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.

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.

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.

Optimization of Pediatric Bowel Management Using an Antegrade Enema Troubleshooting Algorithm.

BACKGROUND: A successful flush is the ability to flush through the appendicostomy or cecostomy channel, empty the flush through the colon, and achieve fecal cleanliness. We evaluated our experience with patients who were having flush difficulties based on a designed algorithm. METHODS: Eight patients with flush difficulties were initially evaluated. Based on the need for additional surgery versus changes in bowel management therapy (BMT), we developed an algorithm to guide future management. The algorithm divided flush issues into before, during, and after flushing. Children aged <20 y who presented with flush issues from September 2018 to August 2019 were evaluated to determine our algorithm's efficacy. Specific outcomes analyzed included changes in BMT versus need for additional surgery. RESULTS: After algorithm creation, 29 patients were evaluated for flush issues. The median age was 8.4 y (interquartile range: 6, 14); 66% (n = 19) were men. Underlying diagnoses included anorectal malformations (n = 17), functional constipation (n = 7), Hirschsprung's disease (n = 2), spina bifida (n = 2), and prune belly (n = 1). A total of 35 flush issues/complaints were noted: 29% before the flush, 9% during the flush, and 63% after the flush. Eighty percent of issues before the flush required surgical intervention, wherease 92% of issues during or after the flush were managed with changes in BMT. CONCLUSIONS: Most flush issues respond to changes in BMT. This algorithm can help delineate which types of flush issues would benefit from surgical intervention and what problems might be present if patients are not responding to changes in their flush regimen.

Laparoscopic vs Open Pyeloplasty in Children: Results of a Randomized, Prospective, Controlled Trial.

PURPOSE: Open dismembered pyeloplasty is the preferred repair for ureteropelvic junction obstruction. Minimally invasive techniques have been applied to the original open approach but no clear advantage has been demonstrated for these technological advances. We evaluate outcomes between transperitoneal laparoscopic and open pyeloplasty in children. MATERIALS AND METHODS: All children 1 to 18 years old with ureteropelvic junction obstruction requiring operative repair were offered enrollment in the study. Patients were prospectively randomized to either laparoscopic or open pyeloplasty through a flank incision. RESULTS: A total of 50 patients in the laparoscopic group and 48 in the open group were enrolled from 2005 to 2014. Mean followup was similar between the groups (13.7 months in the laparoscopic group vs 12.3 months in the open group, p = 0.54). The only significantly different outcomes were for mean operative time, which was 139.5 minutes (range 94 to 213) in the laparoscopic group and 122.5 minutes (83 to 239) in the open group (p <0.01), and mean length of stay, which was 25.9 hours (18 to 143) in the laparoscopic group and 28.2 hours (16 to 73) in the open group (p = 0.02). Analgesic usage, success rate, total charges and all parameters in children older than 11 years were similar between the groups. CONCLUSIONS: Open and laparoscopic dismembered pyeloplasty are comparable and effective methods for repair of ureteropelvic junction obstruction. Although operative time was statistically shorter in the open group and length of stay was shorter in the laparoscopic group, the clinical significance of these variables is questionable. The approach to repair may best be based on family preference for incision aesthetics and surgeon comfort with either approach, rather than more classically objective outcome measures.

Surgical Approaches to Pediatric Ureteropelvic Junction Obstruction.

Approximately 10-30% of infants with hydronephrosis are found to have ureteropelvic junction (UPJ) obstruction. Technological advances in imaging have allowed physicians to better identify the location of the obstruction. The classic repair is the Anderson-Hynes repair which shows a 90-100% success rate and appears superior to many less complex techniques. Is it best to approach this repair through an open incision or laparoscopically with or without a surgical robot? That question remains to be answered and largely depends on how you define "best."

Use of vaginoscopy following vaginoplasty in individuals with congenital adrenal hyperplasia.

STUDY OBJECTIVE: To explore the use of exam under anesthesia with vaginoscopy (EUA-V) following vaginoplasty in patients with congenital adrenal hyperplasia (CAH). DESIGN: Retrospective cohort METHODS: An IRB-approved retrospective chart review was performed of the post-surgical follow-up care of all patients diagnosed with classical CAH who sought care at a tertiary children's hospital from 2000-2017 and had undergone vaginoplasty at that institution. RESULTS: Twenty-six patients met inclusion criteria. The median age at vaginoplasty was 1.25 years (IQR: 0.67, 9). Median post-surgical follow-up was 5.5 years (IQR: 1.5, 9). Twenty six EUA-Vs were performed, on 22 patients. Four were for complication assessment (rectovaginal fistula (1); urethrovaginal fistula (3)-on the same patient). The remaining 22 EUA-Vs, performed on 20 patients with two each undergoing two EUA-Vs, were planned for routine evaluation of vaginal patency. They were done a median of 0.67 years (IQR: 0.25, 2) following surgery and at a median of 6.67 years of age (IQR: 1.75, 12). Of these, five EUA-Vs identified stenosis in five patients (25%), a median of 0.91 years (IQR: 0.5, 7) after surgery, at a median age of 8.42 years (IQR: 2, 10.92). Of the four who did not undergo EUA-V, one had stenosis on clinical exam with ultrasound-verified hematocolpos. The remaining three were all prepubertal at last follow-up. CONCLUSION: The majority of CAH patients following vaginoplasty underwent routine EUA-V, roughly a quarter of which detected stenosis. EUA-Vs may play a beneficial role in routine post-op care following vaginoplasty in patients with CAH.

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.

Using electrospun poly(ethylene-oxide) nanofibers for improved retention and efficacy of bacteriolytic antibiotics.

The aim of this study was to demonstrate targeted delivery of protein-based bactericidal antibiotics using electrospun polymer nanofibers. Previous studies have utilized electrospinning to create nanofibers for the localized delivery of therapeutic agents, including non-steroidal anti-inflammatory drugs (NSAIDs) and low molecular weight heparin. By employing established electrospinning techniques, nanofibers of varying diameters (100-500 nm) were generated from a 0.05 % solution of poly(ethylene-oxide) (PEO) and the antimicrobial peptide, LL-37 was incorporated into the nanofiber meshwork. Initial experiments determined that the strong electric fields caused by electrospinning do not disrupt the antimicrobial properties of LL-37, thus justifying the application of LL-37 as an electrospun component. Disk diffusion assays and especially bacterial filtration studies with E. coli were conducted to quantify the drug delivery potential of the nanofibers. Disk diffusion revealed a small zone of inhibition of about 1 mm around the LL-37-incorporated nanofiber disk. Filtration tests demonstrated that electrospun PEO fibers were capable of delivering LL-37 consistently while still maintaining their antimicrobial abilities.

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.

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.

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.

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.