Relative anemia and perioperative stroke in children with moyamoya.

OBJECTIVES: Surgical revascularization for moyamoya arteriopathy decreases long-term stroke risk but carries a risk of perioperative ischemic complications. We aimed to evaluate modifiable stroke risk factors in children undergoing surgical revascularization for moyamoya. MATERIALS AND METHODS: In this exploratory, single-center, retrospective cohort study, medical records of pediatric patients undergoing surgical revascularization for moyamoya arteriopathy at our center between 2003 and 2021 were reviewed. Candidate modifiable risk factors were analyzed for association with perioperative stroke, defined as ischemic stroke ≤7 days after surgery. RESULTS: We analyzed 53 surgeries, consisting of 39 individual patients undergoing indirect surgical revascularization of 74 hemispheres. Perioperative ischemic stroke occurred following five surgeries (9.4%). There were no instances of hemorrhagic stroke. Larger pre-to-postoperative decreases in hemoglobin (OR 3.90, p=0.017), hematocrit (OR 1.69, p=0.012) and blood urea nitrogen (OR 1.83, p=0.010) were associated with increased risk of perioperative ischemic stroke. Weight-adjusted intraoperative blood loss was not associated with risk of perioperative ischemic stroke (OR 0.94, p=0.796). Among children with sickle cell disease, all of whom underwent exchange transfusion within one week prior to surgery, none experienced perioperative stroke. CONCLUSIONS: Decreases in hemoglobin, hematocrit, and blood urea nitrogen between the preoperative and postoperative periods are associated with increased risk of perioperative stroke. These novel findings suggest that dilutional anemia, possibly due to standardly administered hyperhydration, may increase the risk of perioperative stroke in some children with moyamoya. Further work optimizing both mean arterial pressure and oxygen-carrying capacity in these patients, including consideration of alternative blood transfusion thresholds, is necessary.

Transcranial Doppler in Childhood Moyamoya: An Underutilized Tool?

BACKGROUND: Children with moyamoya are at high risk for incident and recurrent stroke. Transcranial Doppler (TCD) ultrasound is an attractive option to screen high-risk populations for moyamoya and to provide stroke risk stratification information due to its safety and cost-effectiveness. We used TCD to evaluate cerebral blood flow velocities in children with presurgical moyamoya and to determine if velocities differ between children with stable and unstable disease. METHODS: Fourteen participants aged ≤21 years with a radiographic diagnosis of moyamoya or moyamoya-like arteriopathy underwent a research TCD at a median age of 7.2 years. TCDs were performed outside of the setting of acute stroke and before surgical revascularization. Arteriopathy was classified as unstable if the participant had a stroke or transient ischemic attack within three months preceding the TCD. RESULTS: Middle cerebral artery and internal carotid artery (ICA) blood flow velocities were elevated. The median M1 velocity was 138 cm/s (interquartile range [IQR] 106 to 168). Individual M1 flow velocities were a median of 5.0 S.D.s above age-based normative values. The median distal ICA velocity was 146 cm/s (IQR 124 to 163). Individual ICA flow velocities were a median of 5.9 S.D.s above normative values. Participants with unstable arteriopathy had higher M1 velocities compared with those with stable arteriopathy (170 vs 119 cm/s, P = 0.0003). We did not identify velocity differences based on comorbid conditions or age. CONCLUSIONS: These preliminary data suggest that TCD is a promising tool for screening for cerebral arteriopathies in high-risk pediatric populations and assessment for unstable disease.

Unclaimed Bodies in Anatomical Education: Medical Student Attitudes at One U.S. Medical Institution.

