BACKGROUND: Endoscopic resection has been reported for vascular anomalies (VA) previously. However, there is no study comparing endoscopic resection surgery (ERS) with open resection surgery (ORS) in children. We aimed to compare clinical and cosmetic outcomes between two approaches in pediatric VA.
METHODS: Between June 2018 and June 2023, 138 pediatric VA patients undergoing ERS or ORS were retrospectively reviewed. Propensity score matching (PSM) was performed to minimize selection bias. The Scar Cosmesis Assessment and Rating (SCAR) Scale and numerical rating scale (NRS) based on patient satisfaction were used for cosmetic assessment.
RESULTS: After PSM for age, depth of lesion, size of lesion, and site of surgery, 72 patients (ERS = 24, ORS = 48) were analyzed. Patients undergoing ERS had longer operative time (164.25 ± 18.46 vs. 112.85 ± 14.26 min; P < 0.001), less estimated blood loss (5.42 ± 2.15 vs. 18.04 ± 1.62 ml; P < 0.001), and shorter median hospital stay (4.50 [3.00-5.00] vs. 6.00 [5.00-6.00] days; P < 0.001). The follow-up time was 8.04 ± 1.23 month for ERS group and 8.56 ± 1.57 month for ORS group. For aesthetic results, the median overall SCAR score in ERS was lower than that in ORS (2 [1-3] vs. 5 [4-5]; P < 0.001), and the subscales of \"scar spread,\" \"dyspigmentation,\" \"track marks or suture marks,\" and \"overall impression\" were better. The median NRS score was higher (8 [7-8] vs. 6 [5-6]; P < 0.001) and length of scars was shorter (2.18 ± 0.30 vs. 8.75 ± 1.98 cm; P < 0.001) in ERS group than those in ORS group. The incidences of total complications and recurrence showed no significant difference between two groups.
CONCLUSIONS: Endoscopic surgery can be a safe and effective option for pediatric VA in the limbs and trunk. It offers the advantages of improving aesthetic outcomes and reducing postoperative wound healing time.

High temperature requirement A1 (HTRA1) is a member of the serine protease family, comprising four structural domains: IGFBP domain, Kazal domain, protease domain and PDZ domain. HTRA1 encodes a serine protease, a secreted protein that is widely expressed in the vasculature. HTRA1 regulates a wide range of physiological processes through its proteolytic activity, and is also involved in a variety of vascular abnormalities-related diseases. This article reviews the role of HTRA1 in the development of vascular abnormalities-related hereditary cerebral small vessel disease (CSVD), age-related macular degeneration (AMD), tumors and other diseases. Through relevant research advances to understand the role of HTRA1 in regulating signaling pathways or refolding, translocation, degradation of extracellular matrix (ECM) proteins, thus directly or indirectly regulating angiogenesis, vascular remodeling, and playing an important role in vascular homeostasis, further understanding the mechanism of HTRA1\'s role in vascular abnormality-related diseases is important for HTRA1 to be used as a therapeutic target in related diseases.

UNASSIGNED: Situs inversus totalis (SIT) is an uncommon disorder characterized by mirror-image anatomy, which can present unique challenges and potential vascular anomalies in surgical interventions, particularly in gastric cancer patients.
UNASSIGNED: We aim to delineate a rare case of gastric adenocarcinoma in a SIT patient and conduct a thorough review of the existing literature concerning surgical strategies, vascular anomalies, and outcomes observed across varied geographic locales and technological approaches.
UNASSIGNED: A thorough examination of a case involving a 39-year-old male SIT patient who underwent a successful distal gastrectomy with D2 lymph node dissection is presented alongside an expansive literature review. The review encompasses 47 articles, collating data on surgical approaches and vascular anomalies across 49 patients diagnosed with SIT and gastric cancer.
UNASSIGNED: The patient underwent curative distal gastrectomy and Billroth II with Braun anastomosis within 95 minutes, incurring minimal intraoperative blood loss (100ml). Postoperative pathology confirmed moderately to poorly differentiated gastric adenocarcinoma (pT3N0M0), with no signs of recurrence or metastasis after 6 months of S-1 adjuvant chemotherapy. The literature review revealed vascular anomalies in approximately 20% of reported cases, accentuating its surgical significance. Noteworthy variations in surgical strategies, operative times, blood loss, and complications across different surgical modalities were observed, providing a comprehensive view into the practical management of such cases.
UNASSIGNED: Despite the inherent challenges associated with SIT, various surgical techniques can be successfully applied with meticulous preoperative planning and understanding vascular anomalies. This compilation of diverse surgical experiences across numerous documented cases seeks to provide a consolidated resource for refining surgical strategies and enhancing postoperative outcomes for gastric cancer patients with SIT, underscoring the imperativeness of further research in this niche domain.

