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Pediatric dermatologists are frequently consulted to evaluate children for cutaneous signs of systemic disorders. Numerical thresholds of significance have been described in the dermatologic literature for various skin findings where the likelihood of an associated extracutaneous abnormality or known genetic syndrome increases significantly. Knowledge of these numerical thresholds facilitates diagnosis and management, which improves clinical outcomes and avoids severe complications. This review highlights the clinical presentation, complications, evaluation, and numerical significance, when applicable, for the following skin findings: infantile hemangiomas, capillary malformations, café‐au‐lait macules, hypopigmented macules, juvenile xanthogranulomas, pilomatricomas, and angiofibromas. [ABSTRACT FROM AUTHOR]

A 59-year-old woman presented to our clinic with a 3.5× 3-cm protruding mass on her forehead. A skull X-ray revealed a radiolucent osteolytic lesion on the left side of the frontal bone. Additionally, computed tomography showed a 3.1× 1.7× 3.6-cm mass exhibiting a “sunburst” pattern situated between the outer and inner tables of the skull, just superior and lateral to the left frontal sinus. This pattern suggested the presence of an intraosseous vascular malformation (IVM). The lesion was approached via a bicoronal incision. En-bloc resection was performed, removing the mass along with approximately 0.5 cm of the surrounding normal bone without injury to the exposed frontal sinus mucosa. The exposed mucosa was reinforced with a galeal flap, and cranioplasty with bone cement was performed to repair the resulting bony defect. Pathological examination confirmed a diagnosis of intraosseous cavernous-type malformation with mixed cavernous and capillary histological features. We report this case of IVM and review the existing literature, highlighting the satisfactory functional and aesthetic outcomes after surgery. [ABSTRACT FROM AUTHOR]

Objective: This retrospective study aimed to perform a clinicopathological analysis of oral lymphangioma cases diagnosed at a reference service in oral pathology. Materials and Methods: A total of 40 cases with reviewed and confirmed histopathological diagnoses for oral lymphangioma were included in the study. Results: It was found that Caucasian women represented most of the sample, with the tongue and lower lip being the most affected sites. The most frequent clinical presentation of the lesions was as an asymptomatic reddish nodule. As for morphological characteristics, there was a slight predominance of simple lymphangioma (55%). Surgical removal was the therapeutic approach adopted in most cases. Conclusions: Despite OL being rare, it is essential that dentists and other professionals recognize variations in the clinical presentation and histopathological aspects of these lesions, differentiating them from other vascular lesions, and implementing correct treatment in each case. [ABSTRACT FROM AUTHOR]

Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus. Their removal poses significant risk due to their vascularity and diffuse nature, and their genetic underpinnings remain incompletely understood. Our approach involved genetic analyses on 31 tissue samples of ECHs employing whole-exome sequencing and targeted deep sequencing. We explored downstream signaling pathways, gene expression changes, and resultant phenotypic shifts induced by these mutations, both in vitro and in vivo. In our cohort, 77.4% of samples had somatic missense variants in GNA14 , GNAQ , or GJA4. Transcriptomic analysis highlighted significant pathway upregulation, with the GNAQ c.626A>G (p.Gln209Arg) mutation elevating PI3K-AKT-mTOR and angiogenesis-related pathways, while GNA14 c.614A>T (p.Gln205Leu) mutation led to MAPK and angiogenesis-related pathway upregulation. Using a mouse xenograft model, we observed enlarged vessels from these mutations. Additionally, we initiated rapamycin treatment in a 14-year-old individual harboring the GNAQ c.626A>G (p.Gln209Arg) variant, resulting in gradual regression of cutaneous cavernous hemangiomas and improved motor strength, with minimal side effects. Understanding these mutations and their pathways provides a foundation for developing therapies for ECHs resistant to current therapies. Indeed, the administration of rapamycin in an individual within this study highlights the promise of targeted treatments in treating these complex lesions. [Display omitted] Extra-axial cavernous hemangiomas (ECHs) are complex vascular lesions mainly found in the spine and cavernous sinus, and their genetic underpinnings remain incompletely understood. Here, we report somatic mutations of GNA14 , GNAQ , and GJA4 in ECH lesions. Furthermore, we present the utilization of rapamycin in treating an individual with GNAQ mutations. [ABSTRACT FROM AUTHOR]