PHENOMENON: Dissection of cadavers is a common practice in anatomical education. To meet demand for cadavers, some medical institutions facilitate dissection of individuals who did not provide consent during their life. This includes the bodies of individuals who passed away with either no living kin or no kin able to claim and bury their body. Recent literature demonstrates widespread discomfort with this practice among anatomy course directors at U.S. institutions, bringing into question continuation of this practice. However, attitudes among medical students must similarly be assessed as they represent key stakeholders in the dissection process. The purpose of this study was to assess prevailing attitudes among a sample of medical students at one U.S. medical institution regarding the dissection of unclaimed bodies and identify emerging themes in ethical viewpoints. APPROACH: Two-hundred-twelve students (35% response rate) at one U.S. medical institution completed an anonymous online survey. Students came from different class cohorts at various stages of their training. Survey items were developed to capture students' academic and emotional experience with anatomical dissection and to identify emerging themes in attitudes. FINDINGS: Students reported high regard for cadaveric dissection in general with 170 (80%) respondents endorsing it as critical to anatomical education. Regarding dissection of unclaimed bodies, 30% of students found the practice ethical while 47% of students found the practice unethical. Multivariate analysis found that ethical view was directly associated with comfort level (OR= 156.16; 95% CI: 34.04, 716.40). Most students expressed comfort dissecting self-donated bodies (n = 206, 97%), while fewer students expressed comfort dissecting unclaimed bodies (n = 66, 31.1%). This latter finding significantly correlated with gender (t = 3.361. p < 0.05), class cohort (F = 3.576, p < 0.01), but not with religious affiliation or age. Thematic analysis revealed the following themes in student responses: (1) invoking ethical paradigms to either justify or condemn the practice, (2) subjective experiences, and (3) withholding judgment of the practice. INSIGHTS: Many students expressed negative attitudes toward the dissection of unclaimed bodies, with some citing issues of social vulnerability, justice, and autonomy. These findings indicate that many students' ethical code may conflict with institutional policies which permit this practice. Medical school represents a critical time in the professional development of trainees, and development practices which align with the moral code of local institutions and stakeholders is crucial.

Anterior Ear Reconstruction with the Posterior Pull-through Flap.

Background: Defects of the central ear after skin cancer removal remain a common problem for the reconstructive surgeon. The experience with a one-stage, postauricular, skin-island flap passed through the cartilage to reconstruct ear defects is reported. Methods: Patients with an intact ear helix and an anterior full-thickness defect (cartilage defects included) were reconstructed with a skin-island flap based on posterior subcutaneous tissue deep to the flap base. The flap was passed through a generous aperture created in the ear cartilage. The skin at the base was incised superficially after the flap was passed through the cartilage to create a skin-island and avoid burial of epithelium. The flap periphery and postauricular harvest site were sutured with absorbable chromic sutures. Results: Twenty-two patients (six women, 16 men) with ear defects underwent single-stage reconstruction over a 9-year period. The defects reconstructed measured in diameter from 2 cm to 4.5 cm. Six patients required a second skin flap from the preauricular area to close ear canal defects. The pull-through flap added structural support, and prevented ear distortion. No flap necrosis occurred. Venous congestion was common and self-limiting. No epithelial cysts developed. Conclusions: A one-stage postauricular skin-island flap can reliably reconstruct anterior ear defects without distorting shape or position of the ear. Care is needed to provide a generous aperture through the ear cartilage to accommodate the flap. Healing proceeds predictably, and minimal complications are associated with this posteriorly-based, pass-through, skin-island flap in ear reconstruction.

Seizures in Pediatric Moyamoya: Risk Factors and Functional Outcomes.

BACKGROUND: Children with moyamoya arteriopathy are at high risk for stroke and seizures. Risk factors for seizures and the impact of seizures on neurological outcomes in children with moyamoya are unknown. METHODS: This is a single-center retrospective cohort study of children with moyamoya evaluated between 2003 and 2021. Functional outcome was assessed using the Pediatric Stroke Outcome Measure (PSOM). Associations between clinical variables and seizure occurrence were assessed using univariate and multivariable logistic regression. Associations between clinical variables and final PSOM score were assessed using ordinal logistic regression. RESULTS: Eighty-four patients met inclusion criteria, and 34 (40%) children experienced seizure. Factors associated with seizures included moyamoya disease (vs syndrome; odds ratio [OR] 3.43, P = 0.008) and the presence of infarcts on baseline neuroimaging (OR 5.80, P = 0.002). Factors associated with decreased likelihood of experiencing seizures included older age at initial presentation (OR 0.82, P = 0.002) and asymptomatic (radiographic) presentation (OR 0.05, P = 0.006). Both older age at presentation (adjusted OR [AOR] 0.80, P = 0.004) and incidental radiographic presentation (AOR 0.06, P = 0.022) remained significant after adjusting for potential confounders. Seizures were associated with worse functional outcomes as assessed by the PSOM (regression coefficient 2.03, P < 0.001). This association remained significant after adjusting for potential confounders (adjusted regression coefficient 1.54, P = 0.025). CONCLUSIONS: Younger age and symptomatic presentation are associated with increased likelihood of seizures among children with moyamoya. Seizures are associated with worse functional outcomes. Prospective studies should clarify how seizures impact outcomes and how effective seizure treatment modifies this relationship.

