Treatment Of Microcystic Lymphatic Malformations Research Articles

LB1698 Phase 2 study of the safety and efficacy of QTORIN rapamycin in the treatment of microcystic lymphatic malformations

LB1698 Phase 2 study of the safety and efficacy of QTORIN rapamycin in the treatment of microcystic lymphatic malformations

Endovascular Treatment of Klippel-Trenaunay Syndrome

Endovascular Treatment of Klippel-Trenaunay Syndrome

Retrospective analysis of classification and treatment of microcystic lymphatic malformations of tongue

Objective: To explore the clinical classification of microcystic lymphatic malformations of tongue and observe the treatment of microcystic lymphatic malformations of tongue by retrospective analysis, in order to provide reference for clinical practice. Methods: From October 2005 to October 2015, the complete data of 220 cases of microcystic lymphatic malformations of tongue (115 males and 105 females) received and treated in Provincial Special Department of Vascular Anomalies, Linyi Tumor Hospital was analyzed retrospectively. The age ranged from 8 months to 52 years old, with a median age of 16 years old. All patients were followed up for 3 years, and according to their clinical manifestations, they were divided into three types: localized type of 23 cases, diffuse type of 161 cases, and megaloglossia type of 36 cases. Injection with pingyangmycin merely was performed on 58 cases, whereas merely surgery on 20 cases, injection with pingyangmycin combined with high frequency electrocoagulation on 55 cases, and surgery combined with injection with pingyangmycin on 87 cases. The therapeutic effect was evaluated according to the grade 4 standard. The χ(2) test was used for statistical analysis of count data. Rank sum test was used for statistical analysis of ranked data. Results: The percentage of surgery merely of localized type was 87.0% (20/23), significantly higher than that of other types of lesions [0% (0/197)] (χ(2)=178.060, P<0.001). The percentage of injection with pingyangmycin of diffuse type was 100% (161/161), significantly higher than that of other types of lesions [66.1% (39/59)] (χ(2)=60.034, P<0.001). The percentage of surgery combined with injection with pingyangmycin of megaloglossia type was 100% (36/36), significantly higher than that of other types of lesions [27.7% (51/184)] (χ(2)=65.800, P<0.001). After follow-ups for 3 to13 years, there were 0 cases of gradeⅠ, 11 cases of grade Ⅱ, 50 cases of grade Ⅲ, and 159 cases of grade Ⅳ. There were statistically significant differences in clinical efficacy among different clinical types (H=158.668, P<0.001). The percentage of level Ⅳ efficacy of localized type, diffuse type and megaloglossia type were 100% (23/23), 82.6% (133/161) and 8.3% (3/36) respectively. Local mucosa ulcer appeared in 45 cases and was cured through oral care and expectant treatment. Fever occurred in 28 cases and returned to normal within 24 h after expectant treatment. The lingual frenum was shortened in 16 cases. There were 12 cases with mild tongue swelling, 6 cases with influence on eating but without influence on breathing, and recovered spontaneously one week later. Conclusions: The choice of different treatment methods of microcystic lymphatic malformations of tongue should be made according their clinical classification. The only surgical resection is recommended for localized protrude lesions. Injection with pingyangmycin in high tension combined with surgical resection or high frequency electrocoagulation is effective for diffuse lesions. Surgical resection combined with injection with pingyangmycin is suitable for the patients with megaloglossia type.

