Systemic and Ophthalmic Manifestations in Different Types of Refractive Errors in Patients with Down Syndrome.

Background and Objective: This study aims to investigate the prevalence of systemic and ophthalmic manifestations in different refractive groups in children and young adults with Down syndrome (DS). Materials and Methods: The study was a population-based, cross-sectional study that included 141 Caucasian children and young adults with DS. They were classified into the following three groups: myopia DS group (37 subjects, mean age 15.8 years), emmetropia DS group (41 subjects, mean age 11.7 years) and hyperopia DS group (63 subjects, mean age 10.9 years). The participants underwent inspection, slit-lamp examination, cycloplegic refraction, ocular alignment and ocular motility examination. Ten systemic manifestations were analyzed. Results: There was no difference in the prevalence of any systemic manifestations between the groups. Considering the ophthalmic manifestations, there was statistical difference in the distribution of proportions among the three groups for nystagmus (p = 0.011), iris-stromal atrophy (p = 0.048) and strabismus (p = 0.031). The prevalence of strabismus in our DS myopia group was 35.1%, and in DS hyperopia group 38.1%. Conclusions: The results of our study suggest that DS children and young adults with any refractive error do not have a higher chance of additional systemic manifestations. Myopia in DS was associated with a higher prevalence of nystagmus and iris stromal atrophy, whereas astigmatism was found to be more frequent in hyperopia.

Con: The Length of Adult Cardiothoracic Anesthesiology Fellowship Training Should Not Be Extended Beyond One Year.

Fellowship training in adult cardiothoracic anesthesiology (ACTA) is a one-year postgraduate experience with formal accreditation by the Accreditation Council for Graduate Medical Education. ACTA is a competitive and evolving subspeciality. With expanding knowledge, clinical roles and technical skills required of the modern cardiothoracic anesthesiologists, the optimal structure and duration of the fellowship training are worth considering. This manuscript provides supporting rationale for fellowship training in ACTA to remain one year in duration. The expanding responsibilities of the cardiothoracic anesthesiologist and strategies to best train the future of the subspecialty within the current training structure are discussed. It also briefly examines the history and current status of the fellowship training, reviews considerations for increasing fellowship duration, and highlights personal and financial considerations during the training.

Implantation of subcutaneous defibrillator is feasible and safe with monitored anesthesia care.

BACKGROUND: The perioperative anesthesia care during subcutaneous implantable cardioverter-defibrillator (S-ICD) implantation is still evolving. OBJECTIVE: To assess the feasibility and safety of S-ICD implantation with monitored anesthesia care (MAC) versus general anesthesia (GA) in a tertiary care center. METHODS: This is a single-center retrospective study of patients undergoing S-ICD implantation between October 2012 and May 2019. Patients were categorized into MAC and GA group based on the mode of anesthesia. Procedural success without escalation to GA was the primary endpoint of the study, whereas intraprocedural hemodynamics, need of pharmacological support for hypotension and bradycardia, length of the procedure, stay in the post-anesthesia care unit, and postoperative pain were assessed as secondary endpoints. RESULTS: The study comprises 287 patients with MAC in 111 and GA in 176 patients. Compared to MAC, patients in GA group were younger and had a higher body mass index. All patients had successful S-ICD implantation. Only one patient (0.9%) in the MAC group was converted to GA. Despite a similar baseline heart rate (HR) and mean arterial blood pressure (MAP) in both groups, patients with GA had significantly lower HR and MAP during the procedure and more frequently required pharmacological hemodynamic support. Length of the procedure, stay in the postanesthesia care unit, and postoperative pain was similar in both groups. CONCLUSION: This retrospective experience suggests that implantation of S-ICD is feasible and safe with MAC. Use of GA is associated with more frequent administration of hemodynamic drugs during S-ICD implantation.

Implication of Deep-Vascular-Layer Alteration Detected by Optical Coherence Tomography Angiography for the Pathogenesis of Diabetic Retinopathy.

The aim of this narrative mini review is to analyze optical coherence tomography angiography (OCTA) parameters from reports that involved both superficial and deep vascular layers in patients with diabetes and to assess their relevance for the pathogenesis of diabetic retinopathy (DR). Papers published from January 2015 to August 2018 describing the use of OCTA in diabetes were identified and reviewed through a Medline/PubMed search. OCTA studies suggest that parameters are altered in patients with diabetes in all retinal vascular layers. From all included studies that evaluated both the superficial and the deep vascular layer, a number of studies suggested that the deep vascular layer was affected at an earlier stage of DR. OCTA parameter alterations were more prominent in the deep vascular layer than in the superficial vascular layer in patients with DR, and deep-vascular-layer alterations were most evident in patients with diabetic macular edema. Regarding that retinal venules originate from the deep vascular layer of the retina, alteration of OCTA parameters at the deep vascular layer in diabetic patients may imply a predominant affection of the venous side of the retinal vascular system in the pathogenesis of DR.

