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Venous malformation (VM) is a prevalent congenital vascular anomaly characterized by abnormal blood vessel growth, leading to disfigurement and dysfunction. Sclerotherapy, a minimally invasive approach, has become a primary therapeutic modality for VM, but its efficacy is hampered by the rapid dilution and potential adverse effects. In this study, we introduced a series of cationic amphiphilic molecules, fatty alcohol esters (TA6, TA8, and TA9) of tranexamic acid (TA), which self-assembled into low-molecular-weight gels (LMWGs) in water. The TA9, in particular, is released slowly when hydrogel is injected into the vein locally. Then, it damages the venous wall by destroying cell membranes and precipitating proteins, causing inflammation and thrombosis, thickening of the venous wall, effectively inducing irreversible vein fibrosis. Additionally, TA9 can be rapidly degraded into TA in plasma to reduce toxicity caused by diffusion. Overall, this study suggests that the cationic amphiphilic molecule TA9 is a promising sclerosant for VM treatment, offering a novel, effective, and safe therapeutic option with potential for clinical translation., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objective: To compare and analyze the efficacy and safety of different methods for injecting polidocanol in the treatment of venous malformations., Methods: The medical records of patients with venous malformations in our hospital from April 2021 to April 2023 were analyzed retrospectively, and they were divided into control group ( n = 38) and observation group ( n = 20) according to different treatment methods. Control group was injected with polidocanol under ultrasound guidance, while observation group was injected with polidocanol under digital subtraction angiography (DSA) monitoring. Therapeutic effects of observation group and control group after 3-6 months of treatment were compared and analyzed, and complications that can be used to reflect the safety of treatment in two groups were collected and recorded., Results: After treatment, the total effective rates of observation group and control group were 97.37% (37/38) and 75.00% (15/20) respectively, with significant differences (both p < .05). After 3-6 months of treatment, 13 complications occurred in observation group, while 16 occurred in control group. The number of complications in observation group was 6, with an incidence rate of 15.78%; while that in control group was 9, with an incidence rate of 45.00%, with a significant difference ( p < .05). There were no significant differences among other baseline data, age and complications between two groups (all p > .05)., Conclusion: Injection of polidocanol in patients with venous malformations under DSA monitoring was more effective and safer., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

During standard cadaveric dissection we encountered multiple vascular variations in the retroperitoneum: duplicated and dilated left ovarian vein with the coexistence of a persistent right mesonephric artery., (© 2024. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Common capillary malformations are red vascular skin lesions, most commonly associated with somatic activating GNAQ or GNA11 mutations. We focused on capillary malformations lacking such a mutation to identify previously unreported genetic causes. We used targeted next-generation sequencing on 82 lesions. Bioinformatic analysis allowed the identification of 9 somatic pathogenic variants in PIK3R1 and PIK3CA, encoding for the regulatory and catalytic subunits of phosphoinositide 3-kinase, respectively. Recharacterization of these lesions unraveled a common phenotype: a pale capillary malformation associated with visible dilated veins. Primary endothelial cells from 2 PIK3R1-mutated lesions were isolated, and PI3k-Akt-mTOR and RAS-RAF-MAPK signaling were assessed by western blot. This unveiled an abnormal increase in Akt phosphorylation, effectively reduced by PI3K pathway inhibitors, such as mTOR, Akt, and PIK3CA inhibitors. The effects of mutant PIK3R1 were further studied using zebrafish embryos. Endothelium-specific expression of PIK3R1 mutants resulted in abnormal development of the posterior capillary-venous plexus. In summary, capillary malformation associated with visible dilated veins emerges as a clinical entity associated with somatic pathogenic variants in PIK3R1 or PIK3CA (nonhotspot). Our findings suggest that the activated Akt signaling can be effectively reversed by PI3K pathway inhibitors. In addition, the proposed zebrafish model holds promise as a valuable tool for future drug screening aimed at developing patient-tailored treatments., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

The insect wing is one of the most important characteristics that allowed insects to occupy most of the habitats on the planet. Honeybee wings has been the subject of studies on the venation abnormalities. A total of 424 honeybees from 14 locations were collected and all four wings were removed and examined for 19 abnormalities on the forewings and 6 abnormalities on the hindwings. In general, supernumerary veins were the most common abnormalities seen and abnormalities no. 23, 2, 6, 1, 5, 21, 10, 13 had the highest and abnormalities no. 11, 17, 18, 19, 20, and 25 had the lowest frequencies. All of the abnormalities had similar frequencies in the right and left wings in the population. In terms of correlation between 25 abnormalities, abnormality pairs AB3-AB13, AB6-AB7, AB7-AB8, AB10-AB12, AB16-AB17 on the forewing and AB2-AB23, AB12-AB20, AB12-AB24, AB13-AB21, AB16-AB25, and AB17-AB25 between the forewing and hindwing show significant positive correlations and abnormality pairs AB4-AB5, AB7-AB15 and AB8-AB9 on the forewing show significant negative correlations with each other. In terms of the differential occurrence of abnormalities , a few locations differed significantly from other locations. This study provides some insights into the nature of these abnormalities on the honeybee wings., (© 2024. The Author(s).)

Background: Among low-flow vascular malformations, venous malformations are relatively frequent. The pathological patterns vary in severity and are generally characterized by dilated vessels and low-flow blood that over time can organize into phleboliths. Sometimes small capillary and/or lymphatic vessels may be associated, micro- and/or macro-shunts may form alone or in different combinations, and finally adipose tissue may be interposed between the malformed vessels. Magnetic resonance imaging (MRI) is a crucial examination for confirming venous malformations because it can accurately identify different features of the lesions., Objective: The aim of our study was to compare MRI and histopathological findings of venous malformations in children to assess the possibilities and limitations of MRI., Materials and Methods: In a retrospective study, two observers independently evaluated the contrast-enhanced MRI of 26 children with venous malformations. Several radiological parameters were considered and compared with histopathological findings. The agreement between the interobserver radiological evaluation and between histopathological and radiological diagnosis was verified using Cohen's kappa., Results: MRI interobserver agreement was excellent for micro-shunts and good for the remaining findings. The radiological-pathological agreement was perfect for the presence/absence of phleboliths and of macro-shunts and almost perfect for the presence of intralesional adipose tissue, lymphatic component, and micro-shunts., Conclusion: MRI in venous malformations can detect the presence of phleboliths, adipose tissue, and lymphatic components with excellent accuracy and good to excellent interobserver agreement. Furthermore, MR angiography can detect micro-shunts in simple and combined venous malformations with substantial agreement with histopathological findings., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Description of clinical features, radiological characteristics, and management strategies in primary orbital intraosseous venous malformation (OIVM) with pertinent literature review., Methods: A retrospective analysis including clinical, radiologic, operative, and histopathological data of six cases of histopathologically proven OIVM was done. A comprehensive literature review was conducted using online databases and augmented with manual search to identify reported cases of OIVM., Results: Study data showed five females and one male in young to middle-age group, with an average age of 30 years (range: 20-48 years). Proptosis was noted in five cases (83.33%), and the duration of symptoms ranged from 6 months to 10 years. Frontal and zygomatic bones were most frequently affected and expansile bony lesion was the most common CT scan finding. Three patients underwent pre-operative embolization of feeders followed by en bloc excision of mass and surgical reconstruction (50%); one patient was managed with partial excision (16.66%) while two were regularly followed-up after incision biopsy (33.33%). Histopathology revealed vascular spaces with endothelial lining, separated by bony trabeculae in all patients. Follow-up periods ranged from 6 to 48 months and no recurrence or progression were noted., Conclusions: OIVM is an exceptionally rare disorder with a gradually progressive benign course. Ophthalmologists need to be mindful of this entity during patient evaluation as it has propensity for large volume blood loss intra-operatively, owing to its vascular nature. Complete excision with reconstruction of resultant defect is the preferred treatment strategy and without known recurrence.