Lymphographic-Like Technique for the Treatment of Microcystic Lymphatic Malformation Components of

The treatment of microcystic lymphatic malformations remains challenging. Our aim was to describe the lymphographic-like technique, a new technique of slow bleomycin infusion for the treatment of microcyst components of <3 mm, performed at our institution. A retrospective analysis of a prospectively collected lymphatic malformation data base was performed. Patients with at least 1 microcystic lymphatic malformation component demonstrated on MR imaging treated by lymphographic-like technique bleomycin infusion were included in the study. Patient interviews and MR imaging were performed to assess subjective and objective (microcystic lymphatic malformation size decrease of >30%) clinical improvement, respectively. Patients were reviewed 3 months after each sclerotherapy session. Lymphographic-like technique safety and efficacy were assessed. Between January 2012 and July 2016, sixteen patients (5 males, 11 females; mean age, 15 years; range, 1-47 years) underwent the bleomycin lymphographic-like technique for microcystic lymphatic malformations. Sixty sclerotherapy sessions were performed, with a mean of 4 sessions per patient (range, 1-8 sessions) and a mean follow-up of 26 months (range, 5-58 months). We observed no major and 3 minor complications: 1 eyelid infection, 1 case of severe postprocedural nausea and vomiting, and 1 case of skin discoloration. One patient was lost to follow-up. Overall MR imaging objective improvement was observed in 5/16 (31%) patients; overall improvement of clinical symptoms was obtained in 93% of treated patients. The bleomycin lymphographic-like technique for microcystic lymphatic malformations is safe and feasible with objective improvement in about one-third of patients. MR signal intensity changes after the lymphographic-like technique are associated with subjective improvement of the patient's symptoms.

Microcystic Lymphatic Malformation Successfully Treated With Topical Rapamycin.

Microcystic lymphatic malformations (MLM) are low-flow vascular malformations composed of multiple small cysts. MLM usually affect deep-lying structures, which makes their treatment even more difficult and complex. A novel and interesting treatment is rapamycin, a mammalian target of rapamycin inhibitor that when orally administrated has offered favorable results. However, until recently, topical rapamycin had not been used in the treatment of MLM. Case 1 is a girl aged 13 years with extensive MLM affecting the muscles in the right buttock. The patient had received frequent cycles of cryotherapy, but they had failed to control the associated symptoms. In the previous 12 months, the patient had reported greater discomfort, swelling, exudate, and superinfection of the affected region. Because no specific treatment has yet been approved for MLM, and as a step before the use of aggressive systemic or intralesional treatments, it was decided to initiate treatment with 1% rapamycin ointment. After 4 months of treatment, the patient presented a marked improvement, with a significant reduction of associated complications and no major side effects. Case 2 is a boy aged 5 years who underwent surgery for an intergluteal lipoblastoma at 3 weeks of life and developed a MLM on the scar 6 months afterward. The lesion showed slow growth and continuous exudation with frequent episodes of superinfection. Treatments with laser multiplex and intralesional bleomycin were performed unsuccessfully. In the previous 4 months, the patient had been treated with 1% rapamycin ointment with significant improvement and no side effects.

Treatment of deep-seated facial microcystic lymphatic malformations with intralesional injection of pingyangmycin.

Treatment of microcystic lymphatic malformations (LMs) is still a great challenge to physicians in the field of managing vascular anomalies. Several kinds of treatment have been proposed for microcystic LMs, but the responses to these treatment modalities vary considerably among individuals. The aim of the study was to investigate the safety and efficacy of intralesional injection of pingyangmycin for microcystic LMs located in the deep facial region.Twenty-one consecutive patients with deep-seated facial microcystic LMs were treated with intralesional injection of pingyangmycin between March 2010 and April 2015. The patients received 2 to 8 injections, and the average session was 3.7. The therapeutic efficacy was accessed on the basis of the imaging findings and clinical measurements.Among the 21 patients, the clinical responses were excellent in 7 patients (33.33%), good in 9 patients (42.86%), fair in 3 patients (14.29%), and poor in 2 patients (9.52%). No severe side effects were encountered. Furthermore, therapeutic outcomes were significantly associated with lesion location (P = 0.006) and number of injections (P = 0.003).Our study supports that sclerotherapy with pingyangmycin is safe and effective for the treatment of deep-seated facial microcystic LMs.

Fractional carbon dioxide laser ablation for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum) in an adult patient with Klippel-Trenaunay syndrome.

