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Objective: Sickle cell disease (SCD) is an inherited hematologic disorder that impacts approximately 100,000 Americans. This disease is associated with progressive organ damage, cerebral vascular accident, and neurocognitive deficits. Recent guidelines from the American Society of Hematology (ASH) recommend cognitive screening with a psychologist to help manage cerebrovascular risk and cognitive impairment in this population. SCD patients benefit from neuropsychology services and several institutions already have programs in place to monitor cognitive risk. Program Description: We describe a longitudinal neurocognitive evaluation program at our institution that serves all patients with SCD, regardless of disease severity or referral question. The Sickle Cell Assessment of Neurocognitive Skills (SCANS) program was established in 2012. We outline the program's theoretical framework, timepoints for evaluation, test battery, logistics, patient demographics, integration with research programming, and multidisciplinary collaboration to support optimal outcomes. Program Outcomes: Our program has provided 716 targeted neuropsychological evaluations for patients over the last decade. Nearly 26% of patients in the program have been followed longitudinally. The most common diagnoses generated across cross-sectional and longitudinal evaluations include cognitive disorder ( n  = 191), attention-deficit/hyperactivity disorder ( n  = 75), and specific learning disorder ( n  = 75). Approximately 87% of patients who participated in SCANS during late adolescence successfully transitioned from pediatric to adult care. Conclusion: We discuss considerations for developing programming to meet the needs of this population, including tiered assessment models, timing of evaluations, scope, and reimbursement. Program models that utilize prevention-based tiered models or targeted evaluations can assist with serving large volumes of patients.

Purpose: Historically, the presence of gray matter heterotopia was a concern for adverse postnatal neurocognitive status in patients undergoing fetal closure of open spinal dysraphism. The purpose of this study was to evaluate neurodevelopmental outcomes and the onset of seizures during early childhood in patients with a prenatal diagnosis of myelomeningocele/myeloschisis (MMC) and periventricular nodular heterotopia (PVNH)., Methods: All patients evaluated at the Center for Fetal Diagnosis and Treatment with a diagnosis of MMC between June 2016 to March 2023 were identified. PVNH was determined from prenatal and/or postnatal MRI. The Bayley Scales of Infant and Toddler Development (edition III or IV) were used for neurodevelopmental assessments. Patients were screened for seizures/epilepsy., Results: Of 497 patients evaluated with a prenatal diagnosis of MMC, 99 were found to have PVNH on prenatal MRI, of which 35 had confirmed PVNH on postnatal imaging. From the 497 patients, 398 initially did not exhibit heterotopia on prenatal MRI, but 47 of these then had confirmed postnatal PVNH. The presence of PVNH was not a significant risk factor for postnatal seizures in early childhood. The average neurodevelopmental scores were not significantly different among heterotopia groups for cognitive, language, and motor domains., Conclusion: The presence of PVNH in patients with a prenatal diagnosis of MMC does not indicate an increased risk for neurodevelopmental delay at 1 year of age. We did not demonstrate an association with seizures/epilepsy. These findings can aid clinicians in prenatal consultation regarding fetal repair of open spinal dysraphism. Long-term follow-up is required to discern the true association between PVNH seen on prenatal imaging and postnatal seizures/epilepsy and neurodevelopmental outcomes., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Executive function, adaptive function, and behavioral outcomes in congenital diaphragmatic hernia (CDH) survivors have not been well studied., Aim: To evaluate executive and neurobehavioral dysfunction in preschool and early school-aged children with CDH., Study Design: Retrospective cohort study., Subjects: All eligible CDH survivors ages 3 to 7 years enrolled in our follow-up program between February 2020 and February 2021., Outcome Measures: The Behavior Rating Inventory of Executive Function (BRIEF), the Adaptive Behavior Assessment System, 2nd Edition (ABAS-II), and the Child Behavior Checklist (CBCL) were used to assess functional and behavioral outcomes. Summary scores were compared to standard population norms., Results: A total of 100 patients were enrolled during the study period. Of those, 73 parents completed at least one of the questionnaires, resulting in completion of the BRIEF, ABAS-II, and CBCL for 63, 68, and 63 patients, respectively. Preschool children had normal executive function (BRIEF-P) while global executive composite (P = 0.012) and the emotional regulation index (P = 0.010) for school age patients (BRIEF-2) were worse. CDH survivors had favorable adaptive functioning (ABAS-II). Mean CBCL scores for preschool attention problems (P = 0.018), school age attention problems (P = 0.001), and attention deficits hyperactivity problems (P = 0.027) were significantly worse. Prematurity, surrogate markers of disease severity, non-white race, and public insurance status were associated with worse neurobehavioral dysfunction in bivariable analysis., Conclusions: The majority of preschool and school age CDH survivors have age-appropriate executive, adaptive and behavioral functioning. CDH survivors, however, have lower executive function and attention scores compared with the general population., Competing Interests: Declaration of competing interest None of the authors has any conflict of interest to disclose., (Copyright © 2023. Published by Elsevier B.V.)

