开窗置管注射OK-432治疗颈面部淋巴管瘤

目的介绍并评价应用OK-432(沙培林)注射治疗颈面部淋巴管瘤的方法和疗效。方法回顾性分析15例颈面部淋巴管瘤患儿的诊治经过。结果15例患儿均为较大的囊状水瘤,均接受了开窗病灶内留置导管间断注射OK-432,取得满意疗效,病灶区无疤痕及色素沉着,术后无明显并发症,无需再次手术治疗。结论开窗病灶内置管间断注射OK-432治疗颈面部淋巴管瘤特别是水囊瘤,具有微创、安全、效果确实等优点,对于手术难以切除的巨大囊瘤是较好的微创治疗方式。     

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。     

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。     

平阳霉素瘤内注射治疗小儿囊状淋巴管瘤228例疗效分析

目的观察平阳霉素(PYM)瘤内注射治疗囊状淋巴管瘤的疗效。方法收集2000年1月～2005年12月228例应用PYM注射治疗囊状淋巴管瘤患儿的临床资料,男129例,女99例,男女比例1:0.76;年龄最小3个月,最大14岁;瘤体位于颈颌面部147例,躯干45例,四肢36例;瘤体体表面积最小2cm×1.5cm,最大8cm×10cm。对瘤体内7～10d注射1次,3～8次为一个疗程。结果经6个月～5a随访,治愈222例,治愈率97.37%;好转6例,好转率2.63%,总有效率100%。结论PYM治疗小儿囊状淋巴管瘤疗效高,是一种简便、安全的有效方法。     

瘤内注射平阳霉素治疗囊性淋巴管瘤18例分析

目的 了解瘤内注射平阳霉素治疗囊性淋巴管瘤的疗效及不良反应。方法 对2001—2005年收治的18例囊性淋巴管瘤患儿进行了瘤内注射平阳霉素的治疗。注射前通过超声或CT定位,平阳霉素用注射用水溶解成1mg／ml的溶液,总剂量1～18mg（平均4,2mg／次）,注射1—4次。结果 18例中16例（88．9％）肿块明显缩小,其中11例（61．1％）肿块完全消退;2例肿块未缩小。3例进行了手术。瘤内注射治疗后个别病例出现短暂发热和局部肿痛的炎症反应,未出现严重的并发症。结论 瘤内注射平阳霉素对治疗囊性淋巴管瘤有效。     

低浓度平阳霉素注射治疗1110例淋巴管瘤单中心临床分析

目的 探讨镇静下B型超声引导下低浓度平阳霉素注射微创治疗软组织淋巴管瘤的安全性及有效性.方法 1 110例软组织淋巴管瘤患儿,年龄1个月～17岁8个月.病变类型:大囊型淋巴管瘤(囊状水瘤)233例、小囊型淋巴管瘤391例、海绵状淋巴管瘤247例、混合型淋巴管瘤224例、弥漫性淋巴管瘤15例.基础麻醉下行B型超声引导下低浓度平阳霉素注射微创治疗,大囊及小囊性淋巴管瘤浓度为0.4 mg/ml囊内注入,海绵状、混合性及弥漫性淋巴管瘤为0.33 mg/ml浓度瘤内多点注入.单次治疗最大剂量:学龄儿及以上(≥5岁)不超过4 mg;3～5岁不超过2～3 mg,幼儿(＜3岁)不超过2mg;婴儿(＜1岁)不超过1mg;年龄小于6月龄的婴儿用量＜0.5 mg,1月龄内不采用平阳霉素治疗,1～2月龄慎用.每隔2～3个月一次,共1～10余次不等.结果 1 110例软组织淋巴管瘤患儿总的完全缓解率为51.62％,非常好的部分缓解率为41.53％,部分缓解率为4.59％,微缓解率为1.17％,疾病稳定率为1.08％.其中淋巴管瘤各类型中大囊型疗效最好,CR为100％;其次为小囊型,CR为86.96％;CR仅发生在大囊型及小囊型两组.海绵状VGPR 83.40％;混合性VGPR91.07％;弥漫型疗效最差,80％为SD,20％为MR.迟发瘤内出血及迟发感染等并发症发生率为3.42％,无急性感染、皮肤破溃、坏死、硬变、肺纤维化等严重并发症.结论 镇静下B超引导下低浓度平阳霉素注射精准微创治疗软组织淋巴管瘤为安全、有效的治疗方法.     