Increase in the OCT angiographic peripapillary vessel density by ROCK inhibitor ripasudil instillation: a comparison with brimonidine.

PURPOSE: To assess the responses of the superficial peripapillary retinal vessel density (VD) and prelaminar flow index (PLFI) to topical Rho-assisted coiled-coil forming protein kinase (ROCK) inhibitor ripasudil and alpha-2 agonist brimonidine using optical coherence tomography angiography. METHODS: This is a prospective, non-randomized, comparative cohort study. We studied the response of optical coherence tomography angiography (OCTA) parameters to drugs in 24 eyes treated with ripasudil and 23 eyes treated with brimonidine at the Sensho-kai Eye Institute. After division by the signal strength (SS), we compared the responses of peripapillary VD/SS and PLFI/unit area (UA)/SS to topical eye drops in eyes with primary open-angle glaucoma (POAG) and ocular hypertension (OH). RESULTS: In the superficial peripapillary retina, VD/SS increased significantly in the ripasudil-treated eyes (12.5 ± 21.7%, P = 0.018), but not in the brimonidine-treated eyes (- 2.0 ± 13.8%, P = 0.484). In the deeper area of the optic disc, the changes in the PLFI/UA/SS in the brimonidine-treated eyes (+ 0.9 ± 8.9%, P = 1.00) and ripasudil-treated eyes (- 1.3 ± 8.5%, P = 0.241) were not significant. Multivariate discriminant analysis showed that the change in the peripapillary VD/SS was the most important parameter (P = 0.0186) for differentiating ripasudil- and brimonidine-treated eyes. CONCLUSIONS: The topical ROCK inhibitor ripasudil enhanced the peripapillary VD in POAG and OH, whereas the alpha-2 agonist brimonidine did not. The PLFI did not respond to either drug.

Human trafficking for labour exploitation: the results of a two-phase systematic review mapping the European evidence base and synthesising key scientific research evidence.

OBJECTIVES: Our objectives were (1) to systematically map the contours of the European evidence base on labour trafficking, identifying its key characteristics, coverage, gaps, strengths and weaknesses and (2) to synthesise key scientific research. METHODS: We took a two-phase approach: a systematic map followed by a detailed synthesis of key scientific research evidence. Our search strategy included 15 databases, hand searches of additional journals, backwards searches, snowball searches and expert recommendations. We identified and screened 6106 records, mapped 152 and synthesised eight. RESULTS: Overall, the literature was limited and fragmented. Reports produced by official agencies dominated; academic authorship and peer-reviewed outputs were comparatively rare. Few publications met minimum scientific standards. Qualitative designs outweighed quantitative ones. Publications typically described trafficking's problem profile and/or discussed interventions; they rarely assessed trafficking's impacts or evaluated interventions. Even among the key scientific research, the quality of evidence was variable and often low. Particular weaknesses included poor methods reporting, unclear or imprecise results and conclusions not properly grounded in the data. The synthesised studies were all exploratory, also sharing other design features. Common themes identified included: poor treatment of victims; diversity of sectors affected and commonalities among victims; inadequacies of current responses; and barriers to interventions. CONCLUSIONS: There is a lack of high-quality studies into European labour trafficking. Methodological opacity, insufficient rigour and publication in non-indexed locations impede the identification, assessment and synthesis of evidence. Adherence to higher reporting standards would further the field's development and particular research gaps should be addressed.

Refractory Hypotension During Implantation of a 70 mL Total Artificial Heart in a Patient With Pectus Excavatum: A Case Report.

The Syncardia total artificial heart system is the only commercially approved durable device for treating biventricular heart failure patients awaiting heart transplantation. Conventionally, the Syncardia total artificial heart system is implanted based on the distance from the anterior aspect of the 10th thoracic vertebra to the sternum and the patient's body surface area. However, this criterion does not account for chest wall musculoskeletal deformities. This case report describes a patient with a pectus excavatum who developed compression of the inferior vena cava after Syncardia total artificial heart implantation and how transesophageal echocardiography guided chest wall surgery to accommodate the total artificial heart system.

Panel-based next-generation sequencing identifies novel mutations in Bulgarian patients with inherited retinal dystrophies.

