Biological identity of orbital cavernous venous malformations

ABSTRACT Vascular anomalies comprise a wide spectrum of clinical manifestations related to disturbances in the blood or lymph vessels. They correspond to mainly tumors (especially hemangiomas), characterized by high mitotic activity and proliferation of the vascular endothelium, and malformations, endowed with normal mitotic activity and no hypercellularity or changes in the rate of cell turnover. However, the classifications of these lesions go beyond this dichotomy and consist various systems adapted for and by different clinical subgroups. Thus, the classifications have not reached a consensus and have historically caused confusion regarding the nomenclatures and definitions. Cavernous venous malformations of the orbit, previously called cavernous hemangiomas, are the most common benign vascular orbital lesions in adults. Herein, we have compiled and discussed the various evidences, including clinical, radiological, morphological, and molecular evidence that indicate the non-neoplastic nature of these lesions.

Value of dynamic contrast-enhanced MRI in selecting sclerosants for endovascular sclerosis of venous malformation / 上海交通大学学报（医学版）

Objective: To evaluate the value of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) in predicting the efficacy of foam sclerotherapy and selecting sclerosants for endovascular sclerosis of venous malformations. Methods: A retrospective analysis was conducted for 56 patients with venous malformations who underwent intravascular sclerotherapy and DCE-MRI examination from January 2018 to June 2019 in Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine. All the patients were firstly treated with foam sclerotherapy. During the surgery, the surgeons determined whether to subsequently use ethanol, depending on the immediate therapeutic effect of foam sclerotherapy. Among them the 40 cases were treated with foam sclerotherapy only (foam sclerotherapy group) and the other 16 cases (ethanol group) with subsequent ethanol. The basic characteristics and DCE-MRI parameters of the two groups were compared. Logistic regression was used to analyze the risk factors of selecting different sclerosing agents, and the receiver operator characteristic curve was applied to assess the efficacy of these risk factors. Results: There were no significant differences in the gender, age, lesion location, pre-treatment volume and presence or absence of phleboliths between foam sclerotherapy group and ethanol group. The lesion classification, maximum intensity time ratio (MITR) and peak enhancement percentage showed significant differences between the two groups. Multivariate Logistic regression analysis showed that the lesion classification and MITR were two independent factors for the selection of sclerosing agents. The area under curve (AUC) of MITR was 0.947, while the AUC of lesion classification was 0.844. After the combination of these two parameters, the AUC was 0.969 with the sensitivity of 93.8% and the specificity of 90.0%. Conclusion: DCE-MRI can be helpful for clinical selection of appropriate sclerosing agents to improve the effectiveness of venous malformations treatment.

Effect of foam sclerotherapy for the treatment of oropharyngeal venous malformation / 中华耳鼻咽喉头颈外科杂志

Objective@#To evaluate the safety and efficacy of foam sclerotherapy with polidocanol for the treatment of venous malformation in the oropharynx.@*Methods@#The clinical data of 21 children with venous malformation in the oropharynx treated by foam sclerotherapy were retrospectively analyzed. There were 10 males and 11 females, ranging in age from 1 month to 13 years, with a median age of 2.3 years. MRI was performed in all children, and the diagnosis was further confirmed by radiography. After general anesthesia, the oropharynx was exposed by opening device. Scalp acupuncture was used to pucture lesions and polidocanol foam was injected after the nidus was confirmed by digital subtraction angiography(DSA). The follow-up time ranged from 2-29 months, with a mean time of 15 months. The clinical symptoms, imaging data, therapeutic effects and postoperative complications were evaluated.@*Results@#Total numbers of treatment were 52 times, 1-6 times/case; 13 cases were cured, 7 cases was relieved and no response in one case. Postoperative swelling in 13 cases, fever in 3 cases, local mucosal ulcer in 2 cases, difficult extubation in 2 cases. No nerve injury, swallowing function damage and cardiopulmonary accidents were found in all patients.@*Conclusion@#Foam sclerotherapy with polidocanol in the treatment of venous malformation in the oropharynx is a safe and effective method.

