A 15-year review of secondary and tertiary optic nerve sheath fenestration for idiopathic intracranial hypertension.

PURPOSE: Optic nerve sheath fenestration (ONSF) is a common surgical option for patients with idiopathic intracranial hypertension (IIH) with vision loss refractory to medical management. Little is known about the visual benefit of repeated ONSF. The authors aimed to assess the efficacy of secondary and tertiary ONSF in patients with IIH. METHODS: A retrospective chart review was performed on all patients with repeat ONSF for IIH at Emory University from 1999 to 2016. Primary outcome measures included visual acuity, optic nerve head findings, and visual field results. RESULTS: A total of nine eyes in seven patients (five females and two males) with repeat ONSF were identified. Two of the seven patients had repeat ONSF in both eyes, while the remaining five patients had only one eye repeated. Five of seven patients (five eyes) improved or remained stable after the secondary ONSF. Two patients (three eyes) continued to worsen despite the secondary fenestration surgery and underwent tertiary ONSF at an average of 13.2 months (SD 5.5 months) after the failed secondary ONSF. Both patients that underwent the tertiary fenestration showed improvement. Six of the patients had either improvement or stability in their clinical findings at their last documented follow-up, but one continued to worsen despite intervention. CONCLUSIONS: This study suggests that secondary and tertiary nerve sheath fenestration is a viable management option for patients with progressive vision loss from IIH. Repeat ONSFs do not appear to have increased complication or failure rates compared to prior documented studies regarding primary fenestrations.

New Basal Insulins: a Clinical Perspective of Their Use in the Treatment of Type 2 Diabetes and Novel Treatment Options Beyond Basal Insulin.

PURPOSE OF REVIEW: The purpose of this review was to review advances in basal insulin formulations and new treatment options for patients with type 2 diabetes not achieving glycemic targets despite optimized basal insulin therapy. RECENT FINDINGS: Advances in basal insulin formulations have resulted in products with increasingly favorable pharmacokinetic and pharmacodynamic properties, including flatter, peakless action profiles, less inter- and intra-patient variability, and longer duration of activity. These properties have translated to significantly reduced risk of hypoglycemia (particularly during the night) compared with previous generation basal insulins. When optimized basal insulin therapy is not sufficient to obtain or maintain glycemic goals, various options exist to improve glycemic control, including intensification of insulin therapy with the addition of prandial insulin or changing to pre-mixed insulin and, more recently, the addition of a GLP-1 receptor agonist, either as a separate injection or as a component of one of the new fixed-ratio combinations of a basal insulin and GLP-1 RA. New safer and often more convenient basal insulins and fixed ratio combinations containing basal insulin (and GLP-1 receptor agonist) are available today for patients with type 2 diabetes not achieving glycemic goals. Head-to-head studies comparing the latest generation basal insulins are underway, and future studies assessing the fixed-ratio combinations will be important to better understand their differentiating features.

Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study.

BACKGROUND: We evaluated the effects of the site of ventricular pacing on left ventricular (LV) synchrony and function in children requiring permanent pacing. METHODS AND RESULTS: One hundred seventy-eight children (aged <18 years) from 21 centers with atrioventricular block and a structurally normal heart undergoing permanent pacing were studied cross-sectionally. Median age at evaluation was 11.2 (interquartile range, 6.3-15.0) years. Median pacing duration was 5.4 (interquartile range, 3.1-8.8) years. Pacing sites were the free wall of the right ventricular (RV) outflow tract (n=8), lateral RV (n=44), RV apex (n=61), RV septum (n=29), LV apex (n=12), LV midlateral wall (n=17), and LV base (n=7). LV synchrony, pump function, and contraction efficiency were significantly affected by pacing site and were superior in children paced at the LV apex/LV midlateral wall. LV dyssynchrony correlated inversely with LV ejection fraction (R=0.80, P=0.031). Pacing from the RV outflow tract/lateral RV predicted significantly decreased LV function (LV ejection fraction <45%; odds ratio, 10.72; confidence interval, 2.07-55.60; P=0.005), whereas LV apex/LV midlateral wall pacing was associated with preserved LV function (LV ejection fraction ≥55%; odds ratio, 8.26; confidence interval, 1.46-47.62; P=0.018). Presence of maternal autoantibodies, gender, age at implantation, duration of pacing, DDD mode, and QRS duration had no significant impact on LV ejection fraction. CONCLUSIONS: The site of ventricular pacing has a major impact on LV mechanical synchrony, efficiency, and pump function in children who require lifelong pacing. Of the sites studied, LV apex/LV midlateral wall pacing has the greatest potential to prevent pacing-induced reduction of cardiac pump function.

