Chy-3 mice are Vegfc haploinsufficient and exhibit defective dermal superficial to deep lymphatic transition and dermal lymphatic hypoplasia.

Recent advances in molecular lymphology and lymphatic phenotyping techniques in small animals offer new opportunities to delineate mutant mouse models. Chy-3 mutant mice were originally named for their chylous ascites, but the underlying lymphatic disorder was not defined. We now re-examined these mice and applied advanced genotyping and lymphatic phenotyping techniques to pinpoint the specific lymphatic defect in this mouse model. We demonstrated that Chy-3 mice carry a large chromosomal deletion that includes Vegfc and narrowed this region by monitoring the heterozygosity of genetic markers. We found that Chy-3 mice not only exhibited chylous ascites but also lymphedema of the hind paws and, in approximately half of the males, lymphedema of the penis. Visual lymphangiography and immunofluorescence staining showed a hypoplastic dermal lymphatic network, whereas the blood vasculature appeared unaffected. This hypoplastic lymphatic network was functional, and all adult Chy-3 mice exhibited a lateral lymphatic pathway directly connecting the inguinal to the axillary lymph node. The dermal superficial to deep lymphatic connections in upper limbs and in all cervical regions were intact and functionally drained the upper body. Lymphatic tracer was not transported from the dermal to the deep truncal lymphatic system in the lower limbs, even though the deep lymphatic vessels and nodes were present and patent. These findings further delineate the lymphatic phenotype of Chy-3 mice, identify a collateral lymph drainage pathway previously undescribed in other genetic models of lymphedema, and demonstrate a predilection for lymphatic abnormalities of the lower limbs.     

Determination of Bioelectrical Impedance Thresholds for Early Detection of Breast Cancer-related Lymphedema

Background: Bioelectrical impedance technology is a common technique used for the early detection of breast cancer-related lymphedema (BCRL). However, studies on the threshold value established by Inbody 720 device (Biospace, Korea) have been extremely limited. We aimed to determine its reference range and cutoff values.Methods: All patients were recruited from October 2017 to October 2019 at the Peking University People''s Hospital Breast Center. In total, 82 patients with unilateral BCRL and 1305 healthy subjects were recruited in this study. We measured the extracellular fluid (ECF) ratio, extracellular water (ECW) ratio, as well as the single-frequency bioimpedance analysis (SFBIA) ratios at 1 and 5 kHz with the Inbody 720 device. The Youden index-based cutoff points, mean + 2SD and mean + 3SD values of these four indicators for both dominant and nondominant arms were also calculated.Results: Data were collected from 1387 women, including healthy subjects and patients with lymphedema. All statistical analyses were performed with SPSS. Significant differences were found between the two groups in the ECW, ECF, and SFBIA ratios. For the dominant affected arms, the Youden index-based cutoff points for the ECF, ECW, as well as SFBIA ratios at 1 and 5 kHz were 1.009, 1.008, 1.068, and 1.068, respectively. For the nondominant affected arms, the Youden index-based cutoff points were 1.014, 1.013, 1.047, and 1.048, respectively. The mean + 2 standard deviations (SD) and mean + 3SD values were also calculated.Conclusions: We determined the Youden index-based cutoff points, mean + 2SD and mean + 3SD values of the ECF, ECW, as well as SFBIA ratios at 1 and 5 kHz for both dominant and nondominant arms with data from 1305 healthy subjects. Next, the Youden index-based cutoff points, the mean + 2SD and mean + 3SD values were used to recognize patients with lymphedema. We found that the Youden index-based cutoff points and the mean + 2SD showed similar identification capacity on lymphedema, and they seemed to distinguish more patients with lymphedema than mean + 3SD values.     

Microcephaly, lymphedema, chorioretinopathy and atrial septal defect: a case report and review of the literature

Background: The rare congenital combination of microcephaly, lymphedema and chorioretinopathy (MLCD) has been described. Recently, three cases with these clinical characteristics have been diagnosed as having, in addition, various congenital cardiac anomalies, which may be part of this genetic entity that presents with variable expression.
Clinical observation: Here we present a new case of a one-year-old infant who was born with microcephaly and lymphedema and atrial septal defect (ASD) and developed chorioretinopathy at the age of 6 months. This infant had normal neurodevelopment at one year of age.
Conclusion: We recommend that cardiac evaluation and long-term ophthalmologic follow-up should be part of the evaluation in each child born with microcephaly and lymphedema. Family counseling should include the fact that normal to near-normal development may be possible, despite the presence of microcephaly.     

乳腺癌术后上肢水肿诊疗现状分析

对近10年有关乳腺癌术后患侧上肢淋巴水肿的发病原因、诊断、治疗及预防等方面的资料进行综述。乳腺癌术后上肢淋巴水肿严重危害患者的身心健康和生活质量。按摩、压力泵、微波等物理治疗对轻中度水肿有效,但易反复;药物治疗效果不理想;重症患者可考虑手术治疗。水肿一旦形成则很难治愈,应把工作的重点放在预防上;前哨淋巴结活检术取代腋窝淋巴结清扫术,有望从根本上解决上肢淋巴水肿等并发症。     