Detection of Galectin-3 in ocular venous malformation tissue and its clinical significance / 中华整形外科杂志

Objective@#To detect the expression level of Galectin-3 in the ocular Venous Malformation (VM) tissue, and explore its associations with the occurrence and development of ocular VM.@*Methods@#12 fresh ocular VM tissue samples and 12 simplex great saphenous vein varices tissue samples were collected. The mRNA and protein expression levels of Galectin-3 gene were detected by RT-PCR and Western Blot.@*Results@#The relative mRNA and protein levels of Galectin-3 in VM and control group were 5.26×10-3±8.78×10-4, 4.89×10-4±5.37×10-5 and 0.861±0.394, 0.223±0.206, respectively; Galectin-3 expressions in both mRNA and protein levels in ocular VM tissue were obviously higher than those in control group (P<0.05), and an increasing trend was accompanied with aggravating pathological changes in both degree and range of ocular VM.@*Conclusions@#Galectin-3 is highly expressed in ocular VM tissue in both mRNA and protein levels, which may be closely related to ocular VM development and prognosis, and can be used to determine the degree of local invasion of ocular VM. The pathogenesis of ocular VM may be related to Galectin-3, which can be further investigated through gene chip techniques.

Eruption of a venous malformation through an iliac bone harvesting site after trauma

Harvesting grafts from the anterior iliac bone has been associated with various complications. A 50-year-old woman presented to our department with a chief complaint of right inguinal swelling and pain. Autologous bone grafts had been harvested on two previous occasions from the right anterior iliac crest for use in the reconstruction of multiple facial fractures. Computed tomography and magnetic resonance imaging revealed a full-thickness bone defect in the right anterior iliac crest. A mass was noted in the right gluteus minimus, while a multilocular cystic mass extended from the right iliac crest defect to the right inguinal region. Both the inguinal mass and gluteal mass were removed under general anesthesia. Following histopathological analysis, the gluteal mass was diagnosed as a venous malformation(VM). Based on the patient’s clinical course, iliac bone graft harvesting and trauma to the gluteal region triggered hemorrhaging from the VM. Blood components leaked out from the fragile portion of the iliac bone defect, forming a cystic lesion that developed into the inguinal mass. In this case, a coincidental VM resulted in a rare complication of iliac bone graft harvesting. These sequelae could have been avoided by planning for more appropriate ways to collect the grafts.

Telangiectasia hemorrágica hereditaria Síndrome de Osler Weber Rendú y manejo con bevacizumab / Hereditary hemorrhagic telangiectasia Osler Weber Rendu syndrome and management with bevacizumab

Presentamos un caso clínico de una paciente con diagnóstico de telangiectasia hemorrágica hereditaria con múltiples manifestaciones sistémicas debidas a sangrados profusos, anemia severa y malformaciones arteriovenosas pulmonares, hepáticas y falla cardiaca de alto débito, con adecuada respuesta al uso de bevacizumab. (Acta Med Colomb 2015; 40: 66-68).

The case of a patient diagnosed with hereditary hemorrhagic telangiectasia with multiple systemic manifestations due to profuse bleeding, severe anemia and pulmonary arteriovenous malformations, liver and heart failure high debit adequate response to the use of bevacizumab, is presented. (Acta Med Colomb 2015; 40: 66-68).

Association of Blood Group O with Gastrointestinal Bleeding in Continuous-Flow LVAD Patients.

Background: Increasing use of mechanical circulatory devices for advanced heart failure therapy has led to challenges in managing complications that arise from device implantation for long term support either as a bridge to transplant or as destination therapy. Patients with continuous flow left ventricular assist devices (CF-LVADS) show increasing incidence of gastrointestinal (GI) bleeds. Lack of pulsatality and narrow pulse pressure result in initiation and progression of arteriovenous malformations. High shear forces generated lead to destruction of von Willebrand factor (vWF) making it functionally inactive predisposing patients to increasing bleeding. Literature suggests that patients with blood type O have lower baseline levels of von Willebrand factor. It therefore seemed relevant to study the role of blood groups in this population. Methodology: Retrospective data analysis of 119 CF-LVADS patients discharged between 01/2005 and 07/2014. This study was approved by the Institutional Review Board on 08/01/2014. Results: Blood type (p=0.23), gender (p=0.16) and the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile (p=0.86) were not associated with significant incidence of GI bleeding. Blood group O was not associated with a higher incidence of GI bleeding (correlation coefficient =0.07) Patients supported on the HeartMate II (HMII) LVAD experienced more GI bleeding than patients supported on the HeartWare LVAD (HVAD) (p=0.009). HMII LVAD patients who experienced GI bleeding had a mean age of 59.8 years vs. 55.7 years for HMII LVAD patients in the Non-GI bleed group. Age was a significant factor for GI bleeding (p=0.016). Conclusion: Blood group and GI bleeding did not show any significant association in our study. Patients with blood group O did not show increased incidence of GI bleeding despite reported lower baseline levels of von Willebrand factor suggesting contribution of additional factors leading to this complication. The HVAD had a decreased incidence of GI bleeding as compared to Heartmate II. This study is limited by the fact that it was a retrospective analysis in a small population.

