Coats Disease in 9 Patients: A Hispanic Case Series

Background/Aim To describe demographic and clinical characteristics, treatment, and visual prognosis of Coats disease in Hispanic patients. Methods A retrospective chart review was performed on nine patients (ten eyes) diagnosed with Coats disease in our two clinical centrers from 2004 – 2017. Results Mean age at diagnosis was 5.5 years (range 1 – 12 years) and mean follow-up time was 48 months (range 9 – 108 months). Eight patients (89%) were male and had unilateral disease and one (11%) female patient had bilateral disease. In 40% of the cases, patients were asymptomatic. Visual acuity at first presentation was worse than hand motion in 60% of the eyes. Half of the eyes (5/10 eyes, 50%) had exudative retinal detachment (≥ stage IIIA). Vascular ablation with cryotherapy combined with retinal photocoagulation was the most frequent therapeutic approach (40%). Despite anatomical success at 6 months in 100% of the treated eyes, visual outcome at 1 year of treatment was poor (worse than 20/200) in 70% of the cases. Conclusions In our case series, patients were mostly asymptomatic on presentation, with severe stages of Coats disease. Even with anatomical success after surgical treatment in all treated cases, long-term visual prognosis remained very limited.

Characteristics of polypoidal choroidal vasculopathy in the Malaysian population

Introduction: Polypoidal choroidal vasculopathy (PCV) is a distinct clinical entity, characterized by focal hyperfluorescence in the early phase of indocyanine green angiography (ICGA), with or without its associated branching vascular network (BVN).Purpose: To report the angiographic characteristics of PCV on ICGA in presumed PCV patients.Study design: Descriptive cross-sectional study.Materials and methods: This study involved 36 suspected PCV patients who attended the Ophthalmology Clinic, Universiti Kebangsaan Malaysia Medical Centre from June 1, 2012 to May 31, 2013. All patients underwent complete ophthalmologic examination including colour fundus photography, ICGA, and fundus fluorescein angiography. ICGA images of confirmed PCV patients were analysed.Results: Twenty-five out of 36 eyes (69.4%) were diagnosed to have PCV based on ICGA. Mean age of confirmed PCV patients was 66.4 ± 8.42 years, with predominance of males (n = 17) and Chinese ethnicity (n = 19). Best-corrected visual acuity (BCVA) was between 6/6 and 6/18 in 64%. All 25 patients had unilateral disease. Average size of PCV lesions was 1461.4 ± 864.4 μm. The lesions were mostly concentrated in the extrafoveal region (n = 15, 60%). Lesion formation was cluster in 56% (n = 14), single in 32% (n = 8), string in 4% (n = 1), and combination in 8% (n = 2). The majority involved a single discrete area. Polyp pulsation was detected in six eyes, while seven eyes revealed nodular hyperfluorescence when viewed stereoscopically. BVN was evident in 56% (n = 14). Fifteen eyes demonstrated the hypofluorescent halo, while no hyperfluorescent ring was seen in this study population. Late geographical hyperfluorescence (LGH) was noted in seven eyes (28%). There was no significant association between the morphological characteristics of PCV, i.e., size of lesion, location, formation, discrete area involved, and LGH with BCVA.Conclusion: The demographic, clinical, and angiographic features observed in this study were in agreement with other previously published Asian studies. However, we found no association between the morphological characteristics of PCV with BCVA.

Reassessment of postural stimulation testing as a simple tool to identify a subgroup of patients with unilateral primary aldosteronism

Context Adrenal vein sampling (AVS) represents the current diagnostic gold standard for differentiation between unilateral and bilateral primary aldosteronism (PA). Postural stimulation testing (PST) has been used to provide additional diagnostic information. Objective To evaluate diagnostic utility of PST in the differential diagnosis of PA. Design Cohort study. Setting Single tertiary reference center. Patients We analyzed 106 PST performed between 2008 and 2020. Diagnosis of PA and cause of PA were determined according to the Endocrine Society Clinical Practice Guideline, taking into account results of saline infusion testing, AVS, preoperative imaging and outcome after medical or surgical treatment. Main Outcome Measures The suggested cut-offs for the diagnosis of unilateral PA were revisited and optimized for high specificity using receiver operating characteristics (ROC)-analysis. Results 106 patients had confirmed PA (unilateral PA: n=55, bilateral PA: n=29, AVS unsuccessful/declined by patients: n=22). Based on decreased aldosterone plasma concentration of ≥ 28% after 4 hours in the upright position, the PST showed a sensitivity of 36.4% at a specificity of 100% to identify unilateral disease (AUC=0.72, 95% CI=0.62-0.83, p=0.001). In patients with valid testing (drop of cortisol ≥ 10% after 4 hours, n=53) sensitivity of PST rose to 51.4% at a specificity of 100% (AUC=0.77, 95% CI=0.65-0.90, p=0.001). Conclusion The high specificity of 100% for the detection of unilateral PA in patients with decreased aldosterone by at least 28% after 4 hours, makes PST a simple, non-invasive contribution to subtype differentiation in PA.

