Purpose Today, male and female adult and pediatric cancer patients, individuals transitioning between gender identities, and other individuals facing health extending but fertility limiting treatments can look forward to a fertile future. This is, in part, due to the work of members associated with the Oncofertility Consortium. Methods The Oncofertility Consortium is an international, interdisciplinary initiative originally designed to explore the urgent unmet need associated with the reproductive future of cancer survivors. As the strategies for fertility management were invented, developed or applied, the individuals for who the program offered hope, similarly expanded. As a community of practice, Consortium participants share information in an open and rapid manner to addresses the complex health care and quality-of-life issues of cancer, transgender and other patients. To ensure that the organization remains contemporary to the needs of the community, the field designed a fully inclusive mechanism for strategic planning and here present the findings of this process. Results This interprofessional network of medical specialists, scientists, and scholars in the law, medical ethics, religious studies and other disciplines associated with human interventions, explore the relationships between health, disease, survivorship, treatment, gender and reproductive longevity. Conclusion The goals are to continually integrate the best science in the service of the needs of patients and build a community of care that is ready for the challenges of the field in the future.
PURPOSE:Endoscopic sinus surgery (ESS) is typically guided under preoperative computed tomography (CT), which increasingly diverges from actual patient anatomy as the surgery progresses. Studies have reported that the revision surgery rate in ESS ranges between 28 and 47%. This paper presents a method that can update the preoperative CT in real time to improve surgical completeness in ESS.APPROACH:The work presents and compares three novel methods that use instrument motion data and anatomical structures to predict surgical modifications in real time. The methods use learning techniques, such as nonparametric filtering and Gaussian process regression, to correlate surgical modifications with instrument tip positions, tip trajectories, and instrument shapes. Preoperative CT image sets are updated with modification predictions to serve as a virtual intraoperative CT.RESULTS:The three methods were compared in eight ESS cadaver cases, which were performed by five surgeons and included the following representative ESS operations: maxillary antrostomy, uncinectomy, anterior and posterior ethmoidectomy, and sphenoidotomy. Experimental results showed accuracy metrics that were clinically acceptable with dice similarity coefficients > 86%, with F-score > 92% and precision > 89.91% in surgical completeness evaluation. Among the three methods, the tip trajectory-based estimator had the highest precision of 96.87%.CONCLUSIONS:This work demonstrated that virtually modified intraoperative CT scans improved the consistency between the actual surgical scene and the reference model, and could lead to improved surgical completeness in ESS. Compared to actual intraoperative CT scans, the proposed method has no impact on existing surgical protocols, does not require extra hardware, does not expose the patient to radiation, and does not lengthen time under anesthesia.
Ultrasonography may reliably visualize both appropriately positioned and malpositioned femoral-approach catheter tips. Radiography may be used to confirm catheter tip position after placement, but its utility following intraprocedural ultrasound (US) catheter tip verification is unclear. To report the utility of confirmatory radiographs after US-guided tunneled femoral central venous catheter (CVC) placements by interventional radiology in pediatric patients. A total of 484 pediatric patients underwent bedside US-guided tunneled femoral CVC placements in an intensive care setting at a single tertiary children’s hospital between Jan. 1, 2016, and April 20, 2020. Technical success, adverse events, post-procedure radiographic practices and inter-modality catheter tip concordance were recorded. All radiographs were performed within 12 h of catheter placement. The mean patient age was 175±508 days (range: 1 day to 19 years), including 257 (53.1%) males and 227 (46.9%) females. Of the 484 attempted placements, 472 (97.5%) were primary placements. Four hundred eighty-one (99.4%) placements were technically successful. There were three (0.6%) technical failures due to previously undiagnosed iliofemoral venous occlusive disease. Five (1.0%) adverse events occurred. Radiographs were obtained within 12 h of CVC placement in 171 (35.3%) patients, in 120 (70.2%) of whom the indication was recent catheter placement. All 171 (100%) post-placement radiographs showed catheter tip location concordance with the intra-procedural US. In one (0.2%) patient, in whom there was nonvisualization of a guidewire and clinical concern for malposition during US-guided placement, post-procedure radiographs, coupled with multiplanar venography, demonstrated inadvertent paravertebral venous plexus catheter placement. The concordance between intra-procedural US and confirmatory post-procedure radiographs of CVC placements by interventional radiology obviates the need for routine radiographs. Radiographs may be obtained in instances of proceduralist uncertainty or clinical concern.
