淋巴管畸形介入硬化治疗的效果分析

目的回顾性分析介入硬化治疗淋巴管畸形的方法与疗效,探讨淋巴管畸形介入硬化治疗的有效性及安全性。方法收集2016年12月至2019年5月福建省福州儿童医院采用介入硬化治疗的头面颈部、躯干和四肢体表局灶性淋巴管畸形患儿临床资料,回顾其治疗年龄、硬化剂选择及硬化治疗方法。评价治疗前后囊肿体积或大小,评价指标为:优良,体积减小>90%;中等,体积减小>50%;不显著,体积减小<50%;治疗无反应。将优良和中等定义为显著有效。结果29例淋巴管畸形患儿中,巨囊型8例,微囊型8例,混合型13例。平均疗程1.62次,治疗1次18例,治疗2次4例,治疗>2次7例。巨囊型平均治疗1.375次,6例治疗1次;混合型平均治疗1.46次,9例治疗1次;微囊型平均治疗2.13次,治疗均超过2次。随访时间3个月至2年。所有病例治疗优良率为58.7%(17/29),疗效中等率为31%(9/29),不显著率为10.3%(3/29)。巨囊型显著有效率为100%(优良率100%);混合型显著有效率92.3%(优良率53.8%,中等率38.5%);微囊型显著有效率75%(优良率25%,中等率50%)。3种不同类型淋巴管畸形的显著有效率差异无统计学意义(χ2=2.336,P=0.431);对3种不同类型淋巴管畸形的优良率进行比较,差异有统计学意义(χ2=9.77,P=0.009)。巨囊型的优良率明显高于微囊型(P=0.007),混合型优良率与巨囊型(P=0.046)、微囊型(P=0.367)比较均无统计学意义。并发症:2例术后出现发热,3例术后出现囊内出血,2例发生局部色素沉着,1例发生局部感染,无一例出现过敏性休克等全身不良反应或局部组织坏死情况。结论介入硬化治疗淋巴管畸形有显著疗效,其中巨囊型淋巴管畸形疗效最佳,混合型和微囊型次之,且硬化介入治疗的并发症少。介入硬化治疗可作为体表局灶性淋巴管畸形的首要治疗手段。     

囊内出血对儿童大囊型淋巴管畸形硬化治疗效果的影响

目的探讨儿童大囊型淋巴管畸形并发囊内出血的硬化治疗效果。方法将重庆大学附属三峡医院儿外科收治的50例儿童大囊型淋巴管畸形患者按是否发生囊内出血分为出血组(22例)和未出血组(28例),术前根据包块发现时间、彩超、MRI及穿刺结果确诊。在彩超引导下抽尽囊液后向囊腔内注射聚桂醇注射液,局部加压包扎2~3天。两周后包块未消失者采用同样的方法进行第二次治疗,直至包块消失为止。观察第一次硬化治疗后及最终包块大小的变化、治疗次数。包块消失判定为显效,包块缩小50%及以上判定为有效,包块缩小50%以下判定为无效。结果两组均成功进行硬化治疗,第一次治疗后出血组显效4例,有效9例,无效9例,总有效率59.09%;未出血组显效10例,有效14例,无效4例,总有效率85.71%;两组间疗效差异具有统计学意义(χ2=4.539,P<0.05)。最终两组包块均完全消失,有效率均为100%,其中出血组平均治疗(3.55±1.26)次;未出血组平均治疗(2.57±1.35)次,二者比较差异具有统计学意义(t=2.611,P<0.05)。结论儿童大囊型淋巴管畸形并发囊内出血会降低首次硬化治疗效果,最终疗效满意,但需增加治疗次数。     

儿童淋巴管畸形44例诊疗分析

目的：探讨儿童淋巴管畸形的诊疗方法及疗效。方法回顾性分析本院近年来采用数字减影血管造影技术（digital subtraction angiography,DSA）瘤内注射平阳霉素治疗的44例淋巴管畸形患儿临床资料,并进行随访。结果44例患儿均在DSA透视下实施平阳霉素瘤内注射1～7次,治愈14例（32％）,显效16例（36％）,有效13例（30％）,无效1例（2％）。随访6个月至2年,疗效满意,病灶区无瘢痕及色素沉着,术后无明显并发症,总有效率达98％。结论 DSA透视下瘤内注射平阳霉素治疗儿童淋巴管畸形定位准确,损伤小,并发症少,疗效显著,不影响外观和功能,无明显不良反应,对于手术难以切除的淋巴管畸形是一种较好的微创治疗方法。     