PREVALENCE AND CLINICAL FEATURES OF BILATERAL OSTEOCHONDRITIS DISSECANS (OCD) OF THE KNEE IN PEDIATRIC AND ADOLESCENT PATIENTS PRESENTING WITH UNILATERAL SYMPTOMS

Background: Osteochondritis dissecans(OCD) is an idiopathic condition primarily involving the subchondral bone with secondary articular cartilage changes, commonly occurring in the knee. The true prevalence of bilateral OCD in patients presenting with unilateral OCD symptoms is unknown. Hypothesis/Purpose: The goals of this study are to determine the prevalence and characteristics of bilateral OCD in patients with unilateral symptoms and compare to those with unilateral disease. Methods: An electronic medical record database was queried from 2003-2016 to identify and retrospectively review patients 18 years or younger presenting to a single pediatric institution with a diagnosis of OCD of the knee and strictly unilateral knee pain. Contralateral knee imaging of the asymptomatic knee within one year of initial presentation was required. Lesion characteristics were evaluated on both x-ray and magnetic resonance imaging(MRI) assessing size, location, and Hefti staging. Treatment(both surgical and non-operative) and outcomes were recorded. Patients with unilateral OCD were compared with those with bilateral disease using appropriate statistical analyses. Results: Eighty consecutive patients, 63 males(79%) and 17 females(21%), average age of 13.1 years old (range:8-18), were included. 71% of symptomatic lesions were located on the medial femoral condyle and 14% on the lateral femoral condyle with 20 lesions(25%) deemed stable on MRI evaluation. A positive correlation was found between increasing lesion size and severity of MRI/Hefti grade(Figure-1). Twelve patients(15%) were found to have bilateral OCD on contralateral imaging, with five of the contralateral lesions(42%) considered stable on MRI. There was no significant difference in skeletal maturity between patients with bilateral vs. unilateral disease. Fifty-two patients(77%) with unilateral disease underwent surgical intervention, while 9(75%) of those with bilateral disease underwent a surgical procedure on either knee. In patients discovered to have an asymptomatic contralateral lesion, 67% ultimately underwent surgical intervention on the contralateral knee. Comparing patients with unilateral and bilateral disease, no statistical differences were found in terms of patient demographics or lesion characteristics. Conclusion: In patients presenting with unilateral OCD symptoms, there was a 15% prevalence of bilateral disease. There was no difference in age, sex, physeal status or lesion characteristics between patients with unilateral vs. bilateral OCD lesions, and we found no difference in rates of surgical intervention. A consistent relationship between lesion size and Hefti classification was appreciated. Given the prevalence of asymptomatic contralateral lesions and required intervention, our study supports the recommendation for bilateral radiologic knee evaluation for pediatric and adolescent patients presenting with unilateral knee OCD. [Figure: see text]

Spectral-domain optical coherence tomography for evaluating palisades of Vogt in ocular surface disorders with limbal involvement

Spectral-domain optical coherence tomography (SD-OCT) has been used to observe the morphology of the palisades of Vogt (POV) with satisfactory resolutions. In this study, we used SD-OCT to examine the microstructure of the POV in ocular surface disorders with limbal involvement. We detect subclinical limbal pathologies based on five parameters, including (1) decreased epithelial thickness, (2) loss of the sharp stromal tip, (3) loss of the smooth epithelial-stromal interface, (4) dilated stromal vessels, and (5) decreased POV density. Eighteen eyes of 10 patients with advancing wavelike epitheliopathy (AWE) and 15 eyes of 9 patients with phlyctenular keratitis/ocular rosacea were recruited. SD-OCT could detect abnormal changes in the POV in 100% of the lesion sites. In presumed-healthy areas of the diseased eyes diagnosed by slit-lamp biomicroscopy, SD-OCT detected abnormal changes in the POV in 100% of the eyes in both groups. In patients with unilateral disease, abnormal changes in the POV were detected by SD-OCT in 50% and 100% of presumed-healthy eyes diagnosed by slit-lamp biomicroscopy in the AWE group and phlyctenular keratitis/ocular rosacea group, respectively. SD-OCT is powerful in detecting POV changes in ocular surface disorders and can provide useful information that cannot be provided by slit-lamp biomicroscopy.