平阳霉素加确炎舒松-A局部注射治疗小儿淋巴管瘤

目的总结平阳霉素加确炎舒松-A局部注射治疗小儿淋巴管瘤的临床经验，为局部注射治疗淋巴管瘤的推广运用提供依据。方法回顾性分析157例淋巴管瘤患儿的临床资料，对其临床特点、治疗方法及疗效进行评估。结果157例中，囊性120例，海绵状25例，混合性12例。肿瘤直径〉5cm者达91例，其中〉10cm者40例。采用平阳霉素加确炎舒松-A瘤内注射治疗1～5次，痊愈137例，好转19例，无效2例，有效率达98．7％。随访3～7年，疗效巩固。结论药物局部注射治疗小儿淋巴管瘤安全，经济，简便，疗效好。在诊治小儿淋巴管瘤的过程中，应注意鉴别后天性或继发性淋巴管瘤。     

B超引导下注射平阳霉素治疗婴幼儿囊性淋巴管瘤22例

目的总结B超引导下注射平阳霉素治疗囊性淋巴管瘤的经验与疗效。方法对我科2002年～2006年收治的22例囊性淋巴管瘤患儿在B超引导下于瘤内注射平阳霉素。注射前先行B超定位,将平阳霉素用注射用水溶解成1mg/ml的溶液,一次最大用量不超过4mg,每2周注射1次,共注射1～4次。结果本组18例肿块完全消退,4例肿块明显缩小,个别患儿有短暂发热、局部红肿、疼痛等炎症反应。结论B超引导下注射平阳霉素治疗婴幼儿囊性淋巴管瘤安全、有效、经济,值得推广。     

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例均接受3次光纤治疗,疗程结束后进行评估,疗效欠佳者可选择其他方法治疗,随访1～2年。结果 18例中3例淋巴管畸形改善不明显,选择手术治疗。其余15例经激光内照射治疗后,淋巴管畸形明显缩小甚至消失,无一例畸形范围继续增大,有效率为83.3%。6例瘤体未完全消失者每6个月到1年病灶内注射聚桂醇1次,其中4例注射2次,1例注射3次,1例注射4次。10例病灶位于肢体或腋下的患儿中,其中7例病灶位于肢体的患者肢体周径较治疗前缩小平均3.7 cm, 3例病灶位于腋下的患者胸围径较治疗前缩小平均4.03 cm。治疗前患肢与健侧平均差值为4.7 cm;经治疗后,平均差值缩减为2.23 cm。治疗前位于腋下的患侧与健侧平均差值为5.2 cm;经治疗后,平均差值缩减为1.17 cm。18例治疗过程中均无明显不良反应。结论 Nd:YAG激光光纤治疗儿童躯干及四肢部位淋巴管畸形安全可行,有较大的临床应用价值。     

OK-432 therapy of lymphangiomas in children

Between April 1988 and July 1995, 11 children with a lymphangioma were treated with intralesional OK-432 injection. In 7 patients it was the primary therapy and total shrinkage of the lesion was obtained in 5 of them. Two patients did not respond and the children underwent surgery. Following incomplete surgical removal or recurrence of the lymphangioma, intralesional OK-432 injection was used as secondary therapy in 4 patients. Total regression was observed in 2 cases and marked regression in the 2 others. No serious side-effects except fever lasting for 2–3 days and slight tenderness with swelling of the lymphangioma for 3–4 days after the injection was noted. Local inflammatory reaction did not cause any damage to the overlying skin and did not lead to scar formation. Depending on the size, location, and anatomical relationship to the airway, intralesional injections of the lymphangiomas were performed under general anaesthesia and the children were observed for 24 h. There was no recurrence after follow up periods ranging from 2 months to 7 years.     

Cystic lymphangioma with special reference to rare sites

A 10 year retrospective study of 45 cases of cystic lymphangioma (CL) in children is presented. There were 25 females and 20 males. Age ranged from 6 months to 8 years. Common sites were involved in 38 and rare sites in 7 patients. Rare sites were—gluteal region (1), pelvis (1), retroperitoneum (1), mesentery (2), inguinal region (1) and inguinoscrotal region (1). The clinical presentation included sudden increase in size (25), lump abdomen (3), gluteal abscess (1), abdominal distension (1) and inguinal swelling (2). Diagnosis was established preoperatively in 38 cases, and after surgery and histopathology in 7 cases. Near total or subtotal excision was carried out in all cases. Facial nerve palsy (1) and recurrence (2) were the complications of surgery. The study is presented to highlight the occurrence of the cystic lymphangioma at rare sites to avoid diagnostic errors and unnecessary mutilating surgery.     

聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤

目的 探索聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤的方法和经验.方法 回顾性分析2010年9月至2014年8月采用聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤39例的临床资料.39例术前均经彩色超声、CT或MRI检查确诊,治疗时在彩色超声引导下经皮穿刺抽尽囊液后注入药物,对多囊性患儿由深到浅依次抽液后分别注入药物.根据瘤腔位置和大小不同,对眼眶周、口腔、颌下及瘤体容积超过100 ml的18例采用聚桂醇泡沫进行注射,其余21例采用聚桂醇原液进行注射.观察瘤体大小变化、治疗次数和并发症.疗效以瘤体消失,1年后无复发为显效;瘤体缩小50％以上,1年后无增大为有效;瘤体缩小50％以下或治疗后1年内复发为无效.结果 39例治疗1～5次,平均(2.85±0.87)次.显效36例(92.3％),有效2例(5.1％),无效1例(2.6％),总有效率为97.4％(38/39).其中,采用聚桂醇泡沫治疗的次数为(2.72±0.75)次,采用聚桂醇原液治疗的次数为(2.95±0.97)次,两组比较,差异无统计学意义(t=0.26,P＞0.05).有3例第一次注射治疗后当天轻度发热,经物理降温后缓解;5例治疗后局部肿胀,轻微疼痛,观察1～3 d后消失.其余未发现明显并发症.结论 聚桂醇瘤腔内注射治疗小儿囊性淋巴管瘤是一种微创、安全、有效的方法.     

Local bleomycin injection in the treatment of lymphangioma.

AIM: We report here our experience with local bleomycin injection in lymphangioma. PATIENTS AND METHODS: Nine patients with lymphangioma were treated with locally injected bleomycin and followed prospectively. We performed an ultrasound study in all cases to delineate the size, location, nature, and number of compartments of the cyst prior to the injection. Under ultrasound guidance, the content of the cystic cavity was aspirated and 1 - 3 mg/kg bleomycin were injected. The patients were revisited monthly. No attempt at re-injection was made as long as the mass continued to decrease in size. RESULTS: Patients consisted of 4 boys and 5 girls aged between 14 days and 6 years. The localisation of the mass was cervical in 6, cervical, sublingual, and lingual in 1, axillary in 1 and axillary and thoracic in 1. In six children, the mass disappeared totally after a single injection. In one patient a second injection was needed, and surgical excision was performed in two patients with residual solid component. No complication related to bleomycin was seen. CONCLUSION: Local application of bleomycin in children with lymphangioma is a simple, safe, and effective method. In the majority of cases, total healing may be achieved with a single injection.     

�����׶�����ƶ�ͯ��λ���Ķ������ۺ��� ʱ��-��Ч����

??Abstract??Objective The time-effect relationship in treating postural orthostatic tachycardia syndrome ??POTS?? of children with alpha receptor agonist midodrine hydrochloride was analyzed to explore the optimum course of treatment for POTS children. Methods A total of 104 cases of the POTS children were studied from 2005 to 2011 in Peking University First Hospital. A chi-square test was used to evaluate the relationship between effect and time of medication?? and the time-effect curve was constructed. Results According to the 104 cases?? the time accumulative total efficacy for 1 month?? 2 months?? 3 months?? 4 months?? 5 months and 6 months were 19.23%?? 48.08%?? 69.23%?? 73.08%?? 74.04% and 75.96%?? respectively. The time accumulative total efficacy for 3 months was higher than that for 1 month or 2 months ??P??0.05???? but there was no difference in the efficacy among 4 months?? 5 months and 6 months treatment and with 3 months treatment. Conclusion It has the best treatment efficacy when the course of treatment for POTS children with midodrine hydrochloride is 3 months?? and prolonging the duration of treatment does not significantly improve the therapeutic effect.     

Mesenteric cystic lymphangioma in children. Report of a case manifested by anemia

A case of mesenteric cystic lymphangioma revealed by hypochromic anemia and abdominal pain, secondary to an intracystic hemorrhage is reported in a 4-year old child. Etiology, pathology, diagnosis, treatment and prognosis of mesenteric lymphangioma in children are reviewed.     

Six cases of children with a benign cervical tumor who required tracheostomy

Cervical tumors sometimes cause airway obstruction. We have treated six children with benign cervical tumors who required tracheostomy. Two cervical and one glossal lymphangiomata treated with local injection of OK432 after creating a tracheostomy were successfully decannulated after the treatment. One patient with a giant cervical lymphangioma needed an EXIT (ex utero intrapartum treatment) procedure. He underwent tracheostomy at 10 months of age after long-term endotracheal intubation, but he died of sepsis and hypoxic brain damage at 18 months. One patient with a subglottic hemangioma treated with steroids finally achieved closure of the tracheostomy at 2 years of age. A 7-year-old girl with a tracheal schwannoma underwent tracheostomy performed a week after admission, but she already had hypoxic brain damage resulting from problems with intubation. Most patients with a lymphangioma or hemangioma in the cervical region have required early tracheostomy before commencing treatment with OK-432 or steroids. If there is any sign of possible airway compromise, then it is vital to perform an early tracheostomy, even for benign tumors.