Background: Vascular malformations (VaMs) are caused by errors in vascular morphogenesis. Diagnosis and treatment can be complex. Few specialized centers care for these patients, and limited literature exists regarding their characteristics and clinical course. The vascular anomalies clinic (VAC) at the Instituto Nacional de Pediatría (National Institute for Pediatrics) is a multidisciplinary team and has been a reference center for patients with VaMs since 2012. We sought to describe the characteristics of patients cared for at the VAC, types of VaMs, treatments used, and clinical course. Methods: This was a descriptive, observational, retrospective, and cross-sectional study conducted from 2012 to 2022. Results: We included 435 patients with VaMs; the median age of presentation was 1 month. The most frequent signs and symptoms were increased volume (97.2%), superficial color change (65.5%), and pain (43.3%). The most common VaMs were lymphatic (36.7%) and venolymphatic (18.3%). Sclerotherapy was the most frequent treatment (73.4%), followed by medical treatment with sirolimus (18.5%); response to both was excellent/good in > 85% of cases. Conclusion: In this retrospective study of children with VaMs, we found that low-flow malformations were the most common, and sclerotherapy and sirolimus were the most frequently used treatments. The therapeutic response was excellent/good in most cases. [ABSTRACT FROM AUTHOR]

Objective: Gamma Knife radiosurgery (GKRS) is an effective and noninvasive treatment for high-risk arteriovenous malformations (AVMs). Since differences in GKRS outcomes by nidus type are unknown, this study evaluated GKRS feasibility and safety in patients with brain AVMs. Methods: This single-center retrospective study included patients with AVM who underwent GKRS between 2008 and 2021. Patients were divided into compact- and diffuse-type groups according to nidus characteristics. We excluded patients who performed GKRS and did not follow-up evaluation with magnetic resonance imaging or digital subtraction angiography within 36 months from the study. We used univariate and multivariate analyses to characterize associations of nidus type with obliteration rate and GKRS-related complications. Results: We enrolled 154 patients (mean age, 32.14±17.17 years; mean post-GKRS follow-up, 52.10±33.67 months) of whom 131 (85.1%) had compact- and 23 (14.9%) diffuse-type nidus AVMs. Of all AVMs, 89 (57.8%) were unruptured, and 65 (42.2%) had ruptured. The mean Spetzler-Martin AVM grades were 2.03±0.95 and 3.39±1.23 for the compact- and diffuse-type groups, respectively (p<0.001). During the follow-up period, AVM-related hemorrhages occurred in four individuals (2.6%), three of whom had compact nidi. Substantial radiation-induced changes and cyst formation were observed in 21 (13.6%) and one patient (0.6%), respectively. The AVM complete obliteration rate was 46.1% across both groups. Post-GKRS complication and complete obliteration rates were not significantly different between nidus types. For diffuse-type nidus AVMs, larger AVM size and volume (p<0.001), lower radiation dose (p<0.001), eloquent area location (p=0.015), and higher Spetzler-Martin grade (p<0.001) were observed. Conclusion: GKRS is a safe and feasible treatment for brain AVMs characterized by both diffuse- and compact-type nidi. [ABSTRACT FROM AUTHOR]

PIK3CA‐related overgrowth spectrum (PROS) encompasses different clinical entities caused by somatic activating mutations in PIK3CA. Among PROS, CLOVES syndrome represents a severe phenotype with poor survival rate. We present the case of a 4‐month‐old girl with CLOVES syndrome successfully treated with alpelisib, a PIKC3A inhibitor. [ABSTRACT FROM AUTHOR]

This narrative review delves into the intricacies of hemangiomas and vascular malformations (VMs), exploring their characteristics, diagnostic approaches, and therapeutic interventions. Hemangiomas, characterized by an abnormal growth of blood vessels, and VMs, arising from developmental abnormalities in blood vessels, present multifaceted challenges in clinical practice. The review navigates through the clinical manifestations and classifications of these vascular anomalies, emphasizing the importance of accurate diagnosis based on clinical evaluation, imaging techniques, and histopathological assessment. Variations in presentation, ranging from asymptomatic lesions to severe complications impacting vital organs, underscore the necessity for comprehensive understanding and tailored management strategies. Moreover, this review examines current therapeutic modalities encompassing medical, interventional, and surgical interventions. It synthesizes the evolving landscape of treatment options and highlighting the benefits, limitations, and potential adverse effects associated with each approach. In addition, the review underscores the significance of a multidisciplinary approach, integrating expertise from dermatology, radiology, and surgery, to optimize patient care and outcomes. The challenges posed by these vascular anomalies necessitate ongoing research endeavors aimed at refining diagnostic criteria and advancing therapeutic innovations. This review amalgamates existing knowledge and providing clinicians and researchers with a comprehensive understanding of hemangiomas and VMs, thereby fostering continued exploration and innovation in this complex field.