Health related quality of life and social support in pediatric patients with pacemakers.

Prior research evaluating health-related quality of life (HRQOL) among pediatric patients with internal cardiac devices has primarily focused on children with cardiac defibrillators, with scant attention devoted to pacemaker recipients. Social support has been conceptualized as a protective factor that partially accounts for differences in HRQOL. This study compares the HRQOL of children with pacemakers with that of healthy children, and examines associations between HRQOL and social support. Twenty-seven pediatric pacemaker recipients completed measures of HRQOL and social support. Their parents also completed measures of child HRQOL. High concordance was found for child and parent-proxy reports of child HRQOL. Children with pacemakers and their parents both reported relatively low child HRQOL when compared to published normative data for healthy children and parents of healthy children. Family and friends emerged as the sources of support positively associated with the greatest number of HRQOL domains. In conclusion, these findings suggest that pediatric pacemaker recipients experience lower levels of HRQOL compared to healthy peers, and that social support from those closest to the child is associated with their perceived HRQOL.

Imaging in acute ischaemic stroke: assessing findings in light of evolving therapies.

Acute ischaemic stroke (AIS) is a debilitating disease for which effective therapies are now available. Effective identification of candidates for therapy relies heavily on noninvasive imaging that must be interpreted accurately in a short timeframe. This review summarizes the evolution of AIS therapies and the implications for noninvasive imaging. The review concludes with consideration of longstanding assumptions about imaging of ischaemic stroke and potential paradigm shifts on the horizon.

Port malposition in the azygos vein resulting in a veno-broncho and broncho-esophageal fistula: A case report.

We present a case of a port malposition into the azygos vein resulting in both a broncho-esophageal and veno-bronchial fistula. While complications of central venous catheter malposition into the azygos vein are well documented in literature, these unique complications have not yet been described. This case underscores how utilizing state of the art technology like intra-cavity electrocardiography rather that reliance on fluoroscopy can help eliminate catheter malposition and its potential catastrophic consequences.

Radiofrequency modification for inducible and suspected pediatric atrioventricular nodal reentry tachycardia.

INTRODUCTION: AV Node Reentry Tachycardia (AVNRT) is the second most common supraventricular tachycardia (SVT) undergoing pediatric radiofrequency ablation behind accessory pathway reentry tachycardias. AVNRT can be difficult to induce during electrophysiology study (EPS) and dual atrioventricular nodal (AVN) pathways physiology may not be demonstrated in young patients. PURPOSE: This report is the largest single center long term pediatric experience of radiofrequency modification of slow AVN input fibers for inducible or suspected (non-inducible) AVNRT. RESULTS: One hundred thirty-two patients underwent slow input AVN modification from 1993 to 2002. The mean patient age was 13.7 years (4-20 yrs) with 62M/70F. Outpatient tachycardia was documented by ambulatory monitoring in all patients. AVNRT was induced in 98/132 patients during EPS (group A) with mean SVT cycle length of 324 msec (230-570 msec). Initial AVN modification (group A) was successful in 97/98 patients (99%). During 34/132 EPS, AVNRT was non-inducible; dual AVN physiology was present in 19/34 (group B), and 15/34 did not show evidence for dual AVN physiology (group C). These 34 patients underwent empiric AVN modification following discussion with patients' families. Freedom of recurrence from SVT at 1 year was 96% for group A (94/98), 89% (17/19) for group B and 93% (14/15) for group C. 1 major and 6 minor complications occurred. CONCLUSIONS: AVN modification for AVNRT can be performed safely and effectively in pediatric patients with good long-term results. Empiric slow pathway AVN modification for non-inducible SVT results in a high rate of freedom from recurrence of tachycardia.

