A case of 12-year-old male with Seckel syndrome, presented with unilateral glaucoma leading to advanced disc damage hence, visual deterioration. Seckel syndrome being a rare inherited disorder ...characterized by growth delay and unique facial features, had been infrequently reported for ophthalmic anifestation especially glaucoma. Though glaucoma is a rare association in Seckel syndrome, screening at an early stage could help in preventing vision loss.
Adams-Oliver syndrome (AOS) is a rare inherited disorder characterized by aplasia cutis congenita, cutis marmorata telangiectatica congenita, and terminal limb defects. Ocular associations have been ...rarely reported. We report a 6-month-old boy with AOS associated with refractory glaucoma, megalocornea, and anterior polar cataract. To our knowledge, this is the first case of glaucoma to be reported in association with AOS.
Spontaneous expulsive suprachoroidal hemorrhage is a rare ocular condition, which usually occurs after sudden decompression of the eyewall. Most of the cases of expulsive hemorrhage reported had a ...predisposing glaucoma with the combination of corneal pathology. We are reporting a case of spontaneous expulsive suprachoroidal hemorrhage in a glaucoma patient probably due to perpetuated inflammatory reaction and frequent eye rubbing induced by allergic reaction to topical alpha adrenergic agonist in a compromised cornea.
The probability of weaning and of long-term survival of chronically mechanically ventilated cancer patients is unknown, with incomplete information available to guide therapeutic decisions. We sought ...to determine the probability of weaning and overall survival of cancer patients requiring long-term mechanical ventilation in a specialized weaning unit.
A single-institution retrospective review of patients requiring mechanical ventilation outside of a critical care setting from 2008 to 2012 and from January 1 to December 31, 2018, was performed. Demographic and clinical data were recorded, including cancer specifics, comorbidities, treatments, and outcomes. Overall survival was determined using the Kaplan-Meier approach. Time to weaning was analyzed using the cumulative incidence function, with death considered a competing risk. Prognostic factors were evaluated for use in prospective evaluations of weaning protocols.
Between 2008 and 2012, 122 patients required mechanical ventilation outside of a critical care setting with weaning as a goal of care. The cumulative incidence of weaning after discharge from the intensive care unit was 42% at 21 days, 49% at 30 days, 58% at 60 days, 61% at 90 days, and 61% at 120 days. The median survival was 0.16 years (95% CI, 0.12 to 0.33) for those not weaned and 1.05 years (95% CI, 0.60 to 1.34) for those weaned. Overall survival at 1 year and 2 years was 52 and 32% among those weaned and 16 and 9% among those not weaned. During 2018, 36 patients at our institution required mechanical ventilation outside of a critical care setting, with weaning as a goal of care. Overall, with a median follow-up of 140 days (range, 0-425 days; average, 141 days), 25% of patients requiring long-term mechanical ventilation (9 of 36) are alive.
Cancer patients can be weaned from long-term mechanical ventilation, even after prolonged periods of support. Implementation of a resource-intensive weaning program did not improve rates of successful weaning. No clear time on mechanical ventilation could be identified beyond which weaning was unprecedented. Short-term overall survival for these patients is poor.
Purpose: The relative afferent pupillary defect (RAPD) is an important sign of asymmetrical retinal ganglion cell damage. The purpose of this study was to quantify RAPD by a pupillometer (RAPiDo, ...Neuroptics) and assess its correlation with asymmetric glaucoma and manual pupillary assessment. Methods: A total of 173 subjects were enrolled in the study and categorized into glaucoma, n = 130, and control, n = 43. Subjects were all recruited in the Glaucoma Clinic of the Aravind Eye Hospital in Madurai during their follow-up. They were 18 years and older, with best corrected visual acuity of 6/36 or better. Exclusion criteria included all retinal pathologies, optic atrophies, ocular injuries, severe uveitis, cloudy corneas, dense cataracts, or use of mydriatics or miotic drugs. RAPD was assessed in all subjects using an automated pupillometer and the results were compared with the swinging flash light test conducted on the same subjects by an experienced ophthalmologist. We looked at the correlation between RAPD and the intereye difference in cup-to-disc ratio (CDR), mean deviation (MD) of visual field testing, and retinal nerve fiber layer (RNFL) thickness. Sensitivity and specificity were assessed by area under the receiver operator characteristic (AUROC) analysis. Results: Glaucoma patients had significant RAPD (0.55 ± 0.05 log units) when compared with the controls (0.25 ± 0.05 log units), P < 0.001. Significant intereye differences in CDR, MD, and RNFL between glaucoma and control (P < 0.001) were seen. There was a good correlation between the magnitude and sign of RAPD and these intereye differences in CDR (r = 0.52, P < 0.001), MD (r = 0.44, P < 0.001) and RNFL thickness (r = 0.59, P < 0.001). When compared with the experienced ophthalmologist, AUROC was 0.94, with 89% sensitivity and 91.7% specificity. Conclusion: The good correlation between the magnitude of RAPD, as measured by the automated pupillometer, and intereye differences in MD, CDR, and RNFL thickness in glaucomatous, and the good sensitivity and specificity when compared with the experienced ophthalmologist, suggest that pupillometry may be useful as a screening tool to assess asymmetric glaucoma.