Introduction Hemangiomas are benign vascular lesions. The complicated hemangiomas that are causing significant functional or cosmetic problems need surgical excision. This paper presents a novel technique of reconstruction using surgical mesh after removal of a facial hemangioma.Case report A 50-year-old male patient presented with right cheek swelling for more than 40 years causing disproportion of the face. A flap was raised by a nasolabial linear incision, then a 4*2 cm surgical mesh was put in, and the wound closed cosmetically. The excised tissue was sent for histopathological examinations and the results confirmed hemangioma. After more than two years follow up, the patient’s face was symmetrical with normal facial contour.Conclusion The application of surgical mesh for patients who seek functional and cosmetic improvements after hemangioma removal is a constructive method that gives the desired outcome for the patient.

Vascular malformations are intricate anomalies of the circulatory system, presenting a diverse array of clinical manifestations, and posing significant challenges in diagnosis and treatment. The pathogenesis of vascular malformations is explored through the lens of genetic and molecular mechanisms, shedding light on the pivotal role of somatic mutations and dysregulated signaling pathways. Clinical presentations of vascular malformations are widely variable, ranging from cosmetic concerns to life-threatening complications. The utility of imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and angiography, are discussed in detail, emphasizing their role in precise delineation and characterization. Therapeutic strategies for vascular malformations are multifaceted, considering factors such as lesion size, location, potential complications, and patient-specific factors. Traditional interventions, including surgical excision and embolization, are appraised alongside emerging approaches like targeted molecular therapies and minimally invasive procedures. The manuscript underscores the need for an individualized treatment approach, optimizing outcomes while minimizing risks and complications. In summation, this manuscript offers a comprehensive analysis of vascular malformations, encompassing their underlying pathogenesis, clinical nuances, diagnostic methods, and therapeutic considerations. By synthesizing current knowledge and highlighting gaps in understanding, this review serves as a valuable resource for clinicians, researchers, and medical practitioners, fostering an enhanced comprehension of vascular malformations and paving the way for improved patient care and innovative research endeavors.

Object: The aim of the study was to investigate the safety, effectiveness, and peripheral nerve protection in ultrasound-guided microwave ablation (US-guided-MWA) for vascular malformations (VMs) closely related to peripheral nerve. Materials and Methods: From August 2019 to February 2022, 31 patients with 39 VMs received US-guided-MWA. All lesions were confirmed to be closely related to the peripheral nerve by imaging evaluation. Hydrodissection was applied to protect surrounding normal tissue, including peripheral nerves. The patients were followed up at 1day, 2 days, 3 days, 1 week, 1 month, 3 months after operation. Measurements of lesion volume, volume reduction ratio (VRR), sensory and functional abnormalities of adjacent nerves, number of treatments, complication details, personal satisfaction, recurrence, and symptom improvement were recorded. Results: Among the 39 VMs, the maximum volume is 128.58ml, while the minimum volume is 0.99ml. After a mean follow-up of 13.06 ± 4.83 months, the mean numerical rating scale (NRS) score decreased from 5.13 ± 1.65 to 0.53 ± 0.83 (P<0.0001). The mean mass volume was reduced from 18.34 ± 24.68 ml to 1.35 ± 2.09 ml (P=0.0001). The VRR of all lesions was 92.06%. However, the mean number of treatments was only 1.64 ± 0.87. All patients were satisfied with the technique, with a mean satisfaction score (SC) of 9.23 ± 1.13. There were no motor function abnormalities of the related nerves. 10 patients felt numbness in the ablation area after ablation, and gradually recovered after 1 month. Conclusion: US-guided-MWA serves as a novel alternative approach for patients with VMs. Preoperative evaluation of the relationship between VMs and peripheral nerves combined with intraoperative hydrodissection is an effective and safe method to prevent nerve injury. [ABSTRACT FROM AUTHOR]