Background Infants with cystic fibrosis (CF) suffer from gastrointestinal (GI) complications, including pancreatic insufficiency and intestinal inflammation, which have been associated with impaired nutrition and growth. Recent evidence identified altered fecal microbiota taxonomic compositions in infants with CF relative to healthy infants that were characterized by differences in the abundances of taxa associated with GI health and nutrition. Furthermore, these taxonomic differences were more pronounced in low length infants with CF, suggesting a potential link to linear growth failure. We hypothesized that these differences would entail shifts in the microbiome's functional capacities that could contribute to inflammation and nutritional failure in infants with CF. Results To test this hypothesis, we compared fecal microbial metagenomic content between healthy infants and infants with CF, supplemented with an analysis of fecal metabolomes in infants with CF. We identified notable differences in CF fecal microbial functional capacities, including metabolic and environmental response functions, compared to healthy infants that intensified during the first year of life. A machine learning-based longitudinal metagenomic age analysis of healthy and CF fecal metagenomic functional profiles further demonstrated that these differences are characterized by a CF-associated delay in the development of these functional capacities. Moreover, we found metagenomic differences in functions related to metabolism among infants with CF that were associated with diet and antibiotic exposure, and identified several taxa as potential drivers of these functional differences. An integrated metagenomic and metabolomic analysis further revealed that abundances of several fecal GI metabolites important for nutrient absorption, including three bile acids, correlated with specific microbes in infants with CF. Conclusions Our results highlight several metagenomic and metabolomic factors, including bile acids and other microbial metabolites, that may impact nutrition, growth, and GI health in infants with CF. These factors could serve as promising avenues for novel microbiome-based therapeutics to improve health outcomes in these infants.
Abstract Background Spinal fusion surgery is associated with severe acute postsurgical pain and high rates of chronic postsurgical pain in adolescents. Psychological distress, sleep disturbance, and low pain self-efficacy predict higher acute pain and likelihood of developing chronic postsurgical pain. Interventions targeting baseline psychosocial risk factors have potential to interrupt a negative trajectory of continued pain and poor health-related quality of life (HRQL) over time but have not yet been developed and evaluated. This randomized controlled trial will test effectiveness of a digital peri-operative cognitive-behavioral intervention (SurgeryPalTM) vs. education-control delivered to adolescents and their parents to improve acute and chronic pain and health outcomes in adolescents undergoing spine surgery. Methods Adolescents 12–18 years of age undergoing spinal fusion for idiopathic conditions, and their parent, will be recruited from pediatric centers across the USA, for a target complete sample of 400 dyads. Adolescents will be randomized into 4 study arms using a factorial design to SurgeryPalTM or education control during 2 phases of treatment: (1) pre-operative phase (one-month before surgery) and (2) post-operative phase (1 month after surgery). Acute pain severity and interference (primary acute outcomes) and opioid use will be assessed daily for 14 days following hospital discharge. Chronic pain severity and interference (primary acute outcomes), as well as HRQL, parent and adolescent distress, sleep quality, and opioid use/misuse (secondary outcomes), will be assessed at 3 months and 6 months post-surgery. Discussion Demonstration of effectiveness and understanding optimal timing of perioperative intervention will enable implementation of this scalable psychosocial intervention into perioperative care. Ultimately, the goal is to improve pain outcomes and reduce reliance on opioids in adolescents after spine surgery. Trial registration NCT04637802 ClinicalTrials.gov. Registered on November 20, 2020