Lip-Shortening Surgery.

BACKGROUND: Many changes are associated with the aging face. Upper lip lengthening with atrophy, lip thinning, and diminution of the lip margin are commonly seen. METHODS: Lip-shortening surgery over a 32-year period by a single surgeon is reviewed. A direct surgical excision of the upper lip skin at the base of the nose with an irregular or curvilinear incision was used. RESULTS: Facial aesthetics were improved with this direct surgical approach. A more youthful vermilion border and an increased lip projection were achieved. Lip asymmetry and an improvement in lip dynamics were also observed. A high rate of revision surgery (approximately 25%) was found in this series. The highly visible, delicate, central facial landmarks involved in lip shortening magnify small scar irregularities, and revision, although relatively minor, is often necessary. Patient satisfaction is high, as a subjective improvement in lip aesthetics is readily appreciated. Patients frequently request further shortening. CONCLUSIONS: Surgeons need to review the exigent nature of this surgery with their patients and be willing to perform the associated revisions inherent in the procedure. Lip-shortening surgery reliably improves facial aesthetics and should be used by plastic surgeons when treating the aging face.

Are weight or age limits for pediatric laparoscopic pyeloplasty? Results of a multicentric study.

INTRODUCTION: Anderson-Hynes pyeloplasty is the technique of choice for the treatment of pyeloureteral junction obstruction (PUJO) with an excellent success rate. Minimally invasive surgery has become the standard of care for the management of PUJO in children. Although it has been comparable to the open approach at all levels, its diffusion or employment in younger children has not been widely adopted. Our aim is to evaluate laparoscopic pyeloplasty outcomes from international academic centers in children under 1 year of age, focusing on feasibility and outcomes including possible complications. MATERIALS AND METHODS: This is review of consecutive infants under 1 year of age who underwent laparoscopic pyeloplasty between 2009 and 2018 with more than 12 months of follow-up. Seven different training centers with different backgrounds participated in this study. Evaluation was carried out with ultrasound and renogram before and after surgery. Demographic data, perioperative characteristics, complications, and results are described and analyzed. RESULTS: Over 9 years, 124 transperitoneal laparoscopic Anderson-Hynes pyeloplasties were performed on 123 children under 1 year of age; 88 males and 35 females, with 1 case of bilateral PUJO. Of the 124 renal units, 86 were left-sided. Mean age at surgery was 6.6 months (1 week-12 months), with 56% (n = 70) done before 6 months of age. Mean weight at surgery was 6.8 kg (3-12 kg), with 59% (n = 73) weighing less than 8 kg. Mean operative time (skin-to-skin) was 150 min (75-330 min). After a mean follow-up of 46 months (12-84 months), 12 (9%) patients developed complications, with only 1 needing a redo pyeloplasty also done laparoscopically. One child, with deterioration in renal function, underwent nephrectomy. CONCLUSION: Laparoscopic pyeloplasty under 1 year of age and/or less than 12 kilos is feasible with lower complication rate. Furthermore, age younger than 6 months and weight less than 8 kg are no longer limiting factors for a successful pyeloplasty as shown by this multicentre study.

Characterization of Neuropsychological Outcomes in a Cohort of Pediatric Patients with Moyamoya Arteriopathy.

INTRODUCTION: Moyamoya arteriopathy is a severe, progressive cerebral arteriopathy that places affected children at high risk for stroke. Moyamoya has been associated with a range of neuropsychological deficits in adults, but data on many cognitive domains remain limited in the pediatric population and little is known about the neuropsychological profile of children with syndromic moyamoya. METHODS: This is a single-center, retrospective cohort study of children with moyamoya arteriopathy followed at our center who underwent neuropsychological testing between 2003 and 2021. Test scores were extracted from neuropsychological reports. Medical records were reviewed with attention to individual neuropsychological test results, medical comorbidities, presence of infarct(s) on neuroimaging, and history of clinical ischemic stroke. RESULTS: Of the 83 children with moyamoya followed at our center between 2003 and 2021, 13 had completed neuropsychological testing across multiple cognitive domains. Compared to age-based normative data, children in this sample had lower scores in overall intelligence (p = 0.003), global executive functioning (p = 0.005), and overall adaptive functioning (p = 0.015). There was no significant difference in overall intelligence between children with (n = 6) versus without (n = 7) a history of clinical stroke (p = 0.368), though children with any radiographic infarct scored lower in this domain (p = 0.032). CONCLUSION: In our cohort, children with moyamoya demonstrated impaired intelligence and executive functioning, even in the absence of clinical stroke. Neuropsychological evaluation should be considered standard of care for all children with moyamoya, even those without a history of clinical stroke.