Ethanol sclerotherapy of head and neck venous malformations / Escleroterapia com etanol em malformações venosas da cabeça e pescoço

Objective This retrospective study evaluated the results of sclerotherapy with low doses of ethanol for treatment of head and neck venous malformations. Methods We treated 51 patients, 37 females. Median age was 23 years. Patients were treated with percutaneous intralesional injection of alcohol every two weeks and followed up prospectively for a median period of 18 months. Most lesions affected the face and cosmetic disfigurement was the most frequent complaint. Results We performed a median of 7 sessions of sclerotherapy. Complete resolution or improvement was observed in 48 patients presented. Five cases of small skin ulceration, two cases of hyperpigmentation and two of paresthesia were documented; all of them were treated conservatively. Conclusion Percutaneous sclerotherapy with low doses of ethanol is a safe and effective treatment modality for venous malformations affecting the head and neck. .

Objetivo Estudo retrospectivo que analisou os resultados da escleroterapia, com doses baixas de etanol, realizada sob anestesia local para tratamento de malformações venosas na cabeça e pescoço. Métodos Foram tratados 51 pacientes, sendo 37 do gênero feminino. A mediana de idade foi de 23 anos. Os pacientes foram tratados com injeções alcoólicas nas lesões, por via percutânea, em sessões quinzenais, e acompanhados prospectivamente por um período mediano de 18 meses. A maioria das lesões acometia a face e a queixa principal mais frequente foi deformidade estética. Resultados Foram realizadas, em média, sete sessões de escleroterapia. Resolução completa ou melhora foi observada em 48 pacientes. Cinco pacientes apresentaram pequena úlcera cutânea, dois hiperpigmentação e dois parestesia, sendo todos tratados conservadoramente. Conclusão A escleroterapia percutânea com doses baixas de etanol é um método seguro e eficaz para tratar malformações venosas na cabeça e pescoço. .

Malformaciones arteriovenosas. Revisión y análisis estadístico de 52 MAVs tratadas durante el período de 2000-2010. Tercera parte / Arteriovenous malformations. Review and statistical analysis of 52 MAVs treated during the period 2000-2010. Third part

INTRODUCCIÓN: describir nuestra experiencia en el tratamiento de las MAVs analizando: procedimientos endovasculares utilizados, resultados postoperatorios y complicaciones asociadas, estadificación según escalas de Barthel y Rankin modificadas. MATERIAL Y MÉTODOS: la población de estudio se constituyó por 52 pacientes con MAVs la cual fue analizada y estudiada mediante examen neurológico pre y postoperatorio, TC cerebral, RM cerebral y arteriografía de 4 vasos de cuello, la totalidad fue tratada mediante cirugía convencional en nuestro Servicio durante el período comprendido entre los años 2000 a 2010. RESULTADOS: recibieron tratamiento endovascular previo a la cirugía 16 MAVs (30,76 %). Todas fueron operadas. Doce pacientes mejoraron en el postoperatorio (23,07 %), 30 pacientes (57,69%) no sufrieron modificaciones y 10 de ellos (19,23%) empeoraron durante el postoperatorio. La mortalidad fue de 7 casos (13,46 %). CONCLUSIÓN: consideramos a los procedimientos endovasculares y la radiocirugía una herramienta de indudable valor terapéutico. Creemos que el subgrupo de MAVs grados III, IV y V representa una entidad singular que las distingue del resto, como una subtipo que requiere más aun de una compleja toma de decisiones. Tuvimos las mayores complicaciones postoperatorias en MAVs grados III y IV. Nuestra mortalidad postoperatoria coincide con la bibliografía consultada

INTRODUCTION: to describe our experience in treating AVMs based on the endovascular procedures used, postoperative results and associated complications, staging according to Barthel Index and modified Rankin Scale. PATIENTS AND METHOD: we present 52 patients with AVMs which were analysed and studied by Pre and Post-Surgery Neurological exam, brain CT, Brain IRM and four Neck vessels arteriography. All the patients were treated by conventional Surgery at our Department of Neurosurgery for the 2000­2010 period. RESULTS: Sixteen patients with AVMs (30.76%) underwent endovascular treatment prior to surgery. 100% were operated. 12 patients (23.07%) improved their clinical condition in the postoperative period, 30 patients (57.69%) showed no changes, and 10 of them (19.23%) experienced deterioration during the postoperative period. There were 7 mortal cases (13.46%). CONCLUSION: we believe that endovascular procedures and radiosurgery are tools of immense therapeutic value. We also consider that the subgroup of AVMs grade III, IV and V have unique features that distinguish them among the rest as a subtype, thus requiring extreme care when making decisions. Most postoperative complications occurred with AVMs grade III and IV. The mortal cases in the postoperative period coincided with those mentioned in the bibliography consulted