BACKGROUND: Next-generation sequencing (NGS)-based method is being used broadly for genetic testing especially for clinically and genetically heterogeneous disorders, such as inherited retinal degenerations (IRDs) but still not routinely used for molecular diagnostics in Bulgaria. Consequently, the purpose of this study was to evaluate the effectiveness of a molecular diagnostic approach, based on targeted NGS for the identification of the disease-causing mutations in 16 Bulgarian patients with different IRDs. METHODS: We applied a customized NGS panel, including 125 genes associated with retinal and other eye diseases to the patients with hereditary retinopathies. RESULTS: Systematic filtering approach coupled with copy number variation analysis and segregation study lead to the identification of 16 pathogenic and likely pathogenic variants in 12/16 (75%) of IRD patients, 2 of which novel (12.5%): ABCA4-c.668delA (p.K223Rfs18) and RР1-c.2015dupA (p.K673Efs*25). Mutations in the ABCA4, PRPH2, USH2A, BEST1, RР1, CDHR1, and RHO genes were detected reaching a diagnostic yield between 42.9% for Retinitis pigmentosa cases and 100% for macular degeneration, Usher syndrome, and cone-rod dystrophy patients. CONCLUSION: Our results confirm the usefulness of targeted NGS approach based on frequently mutated genes as a comprehensive and successful genetic diagnostic tool for IRDs with significant impact on patients counseling.

Processed electroencephalogram response of patients undergoing carotid endarterectomy: a pilot study.

BACKGROUND: A risk during carotid endarterectomy (CEA) is cerebral ischemia as a result of clamping of the carotid artery. The Cerebral State Monitor is one of several electroencephalogram monitors that reflects level of consciousness of patients during anesthesia. The monitor reports level of consciousness with the empirical parameter Cerebral State Index (CSI) on a 0-100 scale. In this patient-controlled prospective pilot study, we compared the CSI measured from the surgical and control hemispheres of patients undergoing CEA surgery to determine whether the parameter is affected by presumed changes in cerebral blood flow. METHODS: Fifteen consenting CEA patients entered this institutionally approved study. We connected a separate Cerebral State Monitor to the left and right sides of the patient's head and recorded CSI intraoperatively. We compared the control-side data with the surgical-side data. RESULTS: Clamping caused the surgical-side CSI to drop below the control-side CSI. Shunt activation caused the CSI to rise above the control. The greatest difference between the surgical-side CSI and control-side CSI occurred at the completion of the CEA procedure. These observed trends were not statistically significant in this pilot project. CONCLUSION: Our preliminary results suggest that the CSI, and perhaps other similar indexes, may indicate changes in cerebral blood flow acutely during CEA. Our findings lend promise to a new use for these types of electroencephalogram monitors, which are prevalent in operating rooms today.

Temporary extracorporeal circulatory support and pulmonary embolectomy for catastrophic amniotic fluid embolism.

Amniotic fluid embolism is usually a life-threatening complication of an otherwise healthy pregnancy. Medical management of the coagulopathy and cardiovascular collapse is challenging and is often unsuccessful. We present a case and advocate the use of temporary circulatory support and pulmonary embolectomy in what would otherwise have been a fatal scenario.

Feasibility and safety of same day subcutaneous defibrillator implantation and send home (DASH) strategy.

PURPOSE: To assess the feasibility and safety of same-day discharge after S-ICD implantation by implementing a specific analgesia protocol and phone follow-up. METHODS: Consecutive patients presenting for outpatient S-ICD implantation were enrolled between 1/1/2018 and 4/30/2019. An analgesia protocol included pre-operative acetaminophen and oxycodone, intraoperative local bupivacaine, and limited use of oxycodone-acetaminophen at discharge. The primary outcome was successful same-day discharge. Numerical Pain Rating Scale (NPRS) on postoperative day (POD) 1, 3, 14, and 30 and any unplanned health care visits during the 1-month follow-up period were assessed. RESULTS: Out of 53 potentially eligible S-ICD patients, 49 patients (92.5%) were enrolled and successfully discharged on the same day. Mean age of these 49 patients was 47 ± 14 years. There were no acute procedural complications. Severe pain (NPRS ≥ 8) on POD 0, 1, and 3 was present in 14.3%, 14.3%, and 8.2% of patients, respectively. The total in-hospital stay was 534 ± 80 min. Four unplanned visits (8%) due to cardiac or device-related issues occurred during 1-month follow-up, including 2 patients with heart failure exacerbation, one patient with an incisional infection, and one patient with inappropriate shocks. CONCLUSIONS: With the appropriate institutional protocol including specific analgesics and phone follow-up, same-day discharge after outpatient S-ICD implantation is feasible and appears safe for most patients.. Device-related pain can be severe in the first 3 days post-implantation and can be successfully treated with limited supply of narcotic medications.