Phleboliths are reported as calcifications that occur in vascular malformations, associated with changes in blood flow dynamics, thrombus formation and subsequent calcifications. Radiological examination, such as cone beam computed tomography (CBCT) could help in demonstrating the presence of a calcifiied mass. A 45-year-old male was referred to our service with an asymptomatic nodular purplish lesion located on the ventrolateral tongue. Within the lesion, a stony mass was also evident on palpation. A digital dental radiograph demonstrated two circumscribed radiopaque structures. Phleboliths associated with vascular malformation was the main diagnostic hypothesis. The patient underwent a sclerotherapy protocol allowing surgical accessibility to the area. Phlebolyts were surgically removed using electrocoagulation. Histopathological examination revealed phleboliths in the context of a vascular malformation with intense fibrosis. [ABSTRACT FROM AUTHOR]

This narrative review aims to summarise the classification of vascular anomalies, their clinical presentation, and their radiological features to propose a diagnostic algorithm to approach patients with suspected soft tissue vascular anomalies of the extremities. The management of vascular anomalies necessitates a multidisciplinary approach. Clinical presentation and physical examination are sufficient in most cases to achieve a correct diagnosis. This is especially true for small congenital lesions of the skin and subcutaneous tissue. Imaging is used for accurate characterization of these lesions, especially in cases of atypical or vague clinical presentation, and to assess extension in cases of lesions that are larger and localized in deeper tissues. [ABSTRACT FROM AUTHOR]

Bronchial Dieulafoy's disease (BDD), remains poorly understood, with only 88 cases reported globally. Herein, we present the largest case series (n = 7) from a single centre, between 2017 and 2023, retrospectively reviewed, detailing clinical presentations, diagnoses, management and up to 4‐year follow‐up outcomes. Diagnosis relied on characteristic lesions detected through white light bronchoscopy with or without endobronchial ultrasound (EBUS) or narrow band imaging (NBI), along with computed tomography (CT) scans or bronchial angiography. Identification of aberrant vessels beneath lesions and bronchoscopy details were documented. Treatment modalities and follow‐up outcomes until December 2023 were noted. All patients were non‐smokers. Review of imaging findings by an experienced radiologist was crucial in suspected cases due to risk of bleeding and often unconclusive results from biopsy. Management of BDD varied, with six patients undergoing bronchial artery embolization (BAE) and one requiring lobectomy; four patients received additional endobronchial therapy, one died due to malignancy, none experienced recurrence of haemoptysis. Identifying patients with large volume haemoptysis disproportionate to parenchymal disease in CT scans is important. A bronchoscopic surveillance is crucial to avoid biopsy; it can be confirmed using EBUS of NBI. While no established guidelines exist, BAE and endobronchial therapy emerge as valuable interventions, with surgical resection reserved for recurrent cases. [ABSTRACT FROM AUTHOR]

Vascular malformations are intricate anomalies of the circulatory system, presenting a diverse array of clinical manifestations, and posing significant challenges in diagnosis and treatment. The pathogenesis of vascular malformations is explored through the lens of genetic and molecular mechanisms, shedding light on the pivotal role of somatic mutations and dysregulated signaling pathways. Clinical presentations of vascular malformations are widely variable, ranging from cosmetic concerns to life‑threatening complications. The utility of imaging techniques, such as magnetic resonance imaging (MRI), computed tomography (CT), and angiography, are discussed in detail, emphasizing their role in precise delineation and characterization. Therapeutic strategies for vascular malformations are multifaceted, considering factors such as lesion size, location, potential complications, and patient‑specific factors. Traditional interventions, including surgical excision and embolization, are appraised alongside emerging approaches like targeted molecular therapies and minimally invasive procedures. The manuscript underscores the need for an individualized treatment approach, optimizing outcomes while minimizing risks and complications. In summation, this manuscript offers a comprehensive analysis of vascular malformations, encompassing their underlying pathogenesis, clinical nuances, diagnostic methods, and therapeutic considerations. By synthesizing current knowledge and highlighting gaps in understanding, this review serves as a valuable resource for clinicians, researchers, and medical practitioners, fostering an enhanced comprehension of vascular malformations and paving the way for improved patient care and innovative research endeavors. [ABSTRACT FROM AUTHOR]