Initial experience with cardiac resynchronization therapy for ventricular dysfunction in young patients with surgically operated congenital heart disease.

Data regarding cardiac resynchronization therapy (CRT) in pediatric patients are limited. The first reported use of CRT in a young patient with congenital heart disease showed promising early results with improvement in New York Heart Association classification, aerobic capacity, and ventricular contractility 1 month after implant. Other reports have shown acute benefits of CRT in young patients with postoperative right bundle branch block after surgery for congenital heart defects. To date, however, there are no published data regarding CRT for the long-term management of systemic ventricular dysfunction in the young with associated congenital heart disease. This report describes our initial experience with CRT in these patients.

Perceived self-competence, psychosocial adjustment, and quality of life in pediatric patients with pacemakers.

OBJECTIVES: To compare participants' self-competence levels to normative data and examine self-competence as a potential protective factor against poorer health-related quality of life (HRQOL) and psychosocial adjustment in children with pacemakers. METHODS: Twenty-seven children between the ages of 8 and 18 years and their caregivers were recruited from a pediatric pacemaker clinic. Participants completed self-report and parent-proxy measures of children's health-related quality of life (HRQOL), self-competence, and psychosocial functioning, which included externalizing and internalizing symptoms, adaptive skills, and behavioral symptoms. RESULTS: Participants reported significantly lower levels of self-competence compared to healthy norms. Self-competence was significantly and positively correlated with most HRQOL domains. Few significant correlations emerged between self-competence and various domains of psychosocial functioning. CONCLUSION: Self-competence may function as a protective factor against lower HRQOL in children with pacemakers. There was less evidence that self-competence may play a protective role against lower adaptive skills and higher externalizing, internalizing, and behavioral symptoms. Clinical implications of these findings, limitations of the study, and areas for future research are discussed.

Nocturnal continuous glucose and sleep stage data in adults with type 1 diabetes in real-world conditions.

BACKGROUND: Sleep plays an important role in health, and poor sleep is associated with negative impacts on diabetes management, but few studies have objectively evaluated sleep in adults with type 1 diabetes mellitus (T1DM). Nocturnal glycemia and sleep characteristics in T1DM were evaluated using body-worn sensors in real-world conditions. METHODS: Analyses were performed on data collected by the Diabetes Management Integrated Technology Research Initiative pilot study of 17 T1DM subjects: 10 male, 7 female; age 19-61 years; T1DM duration 14.9 ± 11.0 years; hemoglobin A1c (HbA1c) 7.3% ± 1.3% (mean ± standard deviation). Each subject was equipped with a continuous glucose monitor and a wireless sleep monitor (WSM) for four nights. Sleep stages [rapid eye movement (REM), light, and deep sleep] were continuously recorded by the WSM. Nocturnal glycemia (mg/dl) was evaluated as hypoglycemia (<50 mg/dl), low (50-69 mg/dl), euglycemia (70-120 mg/dl), high (121-250 mg/dl), and hyperglycemia (>250 mg/dl) and by several indices of glycemic variability. Glycemia was analyzed within each sleep stage. RESULTS: Subjects slept 358 ± 48 min per night, with 85 ± 27 min in REM sleep, 207 ± 42 min in light sleep, and 66 ± 30 min in deep sleep (mean ± standard deviation). Increased time in deep sleep was associated with lower HbA1c (R2 = 0.42; F = 9.37; p < .01). Nocturnal glycemia varied widely between and within subjects. Glycemia during REM sleep was hypoglycemia 5.5% ± 18.1%, low 6.6% ± 18.5%, euglycemia 44.6% ± 39.5%, high 37.9% ± 39.7%, and hyperglycemia 5.5% ± 21.2%; glycemia during light sleep was hypoglycemia 4.8% ± 12.4%, low 7.3% ± 12.9%, euglycemia 42.1% ± 33.7%, high 39.2% ± 34.6%, and hyperglycemia 6.5% ± 20.5%; and glycemia during deep sleep was hypoglycemia 0.5% ± 2.2%, low 5.8% ± 14.3%, euglycemia 48.0% ± 37.5%, high 39.5% ± 37.6%, and hyperglycemia 6.2% ± 19.5%. Significantly less time was spent in the hypoglycemic range during deep sleep compared with light sleep (p = .02). CONCLUSIONS: Increased time in deep sleep was associated with lower HbA1c, and less hypoglycemia occurred in deep sleep in T1DM, though this must be further evaluated in larger subsequent studies. Furthermore, the consumer-grade WSM device was useful for objectively studying sleep in a real-world setting.