Circulatory parameters in the retrobulbar central retinal artery and vein of patients with diabetes and medically treated systemic hypertension.

BACKGROUND: We aim to study the circulatory parameters in the retrobulbar central retinal artery and vein in diabetic patients with and without medically treated systemic hypertension. METHODS: The study included 108 patients with diabetes that were allocated in four different groups according to the presence of diabetic retinopathy (DR) and hypertension: group 1-patients without DR and without hypertension (n = 23), group 2-patients without DR and with hypertension (n = 21), group 3-patients with nonproliferative DR and without hypertension (n = 36), group 4-patients with nonproliferative DR and with hypertension (n = 28). The circulatory parameters that were evaluated were: peak systolic blood velocity (PSV), end-diastolic blood velocity (EDV), maximum venous velocity (Vmax), minimum venous velocity (Vmin) and the Pourcelot index which were measured using color Doppler imaging. Non-parametric tests were used to test inter-group differences. Spearman's coefficient of correlation was tested between ocular perfusion pressure and the circulatory parameters in each of the patient groups. Contingency table was performed to test the relation of diabetic retinopathy and hypertension to the PSV in the central retinal artery. RESULTS: The PSV and EDV in the central retinal artery was significantly higher in group 1 (p = 0.02, p = 0.04) and group 2 (p = 0.02, p = 0.02) than in group 3. The Pourcelot index in the central retinal vein was significantly lower in group 1 than in group 4 (p = 0.02), and in group 2 than in groups 3 and 4 (p = 0.02, p < 0.01). A significant relationship was detected between the presence of hypertension, the stage of diabetic retinopathy and the PSV in the central retinal artery of our patients (chi(2) = 8.29; p = 0.04). CONCLUSION: Medically treated hypertension affects the retrobulbar circulatory parameters in the central retinal artery and vein in diabetes.

Pulmonary cuff dysfunction after lung transplant surgery: A systematic review of the evidence and analysis of its clinical implications.

BACKGROUND: Pulmonary cuff dysfunction, either due to pulmonary vein obstruction, pulmonary vein stenosis, or pulmonary vein thrombosis, is an uncommon, yet serious complication after lung transplantation. Although there have been numerous reports of its occurrence, there is little consensus regarding the hemodynamic parameters associated with its presentation and diagnostic considerations. This systematic review summarizes the evidence surrounding pulmonary cuff dysfunction after lung transplantation surgery and empirically analyzes its implications. METHODS: Databases were examined for all articles and abstracts reporting on pulmonary cuff dysfunction. Data collected included: number of patients studied; patients' characteristics; incidences of pulmonary vein stenosis and pulmonary vein thrombosis; and timing and imaging modality utilized for diagnosis. RESULTS: Thirty-four full-text citations were included in this review. The point prevalence of pulmonary vein stenosis and thrombosis were 1.4% and 2.5%, respectively. The peak pulmonary cuff velocity associated with dysfunction was found to be 1.59 ± 0.66 m/sec. The diameter of the dysfunctional pulmonary vein was noted to be 0.48 ± 0.20 cm. The majority of diagnoses were made in the early post-operative period using transesophageal echocardiography. Overall, 41.3% of patients (26 of 63) required emergent procedural reintervention, and 32% of patients (20 of 63) diagnosed with pulmonary cuff dysfunction died during their hospital stay. CONCLUSIONS: This systematic review underscores the importance of identifying pulmonary cuff dysfunction after lung transplant surgery, and the usefulness of transesophageal echocardiography for detection of this complication. The clinical implications of these results warrant the further development of identification and management strategies for lung transplant patients.

Discriminatory Power of Superficial Vessel Density and Prelaminar Vascular Flow Index in Eyes With Glaucoma and Ocular Hypertension and Normal Eyes.