Key Clinical Message: While Cerebral vascular malformations exhibit distinct clinical and radiographical features, rare instances of coexisting lesions occur. This case report sheds light on the rare coexistence of brain capillary telangiectasia and venous angioma in a patient presenting with a seizure attributed to frontal lobe bleeding. Though often silent, brain capillary telangiectasia can manifest with serious life‐threatening intracranial bleeding. Therefore, in cases of spontaneous intracranial bleeding, an MRI of the head is crucial to rule out such cerebral vascular malformations. [ABSTRACT FROM AUTHOR]

Copyright of China Journal of Oral & Maxillofacial Surgery is the property of Shanghai Jiao Tong University, College of Stomatology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Vascular anomalies are vascular lesions that are present from childhood. They are classified into tumours or malformations based on clinical and histological features. Benign infantile haemangiomas are the most common vascular tumour and have a predictable self-limiting course. Rarer vascular tumours exist, and some may cause platelet consumption. Management is usually conservative, with active treatment reserved for functional or cosmetic complications (ulceration, or obstruction and distortion of vital structures). Oral propranolol is useful for troublesome lesions. Vascular malformations are structural anomalies of vascular morphogenesis present at birth without cellular proliferation that, in general, grow with the patient. They are sub-classified by vessel type as 'low flow' (capillary, lymphatic and venous) and 'high flow' (arteriovenous) or lesions with a combination of vessel type. They become problematic under certain circumstances, usually puberty and also pregnancy. The most troublesome are extensive lesions, especially venous and arteriovenous. Their effects may be cosmetic, those of a space-occupying lesion, infection, bleeding, pain or coagulopathy. Venous lesions cause consumptive coagulopathy, sometimes with life-threatening risks. Treatment options include medication and symptom control with antibiotics, analgesia, control of menses, compression garments and intervention with laser (capillary type), sclerotherapy, embolization and/or surgical excision. Patients with complex lesions are best managed by a multidisciplinary team and all surgical sub-specialties may be involved. [ABSTRACT FROM AUTHOR]

Copyright of Anaesthesiologie & Intensivmedizin is the property of DGAI e.V. - Deutsche Gesellschaft fur Anasthesiologie und Intensivmedizin e.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Treatment of oropharyngolaryngeal venous malformations (VMs) remains challenging. This study evaluated the effectiveness and safety of fluoroscopy- and endoscopy-guided transoral sclerotherapy for oropharyngolaryngeal VMs in a hybrid operation room (OR). Methods: Patients with oropharyngolaryngeal VMs who underwent transoral sclerotherapy in a hybrid OR were enrolled. Results: Fourteen patients (six females, eight males; median age of 26 years; range, 4–71 years) were analyzed. The symptoms observed were breathing difficulties (n = 3), snoring (n = 2), sleep apnea (n = 1), and swallowing difficulties (n = 1). Lesions were extensive in the face and neck (n = 9) and limited in the oropharyngolarynx (n = 5). A permanent tracheostomy was performed on two patients, while a temporary tracheostomy was performed on five patients. The treated regions were the soft palate (n = 8), pharynx (n = 7), base of the tongue (n = 4), and epiglottis (n = 1). The median number of sclerotherapy sessions was 2.5 (range, 1–9). The median follow-up duration was 81 months (range, 6–141). Treatment outcomes were graded as excellent (n = 2), good (n = 7), or fair (n = 5). The post-treatment complication was bleeding (n = 1), resulting in an urgent tracheostomy. Conclusions: Fluoroscopy- and endoscopy-guided transoral sclerotherapy in a hybrid OR can be effective and safe for oropharyngolaryngeal VMs. [ABSTRACT FROM AUTHOR]

Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital vascular malformation of unknown etiology, characterized by an abnormal cutaneous vascular network. Associated anomalies have been reported in most cases. We report the case of a full-term baby girl who presented purpuric lesions with a reticular pattern on the right lower limb, associated with subcutaneous atrophy of the right knee. The lesions were not affected by heating, suggesting, together with the asymmetry, the diagnosis of CMTC. There was no asymmetry of the lower limbs or other visible anomalies. Dermoscopic examination con- firmed the presence of telangiectasias in the affected areas, while other complementary tests were normal. The vascular lesions showed significant spontaneous improvement in the first two years of life and the child had adequate growth and neurological development throughout the follow-up period. [ABSTRACT FROM AUTHOR]