Retrospective studies have demonstrated the efficacy and safety of pediatric and adolescent transjugular intrahepatic portosystemic shunt (TIPS), but long-term outcomes warrant further investigation. To report on the development of hyperplastic hepatic nodular lesion development in children and young adults (<21 years) with TIPS patency >3 years. Eighteen children and young adults, including 10 (55.6%) females and 8 (44.4%) males, underwent TIPS creation with >3 years’ patency and follow-up evaluation at a tertiary children’s hospital. The mean age at the time of TIPS creation was 12.5±5.1 years (range: 1.5–20.0 years). The mean model for end-stage liver disease (MELD) at the time of TIPS creation was 8.1±1.6 (range: 6–11). Indications for TIPS creation included acute variceal bleeding (8/18, 44.4%), primary (1/18, 5.6%) or secondary (7/18, 38.9%) prevention of varices, portal vein thrombosis (1/18, 5.6%), and splenic sequestration (1/18, 5.6%). Technical successes, intra-procedural parameters, hemodynamic and clinical successes, TIPS patencies, adverse events, imaging evaluations, and follow-ups were recorded. All (100%) TIPS placements were successful; however, a direct intrahepatic portosystemic shunt was created in one (5.6%) patient. Mean reduction of the portosystemic shunt gradient was 9.1±3.3 mmHg (range: 4–16 mmHg). Seventeen (94.4%) patients demonstrated clinical success with resolution of their initial clinical indication for TIPS placement. The 3-year TIPS primary, primary-assisted, and secondary patencies were 83.3% (15/18), 94.4% (17/18), and 100% (18/18), respectively. Two (11.1%) patients developed mild, medically controlled hepatic encephalopathy. One (5.6%) patient developed hepatopulmonary syndrome. Nine (50%) patients developed single or multiple hepatic nodules at a mean imaging surveillance time after TIPS of 4.4±3.0 years (range: 1.5–10.2 years). Six (33.3%) patients developed nodules >1 cm with imaging features most consistent with focal nodular hyperplasia or focal nodular hyperplasia-like nodules. The mean follow-up duration was 5.7±2.9 years (range: 3.0–13.1 years). Long-term (>3 years) portosystemic shunting via TIPS is associated with the development of hepatic nodular lesions in children. Consequently, children with TIPS may need gray-scale assessment of hepatic parenchyma as part of routine ultrasound exams and extended imaging surveillance until more is understood regarding the natural history of induced nodularity.
Purpose Post-operative seizure rates after endoscopic third ventriculostomy (ETV) are not definitively known. We analyzed our institution’s experience for all causes of hydrocephalus in pediatric patients undergoing ETV to determine rates of post-ETV seizure. Methods A retrospective review of institutional pediatric patients undergoing ETV from May 2014 to December 2018. Included were < 21 years, with 1-year follow-up. Exclusion criteria included ventriculoperitoneal shunts (VPS) prior to ETV, VPS within 7 days post-ETV, and prior seizure disorder. Data included age, gender, diagnosis, early post-operative seizure (within 7 days post-ETV), late post-operative seizures (after first 7 days and within first year post-ETV), concomitant choroid plexus cauterization (CPC), VPS conversion within 1 year, and administration of prophylactic antiepileptics. Results Sixty of 81 ETV cases were included; 41% underwent concomitant CPC. Of these, 53% ( n = 32) were male, 46% ( n = 28) female, averaging 5.8 years, with the most common diagnosis neoplasm-related obstructive hydrocephalus (38.3%, n = 23). Early post-operative seizure occurred in 6.7% ( n = 4); late post-operative seizure occurred in 8.3% ( n = 5). Late post-operative seizures were higher in patients experiencing early post-operative seizure versus those without (75% vs 3.7%, p = 0.003). Late post-operative seizure occurred in 13.6% ( n = 3 patients) requiring VPS versus 5.3% ( n = 2 patients) with successful ETV ( p = 0.36). Rates did not correlate with pathology. No patients received prophylactic antiepileptics prior to surgery or exhibiting a seizure. Conclusions Patients with early post-operative seizures have an increased likelihood of developing late post-operative seizures. Pediatric ETV patients may have a lower rate of both early and late post-operative seizure; underlying pathology may influence these rates.
Intravascular ultrasound (IVUS) is used as a diagnostic adjunct to angiography and has become a valuable diagnostic and interventional tool with a well-documented safety profile. The American College of Cardiology and the European Society of Cardiology have published guidelines regarding the use of IVUS in the setting of percutaneous coronary intervention. IVUS has gained popularity in the interventional radiology (IR) community in recent years; however, there are no consensus guidelines for utilization. Furthermore, IVUS remains an infrequently used modality in pediatric IR, likely because of unfamiliarity with the equipment and techniques, as well as concerns over the compatibility of these instruments with pediatric anatomy. IVUS can be safely used as a helpful and sometimes necessary tool for pediatric interventions in appropriately selected patients. The utility of IVUS for reducing both fluoroscopy time and contrast agent volume makes it particularly valuable in pediatric practice. This article presents an overview of both the rotational and phased-array IVUS types and an in-depth discussion on the most common applications of these techniques in the pediatric setting across multiple procedure categories.