Unique Evaluation and Management Considerations for Adolescents with Late Gynecologic and Colorectal Issues in the Setting of Anorectal Malformations.

STUDY OBJECTIVE: There is little guidance for managing pubertally identified Mullerian anomalies in patients with anorectal malformations (ARMs). We sought to assess these unique issues. DESIGN: Retrospective review SETTING: Single-institution study PARTICIPANTS: Natal female patients aged 10-25, with an ARM, cloaca, or exstrophy, who presented from 2009 to 2019 with a gynecologic concern were included. INTERVENTION: Data collection was performed and included the presenting problem, psychological evaluation, fertility and sexuality concerns, and management strategies for these problems. MAIN OUTCOME MEASURES: The main outcome was unique needs that had to be addressed in the young adult population and the type of colorectal and gynecological procedures needed on representation. RESULTS: Twelve patients were identified; all had gynecologic concerns. Ten had ARMs, including cloaca (n = 3) and cloacal exstrophy (n = 5). Median age at representation was 14.6 years (IQR = 12.7, 15.3). Colorectal revisions included posterior sagittal anorectoplasty (n = 1), resection of bowel attached to urogenital sinus (n = 1), and appendicostomy revision (n = 1). Gynecologic issues included dysmenorrhea (n = 8), obstructed Mullerian anomaly (n = 6), and introital stenosis (n = 5). Behavioral health concerns (n = 9) and fertility/sexuality concerns (n = 4) were identified. Median time from first visit to reconstruction was 1.5 years (IQR = 0.5, 1.5), providing multiple visits to achieve consensus among patients and providers before intervention, including vaginal or introital repair (n = 5) and hysterectomy of obstructed uterine horns (n = 3). CONCLUSIONS: Goal-directed follow-up is required before surgical management to identify psychological and reproductive issues in patients with ARMs who have gynecologic concerns. Patient input and psychologic consultation are helpful for patients requiring staged reconstruction.

Latitudes and attitudes: A multinational study of laparoscopic pyeloplasty in children.

PURPOSE: The Anderson-Hynes technique has been the treatment of choice for primary ureteropelvic junction obstruction in children. Laparoscopic approach has shown similar outcomes to open, with advantages of shorter hospital stay and less pain. We reviewed the experience of 11 geographically diverse, tertiary pediatric urology institutions focusing on the outcomes and complications of laparoscopic pyeloplasty. MATERIALS AND METHODS: A descriptive, retrospective study was conducted evaluating patients undergoing Anderson-Hynes dismembered laparoscopic pyeloplasty. Centers from four different continents participated. Demographic data, perioperative management, results, and complications are described. RESULTS: Over a 9-year period, 744 laparoscopic pyeloplasties were performed in 743 patients. Mean follow-up was 31 months (6-120m). Mean age at surgery was 82 months (1 w-19 y). Median operative time was 177 min. An internal stent was placed in 648 patients (87%). A catheter was placed for bladder drainage in 702 patients (94%). Conversion to open pyeloplasty was necessary in seven patients. Average length of hospital stay was 2.8 days. Mean time of analgesic requirement was 3.2 days. Complications, according to Clavien-Dindo classification, were observed in 56 patients (7.5%); 10 (1%) were Clavien-Dindo IIIb. Treatment failure occurred in 35 cases with 30 requiring redo pyeloplasty (4%) and 5 cases requiring nephrectomy (0.6%). CONCLUSION: We have described the laparoscopic pyeloplasty experience of institutions with diverse cultural and economic backgrounds. They had very similar outcomes, in agreement with previously published data. Based on these findings, we conclude that laparoscopic pyeloplasty is safe and successful in diverse geographics areas of the world.

Antibiotic use for asymptomatic bacteriuria in children with neurogenic bladder.

