soft Cervical Collar Improves Surgeon Ergonomics During Simulated Otologic Surgery.

OBJECTIVE: To determine whether surgeon use of a soft cervical collar during endoscopic and microscopic otologic surgery is feasible and impacts surgeon ergonomics as measured by inertial sensors. STUDY DESIGN: Prospective crossover trial. SETTING: US-based otolaryngology training program. PATIENTS: Otolaryngology residents and fellows. INTERVENTIONS: Therapeutic-use of a soft cervical collar during simulated otologic surgery. MAIN OUTCOME MEASURES: Time spent in high-risk angles of neck and back flexion and extension; average angle of neck flexion, extension, rotation, and lateral bending; validated assessment of neck pain; average daily phone use. RESULTS: Fifteen subjects met criteria for inclusion. Ten of 15 (67%) were male. Seven of 15 (47%) were postgraduate year 1-2. Seven of 15 (47%) reported a history of neck pain. None reported prior spinal steroid injections or surgery. Across all subjects, use of the soft cervical collar significantly reduced time spent in high-risk angles of neck flexion/extension during both endoscopic (56% vs. 35%, p < 0.05) and microscopic (60% vs. 32%, p < 0.05) otologic surgery. There was no effect on back flexion or extension. There was no difference in time spent in high-risk neck or back angles between endoscopic and microscopic surgery. Average angles of neck or back flexion, extension, lateral bending, and rotation were not significantly different for subgroups with more operative experience, increased phone use, perception of good posture, or history of neck pain. CONCLUSIONS: Use of a soft cervical collar during simulated otologic surgery significantly reduced time spent in high-risk neck positions. These data support feasibility of soft collar use during otologic surgery and hold promise for reduction in the high rates of neck pain reported by neurotologists. PROFESSIONAL PRACTICE GAP AND EDUCATIONAL NEED: Improving surgeon ergonomics for otologic surgery. LEARNING OBJECTIVE: To identify a therapeutic intervention to mitigate neck pain in surgeons caused by assumption of high-risk cervical neck flexion and extension. DESIRED RESULT: To demonstrate that use of a readily available soft cervical collar reduces risk of neck pain in otologic surgeons. LEVEL OF EVIDENCE: II. INDICATE IRB OR IACUC: Exempt.

Development and Chinese Cross-Cultural Adaptation of the Cochlear Implant Quality-of-Life Survey.

OBJECTIVE: To conduct the Chinese cross-cultural adaptation of the Cochlear Implant Quality of Life-10 Global (CIQOL-10 Global) instrument. PATIENTS: Bilingual Chinese American cochlear implant users. INTERVENTION: Chinese cross-cultural adaptation of the CIQOL-10 Global. MAIN OUTCOME MEASURE: Description of the process of Chinese cross-cultural adaptation of the CIQOL-10 Global and pilot testing of the Chinese CIQOL-10 Global in the target patient population. RESULTS: The CIQOL-10 Global was cross-culturally adapted into Chinese. Ten participants were recruited for pilot testing. There was wide representation from across the target population in terms of age (mean, 44.8 yr; range, 20.2-80.3 yr), sex (5 were male, 5 were female), education, and socioeconomic factors. All participants were able to easily read, comprehend, and fill out the Chinese CIQOL-10 Global. CONCLUSIONS: The Chinese version of the CIQOL-10 Global is now available to provide an overall assessment of quality of life of Chinese-speaking cochlear implant users.

Temporal changes in endolymphatic hydrops on MRI with or without intervention: A systematic review.