Portomesenteric and portosystemic venous occlusive disease may lead to portomesenteric hypertension, variceal bleeding, ascites and hypersplenism. Data regarding endovascular reconstructive strategies in children, however, are limited. To report technical success, outcome and patency of portomesenteric and portosystemic venous reconstruction using VIABAHN VBX balloon-expandable endoprostheses in pediatric patients. Five pediatric patients (median age: 15 years, range: 4–18 years), including 3 (60%) boys and 2 (40%) girls, with portomesenteric or portosystemic venous occlusion or recurrent stenosis, underwent balloon-expandable stent graft reconstruction. Presenting symptoms included acute variceal bleeding, without (n = 2, 40%) or with (n = 1, 20%) splenomegaly, and transfusion-dependent chronic melena (n = 1, 20%). One patient was asymptomatic (n = 1, 20%). Preprocedural imaging included Doppler ultrasound and contrast-enhanced computed tomography (CT) in all patients. Initial imaging showed 4 (80%) occlusions and 1 (20%) recurrent stenosis greater than 50%. Technical aspects of the reconstructions, technical successes, clinical outcomes and adverse events were recorded. Technical success was defined as completion of stent graft reconstruction. Adverse events were categorized according to Society of Interventional Radiology criteria. Clinical success was defined as resolution of the presenting symptoms and/or prevention of portal hypertensive sequela. Venous reconstruction was technically successful in all five patients. Stent graft locations included the main portal vein in 2 (40%), the superior mesenteric vein in 1 (20%), autologous Meso-Rex shunt in 1 (20%) and splenocaval shunt in 1 (20%). Six stent grafts were placed (two stent grafts placed in a single patient). Stent grafts had a median diameter of 7 mm (range: 6–10 mm) and a median length of 59 mm (range: 19–79 mm). Median fluoroscopy time was 36.6 min (range: 13.4–95.8 min) and median air kerma was 301.0 mGy (range: 218.0–1,148.2 mGy). No adverse events occurred. Median clinical follow-up was 18 months (range: 6–29 months). Median imaging follow-up was 17 months (range: 2–29 months). Clinical success was achieved in all patients and maintained during the follow-up period. One patient required follow-up intervention with superior mesenteric vein side extension with a self-expanding bare metal stent due to perigraft stenosis detected on CT 3 months after stent placement. There were no stent graft occlusions. Portomesenteric and portosystemic venous reconstruction using balloon-expandable stent grafts in pediatric patients was feasible and clinically successful in this preliminary experience. Additional studies are warranted.
Background Neurodevelopmental disorders (NDDs), including autism spectrum disorder (ASD) and intellectual disability (ID), are common diagnoses with highly heterogeneous phenotypes and etiology. The genetics-first approach to research on NDDs has led to the identification of hundreds of genes conferring risk for ASD, ID, and related symptoms. Main body Although relatively few individuals with NDDs share likely gene-disruptive (LGD) mutations in the same gene, characterization of overlapping functions, protein networks, and temporospatial expression patterns among these genes has led to increased understanding of the neurobiological etiology of NDDs. This shift in focus away from single genes and toward broader gene–brain–behavior pathways has been accelerated by the development of publicly available transcriptomic databases, cell type-specific research methods, and sequencing of non-coding genomic regions. Conclusions The genetics-first approach to research on NDDs has advanced the identification of critical protein function pathways and temporospatial expression patterns, expanding the impact of this research beyond individuals with single-gene mutations to the broader population of patients with NDDs.