The Use of Long Pulsed Neodymium-Doped Yttrium Aluminum Garnet for the Treatment of Paediatric Venous Malformations

BACKGROUND: Venous malformation in the pediatric population can present with pain, bleeding, or a debilitating deformity that can be difficult to manage. Sclerotherapy, surgery, and more recently, long pulsed neodymium-doped yttrium aluminum garnet (Nd : YAG) laser have been used with variable success rates. OBJECTIVE: Our aims and objectives, were to identify the specific group of patients that would benefit from this treatment. METHODS: A prospective clinical trial was carried out with 59 consecutive patients. The treatment criteria included a large facial deformity and painful or bleeding lesions. One to three treatments were administered at 6~8-week intervals. RESULTS: A total of 59 patients were treated. The average follow-up was 24 months. All patients achieved good to excellent results in pain and bleeding control as well as in the reduction of the size of lesions in the lip and oral mucosa. The treatment however, is not effective in reducing the size of large, relatively high-flow lesions in the limbs. Complications from treatment include skin blistering (n=4), ulceration (n=4), and subsequent hypertrophic scarring (n=3). Three patients had partial recurrence after a 3-year follow-up. CONCLUSION: Complex venous malformations cannot be cured but can be symptomatically controlled with the long-pulsed Nd : YAG laser treatment. The treatment satisfaction is high, and there is a small but definite risk of scarring from treatment.

Effect of foam sclerotherapy of lauromacrogol on peripheral venous malformations / 中华医学美学美容杂志

Objective To investigate the efficacy and safety of lauromacrogol foam sclerosant in the treatment of peripheral venous malformations.Methods 21 patients with peripheral venous malformations were treated with foam sclerotherapy.The sclerosing foam was produced by Tessari's method using 1％ lauromacrogol.When necessary,the injections were repeated at an interval of four weeks,and this process contained 3 to 5 injections.Therapeutic outcomes and safety were established by evaluating size of lesion,symptom,duration of treatment and side-effects of treatment before and after treatment.Results 21 patients were followed up for 3 to 18 months.10 cases showed markedly shrinked or even disappeared of treated malformations,9 cases showed a reduction in size over 50 ％,and 2 cases showed a reduction in size less 50 ％.Local swelling occurred in almost per session,pyrexia in 5 patients,which resolved spontaneously within several days to 1 week.No major complication occurred.Conclusions Foam sclerotherapy using lauromacrogol seems to be a safe and effective therapeutic method for peripheral venous malformations.

Blue rubber bleb nevus syndrome: Prominent oral findings.

Blue rubber bleb nevus syndrome is a rare disorder characterized by multiple cutaneous venous malformations in association with visceral lesions, most commonly affecting the gastrointestinal tract. Oral cavity lesions occur in 59 to 64% of cases. We report a unique presentation of this syndrome in a 25-year-old male patient with prominent oral findings. This is a sporadic case, started during early childhood, progressively increasing in number and size. Oral vascular lesions were part of gastrointestinal involvement. Associated cardiac abnormalities were also observed. An early diagnosis of this syndrome is required as it gets complicated with bleeding, anemia and other systemic complications.

Outpatient percutaneous treatment of deep venous malformations using pure ethanol at low doses under local anesthesia

INTRODUCTION: Venous malformations are the most frequent vascular malformation. Deep venous malformations are located in subcutaneous tissue or in the muscles. Percutaneous sclerotherapy is the treatment of choice, and the use of ethanol at low doses has not yet been described. OBJECTIVE: To analyze the results of treating Deep venous malformations patients with low doses of ethanol. METHODS: Thirty-nine patients treated between July 1995 and June 2007 were followed up prospectively over a median period of 18 months. Twenty-nine were female (74.4 percent) and 10 were male (25.6 percent), with ages ranging from 11 to 59 years (median of 24 years). All of the lesions affected limbs, and the main symptom reported was pain (97.4 percent). Each patient underwent fortnightly alcohol application sessions under local anesthesia on an outpatient basis. The lesions were classified into three groups according to size using nuclear magnetic resonance imaging: small, up to 3 cm (4 patients); medium, between 3 and 15 cm (27 patients); and large, greater than 15 cm (8 patients). RESULTS: The symptoms completely disappeared in 14 patients (35.9 percent) and improved in 24 (61.5 percent). The lesion size reduced to zero in 6 patients (15.4 percent) and decreased in 32 (82 percent). The median number of sessions was 7. There were no complications in 32 patients (82 percent), while 3 presented local paresthesia (7.7 percent), 2 superficial trombophlebites (5.1 percent), 1 skin ulcer (2.6 percent), and 1 case of hyperpigmentation (2.6 percent). CONCLUSION: Outpatient treatment for Deep venous malformations patients using ethanol at low doses was effective, with a low complication rate.