Adrenocorticotropic Hormone–Stimulated Adrenal Venous Sampling Underestimates Surgically Curable Primary Aldosteronism: A Retrospective Cohort Study and Review of Contemporary Studies

Guidelines recommend adrenal venous sampling to determine disease laterality in primary aldosteronism. Adrenocorticotropic hormone (ACTH) stimulation clearly improves the likelihood of successful adrenal vein catheterization but may lead to a decrease in lateralization rates. To examine the impact of ACTH on lateralization, we performed a retrospective analysis of 340 patients with confirmed primary aldosteronism who underwent adrenal venous sampling with a single interventional radiology team using a protocol of sampling both before and after an ACTH bolus. In addition to this original research, we conducted a review of similar studies from the past 5 years to develop a consensus on the impact of ACTH on lateralization for primary aldosteronism. In the original research analysis, following a bolus of ACTH, 58% of patients had a decline in lateralization index which led to discordance between the pre-ACTH and post-ACTH classifications of lateralization in up to 26% of cases. The majority of discordant cases were due to reclassification from unilateral disease pre-ACTH to bilateral disease post-ACTH. In patients who already lateralized with unstimulated sampling, the response to ACTH did not have any impact on surgical outcomes. In a review of contemporary studies, we identified 11 similar studies in the past 5 years, of which 10 reported either no change or a decrease in lateralization index following ACTH, resulting in ≈25% discordance between unstimulated and stimulated lateralization rates. We conclude that ACTH stimulation during adrenal venous sampling can underestimate surgically remediable primary aldosteronism and recommend that the role of ACTH be limited primarily to enhancing selectivity.

Diagnosis of Menière’s disease on MRI: feasibility at 1.5 Tesla

Background Menière’s disease (MD) is clinically characterized by the triad sensorineural hearing loss, tinnitus and/or aural fullness, and vertigo. Endolymphatic hydrops (EH) is the histopathological basis associated with MD, which can be demonstrated on magnetic resonance imaging (MRI). Currently, most studies are done on a 3-T MRI scanner and to date it is believed that EH can only be demonstrated on a 3-T magnet. We report the feasibility of demonstrating EH on a 1.5-T scanner using the standard 20-channel head and neck coil and the current standard 4-h delayed intravenous gadolinium-enhanced three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) sequence. Purpose To investigate whether current standard 4-h delayed intravenous gadolinium-enhanced 3D-FLAIR imaging can demonstrate endolymphatic hydrops on a 1.5-T MRI scanner. Material and Methods The 3D-FLAIR sequence was taken from a 3-T MRI protocol and tested on a volunteer patient with clinically “definite” MD, after 4-h delayed intravenous contrast injection. Good image quality was obtained after reducing both the matrix and the bandwidth, with clear demonstration of EH. Subsequently, eight more patients with unilateral disease were imaged. Five patients had “definite” MD and four had “probable” MD. Results We imaged nine patients with unilateral disease and detected EH in eight of nine ears. One patient with “probable” MD did not show any abnormality, but the images were degraded by motion artifacts. Conclusion At a cost of 2 min extra scanning time compared to a 3-T scanner, EH can be confidently demonstrated with the current standard 3D-FLAIR sequence on a 1.5-T magnet.

Feline ophthalmomyiasis externa caused by Cuterebra larvae: four cases (2005–2020)

Case series summary Described are four cats diagnosed with ophthalmomyiasis externa caused by Cuterebra larvae. Medical records were retrospectively reviewed to identify cats with ophthalmomyiasis externa between 2005 and 2020 at Cornell University Hospital for Animals. Signalment, history, clinical and diagnostic findings, treatment and outcome were recorded. All cats were young (< 3 years of age), had outdoor access and were initially examined during the summer months. All cases had unilateral disease with the right eye affected. Two cases had nictitating membrane lesions and two had orbital disease. Concurrent superficial corneal ulceration was present in three cats. Two cats suffered from pyrexia, suspected secondary to inflammation from the larval infestation. Successful larval removal was performed in all cats, which resulted in improvement of discomfort and clinical signs. A corneal ulcer persisted in one cat, which was lost to follow-up prior to ulcer resolution. Parasite identification confirmed Cuterebra species infestation in all cases. Relevance and novel information To the authors’ knowledge, this is the first report of feline ophthalmomyiasis externa caused by Cuterebra species. Parasite removal was successful in restoring comfort and resolving clinical signs in all cats with adequate follow-up information.