Hydrocephalus is the most common neurosurgical disorder in children, and cerebrospinal fluid (CSF) diversion with shunt placement is the most commonly performed pediatric neurosurgical procedure. CT is frequently used to evaluate children with suspected CSF shunt malfunction to assess change in ventricular size. Moreover, careful review of the CT images is important to confirm the integrity of the imaged portions of the shunt system. Subtle shunt disruptions can be missed on multiplanar two-dimensional (2-D) CT images, especially when the disruption lies in the plane of imaging. The use of volume-rendered CT images enables radiologists to view the extracranial shunt tubing within the field of view as a three-dimensional (3-D) object. This allows for a rapid and intuitive method of assessing the integrity of the extracranial shunt tubing. The purpose of this pictorial essay is to discuss how volume-rendered CT images can be generated to evaluate CSF shunts in the pediatric population and to provide several examples of their utility in diagnosing shunt disruption. We also address the potential pitfalls of this technique and ways to avoid them.
Computational surgical planning tools could help develop novel skull base surgical approaches that improve safety and patient outcomes. This defines a need for automated skull base segmentation to improve the usability of surgical planning software. The objective of this work was to design and validate an algorithm for atlas-based automated segmentation of skull base structures in individual image sets for skull base surgical planning. Advanced Normalization Tools software was used to construct a synthetic CT template from 6 subjects, and skull base structures were manually segmented to create a reference atlas. Landmark registration followed by Elastix deformable registration was applied to the template to register it to each of the 30 trusted reference image sets. Dice coefficient, average Hausdorff distance, and clinical usability scoring were used to compare the atlas segmentations to those of the trusted reference image sets. The mean for average Hausdorff distance for all structures was less than 2 mm (mean for 95th percentile Hausdorff distance was less than 5 mm). For structures greater than 2.5 mL in volume, the average Dice coefficient was 0.73 (range 0.59–0.82), and for structures less than 2.5 mL in volume the Dice coefficient was less than 0.7. The usability scoring survey was completed by three experts, and all structures met the criteria for acceptable effort except for the foramen spinosum, rotundum, and carotid artery, which required more than minor corrections. Currently available open-source algorithms, such as the Elastix deformable algorithm, can be used for automated atlas-based segmentation of skull base structures with acceptable clinical accuracy and minimal corrections with the use of the proposed atlas. The first publicly available CT template and anterior skull base segmentation atlas being released (available at this link: http://hdl.handle.net/1773/46259 ) with this paper will allow for general use of automated atlas-based segmentation of the skull base.
Robot-assisted stereoelectroencephalography (sEEG) is frequently employed to localize epileptogenic zones in patients with medically refractory epilepsy (MRE). Its methodology is well described in adults, but less so in children. Given the limited information available on pediatric applications, the objective is to describe the unique technical challenges and considerations of sEEG in the pediatric population. In this report, we describe our institutional experience with the technical aspects of robot-assisted sEEG in an exclusively pediatric epilepsy surgery unit, focusing on pre-, intra-, and post-operative nuances that are particular to the pediatric population. The pediatric population presents several unique challenges in sEEG, including reduced skull thickness relative to adults, incomplete neurologic development, and often special behavioral considerations. Pre-operative selection of putative epileptogenic zones requires careful multidisciplinary decision-making. Intraoperative attention to nuances in positioning, clamp selection, registration, and electrode placement are necessary. Activity considerations and electrode migration and removal are key post-operative considerations. Robot-assisted sEEG is a valuable tool in the armamentarium of techniques to characterize MRE. However, special considerations must be given to the pediatric population to optimize safety and efficacy.
Study designRetrospective cohort studyObjectivesTo determine prevalence of hereditary multiple osteochondromas (HMO) and utility of MRI surveillance in a prospective Spine at Risk (SAR) program.Summary of background dataUnidentified intraspinal exostoses in HMO can lead to neurologic injury in children during sedated procedures but no MRI guidelines exist. We sought to determine the prevalence and age of intraspinal exostoses from MRIs, and indications for MRI surveillance.MethodsRetrospective review was performed of pediatric HMO patients who underwent total spine MRIs at a single institution after a prospective SAR program was instituted. Charts were reviewed for MRI indication and findings, symptoms, surgery, and location of other exostoses. Fisher's exact test was used to compare categorical variables and T test to compare continuous variables. Predictive value of pelvic/rib exostoses was calculated for intraspinal lesions.ResultsForty-three patients with HMO underwent total spine MRIs with average age of 11.5 years. Fifteen (35%) patients had exostoses on vertebral column, eight (19%) had intra-canal spinal exostoses. Higher prevalence of spine lesions occurred in symptomatic patients than asymptomatic (any spinal lesion: 73% prevalence in symptomatic vs 22% in asymptomatic, p<0.005; intra-canal spinal lesion: 46% vs 9%, p<0.05). Only two of the 11 'symptomatic presentations' could be attributable to intracanal spinal exostoses. Only one intra-canal exostosis found on asymptomatic surveillance was treated surgically. Presence of pelvic or rib exostoses were not strongly predictive of intra-canal lesions (23% PPV, 85% NPV, 63% sensitivity, 51% specificity).ConclusionsEven with the presence of intra-canal exostoses, true symptomatic lesions are rare. Rib and pelvic lesions were not predictive of intra-canal lesions in our population. We recommend obtaining MRIs at time of preoperative evaluation in asymptomatic children old enough to not need sedation, or in patients with true neurologic symptoms to prevent unnecessary sedation of younger children for surveillance MRI.Level of evidenceIII.
