Autoimmune Retinopathy in a Patient with Stiff-Person Syndrome: A Case Report.

PURPOSE: To describe a patient who developed retinal degeneration associated with autoimmune retinopathy (AIR) and who was also found to have anti-glutamic acid decarboxylase (GAD65) autoantibodies and the diagnosis of stiff-person syndrome (SPS). METHODS: Ophthalmologic workup consisted of clinical examination, multi-modality retinal imaging, and electrophysiologic testing. Further neurologic assessment including relevant serum and cerebrospinal fluid studies was also conducted. RESULTS: We highlight the case of a 45-year-old patient who developed subacute, sequential vision loss, along with bilateral lower extremity weakness. On initial presentation, optical coherence tomography (OCT) of the left eye was notable for diffuse attenuation of the outer retinal bands. Fundus autofluorescence demonstrated a ring of hyper-autofluorescence encircling the fovea of the left eye. At fifteen-month follow-up, the right eye also became similarly affected. He was found to have elevated serum and cerebrospinal anti-GAD65 autoantibodies and was diagnosed with both SPS and AIR. CONCLUSION: There is a potential association of anti-GAD65 autoantibodies with the development of AIR.

Central Retinal Artery Occlusion: Visual Outcomes from a Large Northern California Cohort.

PURPOSE: To assess visual acuity (VA) outcomes in a large cohort of patients diagnosed with nonarteritic central retinal artery occlusion (CRAO), and to ascertain whether time from symptom onset to presentation, presenting VA, or conservative treatment delivery (anterior chamber paracentesis, ocular massage, intraocular pressure lowering drugs, hyperventilation, or some combination of those) impacted ultimate VA outcomes. DESIGN: Retrospective cohort study. SUBJECTS: The study included 794 patients who presented with CRAO between 2011 and 2020. Within this cohort, 484 individuals presented within 30 days of symptom onset and had comprehensive documentation regarding the details of their presentation, management, and follow-up ≥ 90 days postdiagnosis. METHODS: Retrospective chart review was conducted for all patients with a diagnosis of CRAO initially identified via International Classification of Diseases coding, followed by confirmation of diagnosis by 2 retina specialists. Cases of arteritic CRAO were excluded. MAIN OUTCOME MEASURES: Visual acuity recovery, defined as improvement from ≤ 20/200 or worse at presentation to ≥ 20/100 ≥ 90 days after diagnosis. RESULTS: Of the 794 identified patients, 712 (89.7%) presented with VA of ≤ 20/200. Similarly, 447 (92.4%) of the 484-patient subset that presented within 30 days and had comprehensive documentation presented with VA ≤ 20/200. Of the 441 of those patients with documented follow-up, 380 (86.2%) remained at that level. Of the 244 patients who presented within 4.5 hours of symptom onset, 227 (93%) presented ≤ 20/200 and 201 (92.6%) of the 217 of those with follow-up data did not improve beyond that threshold. There was no significant difference (P < 0.05) in final VA between patients presenting before versus after 4.5 hours from time of vision loss. There was also no significant difference (P < 0.05) in VA outcomes between patients who did or did not receive conservative treatment. CONCLUSIONS: This large retrospective study further highlights the poor visual prognosis for patients with CRAO. Earlier time to presentation did not seem to impact final VA outcome, nor did conservative treatment efforts. Efficacious evidence-based treatment options are needed for this patient population. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Retinal Dialysis and Associated Rhegmatogenous Retinal Detachment in Patients with Neurofibromatosis Type 1: A Case Series.

PURPOSE: While ophthalmic manifestations of neurofibromatosis type 1 (NF1), including iris Lisch nodules and optic gliomas have been well described, retinal involvement in these patients has yet to be established. Characterizing the relationship between NF1 and the retina is necessary to optimize outcomes for these patients. METHODS: Independent chart review of NF1 patients was conducted. RESULTS: Chart review yielded four patients, with a history of NF1, with subsequent retinal dialysis and rhegmatogenous retinal detachment. These four patients presented to our institution with a rhegmatogenous retinal detachment secondary to a retinal dialysis with no history of trauma. These patients also demonstrated hyperreflective choroidal abnormalities on near-infrared reflectance (NIR) imaging and optical coherence tomography (OCT). CONCLUSION: Seeing that patients diagnosed with NF1 are susceptible to various ocular manifestations and pathologies, routine ophthalmic examinations are essential in maintaining their ocular health and minimizing morbidity.

Multiple Evanescent White Dot Syndrome: Findings from a Large Northern California Cohort.