UNASSIGNED: Infantile hemangioma (IH) has received global attention, resulting in a significant volume of literature. However, there is a lack of bibliometric analyses specifically focusing on IH publications. This study aims to fill this gap by conducting a comprehensive analysis of IH publications, investigating their characteristics, contribution distribution, and developmental trends. By enhancing our understanding of IH and identifying potential research topics and collaborators, this study will contribute to the advancement of the field.
UNASSIGNED: A total of 4333 articles and reviews on IH were collected from the Web of Science (WoS) database, spanning the years 2000-2022. The study encompassed a comprehensive analysis of IH publications, evaluating their quantity and quality. Additionally, we profiled publishing groups based on country, institution, author publication records, and collaboration networks. Lastly, we identified and summarized the prominent research topics.
UNASSIGNED: Annual publications on IH have increased over the past 20 years. The United States has the highest number of publications and the highest total number of citations. Pediatric Dermatology was the most influential journal in the IH field. The citation analysis indicated that the articles published by Léauté-Labrèze in 2008 had the highest number of citations. The articles published by North PE in 2000 and Boye E in 2001 laid a certain research foundation for this field. Concerning institutions, most of the cooperative relationships were established in the same country/region. The United States has the largest number of scientific research institutions and IH researchers, leading most of the cross-country collaboration. The University of California, San Francisco, Medical College of Wisconsin, Harvard University, and Shanghai Jiaotong University were the research centers that published the most IH-related research. Frieden IJ, Mulliken JB, and Drolet BA were the top three most influential authors. Frieden IJ, Garzon MC, and Mulliken JB were the top three authors with the most cited frequency. In addition, keywords and keyword co-occurrence networks prompted that the pathological mechanism of IH, clinical analysis, and other vascular anomalies are research hotspots. Analysis of trending topics suggests that research on IH has evolved from treatment-focused studies towards investigations of other vascular diseases and a series of clinical case studies. Currently, clinical case studies receive the most attention in the field.
UNASSIGNED: This comprehensive bibliometric study provides a thorough analysis of post-2000 publications in the field of IH, offering insights into current research trends for the first time. The findings suggest that future investigations will continue to prioritize understanding IH mechanisms, treatment approaches, and treatment evaluation. Furthermore, the exploration of other vascular diseases and the inclusion of clinical case studies are expected to contribute to advancements in IH clinical practice. By identifying potential collaborators, partner institutions, and new research avenues, this study offers valuable guidance for future in-depth research on IH.

Interventional procedures (IPs) have been widely used to treat vascular anomalies (VA) in recent years. However, patients are exposed to low-dose X-ray ionizing radiation (IR) during these fluoroscopy-guided IPs. We collected clinical information and IR doses during IPs and measured biomarkers including γ-H2AX, chromosome aberrations (CA), and micronuclei (MN), which underpin radiation-induced DNA damage, from 74 pediatric patients before and after IPs. For the 74 children, the range of dose-area product (DAP) values was from 1.2 to 1754.6 Gy∙cm2, with a median value of 27.1 Gy∙cm2. DAP values were significantly higher in children with lesions in the head and neck than in the limbs and trunk; the age and weight of children revealed a strong positive correlation with DAP values. The treated patients as a group demonstrated an increase in all three endpoints relative to baseline following IPs. Children with vascular tumors have a higher risk of dicentric chromosome + centric ring (dic+r) and cytokinesis-block micronucleus (CBMN) after IPs than children with vascular malformations. The younger the patient, the greater the risk of CA after IPs. Moreover, rogue cells (RCs) were found in five children (approximately 10%) after IPs, and the rates of dic+r and CBMN were significantly higher than those of other children (Z = -3.576, p < 0.001). These results suggest that there may be some children with VA who are particularly sensitive to IR, but more data and more in-depth experiments will be needed to verify this in the future.

UNASSIGNED: To retrospectively report our preliminary experience of treating hand arteriovenous malformations (AVMs) with embolo/sclerotherapy.
UNASSIGNED: Retrospectively review the demographics, treatment detail, outcome data, and complications of 13 consecutive patients with hand AVMs from January 2018 to December 2021. We embolize the dominant outflow vein with elastic coils and then use absolute ethanol or polidocanol for intravascular sclerotherapy and bleomycin for interstitial sclerotherapy.
UNASSIGNED: Yakes type II presents in four lesions, type IIIa in six, and type IIIb in three. A total of 29 treatment episodes were conducted for the 13 patients (1 episode for 3 patients, 2 for 4 patients, and 3 for 6 patients; the repeated treatment rate was 76.9%). The mean stretched length of coils for 1 treatment episode was 95 cm. The mean absolute ethanol dosage was 6.8 ml (range 4-30 ml). In addition, 10 ml of 3% polidocanol foam was injected and interstitial sclerotherapy with 150,000 IU bleomycin was performed on every patient. The post-operative arterial-dominant outflow vein pressure index (AVI) increased in the 29 procedures (6.55 ± 1.68 vs. 9.38 ± 2.80, P < 0.05). The Mann-Whitney U test showed that the post-operative AVI was higher in patients without re-intervention (P < 0.05). Local swelling occurred after all the procedures. Blistering occurred in 6 of the patients in 13 (44.8%) of the 29 procedures. Superficial skin necrosis occurred in 3 of the patients in 5 (17.2%) of the 29 procedures. The swelling, blistering, and superficial skin necrosis recovered within 4 weeks. No finger amputation occurred. The follow-up time was 6 months. The 6-month assessment of clinical improvement after the last treatment episode showed that 2 patients were cured, 10 were improved, and 1 remained unchanged. With regard to angiographic evaluation, 9 showed partial response and 4 complete response.
UNASSIGNED: Embolo/sclerotherapy can be effective and safe for hand AVM. The AVI increased significantly after embolo/sclerotherapy, and the index may be valuable in predicting recurrence in further study.