Background: Alar flare reduction (AFR) is a widely used technique in rhinoplasty. Although the impact of AFR on the alar base has been well studied, its effect on the surrounding tissues is largely unknown. This study aims to elucidate the potential effect of AFR on the overall nasal and perinasal anatomy., Methods: AFR was performed on cadavers ( n = 7) with sequential crescent-shaped alar excisions of 2, 4, and 6 mm. Two- and three-dimensional photographs were obtained at baseline and subsequent intervals. Analysis was performed with Adobe Photoshop and Vectra. Standardized landmarks were placed at the nasal tip point (NTP) and alar base point to quantify NTP vector distances, NTP surface distances, and alar crease angle., Results: The surface and vector distances between the NTP and alar base point decreased for increasing AFR intervals. AFR created a surface decrease of 1.90 ± 1.60, 3.54 ± 1.85, and 4.91 ± 1.89 mm, respectively. AFR created a vector decrease of 1.50 ± 1.14, 2.83 ± 1.37, and 3.97 ± 1.38 mm, respectively. NTP projection decreased by 0.54 ± 0.31 mm for 6-mm excision. AFR led to cheek elevation of 0.87 ± 0.70, 1.25 ± 0.60, and 1.96 ± 0.48 mm, respectively. This alar crease elevation blunted the transition between the cheek and upper lip skin at the level of the alar rim with the angle of this transition increasing 26.62 ± 12.78 degrees from baseline to 6 mm., Conclusions: Three-dimensional analysis demonstrates the influence of AFR on the alar base and surrounding perinasal contour. AFR results in nasal tip deprojection, alar crease elevation, and alar flare width narrowing. Further investigation into the impact of modifying the alar base on surrounding structures is warranted., (Copyright © 2023 by the American Society of Plastic Surgeons.)

Children diagnosed with sickle cell disease (SCD) are at risk of the development of neurobehavioural problems early in life. Specific impairments in executive function skills, including working memory, have been documented in school-aged children with SCD. These executive skills are known to strongly contribute to early academic skills and preparedness for entering kindergarten. This study examined working memory and school readiness in preschool children with SCD compared to a healthy control group matched for race, sex and parent education. A total of 84 patients diagnosed with SCD (61.9% haemoglobin [Hb]SS/HbSβ 0 -thalassaemia) and 168 controls completed testing. The mean (SD) ages of patients and controls at testing were 4.53 (0.38) and 4.44 (0.65) years respectively. The SCD group performed worse than controls on measures of executive function, working memory and school readiness (p < 0.01; Cohen's D range: 0.32-0.39). Measures of working memory were associated with school readiness after accounting for early adaptive development. Multiple linear regression models among patients diagnosed with SCD revealed that college education of the primary caregiver was positively associated with school readiness (p < 0.001) after controlling for sex, genotype, age and early adaptive development. These results highlight the need to implement school readiness interventions in young children diagnosed with SCD emphasising executive function skills., (© 2022 British Society for Haematology and John Wiley & Sons Ltd.)

Background: The subnasal lip lift is a surgical technique that elevates the "lip line" (interface between vertical maxillary incisor height and upper lip) to achieve a more youthful aesthetic., Objectives: The authors sought to offer the first ever definition, to their knowledge, of 3-dimensional (3D) changes to the upper lip due to subnasal lip lift., Methods: A lip lift procedure was performed (on cadaveric samples) in a sequential manner from 2.5- to 5.0-mm intervals (n = 13). 3D photographs were taken with the VECTRA H1 system (Canfield Scientific, Fairfield, NJ), and 3D analysis was performed including vermillion height and width, philtral height, sagittal lip projection, vermillion surface area, and incisor show. A subset of samples (n = 9) underwent a modification of the technique by undermining the upper lip subcutaneous tissue off the underlying muscular fascia., Results: Vermillion surface area (baseline range, 1.45-5.52 cm2) increased by an average of 20.5% and 43.1% with 2.5-mm and 5.0-mm lip lift, respectively. Anterior projection of the vermillion increased in all cases by an average of 2.13 and 4.07 mm at 2.5 and 5.0 mm, respectively. Philtral height decreased in all cases by an average of 3.37 and 7.23 mm at 2.5 and 5.0 mm, whereas incisal show increased on average of 1.9 and 4.09 mm, respectively., Conclusions: This study is the first to our knowledge to define the 3D morphometric changes to the upper lip following subnasal lip lift. Quantifying these changes aids the surgeon in preoperative planning and guiding patient expectations., (© The Author(s) 2022. Published by Oxford University Press on behalf of The Aesthetic Society. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

While the focus of craniosynostosis surgery is to improve head shape, neurocognitive sequelae are common and are incompletely understood. Neurodevelopmental problems that children with craniosynostosis face include cognitive and language impairments, motor delays or deficits, learning disabilities, executive dysfunction, and behavioral problems. Studies have shown that children with multiple suture craniosynostosis have more impairment than children with single-suture craniosynostosis. Children with isolated single-suture subtypes of craniosynostosis such as sagittal, metopic, and unicoronal craniosynostosis can have distinct neurocognitive profiles. In this review, we discuss the unique neurodevelopmental profiles of children with single-suture subtypes of craniosynostosis., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Functional abdominal pain (FAP) is a common physical complaint in children and adolescents. Prior research has documented associations between FAP symptoms and mood, especially internalizing behaviors. Limited research is available examining the association between symptom burden and cognitive function in this pediatric population. This study explored associations between FAP symptoms, internalizing behaviors, and cognitive and school function in children and adolescents. Twenty-seven participants (mean age = 12.6 years, range 8.8-16.5; 33% male) diagnosed with FAP completed assessments of cognitive, emotional, and behavioral function, as well as FAP symptom severity. Mean performances on cognitive tests were within age-expected ranges. Within this context, however, higher overall burden of FAP symptoms was associated with slower processing speed, more self-reported metacognitive problems and internalizing behaviors, and more school absences. Cognitive function was systematically associated with internalizing behaviors but not physical symptoms. Overall, findings revealed that FAP may be associated with cognitive inefficiencies in addition to internalizing problems. Cognitive symptoms may be linked to internalizing behaviors associated with FAP.