OBJECTIVE: The pathophysiology of Meniere's Disease (MD) involves endolymphatic hydrops (ELH) of the inner ear. Magnetic Resonance Imaging (MRI) has been shown to detect ELH, but changes in ELH have been poorly described using this modality. Our objective was to review MRI-measured changes in ELH over time and after medical and/or surgical intervention in patients with MD. We secondarily aim to associate changes in ELH with changes in MD symptomatology. DATABASES REVIEWED: Medline, Web of Science, and Embase databases. METHODS: A systematic review of articles was performed to identify studies utilizing MRI to measure ELH changes over time, and after medical or surgical treatment. Articles on non-human subjects and without direct measurement of ELH were excluded. RESULTS: Of 532 studies identified, 12 were included, involving 170 patients (mean age 56.3 years). Ten studies were prospective; two were retrospective. Five studies strictly utilized medical means of intervention, four utilized surgical treatments, one utilized both, and two observed temporal changes without treatment. Across all interventions, 72.1 % of patients exhibited the same or worsening ELH on imaging. In studies reporting vertigo outcomes, 95.9 % of patients exhibited improvement after the treatment period. CONCLUSION: Medical and surgical interventions often yield symptomatic relief of vertigo in MD patients despite stable or increasing ELH volume. MRI may have greater clinical utility in diagnosing ELH as opposed to assessing treatment response.

Cochlear Implantation in Adults With Single-Sided Deafness: A Systematic Review and Meta-analysis.

OBJECTIVE: To determine the clinical outcomes of adult patients with single-sided deafness (SSD) undergoing ipsilateral cochlear implantation. DATA SOURCE: An electronic search of Medline and Embase articles. REVIEW METHODS: A systematic review was performed with a search strategy developed by a licensed librarian to identify studies of adult patients with SSD who underwent ipsilateral cochlear implantation. Articles were managed in Covidence and evaluated by 2 independent reviewers. Risk of bias was assessed and data were extracted, including patient demographics, etiology of deafness, duration of deafness, and postoperative change in speech recognition, tinnitus, sound localization, and quality of life (QoL). A meta-analysis was performed, and pooled mean differences were calculated for each outcome of interest via random effects models by each outcome, as well as subgroup analyses by the individual clinical score used. RESULTS: Of 2309 studies identified, 185 full texts were evaluated, and 50 were ultimately included involving 674 patients. Speech perception scores in quiet and noise, tinnitus control, sound localization, and QoL all significantly improved after implantation. Pooled outcomes demonstrated score improvements in speech perception (standardized mean difference [SMD], 2.8 [95% CI, 2.16-3.43]), QoL (SMD, 0.68 [95% CI, 0.45-0.91]), sound localization (SMD, -1.13 [95% CI, -1.68 to -0.57]), and tinnitus score reduction (SMD, -1.32 [95% CI, -1.85 to -0.80]). CONCLUSIONS: Cochlear implantation in adults with SSD results in significant improvements in speech perception, tinnitus control, sound localization, and QoL.

Endoscopic Versus Microscopic Stapedotomy: A Single-Blinded Randomized Control Trial.

OBJECTIVE: To demonstrate non-inferiority of endoscopic stapedotomy to microscopic stapedotomy for the treatment of otosclerosis. STUDY DESIGN: Single-blinded randomized control trial. SETTING: Tertiary, academic otology-neurotology practice. PATIENTS: Adult subjects with a diagnosis of otosclerosis and a preoperative air-bone gap (ABG) more than or equal to 20 dB undergoing primary stapedotomy. INTERVENTION: Endoscopic or microscopic stapedotomy. MAIN OUTCOME MEASURES: Primary audiometric outcome was postoperative ABG. Secondary audiometric outcomes included speech reception threshold (SRT), word recognition score (WRS), bone- and air-conduction pure tone averages (PTA), change in ABG, and ABG closure rates to less than or equal to 10 dB and less than or equal to 20 dB. RESULTS: Twenty-two patients were recruited. Eleven patients underwent endoscopic stapedotomy and 11 underwent microscopic stapedotomy. The endoscopic group was non-inferior to the microscopic group in terms of postoperative audiometric outcomes (endoscope versus microscope, p-value): ABG (8.1 dB versus 8.1 dB, <0.001), SRT (27.7 dB versus 25.9 dB, <0.001), WRS (92% at 65 dB versus 98% at 62 dB, <0.001), air-conduction PTA (33.5 dB versus 30.8 dB, <0.01), and change in ABG (23.0 dB versus 20.7 dB, <0.0001). ABG closure rates to less than or equal to 10 dB (72.7% versus 81.2%, p = 1.0) and less than or equal to 20 dB (90.9% versus 100%, p = 1.0) were not significantly different. There was no significant difference in operative time, necessity of scutum curettage, or postoperative dysgeusia. No patients required chorda tympani sacrifice. Preoperative tinnitus resolved in three patients in each group postoperatively. CONCLUSIONS: This study is the first randomized control trial to demonstrate non-inferiority of endoscopic to microscopic stapedotomy.