EXPERIMENTAL TREATMENT FOR OBSTRUCTIVE LYMPHEDEMA BY TRANSPLANTATION OF FASCIAL FLAP RICHED IN LYMPH VESSELS

Objective To provide a more effective microsurgical treatment for obstructive lymphedema. Methods Sixty models of rabbits with lymphedema in right hindlimb were devided into three groups： 20 for fascial flap transplantation （group A ）, 20 for no treatment （group B） and 20 for anastomosis of lymph vessels and vein （group C） as control. For group A, the posterior branch of medial vein in the normal hindlimb was cut down together with the fascial strip （ about 3 cm wide） around it and concomitant lymph vessels, then transferred through the block region of the affected limb. The two broken tips of the vein were anastomosed respectively to normal veins beside the block region. Subcutaneous tissue and skin were sutured at last. For group B, no treatment was carried out. But traditional anastomosis of lymph vessels and vein was used for group C. Postoperative variation of volume was observed and lymphoscintigraphy was made. Results The volume curve of the affected limb in group A kept declining after 2 weeks and nearing to that of the normal hindlimb in group B but away from that of the affected limb in group B. The curve of donor limb in group A was similar to that of the normal limb of group B, which meant no secondary lymphedema occurred in donor limb. The volume of the affected limb of group A had significant difference to that of group C at postoperative 24 weeks, which meant the effect of group A was better than C. The lymphatic drainage of the affected limb of group A was obviously better than B and C in lymphoscintigraphic image. Deposit of nuclein was lessened. Conclusion It was verified that the transplantation of vein-lymph vessels-fatty fascial flap had following advantages： decreased technical difficulty, better long-term effect, almost no chronic lymphedema occurred in donor site, extensive adaptation.     

乳腺癌淋巴结清扫术中保留上肢淋巴结的疗效分析

目的 在探讨乳腺癌淋巴结清扫术中保留上肢淋巴结对淋巴水肿及生活质量的影响。方法 选取2013 年1 月-2014 年12 月于贵州省人民医院乳腺科收治的100 例T1-3No Mo 期乳腺癌患者,根据患者及其家属协商选择的手术方法将研究对象分为观察组和对照组,每组各50 例。观察组术前以纳米炭混悬液行经上臂逆行腋窝淋巴结示踪术;对照组行腋窝淋巴结清扫术。于术后1、6 及12 个月时对两组患者进行随访,记录并比较两组患者随访期间各时间点患侧比健侧上肢臂围增加的百分比,术后12 个月时上肢淋巴水肿发生情况,各时间点乳腺癌患者生命质量测定量表（FACT-B）评分。结果 两组术后1、6 及12 个月患侧比健侧上肢臂围增加的百分比比较,差异有统计学意义（P <0.05）。此外,两组12 个月时上肢淋巴水肿总发生率比较,差异有统计学意义（P <0.05）;在预防上肢淋巴水肿的效果方面,观察组的优良率高于对照组（P <0.05）。与治疗前相比,观察组的生活质量评分降低,且两组随访期间各时间点的评分比较,差异有统计学意义（P <0.05）。结论 保留上肢淋巴结的乳腺癌淋巴结清扫术可以有效地预防患者上肢淋巴水肿,改善患者的生活质量,值得在临床上推广使用。     

Risk factors for breast cancer-related upper extremity lymphedema: Is immediate autologous breast reconstruction one of them?

Breast cancer related upper extremity lymphedema (BCRL) reduces the quality of life of those who have had surgery for breast cancer. The aim of this study is to evaluate the risk factors for BCRL and determine whether immediate autologous tissue breast reconstruction is one of them. A case control study was conducted comparing patients with BCRL (n=97) to surgically treated breast cancer patients without BCRL (control, n=126). The groups were matched for age, type of breast surgery and radiation therapy. Postoperative upper extremity infection, body mass index (BMI), occupation (level of hand-use), and immediate autologous tissue breast reconstruction were investigated as a risk factor of BCRL. Mastectomy was performed on 47.6 % (n=60) and 37.2% (n=36) of patients in the control and the BCRL groups, respectively. Eight patients (13.3%) had immediate autologous tissue breast reconstruction in the control mastectomy group. Six of 36 BCRL patients (16.7%) underwent mastectomy with immediate autologous tissue breast reconstruction. There was no significant difference between groups with respect to incidence or method of immediate reconstruction (p=0.65). Patient occupation (level of hand use) was found to be positively correlated to development of BCRL (p=0.0001). Upper extremity infection rate was 22.7% in the BCRL group and 4.0% in the controls (p=0.0001). The mean BMI in the control and BCRL groups 26.8 kg/m² and 29.1kg/m², respectively (p=0.003). In conclusion, in this study characteristics positively associated with development of BCRL included occupation, infection, and increased BMI. Immediate reconstruction of the breast was not found as a risk factor for BCRL. However larger studies are needed, to further evaluate the effect of breast reconstruction on BCRL.     

宫颈癌根治术后下肢淋巴水肿患者自我护理审查指标的制定及障碍因素分析

目的 根据宫颈癌根治术后下肢淋巴水肿自我护理的最佳证据，制定审查指标，并分析障碍因素和促进因素，为促进证据的临床转化提供参考。方法 总结宫颈癌根治术后下肢淋巴水肿自我护理的21条最佳证据，在此基础上确定审查指标、审查方法，并进行障碍和促进因素分析。结果 共制定15条质量审查指标，其中6条审查指标执行率低于60%，障碍因素主要为缺乏规范化管理流程、证据应用配合度不高、健康教育落实不到位、延续护理服务有待提升、自我护理重要性认知不足等。结论 临床科室应用证据时，需要结合促进因素及障碍因素、患者意愿及偏好等有针对性地采取措施，利于最佳证据的临床实践。     

全层病变组织切除加皮肤回植在重度淋巴水肿治疗中的应用

目的探讨一种重度四肢淋巴水肿的手术治疗方法。方法对20例难治性、巨型象皮肿病人,采用病变组织切除联合创面皮肤回植方式治疗。结果畸形、巨大的患肢明显缩小,肢体功能改善,效果显著。随访6-36个月,疗效稳定。结论切除病变组织加皮肤回植治疗重型四肢淋巴水肿,疗效确切。