Introduction: As computer simulation for rhinoplasty continues to rise, the technology's utility extends beyond increasing patient conversion. Virtual simulation of the surgical result can assist with surgical planning and intraoperative decision-making. 3D printed anatomic models or surgical guides based on 3D images may help align surgeons with their original surgical plan. This study aims to evaluate the utility of 3D printed surgical guides as an intraoperative tool to help establish dorsal height and tip position., Methods: Patients undergoing rhinoplasty had preoperative virtual 3D surgical simulations performed. Simulations were used to create a 3D printed nasal kits containing ceramic models of the preoperative nose and simulated nose, sagittal contour guide, and customized postoperative nasal splint. Nasal guides were sterilized for continual intraoperative assessment of profile contour (i.e., dorsal height and tip position). Postoperative 3D images (1-3 months post-op) were then compared to preoperative simulations. The difference between z coordinates and y coordinates determined the difference in projection and rotation, respectively., Results: Fifteen patients met inclusion criteria for this study. With the use of 3D printed surgical guides, the final tip position was on average of 0.8±0.7mm from simulated projection and 0.3±0.2mm from simulated rotation. Similarly, projection for the cartilaginous and bony dorsum was within 1.0±0.8 and 0.8±0.7mm of the simulation, respectively., Conclusion: Virtual simulation is useful in defining aesthetic goals preoperatively, but the potential clinical value extends beyond this. 3D printed rhinoplasty guides extend the simulation's utility to decision-making intraoperatively. This technology offers a novel medium for anatomic reference, which may improve adherence to desired aesthetic goals., Level of Evidence V: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine Ratings, please refer to Table of Contents or online Instructions to Authors www.springer.com/00266 ., (© 2021. Springer Science+Business Media, LLC, part of Springer Nature and International Society of Aesthetic Plastic Surgery.)

Introduction: Sickle cell anemia (SCA) results in numerous adverse effects on the brain, including neurocognitive dysfunction. Hydroxyurea has been utilized extensively for management of SCA, but its effects on brain function have not been established., Methods: We examined prospectively the effects of 1 year of treatment with hydroxyurea on brain function in children with SCA (HbSS/HbSβ 0 -thalassemia) by baseline and exit evaluations, including comprehensive neurocognitive testing, transcranial Doppler ultrasound (TCD), and brain MRI (silent cerebral infarcts [SCI], gray matter cerebral blood flow [GM-CBF], and blood oxygen level-dependent [BOLD] signal from visual stimulation)., Results: Nineteen patients with SCA, mean age 12.4 years (range 7.2-17.8), were evaluated. At baseline, subjects had these mean values: full-scale IQ (FSIQ) 82.8, TCD velocity 133 cm/s, GM-CBF 64.4 ml/100 g/min, BOLD signal 2.34% increase, and frequency of SCI 47%. After 1 year of hydroxyurea, there were increases in FSIQ (+2, p = .059) and reading passage comprehension (+4, p = .033), a significant decrease in TCD velocity (-11 cm/s, p = .007), and no significant changes in GM-CBF, BOLD, or SCI frequency. Hemoglobin F (HbF) was associated with passage comprehension, hemoglobin with lower TCD velocity, and lower GM-CBF with greater working memory. Higher BOLD signal was associated with higher processing speed and lower TCD velocity with higher math fluency., Discussion: Improvements in neurocognition and decreased TCD velocity following 1 year of treatment support hydroxyurea use for improving neurocognitive outcomes in SCA. Understanding the mechanisms of benefit, as indicated by relationships of neurocognitive function with HbF, hemoglobin, and CBF, requires further evaluation., (© 2021 Wiley Periodicals LLC.)

Neurocognitive impairment is common in sickle cell disease (SCD) and is associated with significant functional limitations. In a cross-sectional analysis, we examined the association between hydroxyurea (HU) treatment and neurocognitive functioning from school-age to young adulthood in individuals with SCD. A total of 215 patients with HbSS/HbSβ 0 -thalassaemia (71% HU treated) and 149 patients with HbSC/HbSβ + -thalassaemia (20% HU treated) completed neurocognitive measures at one of four developmental stages: school-age (age 8-9 years), early adolescence (age 12-13 years), late adolescence (age 16-17 years) and young adulthood (ages 19-24 years). For participants with multiple assessments, only the most recent evaluation was included. In multivariable analysis adjusted for social vulnerability, HU treatment and sex, older age was associated with a reduction in overall intelligence quotient (IQ) of 0·55 points per year of life [standard error (SE) = 0·18, false discovery rate adjusted P value (PFDR) = 0.01] for patients with HbSS/HbSβ 0 -thalassaemia. Earlier initiation of HU (n = 152) in HbSS/HbSβ 0 -thalassaemia was associated with higher scores on neurocognitive measures across most domains, including IQ [estimate (SE) 0·77 (0·25)/year, PFDR = 0·01], after adjusting for social vulnerability, sex and treatment duration. These results support the early use of HU to limit the detrimental neurocognitive effects of SCD, while highlighting the need for additional measures to further mitigate neurocognitive deterioration., (© 2021 British Society for Haematology and John Wiley & Sons Ltd.)