Lymphangioma circumscriptum (LC) is a rare, benign, vascular malformation of dilated superficial lymphatic channels. Treatment is typically undertaken for cosmetic reasons or complications such as fluid drainage, pain, and infection risk that can negatively impact quality of life. Management of LC remains challenging because of high recurrence rates, regardless of the treatment modality chosen. Treatment of LC with the fully-ablative carbon dioxide (CO2 ) laser has been reported widely, however, treatment with the fractionated CO2 laser has been limited to one case in the pediatric population. Our objective was to test the usefulness of the fractionated 10,600-nm CO2 laser in the management of symptomatic LC in an adult. We report a case of a 27-year-old female with Klippel-Trenaunay Syndrome (KTS) who presented with a 5-year history of well-circumscribed lesions on the right lateral and anterior thigh that drained clear lymphatic and serosanguineous fluid, respectively. These lesions caused her significant emotional distress because of extensive drainage. After only one treatment utilizing the fractionated 10,600-nm CO2 laser, the patient had improvement with cessation of fluid drainage, and the lesions are still asymptomatic 6 months since initial treatment. The favorable results observed in our patient suggest that fractionated CO2 laser may be an additional well-tolerated, low downtime option for palliation of symptoms in patients with LC. We recognize that short-term follow-up cannot conclusively demonstrate effectiveness and durability of this treatment and that, given the high risk of recurrence, a longer-term observation is needed before making definite conclusions. Lasers Surg. Med. 47:539-541, 2015. © 2015 Wiley Periodicals, Inc.

Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study

BackgroundMicrocystic lymphatic malformations (LM) are congenital birth defects that can cause severe functional or esthetic deformity. At this time, several treatment interventions are possible, but there is no ideal therapy. A recently published article noted a coincidental improvement in microcystic LMs with the use of sildenafil for pulmonary hypertension, but conclusive and reproducible data is lacking regarding its efficacy. Methods and resultsA prospective study was conducted to examine the subjective and objective results associated with sildenafil use in the treatment of microcystic LMs. Patients under the age of 18 were enrolled, and after evaluation with pre-intervention magnetic resonance imaging (MRI) studies, each was given a 6-week course of sildenafil. Subjective outcomes were obtained, and postoperative MRIs were used to objectively quantify changes.Five patients between the ages of 4 and 11 were enrolled in the study. All patients had microcystic LMs of the head and neck. All patients had undergone previous treatment interventions utilizing various modalities, and each was now seeking treatment for functional and cosmetic purposes. All patients had minimal to no response from a subjective standpoint. Comparison of pre- and post-treatment MRIs also demonstrated minimal to no response. ConclusionsThe use of sildenafil for the treatment of microcystic lymphatic malformations did not have any appreciable effect on our selected population of pediatric patients who had been resistant to previous therapies. Further studies would be necessary to determine if other cohorts may benefit.

Closure of large defects after microcystic lymphatic malformations using lateral intercostal artery perforator flap

The surgical treatment of microcystic lymphatic malformations (LMs) has historically been difficult and frustrating because of a high recurrence rate due to incomplete resection. However, complete removal of the multifocal and extensive lesions rely on accurate imaging diagnosis and effective repair methods for the resulting large defect. The purpose of this study was to repair large skin defects due to complete resection of microcystic LMs using lateral intercostal artery perforator (LICAP) flap. Between January 2009 and June 2012, tissue defects in the axillary chest wall region of eight patients aged 13-22 years after microcystic LMs resections were closed using the LICAP flap. Flap donor sites in all patients were closed primarily except in one patient who underwent skin grafting. Before surgery, ultrasound and magnetic resonance imaging (MRI) examination were used to confirm the diagnosis and determine the scope and level of the abnormality for complete resection. All defects after microcystic LM excision were successfully closed using LICAP flaps. The follow-up period ranged from 1 to 3 years (mean 2.1 years). All flaps survived postoperatively. No recurrence occurred. Ultrasound and MRI follow-up also demonstrated flap survival without recurrence of microcystic LMs. No functional loss attributable to the LICAP flap harvest was identified in any case. Surgical resection is necessary for microcystic LMs. Imaging assists in the diagnosis and identification of the scope and level of lesions. The LICAP flap provides good coverage for the large defects and achieves acceptable morphology without functional deficits at flap donor sites. Ultrasound and MRI are safe and accurate diagnostic imaging methods for the pre- and postoperative evaluation of microcystic LMs in patients undergoing surgery.

