并发呼吸道梗阻的小儿脉管畸形的综合治疗

Treatment of infantile vascular malformations associated with airway obstruction

目的总结并发呼吸道梗阻的小儿脉管畸形的临床特点，探讨该类疾病的治疗方法。方法回顾性分析1985年6月至2007年12月间收治的47例并发不同程度呼吸道梗阻的小儿脉管畸形资料，其中男25例，女22例；年龄3个月～14岁，中位年龄2．3岁。静脉畸形27例，大囊型淋巴管畸形17例，微囊型淋巴管畸形3例。20例静脉畸形行无水乙醇病变内注射；7例广泛性静脉畸形中行无水乙醇注射＋手术切除；17例大囊型淋巴管畸形行单纯平阳霉素病变内注射，3例微囊型淋巴管畸形行手术切除＋平阳霉素注射。根据患者呼吸道梗阻程度和治疗需要，27例采用气管插管手术，3例术前紧急气管切开，2例术中预防性气管切开，1例拔管后气管切开。结果30例治疗后留置气管插管24～48h，除1例拔管后出现呼吸困难行气管切开，其余均顺利拔管；6例气管切开患儿分别于术后3周～4个月拔除气管套管。病变内注射治疗1次9例，重复注射2～5次38例。2例无水乙醇注射后出现局部黏膜坏死和1例轻度一过性血尿，5例平阳霉素注射后出现轻中度发热反应，经对症处理1h～3周后恢复正常。全组随访1～23年，治愈38例，有效9例，没有无效患儿。结论对合并呼吸道梗阻的小儿咽喉、纵隔部位的脉管畸形，推荐硬化疗法为主的治疗。静脉畸形可采用无水乙醇，淋巴管畸形可先用平阳霉素治疗，对多部位广泛性病变采用手术＋硬化疗法的综合治疗可能缩短疗程和提高疗效。

Objective To summarize the clinical features of vascular malformations complicated with airway obstruction and to evaluate the therapeutic methods of these disease. Methods Forty-seven children with airway obstruction and dyspnea （25 males, 22 females ） were treated from Jun 1985 to Dec 2007, and their clinical data were retrospectively analyzed. Among 47 patients, there were 27 cases of venous malformations, 17 cases of macrocystic lymphatic malformations, and 3 cases of microcystic lymphatic malformations. Injection with absolute alcohol were performed in 20 patients with venous malformations, whereas both sugery and injection were performed in 7 patients with extensive or multiple lesions. Seventeen patients with macrocystic lymphatic malformations were treated with pingyangmycin injection. While surgery combined with pingyangmycin injection were used in other 3 patients with microcystic lymphatic malformations. According to the degree of airway obstruction and therapeutic conditions, tracheal intubation was performed in 27 patients, urgent preoperative tracheotomy was performed in 3 patients, prophylactic tracheotomy was performed in 2 patients, and postoperative tracheotomy was performed in 1 patient. Results Tracheal intubation was remained for 24 to 48 hours in 30 patients, whose intubation was removed successfully in 29 patients except 1 patient who occurred dyspnea after removal of tracheal intubation resulting in tracheotomy. Tracheal cannula was successfully removed in all 6 patients 3 weeks to 4 months after the tracheotomy. There were 9 patients treated once, whereas injections were repeated 2 to 5 times in 38 patients. Necrosis of mucosa occurred in 2 cases after the injection with absolute alcohol, while temporary hemoglobinuriaone occurred in 1. There were 5 cases of light or mediate fever after the pingyangmycin injection who recovered well after the symptomatic treatment. Follow-up lasted 1 to 23 years, 38 patients cured, 9 patients valid, and no patient invalid. Conclusions It is suggested that sclerotherapy should be the first choice in the treatment of vascular malformations complicated with airway obstruction, in which absolute alcohol should be used in venous malformations compared to pingyangmycin in lymphatic malformations. Combined therapy should be carried out in patients with extensive lesions in order to shorten the course of treatment and to get good therapeutic result.