Aim: To examine whether anesthesia exposure is associated with neurocognitive decline in pediatric medulloblastoma., Methods: Patients were treated at St. Jude Children's Research Hospital and completed ≥2 protocol-directed neurocognitive assessments (n = 107) as part of a multisite clinical trial for pediatric medulloblastoma (NCT00085202). Patients received risk-adapted craniospinal photon irradiation, followed by four cycles of high-dose chemotherapy and stem cell rescue. Neurocognitive testing was completed at study baseline (after surgery and <2 weeks of starting radiation therapy) and annually for 5 years. Data on anesthesia exposure during treatment was abstracted from medical records., Results: Patients were 10.2 years at diagnosis on average (SD = 4.5; 37% female, 73% average-risk). Mean cumulative anesthesia duration was 20.4 h (SD = 15.2; range 0.7-55.6 h). In the overall group, longer anesthesia duration was associated with greater declines in IQ (Estimate = -0.08, P < 0.001), attention (Estimate = -0.10, P < .001) and processing speed (Estimate = -0.13, P < 0.001). Similar results were shown in subgroups of patients who were <7 years at diagnosis (IQ = -0.14, P = 0.027; Attention = -0.25: P = 0.011), ≥7 years at diagnosis (Attention = -0.07, P = 0.039; Processing Speed = -0.08, P = 0.022), treated for high-risk disease (IQ = -0.09, P = 0.024; Attention = -0.11, P = 0.034; Processing Speed = -0.13, P = 0.001), or treated for average-risk disease (IQ = -0.05, P = .022; Attention = -0.08, P = 0.011; Processing Speed = -0.10, P < 0.001)., Conclusion: Greater anesthesia exposure is a risk factor for clinically significant neurocognitive decline, in addition to factors of age at diagnosis and treatment risk arm. This result is notable as there are evidence-based strategies that can limit the need for anesthesia. Limiting anesthesia exposure, as feasible, may mitigate neurocognitive late effects, and thus, improve quality of life for survivors., Competing Interests: Conflict of interest statement Authors have no conflicts of interest to disclose., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Neurocognitive deficits in sickle cell disease (SCD) may impair adult care engagement. We investigated the relationship between neurocognitive functioning and socio-environmental factors with healthcare transition outcomes. Adolescents aged 15-18 years who had neurocognitive testing and completed a visit with an adult provider were included. Transition outcomes included transfer interval from paediatric to adult care and retention in adult care at 12 and 24 months. Eighty adolescents (59% male, 64% HbSS/HbSβ 0 -thalassaemia) were included. Mean age at adult care transfer was 18·0 (±0·3) years and transfer interval was 2·0 (±2·3) months. Higher IQ (P = 0·02; P FDR  = 0·05) and higher verbal comprehension (P = 0·008; P FDR  = 0·024) were associated with <2 and <6 month transfer intervals respectively. Better performance on measures of attention was associated with higher adult care retention at 12 and 24 months (P = 0·009; P FDR  = 0·05 and P = 0·04; P FDR  = 0·12 respectively). Transfer intervals <6 months were associated with smaller households (P = 0·02; P FDR  = 0·06) and households with fewer children (P = 0·02; P FDR  = 0·06). Having a working parent was associated with less retention in adult care at 12 and 24 months (P = 0·01; P = 0·02 respectively). Lower IQ, verbal comprehension, attention difficulties and environmental factors may negatively impact transition outcomes. Neurocognitive function should be considered in transition planning for youth with SCD., (© 2021 British Society for Haematology and John Wiley & Sons Ltd.)

Introduction: Depressive risk is higher for mothers of infants with chronic medical conditions. The present study examined maternal depressive risk and associations with parent and child outcomes among mothers of young children who were randomized to either prenatal or postnatal surgical closure for myelomeningocele., Methods: Using the Management of Myelomeningocele Study database, maternal depressive risk was examined at 3 time points as follows: prior to birth, 12 months, and 30 months post birth. Separate multivariate analyses examined associations among change in depressive risk (between baseline and 30 months), parenting stress, and child outcomes at 30 months., Results: Mean scores were in the minimal depressive risk range at all the time points. Post birth depressive risk did not differ by prenatal versus postnatal surgery. Mean change scores reflected a decrease in depressive risk during the first 30 months. Only 1.1-4.5% of mothers reported depressive risk in the moderate to severe range across time points. Increased depressive risk during the first 30 months was associated with increased parenting stress scores and slightly lower child cognitive scores at 30 months., Conclusion: Most mothers reported minimal depressive risk that decreased over time, regardless of whether their infant underwent prenatal or postnatal surgery. Only a small percentage of mothers endorsed moderate to severe depressive risk, but an increase in depressive risk over time was associated with higher parental stress and slightly lower child cognitive development., (© 2021 S. Karger AG, Basel.)