PURPOSE: To report the clinical and imaging characteristics of multiple evanescent white dot syndrome (MEWDS) from a large single-center cohort. DESIGN: Single-center, retrospective cohort study. PARTICIPANTS: A total of 111 patients previously diagnosed with MEWDS in the Kaiser Permanente Northern California system from 2012 to 2019. METHODS: Two retina specialists reviewed the medical records and all available retinal imaging, including Humphrey visual field testing, fundus photography (FP), OCT, fluorescein angiogram (FA), and fundus autofluorescence (FAF). Patients were excluded from analysis if confirmatory imaging was unavailable. MAIN OUTCOME MEASURES: Patient characteristics, visual acuity, clinical examination and imaging findings, and final diagnosis. RESULTS: Seventy-three patients (65.8%) were confirmed to have the diagnosis of MEWDS. Fifty-eight (79.5%) were female, with a mean (standard deviation [SD]) age at presentation of 35.2 (14.2) years, and mean refractive error of -1.6 diopters. Initial mean (SD) visual acuity was logarithm of the minimum angle of resolution (logMAR) 0.39 (0.31) and improved to mean (SD) logMAR 0.07 (0.15) at final follow-up. Presenting symptoms included blurred vision (82%), scotomas (56%), photopsias (43%), and floaters (23%). Nine patients (12%) had a previously diagnosed autoimmune condition, and 2 patients (3%) had documentation of a recent vaccination. Antecedent upper respiratory infection was documented in 15 of 66 patients (23%). Noted clinical and imaging features include ellipsoid zone disruption (100%), white fundus lesions (92%), FA hyperfluorescence (92%), foveal granularity (74%), vitreous cell (53%), and optic disc edema (52%). Twenty-nine of the 111 patients (26%) were initially misdiagnosed with MEWDS and subsequently given an alternative diagnosis, including other white dot syndromes, syphilis, primary vitreoretinal lymphoma, myopic degeneration, and central serous chorioretinopathy. CONCLUSIONS: Multiple evanescent white dot syndrome is a rare self-limiting condition of the outer retina. Although a distinct set of clinical exam and imaging findings permit recognition of this disease, misdiagnosis is not uncommon.

A case of pentosan polysulfate maculopathy originally diagnosed as stargardt disease.

PURPOSE: To describe a patient with a past diagnosis of Stargardt disease that was later determined to be pentosan polysulfate (PPS) maculopathy. OBSERVATIONS: The patient had clinical and imaging findings uncharacteristic of Stargardt disease. Rather, her fundus resembled the recently described maculopathy ascribed to PPS. After genetic testing was found to be negative for pathologic variants, the patient was asked to cease usage of PPS. CONCLUSIONS AND IMPORTANCE: This case emphasizes the importance of reviewing patient medication profiles prior to rendering a diagnosis of a retinal dystrophy. It is essential that ophthalmologists catch drug toxicities as early as possible, to minimize risk of further irreversible vision loss due to continued medication exposure.

Technical considerations to minimize complications of inguinal lymph node dissection.

Penile cancer is a rare malignancy with a high propensity for regional dissemination. Current guidelines recommend inguinal lymphadenectomy in patients with penile cancer for palpable inguinal lymph nodes or in certain cases of nonpalpable inguinal lymph nodes. For many years, this procedure was performed with a traditional open approach and carried significant morbidity due to severe lymphedema, flap necrosis, wound infections, and seroma formation. The evolution of inguinal lymphadenectomy surgery for patients with penile cancer to a more minimally invasive approach has greatly reduced the morbidity of the procedure. Complications of inguinal lymphadenectomy can be minimized with modifications in surgical approach with the use of endoscopic, robotic, and various reconstructive methods. This review focuses on various intraoperative techniques to reduce morbidity in inguinal lymphadenectomies for penile cancer.

Upper pole urologist-obtained percutaneous renal access for PCNL is safe and efficacious.

INTRODUCTION: Interventional radiologist may be hesitant to obtain upper pole access for percutaneous nephrolithotomy (PCNL) due to a higher complication rate. Renal access gained by urologists may achieve higher stone-free rates with similar complication rates. We evaluate our institution's contemporary results of percutaneous renal access in the upper pole for nephrolithotomy by urologists, which we believe both safe and efficacious. MATERIALS AND METHODS: This retrospective chart review included all PCNL's performed by fellowship-trained endourologists from 2003 to 2014 at a single institution. Inclusion criteria included patients in which renal access was obtained by the urologist via the upper pole for PCNL. Stone-free status was determined by either KUB or CT scan on POD #1. Patients without stones visible on KUB or less than 4 mm on CT were considered stone-free. RESULTS: A total of 144 patients obtained upper pole access for PCNL. There were a total of 53 (37%%) staghorn calculi, of which 35 (66%) were partial staghorn stones. Renal access was obtained above 11th rib in 12.5% (n = 18), between the 11th and 12th rib in 57.6% (n = 83), subcostal in 14.6% (n = 21) and undetermined in the rest. Complications were seen in 18 (12.5%) of patients. Hydropneumothorax requiring chest tube was seen in 8 (5.6%) patients. Postoperative imaging confirmed 93 (64.5%) patients stone-free, and 35 (24.3%) required a second look PCNL. CONCLUSIONS: Our experience with upper pole percutaneous renal access for nephrolithotomy has shown that it has an acceptable complication risk. It should be a part of an endourologist's armamentarium that operate on large burden, complex stones or ureteral pathology.