Novel Strategies for Evaluating and Improving Plastic Surgery Applicant Selection.

SUMMARY: Residency applicant evaluation and selection is a critical part of developing and maintaining a high-quality plastic surgery residency program. Currently, many programs rely on objective measures such as the United States Medical Licensing Exam scores, number of research publications, grade point average, Alpha Omega Alpha Honor Medical Society status, or a combination of these objective metrics. However, there is a growing body of literature suggesting that the current means of residency applicant evaluation and selection may not be the best predictive factors of future resident success. The aim of this study was to identify nontraditional means of evaluating plastic surgery residency candidates and discuss how these means have been implemented at the authors' institution. After reviewing industry hiring practices, the authors propose that standardized interviewing and personality testing can help evaluate some of the previously intangible parts of an applicant that may play a role in teamwork, commitment, and dedication to patient care.

Open Conjunctival Approach for Sub-Tenon's Xen Gel Stent Placement and Bleb Morphology by Anterior Segment Optical Coherence Tomography.

PRCIS: Sub-Tenon's implantation of the Xen Gel stent resulted in significant intraocular pressure (IOP) lowering along with a low rate of postoperative bleb needling, and a favorable bleb morphology on anterior segment optical coherence tomography (AS-OCT). PURPOSE: The aim was to assess clinical outcomes and bleb morphology following sub-Tenon's implantation of the Xen Gel Stent. METHODS: The medical records of patients who underwent sub-Tenon's Xen Gel Stent implantation with intraoperative mitomycin-C through an open conjunctival approach were reviewed. Postoperative IOP and number of glaucoma medications at 1, 3, 6, 9, and 12 months were assessed. Bleb morphology was analyzed at various timepoints using AS-OCT (Topcon DRI OCT version 1.1.1). RESULTS: Twenty-six eyes were included in the study. Mean age was 69.4±8.0 years. Mean preoperative IOP was 28.1±7.8 mm Hg on an average of 3.5±0.9 glaucoma medications. Mean IOP at postoperative month 12 (n=23 eyes) was 12.9±4.0 mm Hg (P<0.01) on an average of 0.3±0.6 (P<0.01) glaucoma medications. Three eyes (12%) required postoperative needle revision. Bleb morphology in the early postoperative period (≤3 mo) was characterized by multiple small subconjunctival microcysts on AS-OCT. At the intermediate (6 to 12 mo) and long-term (>12 mo) timepoints, reduction in microcysts with multiple internal parallel layers of aqueous flow and a uniform pattern were more frequently noted. All functional blebs were characterized by the presence of a posterior episcleral fluid lake. Failed blebs showed absence of aqueous humor around the distal end of the microshunt. CONCLUSION: Following an open conjunctival approach, sub-Tenon's placement of the Xen Gel Stent with significant IOP lowering was achieved. In eyes with good shunt function, bleb morphology by AS-OCT showed a posterior episcleral fluid lake similar to findings following trabeculectomy.

Opioids Are Infrequently Required following Ambulatory Otologic Surgery.