Cerebral cavernous malformation (CCM) or familial cavernomatosis is a rare, autosomal dominant, inherited disease characterized by the presence of vascular malformations consisting of blood vessels with an abnormal structure in the form of clusters. Based on the altered gene (CCM1/Krit1, CCM2, CCM3) and its origin (spontaneous or familial), different types of this disease can be found. In this work we have isolated and cultivated primary endothelial cells (ECs) from peripheral blood of a type 1 CCM patient. Differential functional and gene expression profiles of these cells were analyzed and compared to primary ECs from a healthy donor. The mutation of the familial index case consisted of a heterozygous point mutation in the position +1 splicing consensus between exons 15 and 16, causing failure in RNA processing and in the final protein. Furthermore, gene expression analysis by quantitative PCR revealed a decreased expression of genes involved in intercellular junction formation, angiogenesis, and vascular homeostasis. Cell biology analysis showed that CCM1 ECs were impaired in angiogenesis and cell migration. Taken together, the results obtained suggest that the alterations found in CCM1 ECs are already present in the heterozygous condition, suffering from vascular impairment and somewhat predisposed to vascular damage. [ABSTRACT FROM AUTHOR]

Introduction: Infantile hemangioma with minimal or arrested growth (IHMAG) is an unusual subset of infantile hemangioma, difficult to recognize because they are often mistaken with capillary malformation or other entities. Dermoscopic features of IHMAG have been described only in small case series so far. Objectives: The aim of our study is to evaluate epidemiological, clinical and dermoscopic features in 79 cases of IHMAG with a specific focus on neonates and toddlers with segmental complicated IHMAG and to provide a remarkable dermoscopic criteria to achieve diagnosis. Methods: This case series collected all the cases of IHMAG recorded in our Clinical Registry from January 2012 to March 2022. Results: A total of 79 cases of IHMAG were identified in our study; 53 (67.1%) were localized and 26 (32.9 %) were segmental. Patients showed any complications during the follow up, as ulceration and soft tissue anomalies. One PHACE syndrome and 2 LUMBAR syndromes were included. Our study highlights the main dermoscopic features differentiating IHMAG from infantile hemangiomas and capillary malformations in neonatal patients, highlighting the presence of enlarged, unfocused telangiectatic vessels as remarkable clues. Conclusions: This is a large case series described in literature about this rare entity. We emphasize that segmental IHMAG may be associated with structural abnormalities and may pose a diagnostic challenge especially in its rare facial segmental localization. The use of dermoscopy has allowed us to find typical signs for IHMAG, thus avoiding the execution of invasive methods and ensuring a prompt suspect of a syndrome on segmental neonatal cases.

Klippel–Trenaunay Syndrome (KTS) is a rare congenital vascular disorder characterized by extensive capillary and venous malformations that pose unique challenges during pregnancy. This case report discusses the successful management of a 34‐year‐old pregnant woman with KTS who had two caesarean sections, resulting in the birth of two healthy babies. Despite the lack of evidence‐based guidelines for obstetrical management in KTS, a multidisciplinary team collaborated to devise a high‐risk thrombosis management plan, involving the use of compression stocking and low molecular weight heparin prophylaxis. The patient's elevated risk of thrombosis, exacerbated during pregnancy, informed the decision of caesarean sections, aligning with finding that in most KTS pregnancies, this method of delivery based on obstetric indications and arteriovenous malformations is chosen. This case highlights the importance of systematic and patient‐centered care, advocating for comprehensive obstetric management guidelines to address the unique challenges posed by KTS during pregnancy. Further research is warranted to enhance our understanding and refine guidelines for individuals with vascular abnormalities linked to KTS. [ABSTRACT FROM AUTHOR]

Somatic activating mutations of PIK3CA are associated with development of vascular malformations (VMs). Here, we describe a microfluidic model of PIK3CA-driven VMs consisting of human umbilical vein endothelial cells expressing PIK3CA activating mutations embedded in three-dimensional hydrogels. We observed enlarged, irregular vessel phenotypes and the formation of cyst-like structures consistent with clinical signatures and not previously observed in cell culture models. Pathologic morphologies occurred concomitant with up-regulation of Rac1/p21-activated kinase (PAK), mitogen-activated protein kinase cascades (MEK/ERK), and mammalian target of rapamycin (mTORC1/2) signaling networks. We observed differential effects between alpelisib, a PIK3CA inhibitor, and rapamycin, an mTORC1 inhibitor, in mitigating matrix degradation and network topology. While both were effective in preventing vessel enlargement, rapamycin failed to reduce MEK/ERK and mTORC2 activity and resulted in hyperbranching, while inhibiting PAK, MEK1/2, and mTORC1/2 mitigates abnormal growth and vascular dilation. Collectively, these findings demonstrate an in vitro platform for VMs and establish a role of dysregulated Rac1/PAK and mTORC1/2 signaling in PIK3CA-driven VMs.

