Vertebral Tortuosity Is Associated With Increased Rate of Cardiovascular Events in Vascular Ehlers-Danlos Syndrome.

Background Arterial tortuosity is associated with adverse events in Marfan and Loeys-Dietz syndromes but remains understudied in Vascular Ehlers-Danlos syndrome. Methods and Results Subjects with a pathogenic COL3A1 variant diagnosed at age <50 years were included from 2 institutions and the GenTAC Registry (National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions). Height-adjusted vertebral artery tortuosity index (VTI-h) using magnetic resonance or computed tomography angiography was calculated. Associations between VTI-h and outcomes of (1) cardiovascular events (arterial dissection/rupture, aneurysm requiring intervention, stroke), or (2) hollow organ collapse/rupture at age <50 years were evaluated using receiver operator curve analysis (using outcome by age 30 years) and mixed-effects Poisson regression for incidence rate ratios. Of 65 subjects (54% male), median VTI-h was 12 (interquartile range, 8-16). Variants were missense in 46%, splice site in 31%, and null/gene deletion in 14%. Thirty-two subjects (49%) had 59 events, including 28 dissections, 5 arterial ruptures, 4 aneurysms requiring intervention, 4 strokes, 11 hollow organ ruptures, and 7 pneumothoraces. Receiver operator curve analysis suggested optimal discrimination at VTI-h ≥15.5 for cardiovascular events (sensitivity 70%, specificity 76%) and no association with noncardiovascular events (area under the curve, 0.49 [95% CI, 0.22-0.78]). By multivariable analysis, older age was associated with increased cardiovascular event rate while VTI-h ≥15.5 was not (incidence rate ratios, 1.79 [95% CI, 0.76-4.24], P=0.185). However, VTI-h ≥15.5 was associated with events among those with high-risk variants <40 years (incidence rate ratios, 4.14 [95% CI, 1.13-15.10], P=0.032), suggesting effect modification by genotype and age. Conclusions Increased arterial tortuosity is associated with a higher incidence rate of cardiovascular events in Vascular Ehlers-Danlos syndrome. Vertebral tortuosity index may be a useful biomarker for prognosis when evaluated in conjunction with genotype and age.

Surgical Techniques for Tonsillectomy and Perioperative Respiratory Complications in Children.

OBJECTIVES: The aim of this study was to determine the incidence of perioperative respiratory complications in children following tonsillectomy with cold and hot dissection surgical techniques. STUDY DESIGN: The study was a retrospective cohort study. SETTING: Retrospective chart review was performed for all children presenting for a tonsillectomy at Texas Children's Hospital from November 2015 to December 2017. METHODS: Pre- and intraoperative patient factors, including surgical technique with cold or hot dissection (electrocautery or radiofrequency ablation), and perioperative anesthetic factors were collected to determine the incidence of perioperative respiratory complications. RESULTS: A total of 2437 patients underwent a tonsillectomy at Texas Children's Hospital from November 2015 to December 2017. The incidence of perioperative respiratory complications was 20.0% (n = 487). Sickle cell disease, cardiac disease, reactive airway disease, pulmonary disease, age >2 and <3 years, and obesity, defined as a body mass index >95th percentile for age, were significant for overall perioperative respiratory complications. There was no difference in the incidence of perioperative respiratory complications in children undergoing tonsillectomy by cold or hot dissection. CONCLUSION: Perioperative respiratory complications following tonsillectomy are more affected by patient factors than surgical technique.

Enhanced Recovery after Surgery: Benefits for the Fetal Surgery Patient.

BACKGROUND: The fetoscopic approach to the prenatal closure of a neural tube defect (NTD) may offer similar advantages to the newborn compared to prenatal open closure of a NTD, with a reduction in maternal risks. Enhanced recovery after surgery (ERAS) protocols have been applied to different surgical procedures with documented advantages. We modified the perioperative care of patients undergoing in utero repair of myelomeningocele with the goal of enhancing the recovery. A retrospective study comparing traditional management to the ERAS protocol was conducted. AIMS: Primary aim was to evaluate the length of stay (LOS). Secondary outcomes included pain scores, time to oral intake, opioid-induced side effects, and respiratory complications. METHODS: Thirty patients who underwent a mid-gestation fetoscopic closure of a NTD were included. Data analyzed include demographics, comorbidities, LOS, anatomical location of the NTD, magnesium sulfate doses and duration of administration, oxygen requirements, duration of the postoperative epidural infusion, duration of surgery and anesthesia, incidence of postoperative nausea and vomiting, respiratory complications, time to oral intake, pain scores, and sedation scores. Differences between the treatment groups were compared using the independent sample t-test or Mann-Whitney Ʋ test. RESULTS: Of the 30 patients, 10 patients were managed according to the ERAS protocol and 20 patients according to the traditional management (1:2 ratio). The mean gestational age at the time of intervention for the traditional and ERAS groups was 24.9 ± 0.5 weeks and 24.8 ± 0.5 weeks, respectively. Compared to the traditional group, the LOS was reduced in the ERAS group to 112.5 ± 12.6 h (4.7 ± 0.5 days) from 179.7 ± 87.9 h (7.5 ± 3.7 days) (p = 0.012). The time to oral intake was also shorter 502.6 ± 473.4 min versus 1015.6 ± 698.2 min; p = 0.049. Oxygen requirements were prolonged in the traditional group (1843.7 ± 1262.6 min vs. 1051.7 ± 1078.1 min p = 0.052). The total duration of magnesium sulfate was longer for patients in the traditional group (2125.6 ± 727.1 min vs. 1429.5 ± 553.8 min; p = 0.006). No statistically significant difference in pain scores was observed between the groups. CONCLUSIONS: Establishing an ERAS protocol for fetoscopic in utero repair of NTDs approach is feasible with the advantages of decreased postoperative LOS, reduced oxygen requirements, lower duration of magnesium sulfate infusion, and facilitation of earlier oral intake without compromising the pain scores.

Lipid temporal trends in normal-weight youth.

Atherosclerosis begins in youth, partly driven by excess weight (EW) and abnormal lipids. Despite pediatric obesity worsening, lipids improved. Given the relation between EW and abnormal lipids, changes in normal-weight (NW) youth may be relevant. We examined the proportions and temporal trends of youth with abnormal lipids who were NW versus EW. METHODS: Analysis was done from National Health and Nutrition Examination Surveys 1988-2016. Data were extracted for 10- to 20-year-olds measured with anthropometrics and laboratory testing to determine proportions of NW versus EW with total cholesterol >190 mg/dL, high-density lipoprotein cholesterol (HDL-C) <40 mg/dL, and calculated non-HDL-C >145 mg/dL (N = 14,785). In survey-weighted regression analysis, a weight-status interaction term was used to examine effect modification in the lipid temporal trend. RESULTS: Over time, EW prevalence increased, whereas dyslipidemia decreased (trend P value < .001 for both). For the pooled sample, EW more than doubled the risk of each lipid disorder (P < .0001 for each). However, for each abnormal lipid, 26%-63% were NW. As the temporal trend in abnormal lipids declined, the proportion with abnormal lipids who were NW also declined. On regression analysis, temporal declines in NW and EW differed for HDL-C. CONCLUSIONS: NW constituted more than a quarter to half of youth with abnormal lipids. Over time, youth with abnormal lipids were less often NW. The novel observation that a high proportion of youth with abnormal lipids are NW is relevant to debates on universal lipid screening, the focus on weight reduction in youth lipid management, and conventional wisdom in cardiometabolic health.