Purpose: We evaluate the ability of optical coherence tomography angiography parameters, such as the peripapillary vessel density of the superficial retina and prelaminar flow index of the optic disc (PLFI), to differentiate primary open-angle glaucoma (POAG) and ocular hypertension (OH) from normal eyes. Methods: The vessel density, PLFI, mean deviation of the visual field, circumpapillary retinal nerve fiber layer thickness (cpNFLT), and global loss volume of the ganglion cell complex were evaluated in one eye of 105 subjects with POAG and OH and normal eyes. The discriminatory powers of these parameters were evaluated based on the area under the curve (AUC) of the receiver operation characteristic curve and multiple comparisons. Results: The vessel density (P < 0.001) and PLFI/unit area (PLFI/UA; P = 0.020) in eyes with POAG were significantly less than in normal eyes. The vessel density in eyes with OH was significantly (P = 0.018) reduced, whereas the PLFI/UA, global loss volume and cpNFLT were unaffected. The AUCs of the vessel density to discriminate glaucoma and OH from normal eyes were 0.832 and 0.724, respectively, and were significantly better than the PLFI/UA, in which the AUCs were 0.662 (P = 0.002) and 0.569 (P = 0.038), respectively. The powers of the vessel density and PLFI/UA to discriminate POAG from normal eyes were inferior to the global loss volume (P = 0.006 and <0.0001) and cpNFLT (P = 0.055 and P < 0.0001, respectively). Conclusions: The vessel density and PLFI/UA decreased significantly in glaucomatous eyes. The vessel density was more efficient than the PLFI/UA for differentiating glaucoma and OH from normal eyes.

Perioperative Management of Subarachnoid Hemorrhage in a Patient with Alagille Syndrome and Unrepaired Tetralogy of Fallot: Case Report.

Alagille syndrome (ALGS) is a genetic disorder associated with multisystem dysfunction involving the hepatic, cardiovascular, and neurologic systems. Tetralogy of Fallot (TOF), a congenital cardiac anomaly, is commonly found in these patients. Patients with ALGS may also have an increased risk of cerebrovascular abnormalities and bleeding. Ruptured cerebral aneurysm and subarachnoid hemorrhage (SAH) may be developed, increasing the incidence of morbidity and mortality. Advances in neuroimaging and neurosurgery have allowed early identification and treatment of such vascular abnormalities, improving patients' outcomes and reducing life-threatening complications such as intracranial bleeding. Authors describe the perioperative management of a patient with ALGS and TOF who was admitted to the emergency department due a ruptured intracranial aneurysm with concomitant SAH. Surgical treatment included diagnostic cerebral arteriography with coil embolization of a left posterior communicating artery aneurysm, and placement of right external ventricular drain (EVD). The combination of neuroprotective anesthetic techniques, fast emergence from anesthesia, and maintenance of intraoperative hemodynamic stability led to a successful perioperative management. A multidisciplinary approach in specialized centers is essential for the treatment of patients with SAH, especially in patients with ALGS and complex congenital heart disease such as TOF.

Quantitative Retinal Optical Coherence Tomography Angiography in Patients With Diabetes Without Diabetic Retinopathy.

Purpose: To compare optical coherence tomography (OCT) angiographic parameters in retina and choriocapillaris between control subjects and diabetic patients without diabetic retinopathy (NDR). Correlations were studied between OCT angiography parameters, retinal structure parameters, and systemic characteristics in all subjects. Methods: Sixty-two patients were included in the study: control subjects (n = 33) and patients with NDR (n = 29). Optical coherence topography angiographic parameters were as follows: vessel density (%) (in superficial, deep retinal vessel plexus and in choriocapillary layer) and foveal avascular zone (FAZ) area (mm2) in superficial and deep retinal vessel plexus of parafovea. Split-spectrum amplitude decorrelation angiography (SSADA) software algorithm was used for evaluation of vessel density and FAZ area (nonflow area tool). Spectral-domain OCT was used to assess full, inner, and outer retinal thickness and volume in parafovea. Results: In superficial and deep retina, vessel densities in NDR (44.35% ± 13.31% and 31.03% ± 16.33%) were decreased as compared to control subjects (51.39% ± 13.05%, P = 0.04; and 41.53% ± 14.08%, P < 0.01). Foveal avascular zone in superficial retina of NDR patients (0.37 ± 0.11 mm2) was greater than in controls (0.31 ± 0.10 mm2, P = 0.02). Superficial vessel density significantly correlated with full retinal thickness and volume in parafovea (r = 0.43, P = 0.01; r = 0.43, P = 0.01) and with outer retinal volume in parafovea (r = 0.35, P < 0.05) of healthy subjects. Systolic blood pressure and ocular perfusion pressure significantly correlated with deep vessel density in NDR (r = -0.45, P = 0.02; r = -0.46, P = 0.01), but not in controls. Conclusions: Superficial and deep retinal vessel density in parafovea of diabetic patients without diabetic retinopathy are both decreased compared to healthy subjects. The associations between vessel density with retinal tissue thickness and with subject's clinical characteristics differ between healthy subjects and patients with NDR.