Patients with vascular anomalies (VAs) who receive oral sirolimus may be at high risk of infectious complications. Antibiotic prophylaxis with trimethoprim-sulfamethoxazole (TMP-SMZ) has been advocated. However, there have been few evidence-based analyses on this topic. This study assessed the effect of prophylactic TMP-SMZ on the incidence of infections in VA patients receiving sirolimus monotherapy.
A retrospective, multicenter chart review was performed on all VA patients receiving sirolimus treatment from August, 2013 to January, 2021.
Before January 2017, 112 patients were treated with sirolimus without antibiotic prophylaxis. In the subsequent period, 195 patients were treated with TMP-SMZ for at least 12 months during sirolimus therapy. The percentage of patients with at least one serious infection during the initial 12 months of sirolimus treatment did not differ between the groups (difference, 1.1%; 95% CI - 7.0-8.0%). We observed no difference in the incidence of individual infection or total adverse events between the groups. The rate of sirolimus discontinuation due to adverse events did not differ significantly between groups.
We demonstrated that prophylactic TMP-SMZ does not decrease the incidence of infection or improve tolerance in VA patients receiving sirolimus monotherapy.

Sirolimus (SRL) is a mammalian target of rapamycin (mTOR) inhibitor. The whole blood concentration of SRL is routinely monitored to tailor dosage and prevent toxicity. Currently, the enzyme multiplied immunoassay technique (EMIT) is often applied to perform therapeutic drug monitoring (TDM) of SRL, but the cross-reactivity with various metabolites is of great concern. A more specific method is required, such as liquid chromatography-tandem mass spectrometry (LC-MS/MS). However, no study on the method comparison of the EMIT and LC-MS/MS for the measurement of whole blood SRL concentration in children with vascular anomalies has been reported. This study developed a simple and sensitive LC-MS/MS assay for the determination of SRL. Meanwhile, consistency between LC-MS/MS and the EMIT was evaluated by linear regression and Bland-Altman analysis. Whole blood samples were deproteinized with methanol for erythrocyte lysis, and the resulting solution was injected into the LC-MS/MS system using the positive electrospray ionization mode. The multiple reaction monitoring transitions of m/z 931.7 → 864.6 and m/z 934.7 → 864.6 were used for SRL and SRL-d3 as the internal standards, respectively. The analytes were separated on a C18 column with a gradient mobile phase (0.1 mM formic acid and 0.05 mM ammonium acetate in methanol/ultrapure water). Blood samples collected from children with vascular anomalies undergoing SRL therapy were tested by EMIT and by LC-MS/MS. The linear range of LC-MS/MS was 0.500-50.0 ng/ml and that of the EMIT was 3.50-30.0 ng/ml. A significant positive correlation between the two assays was established with a regression equation described as [ EMIT ] = 1.281 × [ LC-MS/MS ] + 2.450 (r = 0.8361). Bland-Altman plots showed a mean concentration overestimation of 4.7 ng/ml [95% CI: (-3.1, 12.6)] and a positive bias of 63.1% [95% CI: (-36.1, 162.3)] generated by the EMIT more than that of by LC-MS/MS. In conclusion, the two methods were closely correlated, indicating that switching between the two methods is feasible. Considering the overestimation nature of the EMIT assay, switching from the EMIT to the LC-MS/MS method deserves close attention and necessary re-evaluation for the target therapeutic reference range, may be required when methods are switched within the same clinical laboratory or results are compared between different laboratories.

Cerebral cavernous malformation (CCM) is a type of vascular anomaly that arises due to the dyshomeostasis of brain capillary networks. In the past two decades, many advances have been made in this research field. Notably, as a more reasonable current view, the CCM lesions should be attributed to the results of a great number of additional events related to the homeostasis disorder of the endothelial cell. Indeed, one of the most fascinating concerns in the research field is the inflammatory perturbation in the immune microenvironment, which would affect the disease progression as well as the patients\' outcomes. In this work, we focused on this topic, and underlined the immune-related factors\' contribution to the CCM pathologic progression.

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