Cheng Ming-Huei, Liu Tiffany Ting-Fong
Division of Reconstructive Microsurgery, Department of Plastic and Reconstructive Surgery, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Microsurgery. 2020 Oct;40(7):766-775. doi: 10.1002/micr.30622. Epub 2020 Jul 11.
Primary lymphedema is an anomaly of the regional lymphatic system with long symptom duration or severe lymphatic obstruction. Few microsurgical treatments for primary lymphedema have been reported. This aim of this study was to investigate the outcomes of microsurgical treatments in pediatric primary lymphedema patients.
Between 2013 and 2017, pediatric primary lymphedema patients who underwent either lymphovenous anastomosis (LVA) or vascularized lymph node transfer (VLNT) were retrospectively reviewed. Cheng's Lymphedema Grading, Taiwan Lymphoscintigraphy Staging and indocyanine green lymphography were used to select the procedures. No compression garments were used postoperatively. Outcome measurements included circumferential difference, episodes of cellulitis, and Lymphedema-specific Quality of life questionnaire (LYMQoL).
Nine patients with mean age of 9.2 years (range, 2-19 years) with 11 lower and two upper lymphedematous limbs underwent 11 VLNT and two LVA. All VLNT flaps survived. At a mean 38.4-months (range, 16-63 months) of follow-up, the mean circumferential difference in nine unilateral lymphedematous limbs was improved by 6.7 ± 9.9% (p = .066). Two patients with bilateral lower limb lymphedema had mean limb circumference improvements of 1.3 and 6.5 cm, respectively. In nine limbs with cellulitis preoperatively, episodes of cellulitis decreased by 2.67 times/year (p = .007). At a mean 22.3-months of follow-up (range, 13-24 months), the LYMQoL overall score in 6 patients older than 7 years was improved by 3.2 ± 1.1 points (p = .007).
Lymphedema microsurgery significantly improved the episodes of cellulitis and quality of life without utilizing compression garments in pediatric primary lymphedema patients.
原发性淋巴水肿是一种局部淋巴系统异常疾病，症状持续时间长或伴有严重的淋巴阻塞。关于原发性淋巴水肿的显微外科治疗报道较少。本研究旨在探讨小儿原发性淋巴水肿患者显微外科治疗的效果。
回顾性分析2013年至2017年间接受淋巴管静脉吻合术（LVA）或带血管蒂淋巴结转移术（VLNT）的小儿原发性淋巴水肿患者。采用程氏淋巴水肿分级、台湾淋巴闪烁造影分期和吲哚菁绿淋巴造影来选择手术方式。术后未使用压力衣。观察指标包括周径差、蜂窝织炎发作次数和淋巴水肿特异性生活质量问卷（LYMQoL）。
9例平均年龄9.2岁（范围2 - 19岁）的患者，11条下肢和2条上肢淋巴水肿，接受了11例VLNT和2例LVA。所有VLNT皮瓣均存活。平均随访38.4个月（范围16 - 63个月），9例单侧淋巴水肿肢体的平均周径差改善了6.7±9.9%（p = 0.066）。2例双侧下肢淋巴水肿患者的肢体平均周径分别改善了1.3 cm和6.5 cm。术前有蜂窝织炎的9条肢体，蜂窝织炎发作次数每年减少2.67次（p = 0.007）。平均随访22.3个月（范围13 - 24个月），6例7岁以上患者的LYMQoL总分提高了3.2±1.1分（p = 0.007）。
在小儿原发性淋巴水肿患者中，淋巴水肿显微手术在未使用压力衣的情况下显著改善了蜂窝织炎发作次数和生活质量。