Overgrowth syndromes, particularly within the PIK3CA-related overgrowth syndrome (PROS) spectrum, are commonly associated with venous anomalies. The anomalies include spongiform venous malformations and persistent embryonic veins, such as the lateral marginal vein (of Servelle). The anomalous veins pose a significant risk of thromboembolic disease and should be occluded, preferably earlier in life. A thorough understanding of the conditions, anatomy, and interdisciplinary treatment of these complex anomalies is essential for optimal management. This review explores the clinical and imaging diagnosis of overgrowth syndromes and techniques for assessing and treating associated venous anomalies, particularly the endovenous closure of anomalous veins., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Venous malformation (VM) stands as the most prevalent form of vascular malformation, characterized by its diverse morphology. These lesions can manifest in any part of the body, affecting different tissue planes and giving rise to symptoms such as pain, swelling, or physical dysfunction. In the realm of treatment, direct puncture VM sclerotherapy holds its place as the primary approach. This technique involves the administration of a sclerosing agent into the VM channels during contrast phlebography while simultaneously managing the outflow veins through different methods. The process of VM sclerotherapy induces endothelial damage, thrombosis, and fibrosis, resulting in symptom relief through lesion shrinkage. It is crucial to exercise caution techniques and sclerosing agents during VM sclerotherapy to minimize procedural complications, enhance clinical outcomes, and ultimately improve the patient's overall quality of life., (Copyright © 2024. Published by Elsevier Inc.)

Background: The presence of a vascular, blue linear discoloration on the nasal root of infants and young children is a frequent incidental feature, rarely reported in the medical literature. It is related to the trajectory of the transverse nasal root vein (TNRV)., Objective: To study the frequency and clinical characteristics of the vascular discoloration of the nasal root in children., Methods: A prospective study was performed to address the presence or absence of vascular discoloration of the nasal root in all children under 6 years of age attending a pediatric dermatology clinic from November 2022 to November 2023. Data on age and skin phototype (Fitzpatrick classification I-VI) were also collected., Results: Of 701 patients examined, 345 (49.2%) presented with a vascular discoloration of the nasal root. This was present in 97 of 193 (50.3%), 127 of 261 (48.7%), and 121 of 247 (49.0%) patients for the age groups 0-1, 1-3, and 3-6 years, respectively. The presence of vascular discoloration of the nasal root was more frequent in patients with lighter Fitzpatrick skin phototypes: 49 of 69 (71.0%) phototype II, 157 of 290 (54.1%) phototype III, and 137 of 337 (40.7%) phototype IV., Conclusions: A vascular discoloration of the nasal root is a frequent skin feature in infants and children, persisting at least until the age of 6. It does not constitute any medical problem aside from cosmetic concern and parents can be reassured of its benign nature. We propose the medical term "prominent TNRV" to describe this condition., (© 2024 Wiley Periodicals LLC.)

Objective: The International Union of Phlebology recommends measuring at least D-dimer and fibrinogen levels in the diagnosis of extensive extra-truncular venous malformations, with a surface area of 10 cm 2 or those which are deep, as well as prior to any interventional procedure. The aim of the study was to characterise venous malformations associated with a possible vascular complication., Method: This study was an observational and multicentre study. The objective was to explore the presence of a possible coagulation disorder among patients with venous malformation. The primary endpoint was to characterise venous malformations with increased D-dimer levels., Results: The majority of the 72 venous malformations were located in the trunk region, mostly in intramuscular or subcutaneous locations. There were 72 venous malformations with increased D-dimer levels including 3 with biological disseminated intravascular coagulation (elevated D-dimer and fibrinogen <1 g/L). The anticoagulant treatments administered were very heterogeneous in class and dosage, and at the end of the treatment, 17 elevated D-dimers were persistent, 9 venous malformations remained painful and 27 showed thrombotic regression., Conclusion: Venous vascular malformations are probably underestimated and should probably be explored more systematically in terms of coagulation disorder regardless of size or symptomatology. The therapeutic recommendations to treat localised intravascular coagulation with low-molecular weight are not widely applied. Studies are needed, in particular to assess the role of oral anticoagulants in the management of coagulation disorder among patients with venous malformation., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Rationale: Klippel-Trenaunay syndrome (KTS) is a rare congenital venous malformation, it had been found to be caused by mutations of the phosphatidylinositol 4, 5-diphosphate 3-kinase catalytic subunit alpha (PIK3CA) gene. Currently KTS is defined as a triad of skin wine pigmented spots, varicose veins and malformations of the lower extremities, and hypertrophy of bone and soft tissue, involving urinary system up to 6% to 30%. When the urinary system is involved, KTS is often presented as painless massive gross hematuria., Patient Concerns: This article describes a woman who was hospitalized with painless massive gross hematuria. Physical examination revealed significant hypertrophy of the right lower limb with varicose veins, port-wine stains in the skin, and right perineal hemangiomatous changes with swelling. The patient was admitted to hospital 4 times for repeated hematuria and infection., Diagnoses: By physical examination, CT urography, ureteroscopy and cystoscopy, the patient was diagnosed to have Klippel-Trenaunay syndrome, involving the urinary system., Interventions: The patient hematuria improved after multiple indwelling D-J tubes and anti-inflammatory treatment., Outcomes: The final symptoms of hematuria improved significantly, follow-up so far has not recurred., Lessons: This case presents the possibility of painless gross hematuria with KTS. Most of patients can be improved by conservative treatment. Cystoscopic laser therapy is the preferred treatment for poor bleeding control. Cystectomy and nephrectomy should be considered when life-threatening., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Venous malformations (VMs) are the most common vascular malformations, which can be classified as focal, multifocal, or diffuse types. But the risk of focal venous malformations with muscle involvement is not well defined., Methods: This is a single-center, retrospective review of patients treated for focal VMs between February 2021 and February 2022., Results: We assessed 26 patients focal VMs with 47 lesions; 18 (69%) were unifocal, 3 (12%) were dual-focal, and 5 (19%) were multifocal type VMs, and 29 (62%) were intramuscular VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs (relative risk [RR],1.7; 95% confidence interval [CI], 1.148-2.394)., Conclusion: Intramuscular involvement of the body should be considered in focal VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: To retrospectively evaluate sclerotherapy using consecutive polidocanol and bleomycin foam (CPBF) for large untreated venous malformations (VMs) and/or those resistant to prior treatment., Materials and Methods: This retrospective study included all patients treated with CPBF for untreated VMs larger than 10 mL and/or refractory to treatment between May 2016 and October 2019. Baseline and follow-up VM volumes were measured on fat-suppressed T2-weighted magnetic resonance (MR) imaging. Outcome was evaluated on postprocedural MR imaging volumetry and by a retrospective survey assessing clinical response and adverse events. Imaging response was considered good for volume reduction from 50% to 70% and excellent for volume reduction ≥70%. Symptoms and quality-of-life (QoL) scores were compared before and after CPBF sclerotherapy., Results: Forty-five patients (mean age, 16 years; range, 1-63 years; 25 males) with 57 VMs were analyzed and treated by 80 sclerotherapy. Sixty percent (27 of 45) of patients had undergone prior treatment for VM. Median VM volume was 36.7 mL (interquartile range, 84 mL) on pretherapy MR imaging. Good and excellent results after the last sclerotherapy were achieved in 36% (16 of 45) and 29% (13 of 45) of patients, respectively, corresponding to a decrease of >50% in 60% (34 of 57) of VMs. QoL score increased by at least 3 points, regardless of initial symptoms. Most patients did not desire additional sclerotherapy owing to near complete symptomatic relief, even for patients who did not achieve a good response. Swelling, pain, and motor impairment scores significantly improved after CPBF. Adverse events included fever (44%, 15 of 34) and nausea/vomiting (29%, 10 of 34)., Conclusions: CPBF sclerotherapy represents an effective therapy for large and/or refractory VMs with minimal adverse events., (Copyright © 2023. Published by Elsevier Inc.)