OBJECTIVE: To determine the frequency with which postoperative opioid prescriptions are required after ambulatory otologic surgery. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary otology-neurotology practice. PATIENTS: Patients (n = 447) given over-the-counter acetaminophen and ibuprofen following ambulatory otologic surgery between July 1, 2018 and June 30, 2020. INTERVENTION: Opioid prescription upon request. MAIN OUTCOME MEASURES: Patient, disease, and surgical variables such as age, sex, past medical history, chronic pain condition, surgical procedure, primary versus (vs.) revision surgery, and endoscopic vs. microscopic approach were examined for relationship to ad hoc opioid prescription rate. RESULTS: Of 370 adult patients (mean age 49.0 yrs, range 18.0-88.5 yrs), 75 (20.3%) were prescribed opioids for postoperative pain, most commonly oxycodone-acetaminophen 5/325 mg. Of 77 pediatric patients (mean age 8.8 yrs, range 0.7-17.9 yrs), 5 (6.5%) were prescribed postoperative opioid analgesia. In the adult population, chronic pain condition, pain medication use at baseline, canal wall up mastoidectomy, tympanoplasty, tympanomeatal flap, bone removal of the mastoid, postauricular incision, and intraoperative microscopy were independent predictors of opioid pain prescription. When controlling for all significant variables, only chronic pain condition remained significant (odds ratio = 3.94; p = 0.0007). In the pediatric population, atresiaplasty, meatoplasty, and conchal cartilage removal were independently associated with opioid prescription, but none remained significant when analyzed in a multivariate linear model. CONCLUSIONS: Pain following ambulatory otologic surgery may be adequately managed with over-the-counter pain medications in the majority of cases. Opioids may be necessary in adults with preexisting pain conditions.

Cochlear Implantation in Meniere's Disease: A Systematic Review and Meta-Analysis.

OBJECTIVES/HYPOTHESIS: Meniere's disease (MD) is a debilitating condition characterized by hearing loss, vertigo, and tinnitus. The objective of this study was to systematically investigate outcomes in MD after cochlear implantation (CoI), with and without labyrinthectomy. STUDY DESIGN: Systematic review and meta-analysis. METHODS: A systematic review of articles in Medline and Embase was performed to identify all studies of patients with MD who underwent CoI. This analysis evaluates outcomes of speech recognition, pure tone audiometry, vertigo, tinnitus, and quality of life. RESULTS: Of 321 studies identified, 37 were included, involving 216 patients. Mean age at implantation was 61.4 years (range 27-85 years) with average length of follow-up at 1.7 years (range 0-9 years). Forty-four (20.4%) patients underwent labyrinthectomy. Meta-analysis demonstrated significant improvements in audiometric outcomes following CoI. There was a statistically significant improvement in Hearing in Noise Test performance, with a mean difference improvement of 44.7 (95% confidence interval [CI] [8.8, 80.6]) at 6 months and 60.1 (95% CI [35.3, 85.0]) at 12 months. The Freiburger Monosyllabic Test (FMT) and Consonant-Nucleus-Consonant (CNC) also improved significantly, with mean difference improvements of 46.2 (95% CI [30.0, 62.4]) for FMT and 19.3 (95% CI [8.1, 30.4]) for CNC. There was a statistically significant decrease in tinnitus, as measured by a mean difference reduction of 48.1 (95% CI [1.0, 95.2]) in the Tinnitus Handicap Index. CONCLUSIONS: CoI with and without simultaneous labyrinthectomy is a viable treatment option for patients with MD, yielding high rates of tinnitus suppression and vertigo control. Post-CoI MD patients demonstrate similar postoperative speech perception outcomes to non-MD implant recipients. Laryngoscope, 131:1845-1854, 2021.

The Effect of Phacoemulsification on Intraocular Pressure in Eyes with Preexisting Glaucoma Drainage Implants.

