ABSTRACT
Abstract Objective At present, bleomycin has been widely used in the treatment of Lymphatic Malformations (LMs). This study aims to perform a meta-analysis to investigate the effectiveness and influencing factors of bleomycin in the treatment of LMs. Methods We conducted a systematic review and meta-analysis to clarify the relationship between bleomycin and LMs. PubMed, ISI Web of Science and MEDLINE were searched. Results A total of 21 studies (including 428 cases) about bleomycin sclerotherapy for LMs were included in the current meta-analyses. We calculated pooled effective rate and 95% Confidence Interval (95% CI) using random effects model to evaluate the relations between bleomycin and LMs. The results suggested that the effective rate of bleomycin was the combined effective rate was 84.0% (95% CI 0.81‒0.87) and ranged from 39% (95% CI 0.22‒0.56) to 94% (95% CI 0.87-1.02). The heterogeneity among the studies was substantial (I2 = 61.7%, p = 0.000). In subgroup analyses, it was observed that among retrospective study and prospective study, the estimated effective rate was 80.0% (95% CI 0.76‒0.84) and 91.0% (95% CI 0.85‒0.97), respectively. In terms of the dosage, the combined effective rates of weight-based group and fixed-dose group were 86% (95% CI 0.83‒0.90) and 74.0% (95% CI 0.66‒0.82), respectively. There was no significant publication bias in Egger's test (p = 0.059, 95% CI −3.81 to 0.082), but Begg's test did (p = 0.023), and the funnel plot is asymmetric. Conclusion Our study suggested that bleomycin was safe and effective in the treatment of LMs and was primarily dose dependent.
ABSTRACT
Objective:To investigate the safety and efficacy of ultrasound-guided sclerotherapy combined with radiofrequency ablation on the complex lymphatic malformations (LM) in children.Methods:The clinical data of 21 children with complex LM treated with ultrasound-guided sclerotherapy combined with radiofrequency ablation in the First Affiliated Hospital of Zhengzhou University from June 2018 to October 2021 were retrospectively analyzed.Intraoperative and postoperative complications were recorded.Imaging examinations were performed at 1, 3, 6, 9, 12, 18, 24 months postoperatively to observe the recurrence, the volume of the lesions and their reduction rate were calculated, and the efficacy was analyzed. Friedman test was used to compare the lesion volume at different time points before and after surgery, and the reduction rate of lesion volume at 1 month postoperatively and other time points after surgery. Results:A total of 21 children were included in this study, among them, there were 12 males and 9 females, age range from 1 month to 5 years and 6 months, with a median age of 23 months.A total of 26 LM in 21 children were successfully treated, and no serious complications like organ damage occurred during and after surgery.One patient with abdominal LM had a postoperative infection, which was controlled by 3 weeks of catheter drainage.Four LM in 3 children recurred at 3 or 6 months after surgery, while all lesions were significantly narrowed down than those before surgery and they were cured after 1-3 sessions of continued sclerotherapy.There were significant differences in the lesion volumes before surgery and 1, 3, 6, 9, 12, 18 and 24 months postoperatively [222.26(159.57, 316.40) cm 3vs.43.06(22.74, 62.53) cm 3, 31.56(15.49, 45.94) cm 3, 25.21(9.63, 36.22) cm 3, 19.80(6.79, 28.81) cm 3, 12.80(3.93, 20.38) cm 3, 7.13(0, 11.34) cm 3, and 2.79(0, 4.93) cm 3; all P<0.05]. There were significant differences between the volume reduction rates at 1 month postoperatively and 3, 6, 9, 12, 18, and 24 months postoperatively [79.36(73.30, 87.81)% vs.85.40(81.09, 91.61)%, 88.85(84.70, 93.61)%, 91.67(87.87, 95.05)%, 94.15(94.47, 97.35)%, 97.11(95.02, 100.00)%, and 99.04(97.93, 100.00)%; all P<0.05]. Patients were followed up for 24 months, and all of them were cured. Conclusions:Ultrasound-guided sclerotherapy combined with radiofrequency ablation is a minimally invasive, safe and effective therapeutic strategy for children with complex LM.