小儿颈纵隔淋巴管瘤的外科治疗

目的总结小儿颈纵隔淋巴管瘤的特点,探讨其手术方法。方法回顾性分析2005年8月-2011年7月本院收治经术后病理证实的颈纵隔淋巴管瘤患儿31例,男10例,女21例;年龄1个月～4岁,平均年龄11.0个月,肿瘤位于右侧11例,左侧20例。结果 7例经颈部锁骨上横切口手术;24例纵隔病变为主者经后外侧切口常规开胸手术。完整切除14例,其他病例行大部切除,囊壁破坏或囊肿揭顶引流,残留囊壁碘酊烧灼。术中损伤无名静脉1例,术后并乳糜胸5例、喉返神经损伤3例、膈神经损伤2例、复发3例,无死亡病例。结论颈纵隔淋巴管瘤一经诊断应立即手术,切口的选择很重要,应根据CT及MRI结果慎重选择,单一切口多可完成手术。术中不宜强求完整切除,残留囊腔敞向胸膜腔可减少术后复发。     

气管周围淋巴管畸形的治疗方法

目的探讨儿童气管周围淋巴管畸形的治疗方法并总结经验。方法收集2009年10月至2019年10月南京医科大学附属儿童医院烧伤整形外科收治的气管周围淋巴管畸形27例患儿的相关资料。其中,男16例,女11例;中位年龄为8个月,年龄范围为4~14个月。所有患儿均接受磁共振成像检查,提示为T1像等或高信号,T2像高信号,脂肪抑制像呈高信号。根据气管周围淋巴管畸形的病变范围和临床表现将患儿分为3型:咽后壁型13例,咽侧壁型11例,完全弥漫型3例。所有患儿中,5例行单纯口服药物治疗,17例行单纯注射治疗,5例行注射+口服药物治疗。采用4级分级标准对治疗效果进行评价:Ⅰ级(差),瘤体缩小0~25%;Ⅱ级(中),瘤体缩小26%~50%;Ⅲ级(好),瘤体缩小51%~75%;Ⅳ级(优),瘤体缩小76%~100%。结果所有患儿呼吸困难的症状均明显缓解,控制了淋巴管畸形的增长;随访时间范围为1~10年,2例痊愈,25例尚在随访中。疗效Ⅰ级0例,Ⅱ级3例,Ⅲ级9例,Ⅳ级15例。Ⅲ级疗效以咽后壁型最佳,Ⅳ级疗效以咽侧壁型最佳。完全弥漫型瘤体体积减小最明显。19例出现发热,多见于接受平阳霉素注射者,体温多<38.5℃,经对症处理24 h内恢复正常。无过敏性休克、肺纤维化病例。结论早期发现淋巴管畸形对于改善患儿症状极为重要,若延迟治疗或治疗方法不正确,淋巴管畸形增大,会使患儿症状加重甚至危及生命。     

气管周围淋巴管畸形的治疗方法

目的探讨儿童气管周围淋巴管畸形的治疗方法并总结经验。方法收集2009年10月至2019年10月南京医科大学附属儿童医院烧伤整形外科收治的气管周围淋巴管畸形27例患儿的相关资料。其中,男16例,女11例;中位年龄为8个月,年龄范围为4~14个月。所有患儿均接受磁共振成像检查,提示为T1像等或高信号,T2像高信号,脂肪抑制像呈高信号。根据气管周围淋巴管畸形的病变范围和临床表现将患儿分为3型:咽后壁型13例,咽侧壁型11例,完全弥漫型3例。所有患儿中,5例行单纯口服药物治疗,17例行单纯注射治疗,5例行注射+口服药物治疗。采用4级分级标准对治疗效果进行评价:Ⅰ级(差),瘤体缩小0~25%;Ⅱ级(中),瘤体缩小26%~50%;Ⅲ级(好),瘤体缩小51%~75%;Ⅳ级(优),瘤体缩小76%~100%。结果所有患儿呼吸困难的症状均明显缓解,控制了淋巴管畸形的增长;随访时间范围为1~10年,2例痊愈,25例尚在随访中。疗效Ⅰ级0例,Ⅱ级3例,Ⅲ级9例,Ⅳ级15例。Ⅲ级疗效以咽后壁型最佳,Ⅳ级疗效以咽侧壁型最佳。完全弥漫型瘤体体积减小最明显。19例出现发热,多见于接受平阳霉素注射者,体温多<38.5℃,经对症处理24 h内恢复正常。无过敏性休克、肺纤维化病例。结论早期发现淋巴管畸形对于改善患儿症状极为重要,若延迟治疗或治疗方法不正确,淋巴管畸形增大,会使患儿症状加重甚至危及生命。     