PURPOSE: To study the effect of phacoemulsification on intraocular pressure (IOP) control in patients with preexisting glaucoma drainage implants (GDIs). DESIGN: Retrospective, observational case series. PARTICIPANTS: A total of 45 patients (51 eyes) with previously placed GDIs who underwent phacoemulsification between January 2013 and March 2018. METHODS: The list of patients was obtained from billing records. Clinical data were retrieved from the corresponding electronic medical records. MAIN OUTCOME MEASURES: Intraocular pressure, number of glaucoma medications before and after phacoemulsification (postoperative day 1, week 1, months 1, 3, 6, 12, 18, and 24), rate of failure (failure defined as IOP >18 mmHg and IOP increase >20% at 2 consecutive visits after month 1, need for additional glaucoma surgery, or loss of light perception vision), and postoperative complications. RESULTS: Mean follow-up was 23.0 ± 11.1 months. The average interval between GDI surgery and phacoemulsification was 9.4 ± 6.7 months. An Ahmed glaucoma valve (AGV; New World Medical) was implanted in 12 eyes, a Baerveldt glaucoma implant (BGI; Johnson & Johnson Surgical Vision) was implanted in 36 eyes, and a Molteno (Molteno Ophthalmic Limited) glaucoma implant was implanted in 3 eyes. Before phacoemulsification, the mean IOP was 14.4 ± 4.4 mmHg on 2.1 ± 1.3 glaucoma medications. At postoperative month 24, the mean IOP was 12.6 ± 4.4 mmHg (n = 29, P = 0.519) on 2.0 ± 1.6 (P = 0.457) glaucoma medications. The reduction in IOP was significant only at postoperative week 1 (P = 0.031). The cumulative failure rate was 3.9% at 1 year and 11.8% at 2 years. The AGV group had a significantly higher mean IOP before phacoemulsification than the BGI group (P = 0.016). Analysis of covariance, taking the baseline IOP as a covariate, revealed no differences in postoperative IOP and number of glaucoma medications between groups, except for month 18 (1 patient in the BGI group had uncontrolled IOP requiring surgery). Postoperative complications included cystoid macular edema (10%), corneal decompensation (6%), and choroidal effusion (4%). CONCLUSIONS: Phacoemulsification after GDI surgery resulted in a transient reduction in IOP at postoperative week 1. Patients with previously placed AGVs had similar postoperative outcomes compared with those with BGIs.

Downsizing a Baerveldt Glaucoma Implant For the Management of Persistent Postoperative Hypotony: A Case Series.

PURPOSE: The purpose of this study was to describe a surgical technique for treating persistent hypotony after Baerveldt glaucoma implant (BGI) surgery. MATERIALS AND METHODS: The medical records of 10 patients with persistent postoperative hypotony who underwent truncation of one or both wings of a previously placed BGI, combined with external ligation of the tube using a polypropylene suture, were retrospectively reviewed. RESULTS: All 10 eyes that underwent BGI truncation and placement of a single, external, nonabsorbable (polypropylene) tube ligature exhibited resolution of hypotony within 24 hours and resolution of choroidal effusions within the first 2 postoperative weeks. The median time interval between primary BGI surgery and truncation was 5 months (range, 1.5 mo to 8 y). Median postrevision follow-up time was 12 months (range, 5 mo to 16.2 y). The mean preoperative intraocular pressure (IOP) was 2.1±1.0 mm Hg, and the mean IOP rose to 29.2±13.9 mm Hg on postoperative day 1. Mean IOP at week 1, month 1, and month 3 was 20.5±10.4, 19.7±11.8, and 18.0±8.2 mm Hg, respectively, using an average of 1.4±1.4 glaucoma medications at postoperative month 3. Ligature release after BGI revision was performed in 9 (90%) of the 10 patients. The median time to ligature release was 1.5 months (range, 3 wk to 4 y). There was no recurrence of hypotony in any of these patients. At most recent follow-up, the mean IOP was 12.9±6.0 mm Hg on an average of 1.5±1.3 glaucoma medications. Five patients demonstrated improvement in visual acuity from their prerevision best-corrected visual acuity. CONCLUSIONS: Truncation of one or both wings of a BGI and complete closure of the tube with nonabsorbable, but releasable, suture ligature is an effective and safe method for reversing persistent postoperative hypotony while maintaining IOP control.