To evaluate the demographic, clinical, and socioeconomic factors associated with variation in the quality of life (QOL) in caregivers of children with primary congenital glaucoma (PCG) in south ...India.
Cross-sectional survey.
Caregivers of children younger than 18 with diagnosed PCG were prospectively enrolled at Aravind Eye Hospital in Madurai and Coimbatore, India. Participants completed 2 questionnaires, the PHQ-9 (9-item Patient Health Questionnaire) and the CarCGQoL (Caregivers Congenital Glaucoma QOL Questionnaire). Clinical, demographic, and socioeconomic data were obtained for each child-caregiver dyad. Rasch-calibrated scores were calculated for patient-reported outcome measures. Spearman correlation and linear regression were used to analyze data to determine associations with caregiver QOL.
There were 70 caregivers (mean age 32.1, 77.1% female) of 70 children with PCG (mean age 7.7, 37.1% female) included in the study. In univariate and multivariable analyses, child's age (β = −0.04; 95% confidence interval, −0.08 to −0.01) and duration of disease (β = − 0.03; 95% confidence interval, −0.07 to −0.01) were the only factors associated with CarCGQoL. Survey items related to anger, self-confidence, irritability, appetite, and interest in leisure activities had the lowest scores. There was a negative correlation between CarCGQoL and PHQ-9 scores (r = −0.66, P < .01), indicating that worse caregiver QOL was significantly correlated with more depressive symptoms.
This study identified traits associated with QOL decline, as well as the QOL issues most likely to affect caregivers of children with PCG in south India. Findings from this study may be important for designing interventions to improve caregivers' QOL, thereby maximizing their ability to care for children with PCG.
Frank Ter Haar syndrome (FTHS) is a rare autosomal recessive disorder with characteristic skeletal, cardiac, ocular, and craniofacial abnormalities. We report a sibling pair presenting with clinical ...features typical of FTHS, born to consanguineous parents, with a novel mutation in the SH3PXD2B gene on chromosome 5q35.1 that results in premature truncation of the protein encoded. The children presented with brachycephaly, multiple joint contractures, cardiac valvular defects, bilateral megalocornea, and congenital glaucoma. Trabeculotomy combined with trabeculectomy was performed in both siblings to control intraocular pressure. The characteristic clinical features with the underlying genetic defects confirmed the diagnosis of FTHS. Early diagnosis and treatment of congenital glaucoma preserved vision in the children.
Purpose: Data on glaucoma treatment barriers in South India is limited; improved knowledge of barriers could advance disease self-management. We aimed to understand glaucoma treatment barriers in ...South India.
Methods: Glaucoma patients ≥18 taking ≥1 medication at the Aravind Eye Hospital were screened with a validated medication adherence tool. Patients who self-reported poor adherence and/or were >3 months late for follow-up completed semi-structured interviews on treatment barriers. Interviews were audio-recorded, transcribed, translated and back-translated to ensure accuracy. Researchers used grounded theory to code the transcripts and identify themes using NVivo 11.0. Demographic and clinical characteristics were abstracted from the medical record.
Results: 70/167 (42%) had poor self-reported adherence to drops. The 45 interviewed were 62 ± 12 years and took 1.6 ± 0.7 drops. Forty-two (93%) were non-adherent to medication and 21 (47%) were late for follow-up. Top barriers to medication adherence were difficulty obtaining drops (20, 44%), being busy (18, 40%), and the expense (17, 38%). Top barriers to appointment follow-up were distance to the hospital (21, 47%), expense (20, 44%), and no escort (15, 33%). Other important barriers included mistrust in the health system, poor knowledge of glaucoma and family needs.
Conclusions: Previously, 6% of glaucoma patients in South India self-reported poor medication adherence; the 42% identified in this study is in line with glaucoma medication adherence rates globally. Complex factors caused high rates of non-adherence. Societal-level interventions that address systemic barriers and counselling that supports patients' and families' motivation for behaviour change should be implemented.
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