Objective: To examine the contribution of anesthesia exposure during treatment for childhood medulloblastoma to neurocognitive outcomes 3 years after tumor diagnosis., Study Design: In this retrospective study, anesthesia data were abstracted from medical records for 111 patients treated with risk-adapted protocol therapy at St Jude Children's Research Hospital. Neurocognitive testing data were obtained for 90.9% of patients., Results: For the 101 patients (62.4% male) who completed testing, mean age at diagnosis was 10.1 years, and 74.3% were staged to have average-risk disease. Anesthesia exposure during treatment ranged from 1 to 52 events (mean = 19.9); mean cumulative duration per patient was 21.1 hours (range 0.7-59.7). Compared with normative expectations (16%), the group had a significantly greater frequency of at-risk scores (<1 SD) on measures of intelligence (28.7%), attention (35.2%), working memory (26.6%), processing speed (46.7%), and reading (25.8%). Including anesthesia exposure duration to linear regression models accounting for age at diagnosis, treatment intensity, and baseline IQ significantly increased the predicted variance for intelligence (r 2  = 0.59), attention (r 2  = 0.29), working memory (r 2  = 0.31), processing speed (r 2  = 0.44), and reading (r 2  = 0.25; all P values <.001)., Conclusions: In survivors of childhood medulloblastoma, a neurodevelopmentally vulnerable population, greater exposure to anesthesia significantly and independently predicts deficits in neurocognitive and academic functioning. When feasible, anesthesia exposure during treatment should be reduced., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Patients with sickle cell disease (SCD) are at increased risk for neurocognitive impairments. While disease-modifying treatment, such as hydroxycarbamide (hydroxyurea), may decrease this risk, it has not been systematically investigated in children with SCD. We screened neurocognitive functioning in 103 adolescents with SCD (16-17 years, 50% female) and compared outcomes between patients with a history of exposure to hydroxycarbamide (n = 12 HbSC/HbSβ + thalassaemia; n = 52 HbSS/HbSβ 0 thalassaemia) and those never treated with hydroxycarbamide (n = 31 HbSC/HbSβ + thalassaemia; n = 8 HbSS/HbSβ 0 thalassaemia). Demographic distributions were similar between the groups. After adjusting for socioeconomic status, the hydroxycarbamide group had significantly higher scores on nonverbal IQ (HbSC/HbSβ thalassaemia: P = 0·036, effect size [d] = 0·65), reaction speed (HbSS/HbSβ 0 thalassaemia: P = 0·002, d = 1·70), sustained attention (HbSS/HbSβ 0 thalassaemia: P = 0·014, d = 1·30), working memory (HbSC/HbSβ + thalassaemia: P = 0·034, d = 0·71) and verbal memory (HbSC/HbSβ + thalassaemia: P = 0·038, d = 0·84) when compared to those who did not receive hydroxycarbamide. In patients with HbSS/HbSβ 0 thalassaemia, longer treatment duration with hydroxycarbamide was associated with better verbal memory (P = 0·009) and reading (P = 0·002). Markers of hydroxycarbamide effect, including higher fetal haemoglobin (HbF), higher mean corpuscular volume (MCV) and lower white blood cell count (WBC), were associated with better verbal fluency (HbF: P = 0·014, MCV: P = 0·006, WBC: P = 0·047) and reading (MCV: P = 0·021, WBC: P = 0·037). Cognitive impairment may be mitigated by exposure to hydroxycarbamide in adolescents with SCD., (© 2020 British Society for Haematology and John Wiley & Sons Ltd.)

Background: Autologous fat grafting is an increasingly preferred method for aesthetic facial rejuvenation. The authors' group previously described the concept of "lipotopography" as topographic surface changes that occur with fat grafting to discrete facial fat compartments. The purpose of this study was to define the "augmentation zone" of the lateral suborbicularis oculi fat compartment to understand the topographical surface changes following augmentation., Methods: Nine cadaver hemifaces were injected with fat analogue at intervals from 1 to 4 cc. Three-dimensional photographs were taken at baseline and following each 1-cc incremental injection. The interval surface changes were calculated using three-dimensional software including perimeter, diameter, and projection., Results: The augmentation zone of the lateral suborbicularis oculi fat compartment was characterized by a consistent shape and boundary. The shape was an elongated oval bound superiorly by the lid-cheek junction and inferiorly at the level of the zygomaticocutaneous ligament. Vertical and horizontal diameter and perimeter showed initial increases between 1 and 2 cc and then a plateau between 2 and 3 cc. Projection changes demonstrated an initial slow increase from 1 to 2 cc injection followed by nearly linear growth from 2 to 4 cc., Conclusions: Three-dimensional photography and computer analysis provide tools to understand the surface anatomy change in response to fat grafting specific facial fat compartments. Targeted volumization of the lateral suborbicularis oculi fat compartment also results in a unique surface change with consistent shape and anatomical boundaries. The lid-cheek junction and zygomaticocutaneous ligament were observed to restrict the expansion of fat analogue for all injection volumes., Clinical Question/level of Evidence: Therapeutic, V.

This paper describes some of the more common patterns in neurobehavioral deficits and their underlying neuroanatomical basis in myelomeningocele (MMC). Patients with MMC can face a lifetime of specific organ system dysfunction, chief among them spinal cord malformations, orthopedic issues, hydrocephalus, and urological disabilities. In addition, patients can experience specific patterns of neurobehavioral difficulties due to the changes in neuroanatomy associated with the open spinal defect. Although there is variability in these patterns, some trends have been described among MMC patients. It is thought that early recognition of these potential neurobehavioral deficits by treating neurosurgeons and other members of the treatment team could lead to earlier intervention and positively impact the overall outcome for patients. Neurodevelopmental and neurobehavioral follow-up assessments are recommended to help guide planning for relevant treatments or accommodations.