IgG4-Related Ophthalmic Disease Presenting as Meningitis and Panuveitis.

PURPOSE: We report an uncommon case of immunoglobulin gamma 4-related ophthalmic disease (IgG4-ROD) presenting as meningitis and panuveitis. OBSERVATIONS: A 35-year-old male with no prior ophthalmic history presented with headaches, altered mental status, and fever of unknown origin. A lumbar puncture (LP) revealed an elevated white count with lymphocytic predominance, confirming a suspected meningitis. After an extensive work-up, he was discharged on oral acyclovir to cover for presumed aseptic meningitis. The patient initially improved, however, bilateral eye pain, redness, and photophobia 2 weeks after discharge prompted his first visit to the ophthalmology clinic. Exam at that time was consistent with bilateral anterior uveitis for which he was given topical prednisolone and cyclopentolate. In addition to the preceding work-up, quantitative immunoglobulin serology including IgG4 levels was added. At follow-up, he was found to have increased ocular inflammation with vitreitis, nerve head edema, and subclinical macular thickening. Visual acuity (VA) had decreased in both eyes. Serology titers for IgG had resulted in a significant elevation in IgG subclass 4 (IgG4). Optical coherence tomography (OCT) and fundus fluorescein angiography (FFA) confirmed posterior retinal involvement. The patient was diagnosed with presumed bilateral panuveitis secondary to IgG4-ROD. CONCLUSIONS AND IMPORTANCE: IgG4-RD can be a serious condition that requires careful consideration and intuition to diagnose. This report serves to encourage ophthalmologists to consider IgG4-ROD in cases of idiopathic systemic inflammation with ophthalmic involvement.

An Examination of the Frequency of Paravascular Defects and Epiretinal Membranes in Eyes With Early Glaucoma Using En-face Slab OCT Images.

PURPOSE: To examine the frequency of paravascular defects (PDs) and macular epiretinal membranes (ERMs) in eyes categorized as having mild glaucoma or glaucoma suspect using en-face slab analysis of optical coherence tomography (OCT) scans. MATERIALS AND METHODS: Fifty-seven glaucomatous eyes, 44 low-risk suspect eyes, and 101 healthy control eyes were included in the study. The 101 glaucomatous and suspect eyes had a mean deviation better than -6 dB on the 24-2 visual field, and a spherical refractive error between±6 D or axial length <26.5 mm. Two OCT-graders masked to eye classification identified ERMs and PDs on en-face slab images of the macula and peripapillary retina using horizontal B-scans and derived vertical B-scans. RESULTS: Glaucomatous eyes had a significantly higher number of PDs and ERMs than healthy controls (PD, P<0.001; ERM, P=0.046) and low-risk glaucoma suspects (PD, P=0.004; ERM, P=0.043). PDs and/or ERMs were present in 16 of 57 (28.1%) glaucomatous eyes, 2 of 44 (4.5%) suspect eyes, and 3 of 101 (3.0%) control eyes. Further, PDs were present in 11 of the 57 (19.3%) glaucomatous eyes, 1 of the 44 (2.3%) suspect eyes and 0 of the 101 (0%) control eyes, ERMs were seen in 7 of the 57 (12.3%) glaucomatous eyes, 1 of the 44 (2.3%) suspects, and 3 of the 101 (3.0%) control eyes. CONCLUSIONS: Eyes with early glaucoma have a higher frequency of PDs and ERMs than suspects or controls and exhibit PDs even in the absence of ERMs or high myopia.

Daratumumab-induced transient myopic shift.