Background Consent and assent are important concepts to understand in the care of pediatric neurosurgery patients. Recently it has been recommended that although pediatric patients generally do not have the legal capacity to make medical decisions, they be encouraged to be involved in their own care. Given the paucity of information on this topic in the neurosurgery community, the objective is to provide pediatric neurosurgeons with recommendations on how to involve their patients in medical decision-making. Methods We review the essential elements and current guidelines of consent and assent for pediatric patients using illustrative neurosurgical case vignettes. Results The pediatric population ranges widely in cognitive and psychological development making the process of consent and assent quite complex. The role of the child or adolescent in medical decision-making, issues associated with obtaining assent or dissent, and informed refusal of treatment are considered. Conclusion The process of obtaining consent and assent represents a critical yet often overlooked aspect to care of pediatric neurosurgical patients. The pediatric neurosurgeon must be able to distill immensely complex and high-risk procedures into simple, understandable terms. Furthermore, they must recognize when the child's dissent or refusal to treatment is acceptable. In general, allowing children to be involved in their neurosurgical care is empowering and gives them both identity and agency, which is the vital first step to a successful neurosurgical intervention.
Background Isolated persistent left superior vena cava (PLSVC) is a rare vascular anatomic variant, which can be an incidental finding at the time of an endovascular procedure. Case presentation This report describes the technical success, adverse events, and clinical outcomes of transjugular intrahepatic portosystemic shunt (TIPS) creation via isolated PLSVC. Three adult patients with cirrhosis and isolated PLSVC underwent TIPS placement successfully with one major adverse event. Two patients required TIPS revision within 90 days. There were no deaths within 90 days. Conclusions TIPS creation via isolated PLSVC is feasible using standard techniques with a left jugular vein approach. Caution is warranted during the procedure to assess for any aberrant drainage pattern to the left atrium and to prepare for potentially challenging instrument navigation through the coronary sinus.
Background The safety and efficacy of US-guided lumbar puncture in children has been described. In the pediatric setting, children are frequently referred to interventional radiology only after a failed landmark-based attempt. Routine pre-procedure US in these children is useful to determine a safe level for subarachnoid access and to optimize success. Objective To determine whether pre-procedure US improves technical success and safety of US-guided lumbar puncture. Materials and methods We included 47 children. Inclusion criteria were urgent US-guided lumbar puncture in pediatric patients <18 years old. Exclusion criteria were non-urgent lumbar punctures, children referred without an antecedent landmark-based attempt, lumbar punctures performed with fluoroscopic guidance, and procedures performed prior to introducing the diagnostic approach in 2017. We did not evaluate data pertaining to successful landmark-based lumbar punctures performed without subsequent need for additional attempts. We recorded technical successes, adverse events and relevant abnormalities identified on pre-procedural US. Results Thirty-six US-guided lumbar punctures were performed with 100% technical success. Eleven children referred to interventional radiology did not undergo lumbar puncture because of unfavorable US findings or interval clinical improvement obviating the need for lumbar puncture. Thirty-six children underwent US evaluation of the thecal sac prior to potential intervention. Of these 36 with pre-procedural US studies, 12 demonstrated paucity of cerebrospinal fluid and 14 demonstrated an epidural hematoma. Fifteen children who underwent lumbar puncture had a “traumatic tap,” classified as a mild adverse event. No moderate or severe adverse events were recorded. Conclusion Limited spinal US following failed landmark-based lumbar punctures frequently identifies procedure-related complications and can augment patient selection for future image-guided lumbar punctures.