PURPOSE: Patients with neurogenic bladder (NB) often perform clean intermittent catheterization (CIC) and are predisposed to bladder colonization. Antibiotics are not routinely indicated in those with asymptomatic bacteriuria (ASB). The original purpose of this study was to compare patients that received antibiotics for ASB and those that did not. However, because the non-antibiotic group was very small, the final analysis evaluated treatment patterns of ASB in children with NB. METHODS: A retrospective chart review was completed, including patients who presented with urinary tract infection (UTI) and NB managed by CIC. Patients with symptoms of UTI were excluded. Basic demographics, urinalysis, culture results, and antibiotic prescriptions were collected. RESULTS: The sample included 272 patient encounters for 109 unique patients. Of these, 50.7% were female, and the median age was 10.25 years. More than half the urine cultures (56.2%) grew gram-negative organisms, and 31.3% contained 2 or more organisms. Nearly all encounters received treatment with antibiotics. Twenty-three encounters with no culture performed or the culture resulted in no growth received antibiotic therapy. CONCLUSIONS: Antibiotic resistance and antibiotic stewardship are primary concerns in healthcare today. This organization's current practice pattern shows high antibiotic use for ASB in patients with NB. Future studies are required to identify outcomes associated with treatment versus non-treatment in these patients.

Risk Factors and Imaging Biomarkers Associated With Perioperative Stroke in Pediatric Moyamoya Arteriopathy.

Patients with moyamoya arteriopathy are at high risk for developing ischemic stroke in the perioperative period. We sought to evaluate whether preoperative clinical and neuroimaging biomarkers are associated with postoperative stroke and transient ischemic attack in children with moyamoya following revascularization surgery. We performed a retrospective chart review of pediatric patients who underwent revascularization surgery for moyamoya in the last 15 years. Fifty-three patients who underwent 69 surgeries met the inclusion criteria. We recorded clinical predictors of stroke or transient ischemic attack within 7 days following surgery. We used Suzuki stage and Composite Cerebrovascular Stenosis Score to analyze neuroimaging. Significant risk factors for developing postoperative stroke or transient ischemic attack were younger age at surgery (P = .004) and transient ischemic attack less than 1 month prior to surgery (P < .001). Children under 5 and those with recent preoperative ischemic events should be the focus of investigation to evaluate modifiable risk factors and targeted interventions.

Oligomerization, trans-reduction, and instability of mutant NOTCH3 in inherited vascular dementia.

Cerebral small vessel disease (SVD) is a prevalent disease of aging and a major contributor to stroke and dementia. The most commonly inherited SVD, CADASIL, is caused by dominantly acting cysteine-altering mutations in NOTCH3. These mutations change the number of cysteines from an even to an odd number, but the impact of these alterations on NOTCH3 protein structure remain unclear. Here, we prepared wildtype and four mutant recombinant NOTCH3 protein fragments to analyze the impact of CADASIL mutations on oligomerization, thiol status, and protein stability. Using gel electrophoresis, tandem MS/MS, and collision-induced unfolding, we find that NOTCH3 mutant proteins feature increased amounts of inappropriate disulfide bridges, reduced cysteines, and structural instability. Presence of a second protein factor, an N-terminal fragment of NOTCH3 (NTF), is capable of further altering disulfide statuses of both wildtype and mutant proteins, leading to increased numbers of reduced cysteines and further destabilization of NOTCH3 structure. In sum, these studies identify specific cysteine residues alterations and quaternary structure induced by CADASIL mutations in NOTCH3; further, we validate that reductive factors alter the structure and stability of this small vessel disease protein.

Single institution review of Mini-ACE® low-profile appendicostomy button for antegrade continence enema administration.

PURPOSE: Malone antegrade continence enemas (MACE) provide a conduit in which the patient can achieve improved continence, be clean of stool, and gain independence in maintaining bowel function. The Mini-ACE® is a low-profile balloon button that is used to facilitate the administration of antegrade enemas. We sought to describe our practice and short-term outcomes. METHODS: This work is a retrospective review of the Mini-ACE® appendicostomy button from April 2019 to March 2021, with follow-up concluding in October 2021. Patient demographics, colorectal diagnoses, and outcomes were examined. RESULTS: Forty-three patients underwent Mini-ACE® placement; 22 (51%) were male. The average age at Mini-ACE® insertion was 9.2 years (range 3-20 years). The most common diagnoses were functional constipation in 19 (44%), anorectal malformation in 15 (35%), and Hirschsprung disease in 3 (7%), spinal differences 3 (7%). There were no intra-operative complications, but 5 (12%) required prolapse resection. The median length of stay was two days (IQR 1, 4). Patients achieved self-catheterization at 4.5 [3,7] months from MACE creation, with 38 children (88%) reporting excellent success in remaining clean of stool. CONCLUSION: The Mini-ACE® appears to be a safe and low-profile option for antegrade continence enema access. Further research is needed directly comparing complications and patient satisfaction rates between different MACE devices and overall quality of life. LEVEL OF EVIDENCE: Level IV.