Objective: In recent years, genotypic characterization of congenital vascular malformations (CVMs) has gained attention; however, the spectrum of clinical phenotype remains difficult to attribute to a genetic cause and is rarely described in the adult population. The aim of this study is to describe a consecutive series of adolescent and adult patients in a tertiary center, where a multimodal phenotypic approach was used for diagnosis., Methods: We analyzed clinical findings, imaging, and laboratory results at initial presentation, and set a diagnosis according to the International Society for the Study of Vascular Anomalies (ISSVA) classification for all consecutively registered patients older than 14 years of age who were referred to the Center for Vascular Malformations at the University Hospital of Bern between 2008 and 2021., Results: A total of 457 patients were included for analysis (mean age, 35 years; females, 56%). Simple CVMs were the most common (n = 361; 79%), followed by CVMs associated with other anomalies (n = 70; 15%), and combined CVMs (n = 26; 6%). Venous malformations (n = 238) were the most common CVMs overall (52%), and the most common simple CVMs (66%). Pain was the most frequently reported symptom in all patients (simple, combined, and vascular malformation with other anomalies). Pain intensity was more pronounced in simple venous and arteriovenous malformations. Clinical problems were related to the type of CVM diagnosed, with bleeding and skin ulceration in arteriovenous malformations, localized intravascular coagulopathy in venous malformations, and infectious complications in lymphatic malformations. Limb length difference occurred more often in patients with CVMs associated with other anomalies as compared with simple or combined CVM (22.9 vs 2.3%; P < .001). Soft tissue overgrowth was seen in one-quarter of all patients independent of the ISSVA group., Conclusions: In our adult and adolescent population with peripheral vascular malformations, simple venous malformations predominated, with pain as the most common clinical symptom. In one-quarter of cases, patients with vascular malformations presented with associated anomalies on tissue growth. The differentiation of clinical presentation with or without accompanying growth abnormalities need to be added to the ISSVA classification. Phenotypic characterization considering vascular and non-vascular features remains the cornerstone of diagnosis in adult as well as pediatric patients., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: Surgical treatment of venous malformations (VMs) of the hand is challenging. The hand's small functional units, dense innervation, and terminal vasculature can be easily compromised during invasive interventions like surgery or sclerotherapy, leading to an increased risk of functional impairment, cosmetic consequences, and negative psychological effects., Methods: We have conducted a retrospective review of all surgically treated patients diagnosed with VMs of the hand between 2000 and 2019 and evaluated their symptoms, diagnostic investigations, complications, and recurrences., Results: Twenty-nine patients (females, n = 15) with a median age of 9.9 years (range, 0.6-18 years) were included. Eleven patients presented with VMs involving at least one of the fingers. In 16 patients, the palm and/or dorsum of the hand was affected. Two children presented with multifocal lesions. All patients presented with swelling. Preoperative imaging was done in 26 patients and consisted of magnetic resonance imaging in nine patients, ultrasound in eight patients, and both modalities in nine patients. Three patients underwent surgical resection of the lesions without any imaging. Indications for surgery were pain and restriction of function (n = 16), and when lesions were preoperatively evaluated as completely resectable (n = 11). In 17 patients, a complete surgical resection of the VMs was performed, whereas in 12 children, an incomplete resection of VM was deemed due to nerve sheath infiltration. At a median follow-up of 135 months (interquartile range, 136.5 months; range, 36-253 months), recurrence occurred in 11 patients (37.9%) after a median time of 22 months (range, 2-36 months). Eight patients (27.6%) were reoperated because of pain, whereas three patients were treated conservatively. The rate of recurrences did not significantly differ between patients presenting with (n = 7 of 12) or without (n = 4 of 17) local nerve infiltration (P = .119). All surgically treated patients who were diagnosed without preoperative imaging developed a relapse., Conclusions: VMs in the region of the hand are difficult to treat, and surgery is associated with a high recurrence rate. Accurate diagnostic imaging and meticulous surgery may contribute to improve the outcome of the patients., (Copyright © 2023. Published by Elsevier Inc.)

Management of tongue venous malformations can be challenging in the pediatric population due to their heterogeneity in presentation, extent of involvement and functional compromise. It is important to recognize the value of various treatment options in order to guide management of each patient in an individualized fashion. Here we describe a series of patients with tongue venous malformations that are managed using diverse modalities to illustrate the relative benefits and risks of each technique. The challenges of venous malformation treatment can be mitigated by tailoring the approach to each individual patient and malformation. This case series also emphasizes the need and importance of working in the setting of a multidisciplinary vascular anomalies team., Competing Interests: Potential conflicts of interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Purpose: Here, we report our experience treating a patient with Maffucci syndrome and evaluate the outcomes resulting from surgical management combined with sclerotherapy in the treatment of head and neck venous malformations (VMs). A 19-years-old woman with multiple enchondromas and heterauxesis complained of masses in the oral cavity that had gradually increased in size and eventually affected her daily life. A tracheotomy was performed followed by traditional sclerotherapy to treat the oropharyngeal VMs. Next, we surgically excised the VMs of the oral cavity and maxillofacial skin., Results: Magnetic resonance imaging indicated that the oral VMs were nearly eradicated and the oropharyngeal VMs had stabilized. The patient's appearance and normal maxillofacial region function were restored., Conclusion: In summary, local resection combined with sclerotherapy facilitated timely and efficient VMs removal from the head and neck region of a patient with Maffucci syndrome.