Purpose: Sensorineural hearing loss (SNHL) is associated with intellectual and academic declines in children treated for embryonal brain tumors. This study expands upon existing research by examining core neurocognitive processes that may result in reading difficulties in children with treatment-related ototoxicity., Patients and Methods: Prospectively gathered, serial, neuropsychological and audiology data for 260 children and young adults age 3 to 21 years (mean, 9.15 years) enrolled in a multisite research and treatment protocol, which included surgery, risk-adapted craniospinal irradiation (average risk, n = 186; high risk, n = 74), and chemotherapy, were analyzed using linear mixed models. Participants were assessed at baseline and up to 5 years after diagnosis and grouped according to degree of SNHL. Included were 196 children with intact hearing or mild to moderate SNHL (Chang grade 0, 1a, 1b, or 2a) and 64 children with severe SNHL (Chang grade 2b or greater). Performance on eight neurocognitive variables targeting reading outcomes (eg, phonemics, fluency, comprehension) and contributory cognitive processes (eg, working memory, processing speed) was analyzed., Results: Participants with severe SNHL performed significantly worse on all variables compared with children with normal or mild to moderate SNHL ( P ≤ .05), except for tasks assessing awareness of sounds and working memory. Controlling for age at diagnosis and risk-adapted craniospinal irradiation dose, performance on the following four variables remained significantly lower for children with severe SNHL: phonemic skills, phonetic decoding, reading comprehension, and speed of information processing ( P ≤ .05)., Conclusion: Children with severe SNHL exhibit greater reading difficulties over time. Specifically, they seem to struggle most with phonological skills and processing speed, which affect higher level skills such as reading comprehension.

Background: Previous natural history studies have advanced the understanding of sickle cell disease (SCD), but generally have not included sufficient lifespan data or investigation of the role of genetics in clinical outcomes, and have often occurred before the widespread use of disease-modifying therapies, such as hydroxyurea and chronic erythrocyte transfusions. To further advance knowledge of SCD, St. Jude Children's Research Hospital established the Sickle Cell Clinical Research and Intervention Program (SCCRIP), to conduct research in a clinically evaluated cohort of individuals with SCD across their lifetime., Procedures: Initiated in 2014, the SCCRIP study prospectively recruits patients diagnosed with SCD and includes retrospective and longitudinal collection of clinical, neurocognitive, geospatial, psychosocial, and health outcomes data. Biological samples are banked for future genomics and proteomics studies. The organizational structure of SCCRIP is based upon organ/system-specific working groups and is opened to the research community for partnerships., Results: As of August 2017, 1,044 (92.3% of eligible) patients with SCD have enrolled in the study (860 children and 184 adults), with 11,915 person-years of observation. Population demographics included mean age at last visit of 11.3 years (range 0.7-30.1), 49.8% females, 57.7% treated with hydroxyurea, 8.5% treated with monthly transfusions, and 62.9% hemoglobin (Hb) SS or HbSB 0 -thalassemia, 25.7% HbSC, 8.4% HbsB + -Thalassemia, 1.7% HbS/HPFH, and 1.2% other., Conclusions: The SCCRIP cohort will provide a rich resource for the conduct of high impact multidisciplinary research in SCD., (© 2018 Wiley Periodicals, Inc.)

Autologous fat grafting is an important tool for plastic surgeons treating the aging face. Malar augmentation with fat is often targeted to restore the youthful facial contour and provides support to the lower eyelid. The existence of distinct facial fat compartments suggests that a stepwise approach may be appropriate in this regard. The authors describe a three-step approach to malar augmentation using targeted deep malar fat compartmental augmentation, termed the "boomerang lift." Clinical patients undergoing autologous fat grafting for malar augmentation were injected in three distinct deep malar fat compartments: the lateral sub-orbicularis oculi fat, the medial sub-orbicularis oculi fat, and the deep medial cheek (n = 9). Intraoperative three-dimensional images were taken at baseline and following compartmental injections (Canfield VECTRA H1). Images were overlaid between the augmented and baseline captures, and the three-dimensional surface changes were analyzed, which represented the resulting "augmentation zone." Three-dimensional analysis demonstrated a unique pattern for the augmentation zone consistent across patients. The augmentation zone resembled a boomerang, with the short tail supporting the medial lower lid and the long tail extending laterally along the zygomatic arch. The upper border was restricted by the level of the nasojugal interface, and the lower border was defined medially by the nasolabial fold and laterally by the level of the zygomaticocutaneous ligament. Lateral and medial sub-orbicularis oculi fat injections defined the boundaries of the boomerang shape, and injection to the deep medial cheek provided maximum projection. This is the first description of deep malar augmentation zones in clinical patients. Three-dimensional surface imaging was ideal for analyzing the surface change in response to targeted facial fat grafting. The authors' technique resulted in a reproducible surface shape, which they term the boomerang lift.