Adolescent Varicocelectomy: Success at What Cost? Clinical Outcome and Cost Comparison of Surgical Ligation and Percutaneous Embolization.

Objectives: Evaluate clinical outcome, recurrence, morbidity, and cost associated with laparoscopic surgical ligation versus percutaneous embolization of adolescent varicocele. We hypothesize that both approaches are similar in outcomes, complications, and cost. Materials and Methods: A retrospective review of 56 consecutive adolescent males, ≤18 years from 2006 to 2016 with clinical varicocele who underwent laparoscopic surgical ligation or percutaneous embolization. Patient demographics, operative time, postoperative complications, success, varicocele grade, recurrence, and hospital charges were abstracted. Results: Mean age was 14.2 ± 2.1 years; 48 (86%) patients having undergone laparoscopic surgical ligation and 8 (14%) percutaneous embolization. Intervention in 45 (80%) patients was for testicular hypotrophy (mean 27.4% ± 15.6%) and 11 (20%) for pain symptomology. Median follow-up was 17.5 months (range 1-65 months). After ligation, 2 (4%) patients developed hydroceles (1 with subsequent hydrocelectomy) and 6 (12%) varicocele recurrence. There were no cases of hydrocele or varicocele recurrence after percutaneous embolization. Twenty ligation patients had postoperative scrotal ultrasound demonstrating an increase in testicular volume by a reduction in difference in testicular volume from 27.3% ± 14.7% preoperatively to 11.2% ± 13.6% postoperatively (P < .001). There was significant difference in mean operative time between the groups (surgical ligation 41.3 minutes versus percutaneous embolization 117.9 minutes, P < .001) and hospital charges for the procedure (surgical ligation $3983 versus percutaneous embolization $18.165, P < .001). Conclusions: Contrary to our hypothesis, percutaneous embolization has seemingly lower rates of postoperative hydrocele and varicocele recurrence in comparison to surgical ligation but with three times the exposure to general anesthesia and at four times the price.

Neonatal Subpial Hemorrhage: Clinical Factors, Neuroimaging, and Outcomes in a Quaternary Care Children's Center.

BACKGROUND: Subpial hemorrhages are underrecognized, underreported, and poorly understood. The spectrum of their clinical manifestations and consequences in neonates has not been fully described. Here, we describe the demographic, clinical, and radiographic characteristics of neonates with subpial hemorrhages. METHODS: We reviewed the medical records and neuroimaging studies of neonates with subpial hemorrhage who were admitted to our neonatal intensive care unit between September 2009 and December 2020. RESULTS: Of 114 neonates with intracranial hemorrhage, 31 (27%) had subpial hemorrhage. The majority of neonates in our cohort were male (68%) and born at term (55%). The most common imaging indication was apneas and/or seizures in 58%. Common comorbid conditions included cardiorespiratory failure (42%), hypoxic-ischemic encephalopathy (26%), and coagulopathy (23%). Subpial hemorrhages were multifocal in 45% of neonates, located in the temporal lobe in 45% of neonates, and tended to be larger in neonates with coagulopathy, birth trauma, or hydrocephalus requiring neurosurgical intervention. Subpial hemorrhage was associated with another type of intracranial bleed in 77% of cases and with arterial ischemic stroke in 16% of cases. Of 17 patients with more than one year of follow-up data, 14 (82%) have developmental delay and four (24%) have epilepsy. Of 14 patients with follow-up imaging, 10 (71%) had encephalomalacia subjacent to the subpial hemorrhage. CONCLUSIONS: This is the largest cohort of neonates with subpial hemorrhages to date. Outcome data are limited by duration of follow-up and may be confounded by comorbid conditions and other concurrent hemorrhages. Further study is needed to define the spectrum of risk factors and expected neurological outcomes.

Hydronephrosis Classifications: Has UTD Overtaken APD and SFU? A Worldwide Survey.