Ethanol Sclerotherapy for the Management of Craniofacial Venous Malformations: the Interim Results

OBJECTIVE: We wanted to evaluate the safety and feasibility of ethanol sclerotherapy for treating craniofacial venous malformations (CVMs). MATERIALS AND METHODS: From May 1998 to April 2007, 87 patients (40 men and 47 women; age range, 2-68 years) with CVMs underwent staged ethanol sclerotherapy (range, 1-21 sessions; median number of sessions, 2) by the direct puncture technique. Clinical follow up (range, 0-120 months; mean follow up, 35 months; median follow up, 28 months) was performed for all the patients. Therapeutic outcomes were established by evaluating the clinical outcome of the signs and symptoms in all patients, as well as the degree of devascularization, which was determined on the follow-up imaging, in 71 patients. RESULTS: A total of 305 procedures with the use of ethanol were performed in 87 patients. Follow-up imaging studies were performed for 71 of 87 patients. Twenty-three (32%) of the 71 patients showed excellent outcomes, 37 patients (52%) showed good outcomes and 11 patients (16%) showed poor outcomes. Ethanol sclerotherapy was considered effective for 60 patients. All the minor complications such as bulla (n = 5) healed with only wound dressing and observation. Any major complication such as skin necrosis did not develop. CONCLUSION: Percutaneous ethanol sclerotherapy is an effective, safe treatment for CVMs.

Therapeutic Effect of Sclerotherapy on Venous Malformations / 대한피부과학회지

BACKGROUND: Although surgical excision is the standard method for the treatment of venous malformations, this procedure often leads to massive bleeding and cosmetic problems. Sclerotherapy for venous malformations has recently been reported, whereas sclerotherapy for varicose veins, leg telangiectasias and hemorrhoids has well been established. PBJECTIVE: To assess the usefulness of sclerotherapy for venous malformations. METHOD: Fourteen patients who had venous malformation were treated with sclerotherapy using sodium tetradecyl sulfate (Thromboject(R), Omega Laboratories, Ltd., Montreal, Canada). Clinical efficacy was evaluated by physical examination and comparison of photographs. RESULTS: In patients with venous malformations, 71.4% of the eases showed moderate to marked improvement. Side effects were noted in 6 patients, however, they were trivial and transient and no treatment was needed. CONCLUSION: Sclerotherapy can be recommended as an effective method for the treatment of venous malformations. It has milder and fewer side effects than other treatment modalities, and it also yields superior cosmetic results.

Treatment of body surface venous malformations by local injection of Pingyangmycin lipiodol emulsion under fluoroscopic guidance / 医学研究生学报

Objective: To evaluate the method,efficacy and complication of fluoroscopic guided local injection of Pingyangmycin lipiodol emulsion(PLE) in the treatment of body surface venous malformations(VM).Methods:Forty cases of VM,including 22 cases in the maxillofacial region,5 cases in the trunk and 13 cases in the extremities,received PLE local injection under X-ray fluoroscopy.PLE was prepared by mixing 2-8 mg of pingyangmycin(PYM)with 2-8ml of ultra-fluid lipiodol,the dosage was determined by the size of lesions.The effects and complications of PLE treatment were observed. Results:Signs of VM were patches of abnormal venous opacification and washout delay of contrast medium.Thirty four cases received single injection of PLE and 6 cases were injected twice.Excellent and good results were obtained in 20 cases and 16 cases respectively after 6 months post-injection,the therapy was ineffective in the rest four cases.Total efficacy was 90 percent.There were no severe complications such as skin necrosis or ectopic embolization. Conclusion: PLE intra-vascular injection under fluoroscopic guidance was convenient and safe for body surface VM.The merits of PLE treatment include accurate(localization,) safety,and excellent curative effect.