Introduction: Venous malformations affect around 2 per 10,000 newborn and are the most common type of congenital vascular malformation. They are always present at birth and are often misdiagnosed with hemangiomas. Accurate diagnosis and adequate targeted therapy through a multidisciplinary approach is advocated for a successful treatment, considering a combination of modalities in complex cases. We present here the workup and treatment of a venous malformation in a 14 month old child by combining a preoperative sclerotherapy with sodium tetradecyl sulfate (STS) followed by complete surgical excision respecting the calvarium., Case Presentation: A large right extracranial fronto-parietal venous malformation and scalloping of the underlying calvarium, attached to the pericranium, surgically excised after preoperative sclerotherapy with STS in a 14-month-old child., Results: The patient had an uneventful postoperative course with complete resection of the lesion, satisfying cosmetic appearance and no complications. Histopathological examination confirmed a venous malformation., Conclusion: We demonstrated the workup and the rationale of the combined sclerotherapy followed by surgical resection of a large growing extracranial venous malformation. Preoperative direct contrast injection and sclerotherapy allowed to rule out significative extracranial to intracranial venous inflow and reduce blood loss for the surgical procedure, respectively. Complete removal of the malformation minimized the impact on future growth of the calvarium., (© 2022. The Author(s).)

Background: Venous malformations (VMs) and sclerotherapy may disrupt the normal systemic coagulation profile in individuals. This study investigated a correlation between the clinical efficacy of sclerotherapy in the treatment of VMs and the changes in coagulation indexes to provide data that will inform the future application of this therapy., Methods: From September 2019 to September 2020, 61 patients were enrolled in this study to receive sclerotherapy with absolute alcohol. The clinical outcomes and the coagulation profile were assessed., Results: Sclerotherapy induced increasing fibrin (original) degradation products (FDP) and D-dimer (D-D) levels. The changes in FDP and D-D level pretreatment and posttreatment were positively correlated with treatment outcomes. Moreover, a repeated treatment with absolute alcohol may restore normal levels of FDP and D-D., Conclusions: Upregulation of FDP and D-D levels after sclerotherapy results in good therapeutic outcomes. Therefore, monitoring changes in FDP and D-D levels in patients with VMs undergoing sclerotherapy may reflect the effects of sclerotherapy., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Purpose: Treatment for orbital venolymphatic malformations (VLMs) commonly includes 3 major options: sclerotherapy, surgery, and embolization followed by surgical excision. Each has certain advantages, although it is not clear whether all are effective. The authors characterize the clinical course for a series of patients with distensible orbital venous-dominant VLM treated with sclerotherapy and/or embolization with excision., Methods: In this cross-sectional cohort study, patients affected by distensible orbital venous-dominant VLM presenting to the orbital and ophthalmic plastic surgery service from 2014 to 2020 were identified. Patients were included if they presented with a moderate-flow, distensible venous-dominant malformation associated with Valsalva-related symptoms (e.g., pain, proptosis, and diplopia)., Results: Six cases were treated with sclerotherapy. Four underwent multiple treatments, with a mean ± SD of 3.5 ± 2.3 (range 1-7). All patients in this group failed to improve or experienced recurrence of symptoms after sclerotherapy. Twelve cases were treated with embolization and excision. Resolution of symptoms in all 12 cases was noted and maintained for a mean of 3.4 ± 2.1 years. There have been no cases of recurrence. Patients treated with sclerotherapy were more likely to experience recurrence of symptoms compared to those treated with embolization and excision (p < 0.001)., Conclusions: Treatment of distensible venous-dominant moderate-flow orbital VLM with sclerotherapy may provide temporary improvement in some cases. However, in the medium to long term, recurrence was universal in this series. Embolization with excision appears to provide more definitive management, avoiding recurrence in all cases for a mean follow-up of 3 years., Competing Interests: The authors have no financial or conflicts of interest to disclose. The authors alone are responsible for the content and writing of the paper., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Objective: Venous malformations (VMs) are the most frequent vascular malformations and are characterized by dilated and tortuous veins with a dysregulated vascular extracellular matrix. The purpose of the present study was to investigate the potential involvement of microRNA-21 (miR-21), a multifunctional microRNA tightly associated with extracellular matrix regulation, in the pathogenesis of VMs., Methods: The expression of miR-21, collagen I, III, and IV, transforming growth factor-β (TGF-β), and Smad3 (mothers against decapentaplegic homolog 3) was evaluated in VMs and normal skin tissue using in situ hybridization, immunohistochemistry, Masson trichrome staining, and real-time polymerase chain reaction. Human umbilical vein endothelial cells (HUVECs) were used to explore the underlying mechanisms., Results: miR-21 expression was markedly decreased in the VM specimens compared with normal skin, in parallel with downregulation of collagen I, III, and IV and the TGF-β/Smad3 pathway in VMs. Moreover, our data demonstrated that miR-21 positively regulated the expression of collagens in HUVECs and showed a positive association with the TGF-β/Smad3 pathway in the VM tissues. In addition, miR-21 was found to mediate TGF-β-induced upregulation of collagens in HUVECs. Our data have indicated that miR-21 and the TGF-β/Smad3 pathway could form a positive feedback loop to synergistically regulate endothelial collagen synthesis. In addition, TGF-β/Smad3/miR-21 feedback loop signaling was upregulated in bleomycin-treated HUVECs and VM specimens, which was accompanied by increased collagen deposition., Conclusions: To the best of our knowledge, the present study has, for the first time, revealed downregulation of miR-21 in VMs, which might contribute to decreased collagen expression via the TGF-β/Smad3/miR-21 signaling feedback loop. These findings provide new information on the pathogenesis of VMs and might facilitate the development of new therapies for VMs., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: The clinical presentation of venous malformations (VMs) is variable and not well documented. The present study was designed to determine the clinical characteristics and outcomes in association with the type and extent of VM in the extremities., Methods: Patients with a diagnosis of a VM using ultrasound, computed tomography, or magnetic resonance imaging were included in the present study. The VM extent and tissue involvement, signs, and symptoms were retrospectively collected. Those with arteriovenous malformations, Klippel-Trenaunay syndrome, lymphedema, and symptoms from other unrelated diseases were excluded. The VMs were classified as extensive or localized according to whether different tissues or the entire compartment was involved. The clinical outcomes and imaging studies were obtained for both the treated patients and those who had chosen to not undergo any intervention., Results: A total of 72 patients, of whom 26 were men, were included in the present study, with a median age of 30 years. The VM had extended into the subfascial space in 40 patients (55.6%). Patients with VM distributed on their leg had mostly experienced pain, which was more evident during physical activity (n = 40; 80.0%), with swelling worsening with prolonged standing (n = 35; 70.0%). For those with VMs in the upper extremities, the most prominent complaints were venous enlargement (n = 10; 45.5%), pain (n = 9; 40.9%), and swelling (n = 11; 50.0%). The symptoms were related to the extent of the lesion (odds ratio, 7.664; 95% confidence interval, 2.006-29.291; P = .003). Treatment was decided by the extent and depth of the lesions. Excision was performed in 15 patients (20.8%), sclerotherapy in 12 (16.7%), sclerotherapy with phlebectomy or coil embolization in 8 (11.1%), and stripping in 2 (2.8%). An excellent result was achieved with the treatment of localized VMs. For those with extensive VMs, the symptom intensity was reduced or controlled after multiple treatments., Conclusions: VMs of the extremities affect patients' quality of life significantly owing their appearance and the resulting pain. The VM extent was associated with symptom severity. The clinical outcomes have been excellent for localized VMs; however, extensive VMs will require multiple treatments to reduce the symptom intensity., (Copyright © 2021 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Venous anomalies of the lower extremity are a challenge when found as rare variations. Physicians should be aware of morphological variations in this region for correct diagnosis and management of diseases such as deep vein thrombosis., Materials and Methods: During the routine dissection of the lower extremities of a Caucasian male cadaver, a case of persistent sciatic vein was found., Results: A persistent sciatic vein originating from the saphenopopliteal junction coursed proximally and laterally to enter into the adductor magnus muscle, at the right side. Running through the fibres of the adductor magnus anteriorly, the variant vein drained into the deep femoral vein just medial to the vastus medialis muscle., Conclusions: As being a rare congenital vascular anomaly, the persistent sciatic vein should be correctly diagnosed and defined during the radiologic assessments, surgical interventions and blockades for better outcomes and lesser complications.