Background: Patients treated for medulloblastoma who experience posterior fossa syndrome (PFS) demonstrate increased risk for neurocognitive impairment at one year post diagnosis. The aim of the study was to examine longitudinal trajectories of neuropsychological outcomes in patients who experienced PFS compared with patients who did not., Methods: Participants were 36 patients (22 males) who experienced PFS and 36 comparison patients (21 males) who were matched on age at diagnosis and treatment exposure but did not experience PFS. All patients underwent serial evaluation of neurocognitive functioning spanning 1 to 5 years post diagnosis., Results: The PFS group demonstrated lower estimated mean scores at 1, 3, and 5 years post diagnosis on measures of general intellectual ability, processing speed, broad attention, working memory, and spatial relations compared with the non-PFS group. The PFS group exhibited estimated mean scores that were at least one standard deviation below the mean for intellectual ability, processing speed, and broad attention across all time points and for working memory by 5 years post diagnosis. Processing speed was stable over time. Attention and working memory declined over time. Despite some change over time, caregiver ratings of executive function and behavior problem symptoms remained within the average range., Conclusion: Compared with patients who do not experience PFS, patients who experience PFS exhibit greater neurocognitive impairment, show little recovery over time, and decline further in some domains. Findings highlight the particularly high risk for long-term neurocognitive problems in patients who experience PFS and the need for close follow-up and intervention., (© The Author(s) 2017. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com)

Background: Treatment of pediatric medulloblastoma is associated with known neurocognitive deficits that we hypothesize are caused by microstructural damage to frontal white matter (WM)., Methods: Longitudinal MRI examinations were collected from baseline (after surgery but before therapy) to 36 months in 146 patients and at 3 time points in 72 controls. Regional analyses of frontal WM volume and diffusion tensor imaging metrics were performed and verified with tract-based spatial statistics. Age-adjusted, linear mixed-effects models were used to compare patient and control images and to associate imaging changes with Woodcock-Johnson Tests of Cognitive Abilities., Results: At baseline, WM volumes in patients were similar to those in controls; fractional anisotropy (FA) was lower bilaterally (P < 0.001) and was associated with decreased Processing Speed (P = 0.014) and Broad Attention (P = 0.025) performance at 36 months. During follow-up, WM volumes increased in controls but decreased in patients (P < 0.001) bilaterally. Smaller WM volumes in patients at 36 months were associated with concurrent decreased Working Memory (P = 0.026) performance., Conclusions: Lower FA in patients with pediatric medulloblastoma compared with age-similar controls indicated that patients suffer substantial acute microstructural damage to supratentorial frontal WM following surgery but before radiation therapy or chemotherapy. Additionally, this damage to the frontal WM was associated with decreased cognitive performance in executive function 36 months later. This early damage also likely contributed to posttherapeutic failure of age-appropriate WM development and to the known association between decreased WM volumes and decreased cognitive performance., (© The Author(s) 2017. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com)

Throughout history, the technological advancements of conventional clinical photography in plastic surgery have not only refined the methods available to the plastic surgeon, but have invigorated the profession through technology. The technology of the once traditional two-dimensional photograph has since been revolutionized and refashioned to incorporate novel applications, which have since become the standard in clinical photography. Contrary to traditional standardized two-dimensional photographs, three-dimensional photography provides the surgeon with an invaluable volumetric and morphologic analysis by demonstrating true surface dimensions both preoperatively and postoperatively. Clinical photography has served as one of the fundamental objective means by which plastic surgeons review outcomes; however, the newer three-dimensional technology has been primarily used to enhance the preoperative consultation with surgical simulations. The authors intend to familiarize readers with the notion that three-dimensional photography extends well beyond its marketing application during surgical consultation. For the cosmetic surgeon, as the application of three-dimensional photography continues to mature in facial plastic surgery, it will continue to bypass the dated conventional photographic methods plastic surgeons once relied on. This article reviews a paradigm shift and provides a historical review of the fascinating evolution of photography in plastic surgery by highlighting the clinical utility of three-dimensional photography as an adjunct to plastic and reconstructive surgery practices. As three-dimensional photographic technology continues to evolve, its application in facial plastic surgery will provide an opportunity for a new objective standard in plastic surgery.

Lymphedema is a chronic, progressive condition caused by an imbalance of lymphatic flow. Upper extremity lymphedema has been reported in 16-40% of breast cancer patients following axillary lymph node dissection. Furthermore, lymphedema following sentinel lymph node biopsy alone has been reported in 3.5% of patients. While the disease process is not new, there has been significant progress in the surgical care of lymphedema that can offer alternatives and improvements in management. The purpose of this review is to provide a comprehensive update and overview of the current advances and surgical treatment options for upper extremity lymphedema., (© 2016 Wiley Periodicals, Inc.)

Unlabelled: Surgical markings play a crucial role in the planning of plastic surgery procedures. However, despite their importance, they are often imprecise. For instance, when assessing patients in need of autologous fat grafting, surgeons often base markings on estimations of where volume deficiency exists and how much volume will correct the deficiency. In this article, the authors describe a novel approach to lipostructure, guided by a computer-based roadmap. A digital three-dimensional topographic surgical map is created using three-dimensional photography and analytic software and then projected as an image onto the patient in the operating room. This unique concept can be applied to most soft-tissue procedures in plastic surgery., Clinical Question/level of Evidence: Therapeutic, V.