超声引导下硬化联合射频消融治疗儿童复杂淋巴管畸形的疗效分析

目的探讨超声引导下硬化联合射频消融治疗儿童复杂淋巴管畸形(LM)的安全性及疗效。方法回顾性分析2018年6月至2021年10月郑州大学第一附属医院使用超声引导下硬化联合射频消融治疗的21例复杂LM患儿的临床资料, 记录术中、术后并发症发生情况。术后1、3、6、9、12、18、24个月行影像学检查, 观察复发情况, 计算病灶体积及其缩小率, 分析其疗效。采用Friedman检验比较术前与术后不同时间点病灶体积、术后1个月与之后不同时间点病灶体积缩小率。结果本组共纳入21例患儿。其中男12例, 女9例;年龄1个月至5岁6个月, 中位年龄23个月。21例患儿共计26处LM均顺利完成治疗, 术中、术后未出现器官损伤等严重并发症。1处腹部LM患儿术后感染, 置管引流3周后感染得到控制。3例患儿4处LM于术后3个月或6个月随访时复发, 但病灶体积均较术前明显缩小, 继续硬化治疗1～3次后治愈。术前与术后1、3、6、9、12、18、24个月病灶体积比较差异均有统计学意义[222.26(159.57, 316.40) cm3比43.06(22.74, 62.53) cm3、31.56(15.49, 4...     

Nd:YAG激光光纤治疗儿童淋巴管畸形18例

目的 探讨采用Nd:YAG激光光纤治疗儿童躯干及四肢部位淋巴管畸形的疗效. 方法 选择南京医科大学附属儿童医院2018年1月至2019年1月收住入院的18例体表淋巴管畸形患者作为研究对象,术前均行MRI及B超检查,采用光纤插入法进行激光内照射治疗.每3个月治疗一次,重复治疗3次,术后早期持续穿戴弹力裤或绷带.18例均接...     

儿童弥漫性肺淋巴管瘤病

淋巴管瘤病是一种罕见的淋巴管异常增生性疾病。病变通常呈弥漫性、多灶性分布,当累及肺、纵隔、胸膜等胸腔内器官及组织时,也称为弥漫性肺淋巴管瘤病。通常认为,弥漫性肺淋巴管瘤病预后欠佳。近年来,随着遗传学以及细胞信号通路研究领域的突破,对弥漫性肺淋巴管瘤病的发病机制有了新的认识。而靶向药物的应用及展现出来的良好疗效,也为弥漫性肺淋巴管瘤病的治疗带来了新希望。该文着重介绍了儿童弥漫性肺淋巴管瘤病的发病机制、临床表现、诊断方法和治疗进展等。     

Intensive care experience with sclerotherapy for cervicofacial lymphatic malformations.

OBJECTIVE: To describe a cohort of patients needing intensive care support after sclerotherapy for cervicofacial lymphatic malformations. DESIGN: Retrospective review of case records of patients undergoing sclerotherapy between January 2004 and November 2006. SETTING: A tertiary, university-affiliated, pediatric teaching hospital. PATIENTS: Five patients needing admission to a pediatric intensive care unit (PICU) following sclerotherapy with OK432. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Five patients needed a total of 13 PICU admissions. Ages ranged from 4 months to 19 months. All patients had extensive lesions that involved the airways, mediastinum, or floor of the mouth, documented by magnetic resonance imaging. Nine admissions involved elective intubation and ventilation following sclerotherapy due to the extent of lesions. There were four urgent admissions to the PICU with respiratory distress ranging from 3 to 18 days after sclerotherapy. The mean duration of admission was 7 days (total 93 days, range 2-22 days). Total ventilated hours were 1656 hrs with a range of 16.5-370 hrs per admission. Multiple procedures, such as drainage of cysts and further sclerotherapy procedures, were performed before extubation on the PICU. CONCLUSIONS: Children with extensive disease and airway involvement need multiple PICU admissions. The potential for life-threatening respiratory embarrassment is unpredictable following sclerotherapy. Consideration should be given to performing further sclerotherapy while the patients are intubated in the PICU. The PICU provides a safe and secure environment for such procedures.     

Doxycycline sclerotherapy in children with lymphatic malformations: outcomes, complications and clinical efficacy

Background

Lymphatic malformations (LMs) are congenital lesions of the lymphatic system and consist of lymphatic channels and cystic spaces of varying sizes. Recent evidence has shown that LMs respond well to intralesional sclerotherapy.

Objective

The purpose of this retrospective study is to evaluate the outcome and efficacy of using doxycycline in treating macrocystic, microcystic and combined macro- and microcystic LMs in a tertiary-care pediatric center.

Materials and methods

Fifty children (32 boys, 18 girls) underwent doxycycline sclerotherapy for treatment of LMs between January 2005 and October 2010. Demographics, imaging, doxycycline treatment details, complications and follow-up details were documented.