ABSTRACT
Los linfangiomas orbitarios son malformaciones vasculares benignas, de crecimiento lento, abortivas y no funcionales, que se presentan principalmente en la primera década de la vida. Las opciones terapéuticas en estos casos presentan una resolutividad limitada, algunos tratamientos suelen ser agresivos y provocar daños del aparato visual. Se presenta un caso de una paciente femenina de 6 años de edad atendida por proptosis del ojo izquierdo a la que se le realizó el diagnóstico clínico-imagenológico de linfangioma de la órbita, con el objetivo de mostrar el resultado alcanzado en el manejo de la misma mediante el uso del sildenafilo por vía oral, modalidad terapéutica en estudio a nivel mundial en el tratamiento de estas afecciones. El tratamiento con sildenafilo en el linfangioma orbitario demostró ser eficaz en la mejoría del cuadro clínico y por imágenes. Durante el tratamiento no se reportaron reacciones adversas(AU)
Orbital lymphatic malformations are benign, slow-growing, abortive, nonfunctional vascular malformations that occur mainly in the first decade of life. Therapeutic options in these cases present limited resolution, some treatments are usually aggressive and cause damage to the visual apparatus. We present a case of a 6-year-old female patient treated for proptosis of the left eye. The clinical-imaging diagnosis of lymphangioma of the orbit was made to show the results achieved in its treatment through the use of oral sildenafil, a therapeutic modality under study worldwide in the treatment of these conditions. The treatment with sildenafil in orbital lymphangioma proved to be effective in the improvement of the clinical and imaging picture. No adverse reactions were reported during treatment(AU)
Subject(s)
Humans , Female , Child , Vascular Malformations/therapy , Lymphangioma/etiologyABSTRACT
@#Hemangiomas and vascular malformations are common clinical diseases. According to their clinical and imaging characterizations, the International Society for the Study of Vascular Anomalies (ISSVA) has systematically classified infantile hemangioma and vascular malformations, and the classification has been widely recognized and applied. To date, most vascular malformations involve the following important signaling pathways: PI3K/Akt/mTOR and RAS/MAPK/ERK. This discovery has major impacts on the diagnosis and treatment of vascular malformations including the following: the understanding of the biology of vascular malformations has been increased; the understanding of vascular malformations based on genotype has been refined; and the development of targeted drugs for the treatment of vascular malformations has been promoted. Despite facing many challenges, with the development of gene sequencing, molecular biology and imaging technology, the relevance of vascular malformation classification and the accuracy of diagnosis are improving, and this is accompanied by innovations in surgical treatment and sclerotherapy, interventional embolization, and continuous progress in targeted therapy. At present, investigations on vascular malformations are mostly retrospective clinical studies or low-level clinical trials. The purpose of this paper is to review the literature on the treatment of infantile hemangioma, lymphatic malformation, venous malformation and arteriovenous malformation and to review the research progress in evidence-based treatment of infantile hemangioma and vascular malformation.
ABSTRACT
Background: The aim was to study the role of bleomycin and doxycycline as a cheap and readily available sclero-therapeutic agent in the treatment of lymphatic malformations in paediatric populations of poor resource setting.Methods: It was a longitudinal study. A total of 23 paediatric cases with distinct types of lymphatic malformations were treated with injection sclerotherapy. Bleomycin and doxycycline used for microcystic and macrocystic lesion type respectively. The patient was followed up to complete remission. The level of evidence was Level II and type of evidence was prognosis study.Results: Commonest site of lesion was neck (78.3%), followed by cheeck (8.7%), chest, shoulder and suprapubic region. Only 21.7% of patients had good reduction (50-89%) in their lesion volume on first follow up. Overall 43.4% of patients showed a reasonable reduction in lesion volume during the follow-up period. Almost 3/4th of patients improved symptomatically on the first follow up visit. Macrocystic lesion showed an excellent response to treatment (50-89% volume reduction) in 33.3% of cases while only 16.7% of microcystic ad 12.5% of the mixed lesion showed a similar response to treatment.Conclusions: Doxycycline sclerotherapy can be a primary treatment modality in macrocystic and mixed macrocystic lesions. It is inexpensive and widely available and has minimal side effects. In contrast, bleomycine as a sclero-therapeutic agent showed an inadequate response in size reduction of microcystic lesions.