Background: Given the widespread use of facial fillers and recent identification of distinct facial fat compartments, a better understanding of three-dimensional surface changes in response to volume augmentation is needed. Advances in three-dimensional imaging technology now afford an opportunity to elucidate these morphologic changes for the first time., Methods: A cadaver study was undertaken in which volumization of the deep medial cheek compartment was performed at intervals up to 4 cc (n = 4). Three-dimensional photographs were taken after each injection to analyze the topographic surface changes, which the authors define as the "augmentation zone." Perimeter, diameter, and projection were studied. The arcus marginalis of the inferior orbit consistently represented a fixed boundary of the augmentation zone, and additional cadavers underwent similar volumization following surgical release of this portion of the arcus marginalis (n = 4). Repeated three-dimensional computer analysis was performed comparing the augmentation zone with and without arcus marginalis release., Results: Volumization of the deep medial cheek led to unique topographic changes of the malar region defined by distinct boundaries. Interestingly, the cephalic border of the augmentation zone was consistently noted to be at the level of the arcus marginalis in all specimens. When surgical release of the arcus marginalis was performed, the cephalic border of the augmentation zone was no longer restricted., Conclusions: Using advances in three-dimensional photography and computer analysis, the authors demonstrate characteristic surface anatomy changes in response to volume augmentation of facial compartments. This novel concept of the augmentation zone can be applied to volumization of other distinct facial regions., Clinical Question/level of Evidence: Therapeutic, V.

We observed gendered coping strategies and conflict resolution outcomes used by adolescents and parents during a conflict discussion task to evaluate associations with current and later adolescent psychopathology. We studied 137 middle- to upper-middle-class, predominantly Caucasian families of adolescents (aged 11-16 years, 65 males) who represented a range of psychological functioning, including normative, subclinical, and clinical levels of problems. Adolescent coping strategies played key roles both in the extent to which parent-adolescent dyads resolved conflict and in the trajectory of psychopathology symptom severity over a 2-year period. Gender-prototypic adaptive coping strategies were observed in parents but not youth, (i.e., more problem solving by fathers than mothers and more regulated emotion-focused coping by mothers than fathers). Youth-mother dyads more often achieved full resolution of conflict than youth-father dyads. There were generally not bidirectional effects among youth and parents' coping across the discussion except boys' initial use of angry/hostile coping predicted fathers' angry/hostile coping. The child was more influential than the parent on conflict resolution. This extended to exacerbation/alleviation of psychopathology over 2 years: higher conflict resolution mediated the association of adolescents' use of problem-focused coping with decreases in symptom severity over time. Lower conflict resolution mediated the association of adolescents' use of angry/hostile emotion coping with increases in symptom severity over time. Implications of findings are considered within a broadened context of the nature of coping and conflict resolution in youth-parent interactions, as well as on how these processes impact youth well-being and dysfunction over time.

Silent cerebral infarctions (SCI) are the most common neurological injury in children with sickle cell anaemia (SCA), but their incidence/prognosis in early childhood has not been well described. We report clinical, neuroradiological, psychometric and academic follow-up over an average period of 14 years in 37 children with SCA who had magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain between ages 7 and 48 months. Ten patients (27%) younger than age 5 years (Group I) had SCI, as did 12 (32%) older than 5 years (Group II). Fifteen (41%) had no lesions (Group III). Overt stroke or transient ischaemic attack occurred in 5/9 (56%) in Group I. Most Group I patients had progressive MRI abnormalities, concurrent stenosis, decreased cognitive ability, attention/executive function deficits and hindered academic attainment. The proportions of subjects in Group I with subsequent neurological events (P ≤ 0·006), progressive ischaemia (P ≤ 0·001) and vascular stenosis (P ≤ 0·006) were greater than in Groups II and III. Thus, SCI in young children with SCA may predict overt central nervous system events, progressive MRI abnormalities, stenosis, cognitive dysfunction and poor academic performance. Children younger than 5 years may benefit from MRI/MRA testing and should be considered for aggressive intervention when SCI are detected., (© 2015 John Wiley & Sons Ltd.)

Background: A phase II trial of conformal radiotherapy (CRT) for pediatric high-grade glioma (HGG) was performed to evaluate disease control and late effects., Methods: Between July 1997 and January 2003, 34 pediatric patients (median age, 13.2 ± 6.7 years) with HGG were enrolled on an International Commission on Radiation Units and Measurements Report 50-compliant prospective trial using CRT with a 2 cm clinical target volume margin. Baseline and serial evaluations were performed to assess functional outcomes., Results: Median follow-up for the entire group was 18 months (range, 2-134 months). Twenty (58.8%) patients developed local progression, and 6 (17.6%) patients developed distant progression. Progression-free and overall survival at 10 years were 18.8% ± 6.9% and 16.8% ± 6.5%, respectively. At baseline, 40% of patients evaluated for intelligence quotient (IQ) scored below 85. Measures of cognitive function obtained during the first 12 months fit a mixed model with a quadratic function. The relationship between IQ and time was -1.1883 points/month for the linear term and 0.07728 points/month for the quadratic term (P = .0454). IQ decreased between baseline and 6 months and then increased slightly through 12 months. The opposite was found for (all P values of the quadratic term) activities of daily living (P = .0155), socialization (P = .0049), and the composite score (P = .0257) of adaptive behavior., Conclusion: CRT using a 2 cm clinical target volume margin in pediatric HGG demonstrated tumor control comparable to conventional radiation therapy. Disrupted cognitive and adaptive behavioral functioning were present at baseline and throughout the course of disease., (© The Author(s) 2014. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)