Objective: To collect baseline information on the ultrasonographic reporting preferences. Method: A 13-multiple choice questionnaire was designed and distributed worldwide among pediatric urologists, pediatric surgeons, and urologists. The statistical analysis of the survey data consisted of 3 steps: a univariate analysis, a bivariate and a multivariate analysis. Results: Three hundred eighty participants responded from all the continents. The bivariate analysis showed the significant differences in the geographical area, the years of experience and the volume of cases. Most of the physicians prefer the SFU and APD systems because of familiarity and simplicity (37 and 34%, respectively). Respondents noted that their imaging providers most often report findings utilizing the mild-moderate-severe system or the APD measurements (28 and 39%, respectively) except for North America (SFU in 50%). Multivariate analysis did not provide significant differences. Conclusion: Our study evaluates the opinions regarding the various pediatric hydronephrosis classification systems from a large number of specialists and demonstrates that there is no single preferred grading system. The greatest reported shortcoming of all the systems was the lack of universal utilization. The observations taken from this study may serve as basis for the construction of a common worldwide system. As APD and SFU are the preferred systems and the UTD a newer combination of both, it is possible that with time, UTD may become the universal language for reporting hydronephrosis. This time, based on the result of this survey, seems not arrived yet.

Clinical Presentation and Stroke Incidence Differ by Moyamoya Etiology.

INTRODUCTION: Moyamoya arteriopathy, which can be idiopathic or associated with sickle cell disease, neurofibromatosis, Down syndrome, or cranial radiation therapy, is a progressive cerebral arteriopathy associated with high rates of incident and recurrent stroke. Little is known about how these subgroups differ with respect to clinical presentation, radiographic findings, stroke risk, and functional outcomes. METHODS: Using ICD codes, we identified children ages 28 days to 18 years treated for moyamoya arteriopathy at our tertiary care center between 2003 and 2019. Demographic, clinical, and radiographic data were extracted from the medical record. The Pediatric Stroke Recurrence and Recovery Questionnaire was administered to consenting participants. RESULTS: Sixty-nine patients met inclusion criteria (33 idiopathic, 18 sickle cell disease, 11 neurofibromatosis, 6 Down syndrome, 1 cranial radiation therapy). Median follow-up time was 7.7 years; 24 patients had at least 5 years of follow-up data. Frequency of stroke at presentation differed by subgroup (P < .001). Of patients with at least 2 years of follow-up, 33 (55%) experienced stroke. The proportion of patients experiencing stroke differed by subgroup (50% of idiopathic cases, 72% of sickle cell disease, 11% of neurofibromatosis, and 100% of Down syndrome, P = .003). The frequency of bilateral versus unilateral disease (P = .001) and stroke-free survival following presentation (P = .01) also differed by subgroup. CONCLUSIONS: In this single-center cohort, moyamoya subgroups differed with respect to clinical and radiographic characteristics, with neurofibromatosis-associated moyamoya syndrome having a milder phenotype and Down syndrome-associated moyamoya portending a more aggressive course. These findings need confirmation in a larger, multi-center cohort with longer duration of follow-up.

Health Care in the Age of Mass Incarceration: A Selective Course for Medical Students in Their Preclinical Years.

Introduction: While medical school curricula increasingly address health disparities, content regarding health care for persons impacted by incarceration is a persistent and notable gap. There is a high burden of disease among incarcerated populations, and health care challenges continue postincarceration. We developed a course to introduce medical students to the current landscape of mass incarceration in the US and implications for health and health care delivery to people impacted by this system. Methods: We developed a 3.5-hour elective course taken by 19 first-year medical students in its first year and 20 students in its second. The course utilized lecture, case-based discussion, and guest speaker modalities to introduce students to the history of mass incarceration, health care delivery within the carceral system, and challenges in accessing care during and following incarceration. Results: Students received two surveys after completing the course. In the first, 100% of respondents reported outstanding, excellent, or good levels of satisfaction with various elective components, including organization, learning activities, and student discussion. The second found significant increases in knowledge about mass incarceration and incarceration health issues, in addition to significant increases in interest in advocating or providing health care for incarcerated populations. Discussion: Given current mass incarceration practices, students will encounter patients impacted by this system. This elective course sought to better prepare students to effectively care for these patients. We were limited by time availability, and future directions include incorporating a standardized patient exercise, trauma-informed care principles, and providers working within the carceral system.