Venous malformation (VM) and cavernous venous malformation (CVM) are two types of vascular malformations. Even if the two diseases are similar in appearance and imaging, the distinct cellular components and signaling pathways between them might help distinguish the two from a molecular perspective. Here, we performed single-cell profiling of 35,245 cells from two VM samples and three CVM samples, with a focus on endothelial cells (ECs), smooth muscle cells (SMCs) and immune microenvironment (IME). Clustering analysis based on differential gene expression unveiled 11 specific cell types, and determined CVM had more SMCs. Re-clustering of ECs and SMCs indicated CVM was dominated by arterial components, while VM is dominated by venous components. Gene set variation analysis suggested the activation of inflammation-related pathways in VM ECs, and upregulation of myogenesis pathway in CVM SMCs. In IME analysis, immune cells were identified to accounted for nearly 30% of the total cell number, including macrophages, monocytes, NK cells, T cells and B cells. Notably, more macrophages and monocytes were discovered in VM, indicating innate immune responses might be more closely related to VM pathogenesis. In addition, angiogenesis pathway was highlighted among the significant pathways of macrophages & monocytes between CVM and VM. In VM, VEGFA was highly expressed in macrophages & monocytes, while its receptors were all abundantly present in ECs. The close interaction of VEGFA on macrophages with its receptors on ECs was also predicted by CellPhoneDB analysis. Our results document cellular composition, significant pathways, and critical IME in CVM and VM development., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

The often inexorable growth and expansion of congenital vascular malformations can result in substantial morbidity and, in some cases, premature death of these patients. Despite this, patients suffering from such lesions are often erroneously diagnosed and/or inadequately treated, due to a lack of expertise among primary care practitioners as well as specialists. Venous malformations are the most common type of congenital vascular malformations. Over the last two decades management of these lesions has significantly improved, predominantly due to the introduction and implementation of multidisciplinary team concept as well as improvement in diagnostic and treatment modalities. Relatively recently genetic studies are providing more insights into underlying pathophysiological mechanisms responsible for the development and progression of venous malformations and pharmacotherapy is becoming extensively evaluated for safety and efficacy in the treatment of these often challenging vascular lesions.

Background: Venous malformations (VMs) are congenital vascular malformations that grow progressively and never resolve on their own. Cutaneous VMs are difficult to treat due to risk of injury and deformation. The purpose of this study was to examine the safety and efficacy of a modified neodymium-doped yttrium aluminum garnet laser (Gentle YAG) in the management of cutaneous VMs. Methods: Retrospective chart review of patients undergoing Gentle YAG therapy for cutaneous VMs and a blind prospective evaluation of photographs, performed by 10 reviewers, before and after treatment for growth, stability, improvement, or resolution of VMs. Results: Forty-five patients (18 males and 27 females) who underwent Gentle YAG therapy for a cutaneous VM were identified. Based on photographic review, Gentle YAG therapy elicited improvement in the appearance of VMs in 72% of the patients, χ 2 (1, N  = 45) = 25.94, p  < 0.0001, with reviewers noting complete resolution in 8.2%, significant improvement in 34.5%, some improvement in 29.3%, and no growth or improvement in 20.9% of patients. Growth of the VM was noted in 7.3% of patients. Three (6.7%) patients reported complications from the treatment, which included infection, bleeding, blister, and color change. Four patients (8.9%) reported pretreatment pain, which resolved in three (75.0%) after treatment. Conclusions: Gentle YAG therapy can provide safe and effective treatment for cutaneous VMs and should be considered in the multimodal management of VMs.

Background: Our aim was to evaluate the benefit of surgical resection of the venous malformations (VMs) of the external female genitalia., Methods: Over the period of 2009-2019, 18 consecutive females underwent surgical resection for vulvar VM. Evaluations included preoperative Doppler ultrasound, MRI, and pre-and postoperative photographic imaging. The main outcomes were: residual pain, cosmetic distortion, residual VM, and quality of life., Results: Over a 10 year periods, 18 females, mean age 35 years (range 9-71) were included in this study. All patients were symptomatic: 16 had intermittent pain or discomfort, 1 had bleeding and 2 requested cosmetic treatment. Of these cases, there were 5 isolated vulvar VM, 12 associated VM: 3 of the clitoral hood, 3 troncular pelvic vein insufficiency and 12 of the lower limb. Eight patients had undergone previous procedures: 2 sclerotherapy treatments (1-3 sessions), 4 partial surgical resections. There were 18 single resections in the vulva (7 focal, 11 complete), 2 partial resections in clitoral hood and 2 had resection of a VM in the perirtoneovaginal canal at the same time. The mean follow-up was 42. 9 months (range 6-120). Two patients were lost to followup at 6 months. For all patients, elimination of pain and soft tissue redundancy was achieved. Two patients had persistent discomfort and 2 requested cosmetic treatment., Conclusion: Surgical resection of vulvar VM can be the best approach with few postoperative complications, good functional and cosmetic results. Appropriate preoperative evaluation is required to identify isolated VM or VM associated with ovarian vein or internal iliac vein insufficiency requiring to be treated before surgery., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Objective: (1) Review a multidisciplinary vascular anomalies center's practice regarding periprocedural anticoagulation for venous malformations (VM) and the associated risk of thromboembolic and disseminated intravascular coagulation (DIC) events. (2) Compare the risk of thromboembolic events and DIC post-procedure between head and neck (H&N) and extremity VM patients., Methods: An Institutional Review Board (IRB)-approved, retrospective chart review was performed on 120 VM patients. A thromboembolic event was defined as a thrombus formation post-sclerotherapy or post-surgery within 2 months in a distant or local venous structure not directly addressed by the procedure., Results: There were 39 cases involving the H&N and 81 cases based at the extremities. There were eight cases of post-procedure thrombus formation within the extremity VM group (8/71; 11.3%) as opposed to 0 cases in the H&N group (OR: 0, 95% CI .00-.09), p = .049. There was no difference in incidence of post-procedure thromboembolic events between those with elevated D-dimer (H&N: 0%, extremity: 22.7%, 5/22) and normal D-dimer values (H&N: 0%, extremity: 6.3% [1/16], P = .370). There was no difference in incidence of post-procedure thromboembolic events between those who received periprocedural anticoagulation (H&N: 0%, extremity: 21%, 4/19) and those who did not (H&N: 0%, extremity: 8.2%, 4/49), (Extremity: OR: 3.00, .67-13.50, P = .206)., Conclusion: Post-procedure thromboembolism is rare in the treatment of venous malformations, especially in the head and neck subsite. Regardless of anticoagulation use, there were no thromboembolic events for H&N VM patients. Such events are rare, and the odds may approach zero, especially with small sample size., Level of Evidence: 4 Laryngoscope, 131:1163-1167, 2021., (© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).)