External Validation of Prediction Models for Unilateral Primary Aldosteronism

Primary aldosteronism (PA) is the most common cause of remediable hypertension. Treatment is informed by establishing whether disease is unilateral (localized to one adrenal gland) or bilateral. Adrenalectomy is the guideline-recommended treatment of choice for unilateral PA. However, the currently recommended subtyping test, adrenal vein sampling (AVS), is often limited in accessibility. Thus, prediction models have been developed to diagnose unilateral PA and therefore bypass AVS. However, their generalizability remains unknown. In this retrospective study, we aimed to externally validate the performance of prediction models for unilateral PA in a large population of PA patients at a Canadian referral center who underwent AVS during 2006–2018. The presence of unilateral disease was indicated by a lateralization index of &gt;3 on AVS. We identified 6 clinical prediction models from the literature. The discrimination and calibration of each model were systematically evaluated. For the original models, the derivation cohorts were based out of Japan, France, Italy, and England, with mean age between 46–54 years and 43–56% being male. The derivation cohorts were generally small, with 4 of the 6 studies reporting less than 50 people with unilateral PA. Common variables reported to be predictive of unilateral PA included male sex, hypokalemia, elevated aldosterone-renin ratio, and the presence of a unilateral adrenal nodule on imaging. The validation cohort included 342 PA patients who underwent successful AVS (average age, 52.1 years; 58.8% male). Among them, 186 (54.4%) demonstrated unilateral disease, and the remaining 156 (45.6%) were considered to have bilateral disease. The baseline characteristics of the validation cohort were broadly similar to those of the derivation cohorts, except for potential differences in ethnicity. When applying the models to the validation cohort, subjects were excluded if any candidate variables were missing. All 6 models demonstrated poor discrimination in the validation set (C-statistics; range, 0.59–0.72), representing a marked decrease compared to the derivation sets where they were reported (range, 0.80–0.87). Assessment of calibration by comparing observed and predicted probabilities of the unilateral subtype revealed significant miscalibration. Calibration-in-the-large for every model was &gt;0 (range, 0.36–2.23), signifying systematic underprediction of unilateral PA. Calibration slopes were all &lt;1 (range, 0.35–0.85), indicating poor performance at the extremes of risk. These results suggest that the original models were optimistic due to overfitting in the derivation cohorts and therefore lack generalizability. This is primarily because these models were developed in small data sets. In conclusion, clinical assessment with prediction models for unilateral PA cannot be readily used to bypass AVS in the general PA population.

Utility of Plasma Metanephrines in Adrenal Venous Sampling in Primary Aldosteronism

Introduction: Adrenal Venous Sampling (AVS) is the most reliable means of identifying surgically curable subtypes of primary aldosteronism (PA). Cortisol levels are used to determine cannulation success and lateralization. However, cortisol has a variable secretion pattern and long-half life, and can be co-secreted by adrenal adenomas, leading to misinterpretation of results. Plasma metanephrines (MN) are a possible alternative analyte. MN levels are unaffected by stress, have a short half-life of 3–6 minutes and are released continuously by the adrenals, resulting in very high concentration gradients between the adrenal veins (AV) and peripheral veins (PV), thus providing a sensitive means to determine cannulation success. Premise:The objective of this study was to see if MN can be used in lieu of cortisol in AVS. A secondary end-point was to see if the data was particularly useful in patients who are known co-secretors of cortisol. Methods: Data from AVS carried out without cosyntropin stimulation, from October 2018 to March 2020, were analysed retrospectively. Of these, 51 had additional samples drawn for MN at the time of the procedure and were recruited. Six patients were identified as having autonomous cortisol secretion as they failed an overnight dexamethasone suppression test (ONDST). The data was analysed using cortisol and MN separately and then compared with regards to their selectivity and lateralization index. Data was also analysed to see if known co-secretors had an elevated cortisol/MN ratio of more than 2 on the affected side as described in previous papers. Results: When compared to cannulation and lateralization outcomes using cortisol, similar results were obtained using, a MN AV/PV ratio of more than 12 to indicate successful cannulation and an aldosterone/MN ratios of greater than 5 to confirm lateralization. Contralateral suppression to less than 0.5 for aldosterone/MN below the PV was seen in unilateral disease. With regards to the six co-secretors, all had elevated cortisol/MN ratios of more than 2 on the affected side. Three had concordant results but the other three had discrepant results, with MN analysis suggesting unilateral disease and cortisol measurements suggesting bilateral disease. Two had undergone surgery with biopsy confirming unilateral disease that correlated with MN analysis. The third is under medical management. Conclusion: This is the first study evaluating the use of MN to determine lateralisation of aldosterone production in PA. Further studies are needed, but using MN may be a more reliable alternative to cortisol in the analysis of AVS before definitive surgery in particular in patients with cortisol co-secretion.