ABSTRACT
@#The etiology and pathogenesis of hemangiomas and vascular malformations are still unclear and face many challenges in terms of treatment. This article focuses on the etiology and genetic mechanism of common vascular tumors (such as infantile hemangiomas, congenital hemangioma and pyogenic granuloma) and vascular abnormalities (such as sporadic venous malformations, blue rubber bleb nevus syndrome, hereditary cutaneomucosal venous malformations, glomuvenous malformations, verrucous venous malformations, lymphatic malformations, and arteriovenous malformations). Some gene mutations have been identified and established. Several mutations in key proteins in the signaling pathways of endothelial cells (ECs) have been shown to play a major role in the pathogenesis of vascular abnormalities. Mutations in PIK3CA and G-protein coupled receptors were most frequently identified. The detection of genetic or somatic gene mutations is important for elucidating the underlying molecular mechanisms and developing effective therapeutic approaches.
ABSTRACT
More than seventy percent of lymphatic malformations (LMs) occur in head and neck region. The management of head and neck LMs is challenging,especially for diffuse and infiltrative cases.It can cause deformity and death in severe patients. Surgical resection is the main method to treat LMs in the past and sclerotherapy has also greatly improved the treatment out come of LMs over the past 20 years. However, it is still hardly to achieve satisfactory prognosis for the patients with extensively infiltrative lesions, regardless of surgical resection or sclerotherapy. In the past five years, some scholars have made a preliminary exploration of new treatment methods, such as oral medicine, to diversify the treatment options of head and neck LMs. This article will review the general situation, biological properties, clinical characteristics, diagnostic methods, and current treatment strategies for LMs as well as trends in management of LMs.
ABSTRACT
Objective:To investigate the efficacy and advantages of fluoroscopy sclerotherapy treatment for the faciocervical lymphatic malformations (LMs) under digital subtraction angiography (DSA) in children. Method:Retrospective analysis of 48 cases of lymphatic malfromations who were treated with intratumoral injection of Pingyangmycin and iohexol under DSA and fluoroscopy, and followed up in Kunming children's hospital. Result:Forty-eight cases of children were treated 1 to 5 intratumoral injection of Pingyangmycin and iohexol that carried out under DSA and fluoroscopy. Follow up ranged from 6 to 18 months, 22 cases (45.83%) were cured, 19 cases (39.85%) were markedly effective, 5 cases (10.42%) were effective and 2 cases (4.17%) were ineffective. The total effective rate was 95.83% with no scarring, pigmentation and significant postoperative complications. Conclusion:Intratumoral injection of Pingyangmycin under DSA is an effective and safe method for treatment of lymphatic malformations in children and has the advantages of positioning accuracy, less damage, fewer complication and no influence on apperarence and function.
ABSTRACT
Objective To investigate the therapeutic effect of Pingyangmycin combined with Triamcinolone Acetonide on the huge faciocervical lymphatic malformations (LMs) in infants.Methods Sixty-seven infants with LMs located in head and neck from January 2009 to June 2014 were retrospectively analyzed in Kunming Children's Hospital.Thirty-five males and 32 females were enrolled,aged from 1 month to 4 years,with a median age of 1.3 years.Computed tomography and ultrasonography were used to evaluate the location,size and extent of LMs before treatment in all the patients.The size of lesion varied from 5.2 cm ×7.5 cm to 9.2 cm × 10.5 cm.All patients were given local injection of Pingyangmycin combined with Triamcinolone Acetonide after puncturing fluid with uhrasonography guiding under general anesthesia.The injection was repeated every 30 d when necessary.Results The number of injections varied from 2 to 5 times,with a median number of 3.9 times.All cases were followed up for 5 to 36 months.Thirty-two cases (62.68％) were cured,improvement in 19 cases (28.36％) and no effect in 6 cases (8.96％).The total effective rate was 91.04％.There was no severe allergic reaction or pulmonary fibrosis.Secondary operation was performed after 6 months in 12 cases.Two post-operative complications were found,1 was minor paralyses of mandibular branch of facial nerve,with mouth askew,the other was trachyphonia,who were both improved after rehabilitation treatment.Conclusions In order to avoid serious complications,the huge LMs may be given local injection of Pingyangmycin combined with Triamcinolone Acetonide after puncturing fluid with ultrasonography guiding.Graded sclerotherapy provides for a less invasive and shorter course of treatment.The complications and risk of secondary resection increase slightly if sclerotherapy has no curative effect.