Results

The mean age at first treatment was 5.9?years (3?days–17.8?years), median 4.2?years. Cervicofacial (19/50 children) and truncal (16/50 children) locations were the most frequently affected. One hundred forty-six sclerotherapy sessions were performed in 50 children (mean 2.9/child). The mean doxycycline dose/kg body weight for 146 sessions was 15.3?mg/kg (0.6–85.7?mg/kg). Complications occurred in 4/146 procedures. Clinical follow-up showed excellent response in 14/16 children with macrocystic LMs, 21/27 children with combined LMs and 4/7 children with microcystic LMs.

Conclusion

Doxycycline is a safe and effective sclerosant agent for treating LMs in children, with a low complication rate.     

Intralesional sclerotherapy for subcutaneous venous malformations in children

Background  Venous malformations (VMs) involve multiple anatomical spaces and encase critical neuromuscular structures, making surgical treatment difficult. Recently sclerotherapy has been suggested as the primary treatment for VMs instead of surgical intervention. This report represents eight cases of children with VMs treated with direct percutaneous injections of sclerosing agents, such as ethanol, polidocanol or ethanolamine oleate. Methods  All eight patients had large lesions (>3 cm) located on the head, foot, neck and face. Sclerotherapy was performed in an angiographic suite under general anesthesia. Prior to sclerotherapy, percutaneous phlebography was performed in order to visualize the dynamic situation inside the lesion and the draining flow into the adjacent venous vascular system. A 2–15 ml of sclerosing agent was injected into VM lesions under fluoroscopy. Results and conclusions  An evaluation by MRI examination showed that 6 out of 8 patients had remission, and alleviation of their symptoms without major complications, furthermore one of the lesions apparently disappeared. Intralesional sclerotherapy provides a simple, safe and effective treatment for VMs in the subcutaneous lesions in children.     

Site-specific induction of lymphatic malformations in a rat model for image-guided therapy

Background Lymphatic malformation is a common benign mass in children and adults and is representative of a derangement in lymphangiogenesis. These lesions have high recurrence rates and significant morbidity associated with surgery. Several sclerotherapy regimens have been developed clinically to treat lymphatic malformations; however, an animal model has not been developed that is adequate to test the efficacy of image-guided therapeutic interventions. Objective To develop an animal model suitable for evaluation of percutaneous treatments of lymphatic malformations. Materials and methods Male Harlan Sprague-Dawley rats (n = 9) received two US-guided injections of Incomplete Freund’s Adjuvant (IFA) over a 2-week period. All nine rats were injected twice into the peritoneum (IP); a subgroup (n = 3) received additional injections into the neck. Three animals that received IP injections of saline were used as controls. The injection sites were monitored for the development of lesions by high-resolution ultrasonography at 2-week intervals for 100 days. High-resolution (4.7 Tesla) magnetic resonance imaging was then performed on two animals noted to have developed masses. The rats were sacrificed and histologic examination of the identified lesions was performed, including immunohistochemical staining for vascular (CD31) and lymphatic (Flt-4 and Prox-1) endothelium. Results All animals injected with IFA developed cystic lesions. The three animals injected at dual sites were noted to have both microcystic and macrocystic malformations in the neck and microcystic plaque-like lesions in the peritoneum. The macrocystic malformations (≥5 mm) in the neck were detected by ultrasonography and grossly later during necropsy. Histopathologic analysis revealed the cystic spaces to be lined by lymphatic endothelium supported by a connective tissue stroma. Control animals did not exhibit detectable lesions with either ultrasonography or necropsy. Conclusion This model represents a promising tool for translational development of image-guided interventions for lymphatic malformations. It may also serve as a model for the study of lymphangiogenesis and the development of anti-lymphangiogenic therapies.     

Primary lymphatic dysplasia in children: chylothorax,chylous ascites,and generalized lymphatic dysplasia

Primary lymphatic dysplasia, a congenital maldevelopment, interferes with function of the lymphatic system and causes effusion of chyle or lymph into the limbs and pleural or peritoneal cavity. Between 1955 and 1982, 38 Mayo Clinic patients were found to have a chylous effusion or dysplasia of the lymphatic system. In 22, the condition was secondary to surgery or other medical problems and in 16 it was primary. These cases were separated into three categories: chylothorax, chylous ascites, and generalized lymphatic dysplasia. Conservative therapy, such as a restricted fat diet or total parenteral nutrition with repeated thoracentesis or paracentesis, was effective in the children with isolated abnormalities of the lymphatic system (75% resolution rate, no deaths). All five children with documented generalized dysplasia reported in the literature had died; of the three reported here, one has died and two have become progressively worse.Abbreviations CT computed tomography - TPN total parenteral nutrition