Objective: Embolo-sclerotherapy (EST) is the mainstay therapy for peripheral vascular malformations that involves the exposure of patients to ionizing radiation. We analyzed the radiation exposure to patients from EST of peripheral vascular malformations during a 5-year period in a single specialist center., Methods: All patients who had undergone EST at a single specialist center for peripheral vascular malformations from January 1, 2013 to January 8, 2018 were identified from a prospectively collected database. Data collection included basic demographics, procedure date, anatomic site, type of vascular malformations, and procedural details. Radiation exposure, measured as the dose-area product (DAP) and fluoroscopy time, of all patients who had undergone EST during the study period were retrospectively reviewed. Statistical analysis was performed using the Mann-Whitney U and Kruskal-Wallis tests for comparison between subgroups. P < .05 was considered statistically significant., Results: A total of 237 patients (median age, 30 years; range, 1-73 years) had undergone 419 EST sessions during the study period. Of the 237 patients, 61 (25.7%) had had arteriovenous malformations (AVMs) and had undergone 140 EST sessions (33.4%) and 176 (74.3%) had had venous and lymphatic malformations and had undergone 279 EST sessions (66.6%). Patients with AVMs had undergone a median of 2 procedures (range, 1-13) compared with a median of 1 (range, 1-6) for venous and lymphatic malformations within the study period. The median DAP for the single and cumulative EST for peripheral vascular malformations was 1.26 Gycm 2 (range, 0.00-698.36 Gycm 2 ) and 1.91 Gycm 2 (range, 0.00-1300.24 Gycm 2 ), respectively. The median fluoroscopy time for single and cumulative EST was 19 seconds (range, 1-3846 seconds) and 30 seconds (range, 1-5843 seconds), respectively. Significantly greater patient radiation exposure, in DAP and fluoroscopy time, was measured for single and cumulative EST for AVMs compared with venous and lymphatic malformations (P < .01 for both; Mann-Whitney U test). A significant difference in DAP but not fluoroscopy time was found when the anatomic areas of vascular malformations were compared., Conclusions: Patient radiation exposure for EST for peripheral vascular malformations, measured in DAP and fluoroscopy time, appeared to be generally less than that reported for endovascular arterial and deep venous interventions. However, some patients with peripheral vascular malformations received relatively high radiation doses. Further studies to investigate the risk factors and long-term side effects of radiation exposure in these patients and strategies to reduce these are required., (Crown Copyright © 2020. Published by Elsevier Inc. All rights reserved.)

Objective: Bleomycin is one of the most commonly used agents in sclerotherapy for slow-flow vascular malformations worldwide. However, its efficiency remains unknown. The objective of the present study was to assess whether reversible electroporation combined with bleomycin would increase the sclerotherapy effect in patients with previously unsuccessfully treated venous malformations (VMs)., Methods: We performed, to the best of our knowledge, the first retrospective observational case series from January 2019 to January 2020 of 17 patients (20 lesions) with symptomatic VMs, who had previously undergone at least two unsuccessful invasive treatments. Reversible electroporation was performed with various electrodes and directly injected bleomycin. All patient records, magnetic resonance imaging data, documentation of previous treatments, and data regarding the intervention, complications, and clinical symptoms were analyzed., Results: The 17 VM patients (mean age, 20.8 ± 8.2 years; 9 females) had previously undergone an average of 4.2 invasive treatments. These patients had subsequently undergone 22 electrosclerotherapy sessions of 20 lesions. The median dose of bleomycin was 3 mg. The median magnetic resonance imaging-derived lesion volume before treatment was 24.9 cm 3 , which had decreased by 86% to 3.5 cm 3 after treatment. After 3.7 months, eight patients were asymptomatic without residual symptoms and nine patients showed improvement., Conclusions: Bleomycin electrosclerotherapy appears to be an effective therapy for patients with VMs resistive to previous invasive therapy. Because of the small sample size and short follow-up period, our results should be examined further using a larger patient population., (Copyright © 2020. Published by Elsevier Inc.)

Background: Stereotactic radiosurgery such as Gamma Knife radiosurgery (GKRS) has been shown to have a good treatment effect for orbital cavernous venous malformation (CVM). However, radiation-induced retinopathy or optic neuropathy is a vision-threatening complication of orbital irradiation. Predicting the post-treatment visual outcome is critical., Methods: Clinical and radiological outcomes were investigated in 30 patients who underwent GKRS for orbital CVM between July 2005 and February 2020. Measurement of peripapillary retinal nerve fiber layer (pRNFL) thickness using optical coherence tomography (OCT) was obtained in 14 patients., Results: The median clinical and radiological follow-up periods were 46.6 months (range, 15.9-105.8) and 27.5 months (range, 15.4-105.8), respectively. Twenty-eight patients underwent multisession (4 fractions) GKRS. The median cumulative marginal dose was 20 Gy (range, 16-24). Two patients underwent single-session GKRS. Marginal doses were 15 Gy and 10.5 Gy in each patient. The volume of CVM decreased in 29 (97%) patients. Visual acuity was improved in 6 (20%) patients and was stable in 22 (73%) patients. Visual field defect and exophthalmos were improved in all patients. Serial investigation of OCT showed no statistically significant difference in pRNFL thickness after GKRS. Patients with normal average pRNFL thickness showed better visual recovery than patients with thin average pRNFL thickness., Conclusions: GKRS is an effective and safe treatment option for orbital CVM. The pRNFL thickness before GKRS can be a prognostic indicator for visual recovery in orbital CVM after GKRS.