ABSTRACT
Introducción: El higroma quístico es una dilatación difusa de los conductos linfáticos. Puede diagnosticarse prenatalmente a través de una ecografía obstétrica. La incidencia aproximada es de 1/6,000 nacidos vivos y de 1/750 abortos espontáneos. Esta lesión puede presentarse a nivel cervical en la parte inferolateral del cuello, donde aparece con grandes cavidades únicas o multiloculares. En general, se producen por la falta de conexión de los vasos linfáticos con los sacos linfáticos yugulares, o de estos con el sistema de drenaje venoso. Caso clínico: Con el fin de enfatizar sobre la notificación de estas enfermedades y las opciones de tratamiento no quirúrgico, se presenta una paciente con higroma quístico cervical (cara lateral del cuello) con compromiso de la vía aérea y digestiva por la extensión del tumor. Se trató con etanol puro por medio de múltiples infiltraciones guiadas por ultrasonido. Conclusiones: Dependiendo de las características de la lesión, el tratamiento puede ser quirúrgico, farmacológico o mixto. Cuando la extensión es importante o se relaciona con órganos vitales, la mejor opción de tratamiento es, en primer lugar, reducir el tamaño de la lesión y el compromiso de los órganos contiguos. Esto se hace por medio de escleroterapia. Posteriormente, de ser necesario, se realiza cirugía.
Background: Cystic hygroma is a diffuse dilatation of the lymphatic system, which can be prenatally diagnosed by ultrasound. The incidence is 1/6,000 live births and 1/750 spontaneous abortions. This malformation can occur at the cervical level located in the inferior lateral part of the neck where it appears with large single or multilocular cavities. It is generally caused by a lack of connection with jugular lymphatic channels or with the venous drainage system lymph sacs. Case report: In order to emphasize these diseases and non-surgical treatment options, we present a patient with a cervical cystic hygroma that compromises the airway and digestive tract due to tumor extension and treatment with pure ethanol with clinical improvement. Conclusions: Depending on the characteristics of the lesion, treatment options are surgery, pharmacological or mixed. When the extension involves vital organs, the best option is to reduce the size of the lesion and the compromise of the adjacent organ. This is done by sclerotherapy and, if necessary, surgery.
ABSTRACT
BACKGROUND: Surgical excision and alcohol sclerotherapy have been used to treat congenital vascular malformations (CVM) with a significant success rate but the methods have also left marked morbidity. The alternative, sclerotherapy using ordinary sclerosants, although resulting in trivial complications, has a relatively low cure rate and is rarely used in Korea for CVM management. OBJECTIVE: To evaluate the effects and side effects of sclerotherapy using ordinary sclerosants such as polidocanol (POL) and sodium tetradecyl sulfate (STS) for the treatment of CVM of a venous and lymphatic type. METHODS: To confirm the long-term effects and safety with at least a 3-year follow-up, we chose a total of 26 patients who had undergone sclerotherapy between 2000 to 2004. There were 22 venous malformations (VMs) and 4 lymphatic malformations (LMs) which were rather small and superficial, not beyond muscular fascia. Sclerotherapy using POL and STS was performed by blind intraluminal and/or intralesional injection without the aid of imaging methods such as ultrasound examination. The results were evaluated by the patients' subjective satisfaction, physical examination, comparison of photographs and/or radiological examinations, then they were classified into 4 groups; excellent (improvement >75%), good (50~75%), fair (25~49%) and poor (<25%). RESULTS: Twenty two VMs comprised 14 in the excellent group (66.7%), 6 in the good group (25.0%), 1 in the fair group (4.2%) and 1 in the poor group (4.2%). Two LMs of macrocystic type revealed excellent results but the other two showing microcystic type revealed poor results. The dose of sclerosant was 0.1 to 2 ml in every session and a total of 1 to 6 sessions (average: 2.2) were performed. Only one VM showed hyperpigmentation as a side effect. CONCLUSION: Sclerosants for ordinary varicose vein eradication can be used on the treatment of small and superficial venous malformations and macrocystic-lymphatic malformations with relative efficacy and safety.