Intralesional injection of pingyangmycin is a safe and effective treatment for microcystic lymphatic malformations in the tongue

To evaluate the efficacy and safety of intralesional injection of pingyangmycin for the treatment of microcystic lymphatic malformations (LMs) in the tongue. Eighteen patients with tongue microcystic LMs were treated with intralesional injection of pingyangmycin. The concentration of the drug was 1 mg/mL with an addition of dexamethasone. Repeated injections were performed at an interval of 3–4 weeks. The results were evaluated by clinical examinations and Doppler ultrasonography scan. The follow-up period was 12 months to eight years after the last treatment and the mean follow-up time was three years. All patients received 1–8 injections (mean, 3.0 injections) for the whole course of treatment. The total dose of pingyangmycin administered was 8–64 mg (mean, 24 mg). Fifteen patients had complete response with no cosmetic or functional problems.Three patients with macroglossia had a reduction of 50–90% in the lesion size and needed secondary surgery. No serious complications were encountered. The results suggested that intralesional injection of pingyangmycin is an effective and safe treatment for microcystic LMs in the tongue, and can be used as the first-line treatment protocol.

Current treatment paradigms in the management of lymphatic malformations

Lymphatic malformations (LMs) are benign abnormalities of the lymphatic system that can be significantly infiltrative and intimately involve critical structures of the head and neck, making their management difficult. Historically, LMs have been managed by surgical excision, but this treatment approach results in significant morbidity and a high recurrence rate secondary to subtotal resection. As an alternative to surgery a variety of drugs have been used as intralesional sclerosants and immunotherapeutics. These agents offer improved outcomes with lower morbidity as compared to surgery when targeted to macrocystic LMs. However intralesional therapy is not effective in the treatment of microcystic LMs. The development of treatments that are effective for all types of LMs will require further understanding of lymphangiogenesis and the pathogenesis of LMs.

Low power radiofrequency ablation for symptomatic microcystic lymphatic malformation of the tongue

Although many head and neck surgeons agree that complete surgical excision is the treatment of choice for lymphatic malformation, the infiltrating nature of microcystic lymphatic malformations and the involvement of vital structures of the head and neck make total removal nearly impossible in most cases. Recently radiofrequency ablation was introduced for the treatment of microcystic lymphatic malformations of the oral cavity; it destroys lesion tissues at low temperature (40-70 degrees C) with minimal damage to adjacent structures. However, high energy (800-1200 J) and general anesthesia for radiofrequency ablation can hinder the easy and repetitive application of radiofrequency to patients. To overcome this limitation of radiofrequency ablation for microcystic lymphatic malformations, we used the same technique with a lower power (6 W, less than 100 J per site) radiofrequency ablation in an office-based setting under local anesthesia in a child with a microcystic lymphatic malformation of the whole tongue, that was associated with recurrent bleeding and swelling. The repetitive low power energy radiofrequency ablation of the microcystic lymphatic malformation of the tongue was safe and effective; it provided the patient with symptomatic relief without recurrence at follow-up. Therefore, our modification of radiofrequency ablation of the microcystic lymphatic malformations could be technically feasible and easily applicable; our result suggests that it can be a useful alternative treatment option to relieve symptoms from microcystic lymphatic malformation of the oral cavity.