PURPOSE: To describe an unprecedented case of transient myopic shift induced by a chemotherapeutic agent, daratumumab. OBSERVATIONS: A 43-year-old emmetropic female with multiple myeloma experienced sudden onset of myopic shift during her first intravenous dose of daratumumab, an increasingly common FDA-approved chemotherapeutic agent. Her myopia was corrected with -4D lenses in both eyes, and the patient reports cessation of symptoms and disuse of lenses after two days. CONCLUSIONS AND IMPORTANCE: A number of medications have been documented to induce transitory myopic shift, and this report now includes daratumumab among such agents. Further clinical findings regarding the mechanism and frequency of daratumumab-induced myopic shift are needed to further develop our understanding of its tangential effect on the eye.

Aicardi syndrome in a 20-year-old female.

PURPOSE: To describe an unusual case of Aicardi Syndrome that both affirms hallmark characteristics of the condition and introduces new observations. OBSERVATIONS: We report the case of a 20-year-old woman with Aicardi Syndrome who presented in respiratory distress with bradycardia and died soon thereafter. She had a history of severe mental retardation, seizure disorder, advanced scoliosis and numerous contractures in addition to congenital ocular malformations resulting in bilateral blindness. The case is notable for her age and longevity, as most patients with Aicardi Syndrome expire much younger, as well as the presence of intact nuclei under the posterior lens capsule. CONCLUSIONS AND IMPORTANCE: Aicardi Syndrome is a rare X-linked cerebro-retinal disorder typified by seizures, agenesis of the corpus callosum, and chorioretinal lacunae. Documenting alterations from and additions to this triad of symptoms is critical to better understanding both the syndrome itself, as well as the full breadth of its clinical impact on the patient.

Deep Defects Seen on Visual Fields Spatially Correspond Well to Loss of Retinal Nerve Fiber Layer Seen on Circumpapillary OCT Scans.

Purpose: To examine the structure-function relationship in glaucoma between deep defects on visual fields (VF) and deep losses in the circumpapillary retinal nerve fiber layer (cpRNFL) on optical coherence tomography (OCT) circle scans. Methods: Thirty two glaucomatous eyes with deep VF defects, as defined by at least one test location worse than ≤ -15 dB on the 10-2 and/or 24-2 VF pattern deviation (PD) plots, were included from 87 eyes with "early" glaucoma (i.e., 24-2 mean deviation better than -6 dB). Using the location of the deep VF points and a schematic model, the location of local damage on an OCT circle scan was predicted. The thinnest location of cpRNFL (i.e., deepest loss) was also determined. Results: In 19 of 32 eyes, a region of complete or near complete cpRNFL loss was observed. All 19 of these had deep VF defects on the 24-2 and/or 10-2. All of the 32 eyes with deep VF defects had abnormal cpRNFL regions (red, 1%) and all but 2 had a region of cpRNFL thickness <21 µm. The midpoint of the VF defect and the location of deepest cpRNFL had a 95% limit of agreement within approximately two-thirds of a clock-hour (or 30°) sector (between -22.1° to 25.2°). Individual fovea-to-disc angle (FtoDa) adjustment improved agreement in one eye with an extreme FtoDa. Conclusions: Although studies relating local structural (OCT) and functional (VF) measures typically show poor to moderate correlations, there is good qualitative agreement between the location of deep cpRNFL loss and deep defects on VFs.

Progression of Local Glaucomatous Damage Near Fixation as Seen with Adaptive Optics Imaging.