Football Equipment Removal Improves Chest Compression and Ventilation Efficacy.

OBJECTIVE: Airway access recommendations in potential catastrophic spine injury scenarios advocate for facemask removal, while keeping the helmet and shoulder pads in place for ensuing emergency transport. The anecdotal evidence to support these recommendations assumes that maintaining the helmet and shoulder pads assists inline cervical stabilization and that facial access guarantees adequate airway access. Our objective was to determine the effect of football equipment interference on performing chest compressions and delivering adequate ventilations on patient simulators. We hypothesized that conditions with more football equipment would decrease chest compression and ventilation efficacy. METHODS: Thirty-two certified athletic trainers were block randomized to participate in six different compression conditions and six different ventilation conditions using human patient simulators. Data for chest compression (mean compression depth, compression rate, percentage of correctly released compressions, and percentage of adequate compressions) and ventilation (total ventilations, mean ventilation volume, and percentage of ventilations delivering adequate volume) conditions were analyzed across all conditions. RESULTS: The fully equipped athlete resulted in the lowest mean compression depth (F5,154 = 22.82; P < 0.001; Effect Size = 0.98) and delivery of adequate compressions (F5,154 = 15.06; P < 0.001; Effect Size = 1.09) compared to all other conditions. Bag-valve mask conditions resulted in delivery of significantly higher mean ventilation volumes compared to all 1- or 2-person pocketmask conditions (F5,150 = 40.05; P < 0.001; Effect Size = 1.47). Two-responder ventilation scenarios resulted in delivery of a greater number of total ventilations (F5,153 = 3.99; P = 0.002; Effect Size = 0.26) and percentage of adequate ventilations (F5,150 = 5.44; P < 0.001; Effect Size = 0.89) compared to one-responder scenarios. Non-chinstrap conditions permitted greater ventilation volumes (F3,28 = 35.17; P < 0.001; Effect Size = 1.78) and a greater percentage of adequate volume (F3,28 = 4.85; P = 0.008; Effect Size = 1.12) compared to conditions with the chinstrap buckled or with the chinstrap in place but not buckled. CONCLUSIONS: Chest compression and ventilation delivery are compromised in equipment-intense conditions when compared to conditions whereby equipment was mostly or entirely removed. Emergency medical personnel should remove the helmet and shoulder pads from all football athletes who require cardiopulmonary resuscitation, while maintaining appropriate cervical spine stabilization when injury is suspected. Further research is needed to confirm our findings supporting full equipment removal for chest compression and ventilation delivery.

The Value of Pathological Examination of the Foreskin Following Circumcision.

INTRODUCTION: We determined the clinical impact and value of routine histopathological examination of the foreskin following circumcision. METHODS: We performed a retrospective study of 225 consecutive adult circumcisions. Indications for circumcision were categorized as benign or malignant based on preoperative clinical evaluation. Histopathological results were similarly classified as benign or malignant. Preoperative clinical impression and postoperative histological diagnosis were compared and reported as concordant (in agreement) or discordant (in disagreement). The cost impact of histopathology examination was analyzed with respect to study findings. RESULTS: Of the 225 patients 209 (92.9%) had clinically benign disease on preoperative evaluation and 16 (7.1%) had foreskin lesions suspicious for malignancy. Mean age was 57.0 years (range 23 to 92). Patients were younger in the benign group than in the malignant group (56.5 vs 62.8 years, p = 0.018). Black patients represented 65.8% of the study population and were similarly distributed between the 2 groups (p = 0.405). There was no statistical difference in patient height, weight, body mass index or comorbidities between the 2 groups. Preoperative clinical impression and postoperative histological diagnosis were concordant in all 209 patients in the benign group. Of the 16 patients suspected to have malignant disease preoperatively 9 (56.2%) had malignancy and 7 (43.8%) had benign disease on histopathological examination. CONCLUSIONS: Routine histological examination of a foreskin specimen in the absence of clinical suspicion for malignancy appears to have diminished benefit in the setting of benign preoperative indications. Omitting this traditional practice in patients with benign surgical indications may positively impact health care costs without compromising quality of care.

Spontaneous Subdural Hematoma and Intracystic Hemorrhage in an Arachnoid Cyst.

We report the case of 22-year-old man with nontraumatic intracystic hemorrhage into a middle cranial fossa arachnoid cyst associated with a contiguous subacute subdural hematoma. Arachnoid cysts are benign intra-arachnoidal fluid collections frequently detected incidentally during neuroimaging. Rare complications of arachnoid cysts such as intracystic hemorrhage or subdural hematomas and subdural hygromas typically occur after head trauma. Our review of the literature identified fewer than 30 cases of arachnoid cysts with complicating intracystic hemorrhage and ipsilateral subdural hematomas.