Impact of the permanent ventricular pacing site on left ventricular function in children: a retrospective multicentre survey.

BACKGROUND: Chronic right ventricular (RV) pacing is associated with deleterious effects on cardiac function. OBJECTIVE: In an observational multicentre study in children with isolated atrioventricular (AV) block receiving chronic ventricular pacing, the importance of the ventricular pacing site on left ventricular (LV) function was investigated. METHODS: Demographics, maternal autoantibody status and echocardiographic measurements on LV end-diastolic and end-systolic dimensions and volumes at age <18 years were retrospectively collected from patients undergoing chronic ventricular pacing (>1 year) for isolated AV block. LV fractional shortening (LVFS) and, if possible LV ejection fraction (LVEF) were calculated. Linear regression analyses were adjusted for patient characteristics. RESULTS: From 27 centres, 297 children were included, in whom pacing was applied at the RV epicardium (RVepi, n = 147), RV endocardium (RVendo, n = 113) or LV epicardium (LVepi, n = 37). LVFS was significantly affected by pacing site (p = 0.001), and not by maternal autoantibody status (p = 0.266). LVFS in LVepi (39 ± 5%) was significantly higher than in RVendo (33 ± 7%, p < 0.001) and RVepi (35 ± 8%, p = 0.001; no significant difference between RV-paced groups, p = 0.275). Subnormal LVFS (LVFS < 28%) was seen in 16/113 (14%) RVendo-paced and 21/147 (14%) RVepi-paced children, while LVFS was normal (LVFS ≥ 28%) in all LVepi-paced children (p = 0.049). These results are supported by the findings for LVEF (n = 122): LVEF was <50% in 17/69 (25%) RVendo- and in 10/35 (29%) RVepi-paced patients, while LVEF was ≥ 50% in 17/18 (94%) LVepi-paced patients. CONCLUSION: In children with isolated AV block, permanent ventricular pacing site is an important determinant of LV function, with LVFS being significantly higher with LV pacing than with RV pacing.

Catheter ablation of primary supraventricular tachycardia substrate presenting as atrial fibrillation in adolescents.

BACKGROUND: Atrial fibrillation is rarely encountered in children and adolescents, and these cases are usually secondary to myocardial diseases, electrical abnormalities, or scar related in postoperative congenital heart disease patients. Untreated sustained atrial fibrillation may lead to tachycardia-induced cardiomyopathy and/or systemic thromboembolization. OBJECTIVE: The objective here is to describe our findings in four adolescent patients presenting with recurrent atrial fibrillation. DESIGN: We report here the results of the findings in four patients who presented with recurrent clinical atrial fibrillation. RESULTS: Each of the four underwent electrophysiologic study that revealed a primary reentry or automatic supraventricular tachycardia (SVT) substrate, which was able to be treated with radiofrequency ablation. In three of the four cases, elimination of the primary substrate prevented subsequent recurrence of SVT symptoms or documented SVT and/or atrial fibrillation. CONCLUSION: Children and adolescents presenting with atrial fibrillation warrant an exhaustive search for a treatable primary cause of myocardial or electrical disease. If present, a primary SVT substrate may be successfully ablated to prevent recurrence of atrial fibrillation and any associated complications. Pulmonary vein isolation is rarely indicated in adolescents and should be avoided.