Portosystemic venous shunt (PSVS) is a vascular anomaly between the portal and systemic veins, resulting in several critical complications. Although PSVS is often associated with congenital heart diseases, the clinical association between Fontan circulation and PSVS has not been elucidated. This study aimed to investigate the clinical features of Fontan patients with PSVS. Two hundred thirteen patients who underwent Fontan procedure are being followed up at Adult Congenital Heart Disease clinic in Kyushu University Hospital. Among them, 139 adult patients underwent cardiac catheterization between January 1, 2011 and September 30, 2019. Medical records were reviewed to investigate the laboratory, echocardiography, and cardiac catheterization findings, as well as clinical manifestations and outcomes. Eleven Fontan patients received the diagnosis of PSVS. The median age at cardiac catheterization was 25 (range 18-45) years. Fontan operation was performed using extracardiac conduit or lateral tunnel 22 (16-35) years previously. Ten patients presented with chronic heart failure [New York Heart Association class 2 (n = 5) and 3 (n = 5)]. The median level of peripheral oxygen saturation was 87 (70-95)%. Cardiac catheterization showed increased cardiac index [5.3 (2.72-14.3) L/min/m 2 ] with or without high central venous pressure [18 (9-25) mmHg]. Although the pulmonary vascular resistance was within the normal range, the systemic vascular resistance was decreased [7.08 (1.74-18.6) Wood units]. Fontan patients complicated with PSVS had increased cardiac output. The presence of PSVS in Fontan circulation might be associated with unfavorable long-term outcome.

Purpose of Review: To provide an update on recent developments in the understanding, diagnosis, and treatment of CSF-venous fistula (CVF)., Recent Finding: CVF is a recently recognized cause of spontaneous intracranial hypotension (SIH), an important secondary headache, in which an aberrant connection is formed between the spinal subarachnoid space and an adjacent spinal epidural vein permitting unregulated loss of CSF into the circulatory system. CVFs often occur without a concurrent epidural fluid collection; therefore, CVF should be considered as a potential etiology for patients with SIH symptomatology but without an identifiable CSF leak. Imaging plays a critical role in the detection and localization of CVFs, with a number of imaging techniques and provocative maneuvers described in the literature to facilitate their localization for targeted and definitive treatment. Increasing awareness and improving the localization of CVFs can allow for improved outcomes in the SIH patient population. Future prospective studies are needed to determine the diagnostic performance of currently available imaging techniques as well as their ability to inform workup and guide treatment decisions.

Mesenteric arteriovenous vasculopathy (MAVD/V) is an extremely rare and poorly understood disease and its incidence is probably underestimated. It is an uncommon, non-inflammatory and non-atherosclerotic form of mesenteric vascular injury, first reported in 2016, with characteristic histopathologic evidence of fibromuscular dysplasia-like vascular changes. We present the case of a chronically ill 84-year-old female with a 5 year history of recurrent small bowel obstruction, who underwent segmental resection of the small bowel. Intraoperative examination showed bowel stricture with fibrosis, intraluminal pill fragments and creeping mesenteric adipose tissue clinically compatible with Crohn's disease. Histological examination showed acute and chronic mucosal injury characterized by crypt distortion, ulcerations with granulation tissue, pseudo-pyloric metaplasia, areas of fibrosis and serosal adhesions. Multiple blood vessels (including both veins and arteries) demonstrated wall hyalinization, elastic degeneration and non-atherosclerotic luminal occlusion. The pattern of the mucosal injury is, in this case, potentially a consequence of acute and chronic ischemic processes secondary to mesenteric arteriovenous vasculopathy., (Copyright © 2020 Sociedad Española de Anatomía Patológica. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: Hangman fracture or traumatic spondylolisthesis of the axis associated with a traumatic vertebral venous fistula (VVF) is a rare entity and sparsely reported in literature. Standard recommendations for management of such rare and complex scenarios are not available and hence the strategy has to be individualized on a case-by-case basis., Methods: We report a 70-year-old man having an unstable hangman fracture with VVF. Both pathologies were simultaneously managed uniquely. The VVF was managed by endovascular occlusion. The fracture was managed by anterior fusion alone as posterior fusion was deemed riskier in the aftermath of a recently occluded VVF. The patient had good neck function and bony fusion at 1-year follow-up., Conclusion: This case report emphasizes the need for timely recognition and management of a VVF, which can rarely coexist with hangman fracture, and discusses the interesting surgical paradigms in the management. We also present a review of literature., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Intramuscular venous malformations, often erroneously called "intramuscular hemangiomas," present to pediatric orthopaedic surgeons either as a differential diagnosis of tumor or as a cause of muscle pain. Treatment options include injection sclerotherapy or surgery. There is some literature to indicate that sclerotherapy can reduce pain, but little evidence on the effectiveness of surgery. The primary aim of this study was to evaluate the efficacy of surgical resection for intramuscular venous malformations, with a secondary aim to evaluate the natural history and presentation of intramuscular venous malformations to improve clinician understanding of this condition., Methods: A retrospective chart analysis was performed of cases identified from a vascular anomalies database from January 2004 and December 2018. Primary outcome was change in preoperative and postoperative pain. Natural history of the lesion was assessed, including age when the lesion was first noticed, when it became painful, and when it required treatment., Results: Fifty-four cases were identified in the study period of which 40 underwent surgery. Pain improved in 36 of 39 patients (92.3%) who had pain before surgery and 29 (74.4%) were pain free after surgery. All 13 patients who required whole muscle excision to resect the lesion experienced an improvement in pain and 10 (76.9%) were pain free. A history of previous intervention, with surgery or sclerotherapy showed a trend towards less successful surgical outcomes. Patients presented across a wide age range from infancy to adulthood, but the most common presentation was pain with exercise between 9 and 16 years of age, with presence of a mass in about half of cases., Conclusions: Surgery, when performed by a surgeon with appropriate experience, is an effective first-line treatment for painful intramuscular venous malformations, offering pain relief in the majority of cases. Magnetic resonance imaging and ultrasound are diagnostic in most cases. The majority of lesions are resectable, meaning they can be removed with a margin leaving a functional limb. Sometimes resection of a whole muscle is required., Level of Evidence: Level IV-case series.

