Optic nerve hypoplasia (ONH) is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65%) than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED).
The comparative studies on grading in subarachnoid hemorrhage (SAH) had several limitations such as the unclear grading of Glasgow Coma Scale 15 with neurological deficits in World Federation of Neurosurgical Societies (WFNS), and the inclusion of systemic disease in Hunt and Hess (H&H) scales. Their differential incremental impacts and optimum cut-off values for unfavourable outcome are unsettled. This is a prospective comparison of prognostic impacts of grading schemes to address these issues. SAH patients were assessed using WFNS, H&H (including systemic disease), modified H&H (sans systemic disease) and followed up with Glasgow Outcome Score (GOS) at 3 months. Their performance characteristics were analysed as incremental ordinal variables and different grading scale dichotomies using rank-order correlation, sensitivity, specificity, positive predictive value, negative predictive value, Youden's J and multivariate analyses. A total of 1016 patients were studied. As univariate incremental variable, H&H sans systemic disease had the best negative rank-order correlation coefficient (-0.453) with respect to lower GOS (p < 0.001). As univariate dichotomized category, WFNS grades 3-5 had the best performance index of 0.39 to suggest unfavourable GOS with a specificity of 89% and sensitivity of 51%. In multivariate incremental analysis, H&H sans systemic disease had the greatest adjusted incremental impact of 0.72 (95% confidence interval (CI) 0.54-0.91) against a lower GOS as compared to 0.6 (95% CI 0.45-0.74) and 0.55 (95% CI 0.42-0.68) for H&H and WFNS grades, respectively. In multivariate categorical analysis, H&H grades 4-5 sans systemic disease had the greatest impact on unfavourable GOS with an adjusted odds ratio of 6.06 (95% CI 3.94-9.32). To conclude, H&H grading sans systemic disease had the greatest impact on unfavourable GOS. Though systemic disease is an important prognostic factor, it should be considered distinctly from grading. Appropriate cut-off values suggesting unfavourable outcome for H&H and WFNS were 4-5 and 3-5, respectively, indicating the importance of neurological deficits in addition to level of consciousness.
Background and Purpose:Several studies report good outcomes in selected patients of aneurysmal subarachnoid hemorrhage (aSAH). The purpose of our study is to project the clinical characteristics, management, and overall outcome of patients with aSAH presenting to a tertiary care center in India.Materials and Methods:A prospective study was conducted over a period of 10 months and all patients presenting with aSAH were studied. Patients presenting in all grades and managed with any type of intervention or managed conservatively were included to characterize their clinical and radiological profile at admission, during management, and at discharge. Outcome was assessed with the Glasgow Outcome Score (GOS) at 3 months follow-up.Results:Out of the 482 patients [mean age: 51.3 (±13.5); M: F = 1:1], 330 patients were fit to be taken up for intervention of the ruptured aneurysm, while 152 patients were unfit for any intervention. At 3 months follow-up, good outcome (GOS 4 and 5) was observed in 159 (33%), poor outcome (GOS 2 and 3) in 53 (11%), and death in 219 (45.4%) patients, while 51 patients (10.6%) were lost to follow-up. Most (95%) of the patients in the non-intervention group expired, and hence the high mortality rate, as we have analyzed the results of all patients of all grades, regardless of the treatment given. The predictors of poor outcome (GOS 1, 2, and 3) at 3 months follow-up, using multinomial regression model, were: World Federation of Neurological Surgeons (WFNS) grade IV and V (at admission and after adequate resuscitation) [odds ratio (OR): 35.1, 95% confidence interval (CI): 10.8-114.7] and presence of hypertension as a co-morbid illness [OR: 2.7, 95% CI: 1.6-5.6]. All patients showing acute infarction on computed tomography scan at presentation had a poor outcome.Conclusions:Despite recent advances in the treatment of patients with aSAH, the morbidity and mortality rates have failed to improve significantly in unselected patients and natural cohorts. This may be attributed to the natural history of aSAH, and calls for new strategies to diagnose and treat such patients before the catastrophe strikes.
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