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.

Effectiveness and Safety of Treatment with Direct Oral Anticoagulant Rivaroxaban in Patients with Slow-Flow Vascular Malformations: A Case Series.

BACKGROUND: Slow-flow vascular malformations (VM) can be associated with localized intravascular coagulopathy (LIC) that is characterized by elevated D-Dimer levels and low fibrinogen and platelets. This can lead to bleeding and clotting tendencies, which can give rise to functional limitations such as pain and swelling and even progress to disseminated intravascular coagulopathy. METHODS AND RESULTS: We conducted a chart review of four patients with evidence of LIC who were started on rivaroxaban. We found an improvement of D-Dimer and/or fibrinogen levels in all four patients. They also had an improvement of pain and functionality. CONCLUSIONS: We report on four patients in whom anticoagulation with a direct oral anticoagulant, rivaroxaban, was effective in controlling signs and symptoms of consumptive coagulopathy with no evidence of bleeding from the use of rivaroxaban.

Comparison of Emla cream and topical lidocaine tape for pain relief of V-beam laser treatment.

The authors compared the analgesic effects of two topical applications; Emla® cream (Sato Pharmaceutical Co. Ltd., Tokyo, Japan) and 60% lidocaine tape (Penles® tape; Maruho, Osaka, Japan). The authors examined 20 outpatients with capillary malformations. Emla® cream or Penles® tape was applied 1 h before laser irradiation using V-Beam. After V-Beam irradiation, pain intensity was measured using Pain Vision® (NIPRO, Osaka, Japan), while subjective pain was converted into numerical values using the VAS system. In the assessment using Pain Vision®, pain was significantly lower in patients who received Emla® cream (Welch test), whereas no difference was observed in VAS ratings between the two applications (student's t test). The objective system showed that Emla® cream had a significantly greater analgesic effect. We plan to evaluate the analgesic effects of these topical anesthetics by applying them to other anatomical areas.

Indications of Potassium Titanyl Phosphate Laser Therapy for Slow-Flow Vascular Malformations in Oral Region.

BACKGROUND: Indications for laser therapy for slow-flow vascular malformations in the oral and maxillofacial regions have not been clearly documented. The authors aimed to estimate the frequency of resolution of slow-flow vascular malformations and to identify risk and prognostic factors associated with resolution in potassium titanyl phosphate (KTP) laser treatment. METHODS: This study was designed as a prospective cohort study. Patients who had diagnosed slow-flow vascular malformations were continuously assigned to receive KTP laser therapy. All patients had intralesional laser photocoagulation performed under local anesthesia. Administered power of the KTP laser was fixed at 2 watts throughout the procedure in all patients. The primary endpoint was to understand the frequency of resolution of slow-flow vascular malformations in KTP laser treatment. Secondary endpoints were: treatment outcomes based on lesion size; treatment outcomes based on location; treatment outcomes based on total energy in joules; types of complications. Treatment outcomes were judged by a clinical assessment as well as reduction in lesion size on magnetic resonance imaging. RESULTS: Data were obtained from 26 patients (9 men, 17 women) with 38 lesions. The average lesion size was 13.5 ±â€Š7.7 mm. Treatment outcomes based on lesion size showed that cure and regression were obtained in lesions less than 30 mm in size. However, lesions larger than 30 mm showed no response. Lesions in the tongue and lips showed higher cure rates than in other areas. Treatment outcomes based on administered total energy in joules showed that 68% of lesions were treated and responded well at less than 400 joules. Complication rate was relatively high in the buccal mucosal lesions. Immediate postoperative complications such as necrosis were more common in high-energy administration than in low-energy administration. CONCLUSION: Our results indicated that KTP laser therapy was effective for slow-flow vascular malformations less than 30 mm in size without significant side effects.

Mitral isthmus block in a case of persistent left superior vena cava.

Persistent left superior vena cava (LSVC), an uncommon venous anomaly, could be an arrhythmogenic source of atrial fibrillation. Multiple electrical connections were reported between the LSVC and the left atrium, which may negatively impact the achievement of conduction block during linear ablation of left mitral isthmus. We describe a case with perimitral atrial flutter (AFL) in a patient with isolated LSVC. AFL was successfully treated and complete perimitral conduction block was achieved following a lengthy epicardial radiofrequency application.

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.