Objective: To evaluate the clinical effectiveness of sclerotherapy agents in low-flow vascular malformations (LFVM) and identify clinical/imaging features to predict response., Methods: A retrospective analysis of hospital records of symptomatic LFVM patients who underwent phlebosclerotherapy from January 2015 to April 2018 was done. Patients were subdivided into venous malformations (VM) and lymphatic malformations (LM). Out of 246 cases, 223 patients (132 males, 91 females; age range, 2-52 years) had VM and 23 (13 males, 10 females; age range, 3 months to 45 years) had LM. The clinical response was graded as excellent (>60%), good (30%-60%), and poor (<30%). More than 30% was considered as acceptable response. The χ 2 test was performed for correlation between clinical response and clinical, sonographic, magnetic resonance imaging, phlebographic parameters followed by multilinear regression., Results: Cavitary (43%) and spongy (37.7%) were the most common phlebographic patterns seen among VM and a cavitary pattern (87%) was most frequent in LM. Sodium tetradecyl sulphate and bleomycin were most commonly used sclerosants in VM and LM, respectively. The mean number of sessions was 4.35 (range, 1-23) in VM and 2.64 (range, 2-7) in LM. Among VM, 114 patients (51.1%) had excellent response to treatment (>60%) and 75.8% patients had an acceptable response (>30%). All patients with LM had an acceptable response (excellent response in 86.9%). Clinical disfigurement, discoloration, diffuse involvement, dysplastic venous morphology on phlebogram, and late and indirect draining vein correlated with poor response to sclerotherapy in VM (P = .003, P = .036, P = .007, P = .008, P = .003, and P = .035, respectively). Cystic components on ultrasound examination and direct draining vein were seen more often in excellent responders (P = .004 and P = .007) in addition to absence of disfigurement, discoloration, and diffuse involvement (P = .032, P = .003, and P = .002). Mod els comprising clinical disfigurement, dysplastic veins, and late draining vein had the greatest predictive value for poor response (R 2  = 0.256). Also, the best model for predicting excellent response comprised presence of direct draining vein and absence of skin discoloration (R 2  = 0.109). Eleven instances of minor complications occurred among a total of 1032 sessions, seven with sodium tetradecyl sulphate and four with polidocanol., Conclusions: Acceptable response to sclerotherapy was achieved in majority of LFVM with extremely low complication rates. Clinicoradiologic features, especially phlebographic findings, correlated with response to sclerotherapy., (Copyright © 2020 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Incidence of vascular anomalies in donor kidneys varies from 18% to 30% and presents a challenge for a transplant surgeon in kidney transplant. Here we present our personal experience for man - agement of the complicated and unexpected cases., Materials and Methods: A total of 250 kidney transplants (226 living, 24 deceased) were performed in a period of 24 years; mean donor age was 55 years (range, 25-86 years), and mean recipient age was 38.6 years (range, 14-66 years). We analyzed the surgical techniques, complications and outcomes, rejection episodes, kidney function, and graft and patient survival rates., Results: Of 250 nephrectomies, 209 had a single artery (83.6%), 34 had 2 arteries (13.6%), and 7 had 3 arteries (2.8%). Of 34 double arteries, 14 had 2 main arteries, 15 had a main and a polar artery, and 5 had an aortic Carrel patch after deceased donation. According to the size, type, and position, the anastomoses were performed with branches of hypogastric, epigastric inferior, iliac external, and main renal artery, intracorporeally or in bench surgery. Regarding veins, 1 double inferior vena cava, 1 left-side inferior vena cava, 4 retroaortic, 2 circumaortic, 10 large lumbar veins draining into the left renal veins, and 8 cases with 2 or more different size renal veins were managed. In 9 cases with short right renal vein, an extension with vena cava (a "Barry cavoplasty") was performed in deceased donor organs. No serious surgical complications related to vascular anomalies were observed. There were no statistical differences in 1-, 6-, and 12-month graft survival rates between the groups with or without vascular anomalies., Conclusions: Vascular anomalies should no longer be considered a contraindication for transplant, if careful anastomosis is performed in every case to avoid ischemia and further complications. Therefore, management of vascular anomalies could be a graftsaving procedure.

Background: Pulsed dye lasers (PDLs) are well-established for treatment of capillary malformations but are unable to penetrate the depth needed to treat deeper vascular lesions. A combined approach using a deeper penetrating wavelength with a "superficial" wavelength could more comprehensively treat vascular malformations than PDL alone., Objective: To evaluate the safety and efficacy of the long-pulsed 1064-nm neodymium:yttrium-aluminum-garnet (LP 1064-nm Nd:YAG) in conjunction with the 532-nm potassium titanyl phosphate (532-nm KTP) laser wavelengths for treatment of capillary venous and venous malformations., Methods: In this retrospective single-center study, we queried patient records who underwent treatment with the 532-nm KTP and LP 1064-nm Nd:YAG laser wavelengths. A blinded panel of 3 physicians evaluated improvement in lesion color, elevation, texture, and overall architecture on a four-point scale: 0% to 25%; 26% to 50%, 51% to 75%, and 76% to 100%., Results: Our cohort consisted of 23 cases. Sixteen cases had sufficient information for clinical assessment. Treatment number and parameters varied depending on lesion, skin type, and end point. Clinical assessment of treatment effectiveness revealed average scores of 51% to 75% improvement for color, elevation, texture, and overall architecture., Conclusion: This study illustrates that 2 wavelengths, 532-nm KTP to target superficial components and LP 1064-nm Nd:YAG for deeper components, can safely and effectively treat both capillary venous and venous malformations.

Background: Venous malformations (VM) are common vascular malformations. Percutaneous injection of sclerosants into the lesion has become mainstream therapy. The most commonly used sclerosants are ethanol, polidocanol, bleomycin, and the like. But few articles have reported that sclerosants are more effective and safer., Methods: We performed a search on Cochrane, Embase, PubMed, China National Knowledge Infrastructure, CBM, and Wan Fang databases of Controlled Trials (from January 1, 2010, launch up to April 10, 2019) reporting outcome of intralesional ethanol, polidocanol, and bleomycin injections in patients with VM (n ≥ 20). A meta-analysis was conducted using Rev-man 5.3 software., Results: A total of 9 articles, 632 participants and 676 lesions were included. Quality of evidence was generally low. Meta-analysis showed that absolute ethanol treatment was better than polidocanol in treating VM (p = .001), and absolute ethanol elicited a better response than bleomycin (p = .01). Ethanol therapeutic effect was not statistically significant compared with ethanol alone (p = .07), but the combination effect was better than polidocanol (p = .04). Ethanol treatment showed significantly more adverse reactions than polidocanol and combination therapy., Conclusion: Absolute ethanol combined with polidocanol is more effective in treating VM and has fewer adverse reactions.

Background: Diffuse venous malformations that involve all tissues in the upper limb and ipsilateral chest wall are known as "phlebectasia of Bockenheimer." The authors describe their experience with management of this uncommon vascular anomaly., Methods: The authors' Vascular Anomalies Center registry comprised 18,766 patients over a 40-year period. This review identified 2036 patients with venous malformations of the extremities (10.8 percent), of whom only 80 (0.43 percent) had Bockenheimer disease. The authors retrospectively analyzed patient characteristics, diagnostics, treatments, and complications., Results: The venous malformation was first noted at birth or within the first few years of life with slow and gradual progression. Pain was related to engorgement of the limb. Thromboses and phleboliths were common, but diffuse intravascular coagulopathy occurred in only 12 patients (15 percent). Skeletal involvement was demonstrated as lytic lesions, cortical scalloping, osteopenia, and pathologic fractures. Management included compression garments (100 percent), sclerotherapy (27.5 percent), and resection of symptomatic areas in 35 percent of patients. Adjunctive pharmacologic medication was given in 7.5 percent. Following resection, 17 patients (60 percent) had one or more complications: hematoma, wound dehiscence, flap loss, contracture, and psychosis. There were no deaths. Symptoms improved in all patients with useful functional outcomes., Conclusions: The decision to pursue compression, sclerotherapy, pharmacologic treatment, or resection alone or in combination was made by an interdisciplinary team. Although extensive venous malformations cannot be completely ablated, debulking of symptomatic regions, resection of neuromas, and noninvasive treatments improve the quality of life. Despite the bulk and weight of the arm, forearm, and hand, and the ominous appearance on magnetic resonance imaging, these patients remain functional., Clinical Question/level of Evidence: Therapeutic, V.