Purpose-To understand better the influence of retinal blood vessels (BVs) on the interindividual variation in the retinal nerve fiber layer (RNFL) thickness measured with optical coherence tomography (OCT).Subjects and Methods-RNFL thickness profiles were measured by OCT in 16 control individuals and 16 patients. The patients had advanced glaucoma defined by abnormal disc appearance, abnormal visual fields, and a mean visual field deviation worse than − 10 dB.Results-In general, the OCT RNFL thickness profiles showed 4 local maxima, with the peak amplitudes in the superior and inferior regions occurring in the temporal (peripapillary) disc region. There was considerable variability among individuals in the location of these maxima. However, the 4 maxima typically fell on, or near, a major BV with the temporal and inferior peaks nearly always associated with the main temporal branches of the superior and inferior veins and arteries. In the patients' hemifields with severe loss (mean visual field deviation worse than − 20 dB), the signals associated with the major BVs were in the order of 100 to 150 µm.Conclusions-The variation in the local peaks of the RNFL profiles of controls correlates well with the location of the main temporal branches of the superior and inferior veins and arteries. This correspondence is, in part, due to a direct BV contribution to the shape of the OCT RNFL and, in part, due to the fact that BVs develop along the densest regions of axons. Although the overall BV contribution was estimated to be relatively modest, roughly 13% of the total peripapillary RNFL thickness in controls, their contribution represents a substantial portion locally and increases in importance with disease progression.

Purpose-To determine if adjusting for blood vessel location can decrease the inter-subject variability of retinal nerve fiber (RNFL) thickness measured with optical coherence tomography (OCT).Subjects and Methods-One eye of 50 individuals with normal vision was tested with OCT and scanning laser polarimetry (SLP). The SLP and OCT RNFL thickness profiles were determined for a peripapillary circle 3.4 mm in diameter. The midpoints between the superior temporal vein and artery (STva) and the inferior temporal vein and artery (ITva) were determined at the location where the vessels cross the 3.4 mm circle. The average OCT and SLP RNFL thicknesses for quadrants and arcuate sectors of the lower and upper optic disc were obtained before and after adjusting for blood vessel location. This adjustment was done by shifting the RNFL profiles based upon the locations of the STva and ITva relative to the mean locations of all 50 individuals.Results-Blood vessel locations ranged over 39° (STva) and 33° (ITva) for the 50 eyes. The location of the leading edge of the OCT and SLP profiles was correlated with the location of the blood vessels for both the superior [r=0.72 (OCT) and 0.72(SLP)] and inferior [r=0.34 and 0.43] temporal vessels. However, the variability in the OCT and SLP thickness measurements showed little change due to shifting. After shifting, the difference in the coefficient of variation ranged from −2.1% (shifted less variable) to +1.7% (unshifted less variable). Conclusion-The shape of the OCT and SLP RNFL profiles varied systematically with the location of the superior and inferior superior veins and arteries. However, adjusting for the location of these major temporal blood vessels did not decrease the variability for measures of OCT or SLP RNFL thickness. Measures based upon OCT RNFL thickness generally show good sensitivity and specificity for detecting glaucomatous damage. [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] However, there is considerable inter-subject variation in the RNFL thickness profiles, even among normal controls. For example, although the RNFL profiles from the two eyes of an individual are very similar, 20,21 the profiles differed markedly in both amplitude and waveform across individuals. 22 If inter-individual variability can be reduced, it should be possible to improve the sensitivity and specificity of tests based upon OCT RNFL thickness. KeywordsRecent evidence suggests that the location of the superior and inferior temporal blood vessels (BVs) may account for a portion of the inter-subject variability. 22 In particular, the location of the major peaks in the RFNL profiles coincide with the approximate location of the superior and inferior temporal BVs. In part, this is due to the direct contribution of these BVs to RNFL thickness as measured by OCT22 -24 and, in part, due to the correlation between the location of these BVs and the location of the arcuate bundles.23 We have previously suggested that adjusting for the location of these BVs might decrease variabilit...

Carnitine palmitoyltransferase-II (CPT-II) deficiency can be detected through newborn screening with tandem mass spectrometry. We report a 4-year-old patient with rhabdomyolysis due to CPT-II deficiency, which was initially missed by newborn screening. The patient presented with a 2-day history of fevers, upper respiratory infection, diffuse myalgia, and tea-colored urine. Her medical history was notable for frequent diffuse myalgia when ill. She was demonstrated to have homozygous mutation c.338C>T, p. S113L in CPT2, which is typically found in the adult-onset, myopathic form of the disease. An unknown number of CPT-II deficient patients with normal newborn screening have not yet presented to medical care with the adult-onset, myopathic form of disease. We conclude that (1) not all cases of CPT-II deficiency are currently detected through newborn screening, even when blood is appropriately collected on day 2 of life and (2) CPT-II deficiency should be kept on the differential for patients presenting with rhabdomyolysis, even if the newborn screening results were normal.

Prolonged critical illness in children has emotional consequences for both parents and providers. In this observational cohort study, we longitudinally surveyed anxiety and depression in parents and moral distress in pediatric intensive care unit (PICU) providers (attendings, fellows, and bedside registered nurses) and explored their trajectories and relationships. Anxiety/depression and provider moral distress were measured using the Hospital Anxiety and Depression Scale and the Moral Distress Thermometer, respectively. The relationships of parental and provider distress were evaluated using Spearman's correlations, and their trajectories and potentially associated variables were explored using quadratic random slope and intercept models. Predetermined associated factors included demographic and clinical factors, including parent psychosocial risk and intubation status. We found parental anxiety and depression decreased over their child's admission, and parental psychosocial risk was significantly associated with anxiety (coefficient = 4.43, p < 0.001). Clinicians in different roles had different mean levels and trajectories of moral distress, with fellows reporting greater distress early in admissions and nurses later in admissions. Parental anxiety/depression and provider distress were significantly, though moderately, correlated. We conclude that anxiety and depression in parents of children with prolonged PICU admissions and the moral distress of their clinicians correlate and vary over time and by provider role.