PURPOSE: Deep glaucomatous defects near fixation were followed over time with an adaptive optics-scanning light ophthalmoscope (AO-SLO) to better understand the progression of these defects and to explore the use of AO-SLO in detecting them. METHODS: Six eyes of 5 patients were imaged with an AO-SLO from 2 to 4 times for a range of 14.6 to 33.6 months. All eyes had open-angle glaucoma with deep defects in the superior visual field (VF) near fixation as defined by 10-2 VFs with 5 or more points less than -15 dB; two of the eyes had deep defects in the inferior VF as well. AO-SLO images were obtained around the temporal edge of the disc. RESULTS: In 4 of the 6 eyes, the edge of the inferior-temporal disc region of the retinal nerve fiber (RNF) defect seen on AO-SLO moved closer to fixation within 10.6 to 14.7 months. In 4 eyes, RNF bundles in the affected region appeared to lose contrast and/or disappear. CONCLUSIONS: Progressive changes in RNF bundles associated with deep defects on 10-2 VFs can be seen within about 1 year with AO-SLO imaging. These changes are well below the spatial resolution of the 10-2 VF. On the other hand, subtle thinning of regions with RNF bundles is not easy to see with current AO-SLO technology, and may be better followed with OCT. TRANSLATIONAL RELEVANCE: AO-SLO imaging may be useful in clinical trials designed to see very small changes in deep defects.

Defects Along Blood Vessels in Glaucoma Suspects and Patients.

PURPOSE: To examine the relationship between small hypodense regions ("holes") in the retinal nerve fiber layer (RNFL) seen on circumpapillary optical coherence tomography (OCT) images of glaucoma patients and suspects and the paravascular inner retinal defects (PIRDs) seen with OCT line scans near blood vessels in individuals without optic nerve disease but with high myopia and/or epiretinal membranes (ERMs). METHODS: Based upon the availability of wide-field, swept-source OCT scans, 19 eyes from 15 glaucoma patients or suspects were selected from a larger group of eyes with holes on circumpapillary frequency-domain OCT scans. Paravascular defects (PDs) were identified using en face slab images generated (ATL 3D-Suite) from 52-µm slabs just below the vitreal border of the inner limiting membrane by averaging reflective intensity. Paravascular defects were confirmed with B-scans from these images. RESULTS: For 13 of the 19 eyes, the appearance of the PDs fit the previously described PIRDs and extended well beyond the circumpapillary region. In the other 6 eyes, the PDs were restricted to a small region and did not fit the previously described appearance of PIRDs. In these eyes, the holes were associated with an arcuate defect of the RNFL. Of the 13 with PIRDs, 9 had ERMs and/or high myopia previously associated with PIRDs in otherwise healthy eyes. CONCLUSIONS: Holes seen on circumpapillary OCT scans of glaucoma patients and suspects are associated with local glaucomatous damage, as well as with PIRDs associated with high myopia and ERMs.

Details of Glaucomatous Damage Are Better Seen on OCT En Face Images Than on OCT Retinal Nerve Fiber Layer Thickness Maps.

PURPOSE: High-resolution images of glaucomatous damage to the retinal nerve fiber layer (RNFL) were obtained with an adaptive optics-scanning light ophthalmoscope (AO-SLO) and used as a basis for comparisons between en face slab images and thickness maps derived from optical coherence tomography (OCT) scans. METHODS: Wide-field (9 × 12 mm) cube scans were obtained with swept-source OCT (DRI-OCT) from six eyes of six patients. All eyes had a deep defect near fixation as seen on a 10-2 visual field test. Optical coherence tomography en face images, based on the average reflectance intensity, were generated (ATL 3D-Suite) from 52-µm slabs just below the vitreal border of the inner limiting membrane. The RNFL thickness maps were generated from the same OCT data. Both were compared with the AO-SLO peripapillary images that were previously obtained. RESULTS: On AO-SLO images, three eyes showed small regions of preserved and/or missing RNFL bundles within the affected region. Details in these regions were seen on the OCT en face images but not on the RNFL thickness maps. In addition, in the healthier hemi-retinas of two eyes, there were darker, arcuate-shaped regions on en face images that corresponded to abnormalities seen on AO-SLO. These were not seen on RNFL thickness maps. CONCLUSIONS: Details of local glaucomatous damage, missing or easily overlooked on traditional OCT RNFL thickness analysis used in clinical OCT reports, were seen on OCT en face images based on the average reflectance intensity. While more work is needed, it is likely that en face slab imaging has a role in the clinical management of glaucoma.