Abstract Nanotechnology has demonstrated immense potential in various fields, especially in biomedical field. Among these domains, the development of nanotechnology for diagnosing and treating vascular anomalies has garnered significant attention. Vascular anomalies refer to structural and functional anomalies within the vascular system, which can result in conditions such as vascular malformations and tumors. These anomalies can significantly impact the quality of life of patients and pose significant health concerns. Nanoscale contrast agents have been developed for targeted imaging of blood vessels, enabling more precise identification and characterization of vascular anomalies. These contrast agents can be designed to bind specifically to abnormal blood vessels, providing healthcare professionals with a clearer view of the affected areas. More importantly, nanotechnology also offers promising solutions for targeted therapeutic interventions. Nanoparticles can be engineered to deliver drugs directly to the site of vascular anomalies, maximizing therapeutic effects while minimizing side effects on healthy tissues. Meanwhile, by incorporating functional components into nanoparticles, such as photosensitizers, nanotechnology enables innovative treatment modalities such as photothermal therapy and photodynamic therapy. This review focuses on the applications and potential of nanotechnology in the imaging and therapy of vascular anomalies, as well as discusses the present challenges and future directions. Graphical Abstract

Introduction: Congenital vascular malformations (CVMs) are malformed vessels developed due to arrested development at various stages of embryogenesis. Arterial venous malformations (VMs) are complex primitive networks of arterial and venous channels (a nidus) that bypass the normal capillary bed. The management of CVM remains challenging because of their unpredictable behavior, high recurrence rate, and need for multidisciplinary management. In this study, we report on the incidence and management of CVM in our institution and its outcome. Materials and Methods: This is a retrospective analysis of CVM patients who were admitted in our institute from January 2010 to June 2023. In this study, 321 patients who presented with CVM are considered. Once a CVM was diagnosed, all underwent angiographic confirmation as a roadmap for treatment. Embolization, sclerotherapy, and surgical procedures were instituted by the multidisciplinary team with periodic follow-up. Results: Out of the 321 patients analyzed, VM predominates with 202 cases (62.9%), out of which 105 (51.9%) were treated with sclerotherapy, 66 (28.21%) were treated with excision, 40 (19.8%) were managed conservatively. Among 108 arteriovenous malformation (33.64%), 33 (30.55%) patients were treated with excision followed by repair and flap cover/split skin graft, 32 (29.62%) were treated with glue Embolization, 7 (6.48%) were treated with embolization followed by excision, one patient presented with life-threatening bleeding was treated by emergency ECA ligation followed by elective excision, 21 (19.44%) were managed conservatively and 13 (12.03%) patients were not willing for surgery. Seven (2.1%) lymphovenous malformation and 4 (1.24%) lymphatic malformations patients were also treated. Conclusion: Diagnosis and management of CVMs by a multidisciplinary approach that integrates surgical therapy with embolization and sclerotherapy appear to improve the results and management.

Objective: The incidence of left-sided inferior vena cava (IVC) is extremely low. However, without a preoperative diagnosis of left-sided IVC, the risk of intraoperative vascular injury during para-aortic lymph node (PAN) lymphadenectomy is high. Case report: Herein, we present two cases in which left-sided IVCs were diagnosed using preoperative imaging. PAN lymphadenectomies were safely performed in these patients with endometrial cancer. In the first case, the left-sided IVC crossed the abdominal aorta after the left renal and gonadal veins had drained into it and joined the right renal vein. In the second case, the left-sided IVC crossed the abdominal aorta after the left renal and gonadal veins flowed into it and the ascending lumbar vein flowed into the right side. Conclusion: These cases demonstrate that even in the presence of vascular malformations, PAN lymphadenectomy can be performed safely by employing preoperative anatomical imaging analysis and judicious intraoperative surgical maneuvers to avoid vascular injury.

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning.