Comparison of bleomycin polidocanol foam vs electrochemotherapy combined with polidocanol foam for treatment of venous malformations.

OBJECTIVE: This study aims to investigate the difference in safety and efficacy between two treatments for venous malformations (VMs), electrochemotherapy combined with polidocanol foam (ECP) and bleomycin polidocanol foam (BPF), providing alternative therapies for VMs. METHODS: We conducted a retrospective review of 152 patients with VMs treated with ECP and BPF. Pre- and post-treatment magnetic resonance images (MRIs) were collected, and clinical follow-up assessments were performed. Imaging results were used to calculate lesion volume changes. Clinical outcomes included changes in pain and improvements in perceived swelling. Patients were followed up at 1 week and 6 months after surgery. All emerging complications were documented in detail. RESULTS: Of the 152 patients, 87 (57.2%) received BPF treatment, and 65 (42.8%) received ECP treatment. The most common location of VMs was the lower extremities (92/152; 60.2%), and the most common symptom was pain (108/152; 71.1%). Forty-three patients had previously undergone therapy in the BPF group (43/87; 49.4%), whereas 30 patients had received prior treatment in the ECP group (30/65; 46.2%). The study found that the percentage of lesion volume reduction in the BPF group was not significantly different from that in the ECP group (75.00% ± 17.85% vs 74.69% ± 8.48%; P = .899). ECP was more effective when the initial lesion volume was greater than 30 mL (67.66% ± 12.34% vs 73.47% ± 8.00%; P = .048). Patients treated with BPF had significantly less posttreatment pain than those treated with ECP, in different baseline lesion size. In the overall sample, pain relief was significantly higher in the BPF group than in the ECP group (4.21 ± 1.19 vs 3.57 ± 0.76; P = .002). However, there was no difference in pain relief between the two groups for the treatment of initially large VMs (4.20 ± 0.94 vs 3.70 ± 0.87; P = .113). The ECP group was significantly more likely to develop hyperpigmentation (5/87; 5.75% vs 11/65; 16.92%; P = .026) and swelling (9/87; 10.34% vs 16/65; 24.62%; P = .019) 1 week after surgery than the BPF group. CONCLUSIONS: Our study demonstrates that both BPF and ECP are effective treatments for VMs, with BPF being a safer option. ECP is a better choice for patients with the initial lesion volume greater than 30 mL, but it is more likely to lead to early swelling and hyperpigmentation.

Novel treatment of recurrent orbital venolymphatic malformation with sirolimus and rivaroxaban.

Orbital venolymphatic malformations are rare vascular malformations that typically appear early in life and harbor acute and chronic threats to vision. Historically, there are four categories of management: observation, medication, sclerotherapy, and surgery. Currently, there is neither a gold standard for treatment nor randomized control trials comparing treatments.The authors present a 20-year-old male who presented with spontaneous hemorrhage of an orbital venolymphatic malformation occurring with increased frequency and involving more of the posterior orbit. Surgery and sclerotherapy were not feasible options due to the extensive intraorbital and intracranial involvement of the venolymphatic malformation. Systemic steroids treated symptoms but was not curative. To this end, a combination of sirolimus, an mTOR inhibitor, and rivaroxaban, a factor Xa inhibitor, were used to reduce the size of the lesion and minimize the risk of thromboembolic events. This treatment has successfully kept the patient's symptoms in remission for greater than 2 years.

Single Center Experience of Sirolimus Therapy in Head and Neck Low-flow Vascular Malformations.

OBJECTIVE: Recently, studies have shown that sirolimus is clinically efficacious in the treatment of some low-flow vascular malformations (LFVM). This study aimed to assess the efficacy and safety of sirolimus in treating complex head and neck (H&N) LFVM that were challenging and/or refractory to standard treatment. METHODS: Each patient had baseline and 6-months assessments consisting of clinical history and examination, quality of life (QoL) questionnaires, laboratory investigations, MRI and medical photography. Patients were followed up 1-week and then 1-monthly for 6-months. Wilcoxon signed-rank test was used to compare pre-and 6-months treatment in all 8 domains of RAND 36-Item Short Form Health Survey (SF-36), hospital anxiety and depression scale (HADS), and visual analog score for pain (VAS-P). P < 0.05 was considered significant. RESULTS: Seven patients (median age 43 years, range 23-65 years) were recruited. Six patients completed the six-months course of therapy with 1 patient withdrawing due to intolerable side effects. All six patients reported reduction of swelling with and without other symptom improvement related to the vascular malformations while on treatment. However, at 1-month review after discontinuation of sirolimus, 5 patients reported return of initial symptoms. Overall, patients demonstrated an improvement in QoL six-months treatment but there was no statistical significance (P > 0.05) in all 8 domains of SF-36, HADS and VAS-P. Five patients demonstrated a minimum 10% decrease in lesion size six-months treatment (median 21%, range 13-40%). A Wilcoxon signed-rank test showed that sirolimus treatment did elicit a statistically significant change in lesion size in either direction (Z = -1.992, P = 0.046). The most common side effects found were dyslipidaemia (n-4) and mouth ulcers (n = 2). CONCLUSION: In our preliminary experience, sirolimus is effective and safe in treating patients with complex H&N LFVM. This provides an alternative treatment where standard treatment is challenging and/or refractory.

Diode Laser Photocoagulation of Intraoral and Perioral Venous Malformations After Tridimensional Staging by High Definition Ultrasonography.

Objective: To determine optimal settings for use of diode lasers to treat vascular malformations in the head and neck. Background: Diode lasers are generally accepted as effective tools to treat vascular malformations in the head and neck, yet there are no standardized treatment protocols for this treatment approach. Settings for these lasers, in addition to laser type and size and lesion depth are important variables for designing appropriate treatments for vascular malformations. Materials and methods: Vascular venous malformations (VeMs) were classified into five groups according to high-definition ultrasound measurements of superficial dimensions (<1, 1-3, and >3 cm) and lesion extension depth (≤5, and >5 mm). Using a 800 ± 10 nm diode laser for surgical procedures, we treated lesions by two different approaches: trans-mucous/cutaneous photocoagulation (8 and 12 W pulsed wave for cutaneous and mucosal lesions, respectively) and intralesional photocoagulation using a laser set to 13 W in the pulsed wave modality to treat both cutaneous and mucosal lesions. Results: We treated 158 vascular VeMs. According to preoperative staging, there were 52 lesions in Group A (superficial dimension <1 cm), 28 in Group B1 (superficial dimension from 1 to 3 cm, extension depth ≤5 mm), 16 in Group B2 (superficial dimension from 1 to 3 cm, extension depth >5 mm), 42 in Group C1 (superficial dimension >3 cm, extension depth ≤5 mm), and 12 in Group C2 (superficial dimension >3 cm, extension depth >5 mm). All Group A lesions showed complete healing after a single laser treatment, whereas 7%, 10%, 12%, and 16% of lesions classified in Groups B1, B2, C1, and C2, respectively, required further treatments. Conclusions: The laser settings defined in this study were associated with a reduced number of laser applications needed for effective treatment of vascular VeMs. Moreover, the cooling of irradiated tissues, together with prolonged application of a topical regenerative gel, provided good treatment acceptability, reduced the number of postoperative complications, and promoted faster healing of covering tissues.

[Effect of foam sclerotherapy for the treatment of oropharyngeal venous malformation].

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.

Artery of Percheron Infarct - a diagnostic and prognostic conundrum!

A 39 year old male was found unconscious at home. On arrival to the hospital the patient was profoundly somnolent but easily rousable, with no focal neurological deficits. Imaging confirmed bilateral thalamic infarcts from the occlusion of Artery of Percheron, a rare anatomic variant which is a single arterial trunk supplying the thalamus and midbrain bilaterally. Anti-platelet therapy was initiated as soon as the diagnosis was established and the patient showed a rapid remarkable recovery over the next 48 hours. He continued to improve subsequently and was at baseline functional status at 6 months. Extensive investigations for etiologies were mostly unrevealing. In such patients presenting with drowsiness/somnolence, a posterior circulation stroke should be considered if no evidence of other more common causes are found. A CT head must be followed by an MRI to confirm the diagnosis and subsequent focus should be on eliciting risk factors and careful evaluation for etiologies.

A case of extensive pharyngeal vascular malformation successfully treated with Kampo medicine.

OBJECTIVE: To present the efficacy of Japanese-traditional medicine (Kampo) for a case with vascular malformation. METHODS: A case study and literature review. PATIENT: A 62-year-old female presented with dysphagia and spitting blood. Esophagogastroduodenoscopy showed a longitudinal lobulated and septated mass in the posterior pharynx. On MR imaging, the mass showed hyperintensity on T2-weighted images and heterogeneous enhancement on Gadlinium-enhanced T1-weighted images, suggestive of a low-flow vascular malformation. INTERVENTION: According to the Kampo diagnosis, kamisyouyousan and ninjinyoueito were prescribed to this patient. The effect of Kampo medicine was evaluated with improvement of her symptoms and volumetry of MRI findings. RESULT: The longitudinal pharyngeal mass was markedly decreased and her symptoms disappeared after 2 years of Kampo administration. CONCLUSIONS: Kampo medicine can be a novel alternative therapy for VM.

Supraventricular tachyarrhythmias in patients with a persistent left superior vena cava.

Aims: A persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. This venous anomaly can impact the evaluation and treatment of supraventricular tachyarrhythmia (SVA). The aim of this study was to assess the proportion and characteristics of PLSVC in adult SVA patients. Methods and results: From July 2002 to July 2012, clinical and procedural data from databases of 10 cardiac electrophysiology laboratories in the Yeungnam region of the Republic of Korea were reviewed. Of 6662 adult SVA patients who underwent an EP study or catheter ablation of SVA during the 10-year study period, 18 patients had PLSVC (mean age 47.6 ± 14.8 years, 10 men). The proportion of PLSVC in adult SVA patients was 0.27% (18/6662). SVA type and procedural outcomes of radiofrequency (RF) catheter ablation in these patients were investigated and the results were as follows: successful slow pathway modification in six of seven patients with atrioventricular nodal reentrant tachycardia (AVNRT), successful ablation of accessory pathway in three of four patients with atrioventricular reentrant tachycardia, and successful ablation of atrial tachycardia (cavotricuspid isthmus-dependent in two, septal macroreentry in one, focal from the PLSVC in one) in three of four patients. In one patient with junctional tachycardia, catheter ablation failed. In two patients with atrial fibrillation, catheter ablation was successful. Conclusion: Among adult SVA patients who underwent an EP study or RF catheter ablation during the 10-year study period, 0.27% had PLSVC. The most common type of SVA was AVNRT. The success rate of catheter ablation was 82% in SVA patients with PLSVC. There were no procedure-related complications.

Modified Soave procedure for the treatment of vascular malformations involving anorectum and sigmoid colon.

PURPOSE: The objective of this study is to introduce a modified Soave procedure for the treatment of vascular malformations involving the anorectum and sigmoid colon (VMARS) in children. METHODS: Cases of 12 children with VMARS between 2000 and 2008 were reviewed and analyzed. The confirmed diagnosis was established by barium enema, colonoscopy, computed tomography, and magnetic resonance imaging. All the patients underwent a modified Soave procedure. In 11 patients, Sarasola-Klose hemorrhoidectomy was used for the distal part of endorectal dissection. RESULTS: The mean length of resected bowel was 22.5 cm, ranging from 17 to 28 cm. Histologically, the surgical specimens showed that the lesions were venous malformation. Postoperative recovery was uneventful. The patients had good continence with no rectal bleeding, but intermittent fecal soiling was noted in one case. CONCLUSIONS: VMARS forms a unique subset of patients with vascular malformations who have rectal bleeding. The combination of Soave procedure and Sarasola-Klose hemorrhoidectomy is a safe and effective procedure for VMARS.

Venous malformation of the eyelid treated with pulsed-dye-1064-nm neodymium yttrium aluminum garnet sequential laser: an effective and safe treatment.

Venous malformations of the skin and subcutaneous tissue are compressible, blue-purple tumors that are present at birth. According to the location and symptoms caused, venous malformations can be treated with surgery, sclerotherapy, or a combination of both. Laser therapy can also be used, especially when surgery is contraindicated. We report the case of a 24-year-old man who presented with a venous malformation on the upper and lower left eyelids, which provoked a mechanical ptosis. Treatment with sequential pulsed-dye neodymium yttrium aluminum garnet (PDL-Nd:YAG) laser was performed. After 2 treatments, a marked reduction of volume and blanching of the venous malformation was observed, with satisfactory cosmetic results. The sequential PDL-Nd:YAG laser seems to be an effective and safe therapy for the treatment of cutaneous venous malformations. It penetrates deeper than pulsed-dye laser alone, and because it allows the use of lower fluencies than Nd:YAG laser alone, it reduces the risk of adverse effects.