Accurate and reproducible means of measuring the portosystemic gradient are essential for risk stratification and treatment of portal hypertension. To report the reliability of hepatic venous pressure gradients in children with intrahepatic veno-venous collateralization. Between January 2012 and December 2019 (96 months), 39 patients with native livers underwent wedge hepatic venography and hepatic venous pressure gradient measurements at a tertiary pediatric center. All archived images were reviewed for balloon isolation of the hepatic vein and hepatic vein-to-hepatic vein (HV-HV) collaterals. HV-HV collaterals were categorized as present on the basis of non-catheterized segmental venous opacification despite appropriate balloon isolation. Hepatic venous pressure gradient was defined as the difference of wedge and free hepatic venous pressures. Wedge portosystemic gradient was defined as the difference between wedge hepatic venous pressure and right atrial (RA) pressures. For patients subsequently undergoing portal venous catheterization, portosystemic gradient was defined as the difference between main portal vein and RA pressures. Thirteen of 39 (33.3%) patients demonstrated HV-HV collaterals on wedge hepatic venography. The mean hepatic venous pressure gradient was 5.2±3.8 mmHg (range: 0–15 mmHg). The mean hepatic venous pressure gradient was 3.6±2.6 mmHg (range: 0–9 mmHg) in the presence of HV-HV collaterals and 5.9±4.2 mmHg (range: 1–15 mmHg) in the absence of HV-HV collaterals (P=0.043). Twelve (30.8%) patients were found to have varices: 10 gastroesophageal, 1 rectal and 1 stomal. The mean hepatic venous pressure gradient in patients with varices was 5.4±47 mmHg (range: 0–15 mmHg). For patients with varices, mean hepatic venous pressure gradient was 3.0±2.7 mmHg (range: 0–9 mmHg) in the presence of HV-HV collaterals and 10.3±4.1 mmHg (range: 5–15 mmHg) in the absence of HV-HV collaterals (P=0.004). Four (10.3%) patients had extrahepatic portal vein occlusion: 3 with cavernous transformation and 1 with type Ib Abernethy malformation. All patients with extrahepatic portal vein occlusion demonstrated HV-HV collaterals compared with 8 of 35 (22.9%) patients without extrahepatic portal vein occlusion (P=0.002). Four of 39 (10.3%) patients underwent direct portal pressure measurements: 3 via transhepatic and 1 via trans-splenic portal access. All had demonstrated HV-HV collaterals on wedged imaging. One had extrahepatic portal vein occlusion. The mean time between wedge portosystemic gradient and portosystemic gradient measurement was 3.75 days (range: 0–8 days). The mean wedge portosystemic gradient was 4.5±3.1 mmHg (range: 2–9 mmHg) and the mean portosystemic gradient was 14.5±3.7 mmHg (range: 12–20 mmHg) (P=0.006). HV-HV collateralization is frequently observed in children undergoing wedged portal venography and leads to misrepresentative hepatic venous pressure gradients. All patients undergoing hepatic venous pressure gradient measurement should have wedged venography to identify HV-HV collaterals and to qualify measured pressures. Additional techniques to obtain representative pressures in the presence of HV-HV collaterals warrant further investigation.
Purpose of Review Tics often draw negative attention and may interfere with social and academic performance. When enduring a year or longer (“persistent”), tic disorders are usually associated with one or more nontic coexisting conditions that can exacerbate tic severity and pose their own social and academic interferences. This review examines the range of these vulnerabilities and explores strategies and interventions to help affected children and youth cope with tics in school and with peers. Recent Findings Stigma and bullying are frequent outcomes, which contribute to coping challenges that emerge from the interferences of tics and coexisting conditions. Increasingly, research and treatment of tic disorders in children and youth emphasize a holistic, collaborative approach among child and family, educators, peers, and clinicians. Summary Interventions that target psychosocial and functional impacts of tics and coexisting conditions at school and with peers can have wide-reaching effects in improving effective coping. Management emphasizes psychoeducation and resilience building alongside tic-reduction therapies. Methods to improve access to educational and therapeutic resources, including virtual access, are being explored.