Lymphology最新文献

Microcystic lymphatic malformations as described in the international literature form a subgroup of low-flow congenital vascular malformations (VM) resulting from irregular embryological development. Microcystic lesions normally manifest as an accumulation of lymph- and blood-filled vesicles that, when externalized, cause skin maceration with consequent pain and potential infection resulting in the impairment of the patient's quality of life. There is no consensus on a standardized algorithm nor clear guidelines for successful treatment of this type of lymphatic malformation, and treatment options employed often result in ambivalent and transient outcomes with a high rate of recurrence. The topical formulation of tacrolimus is a well-known FDAapproved anti-T cell agent that was recently identified as a potent activator of ALK1, which is involved in several processes and functions including angiogenesis. We investigated if topical administration of tacrolimus may be an effective therapy for directly targeting cutaneous microcystic lymphatic malformations as a complement to systemic treatment. The study enrolled four patients with cutaneous microcystic lymphatic malformations: three male (ages: 13,15,18) and one female (age: 30). Two of the patients presented lesions on their backs, one patient on the left hand and one on the left lower limb. All four patients received treatment with topical tacrolimus 0.1% twice a day for 10 weeks on a previously selected area for application. Weekly clinical follow-ups were conducted along with close physician-patient contact. All patients displayed a satisfactory response after treatment. Lymphorrhea and bleeding were stopped in all cases and the esthetic aspect of lesions improved in two patients. To date, all patients presented no clinically significant changes to the size or extension of the lesion. Topical tacrolimus treatment is a promising and reasonable option for microcystic lymphatic malformations. Our results encourage further exploration in larger populations with the consideration that it is a safe and effective alternative or complementary therapy to systemic treatment.

Patients treated for breast cancer are at risk of developing breast cancer-related lymphedema (BCRL). A significant proportion of patients treated for breast cancer are opting to undergo a contralateral prophylactic mastectomy (CPM). Currently, it remains unclear as to whether the relative volume change (RVC) equation may be used as an alternative to the weight adjusted change (WAC) equation to quantify BCRL in patients who undergo CPM. In order to simplify BCRL screening, our cohort of patients who underwent a CPM (n=310) was matched by BMI to a subset of patients who underwent unilateral breast surgery (n=310). Arm volume measurements were obtained via an optoelectronic perometer preoperatively, postoperatively, and in the follow-up setting every 6-12 months. The correlation of ipsilateral RVC and WAC values for those who underwent bilateral surgery was calculated (r=0.60). Contralateral WAC values for patients in both cohorts were compared, and there was no significant difference between the two distributions in variance (p=0.446). The RVC equation shows potential to be used to quantify ipsilateral postoperative arm volume changes for patients who undergo a CPM. However, a larger trial in which RVC and WAC values are prospectively assessed is needed.

To determine the historical use and utility of various lymphatic imaging modalities in Noonan syndrome (NS) patients, we performed a comprehensive literature review by collecting the published medical imaging of NS lymphatic dysplasias. We correlated imaging findings with clinical phenotypes and treatment. Our analysis of lymphatic imaging modalities provides an algorithmic approach to imaging and patient care across the spectrum of NS developmental defects. A total of 54 NS cases have been published since 1975. Using the observations reported in 15 reviewed publications, an association was made between disruptions in central lymphatic flow and poor clinical presentations/outcomes in NS patients.

Secondary lower extremity lymphedema is a common complication of treatment for gynecological cancers. Conservative therapy plays an important role in the treatment of patients with secondary lower extremity lymphedema; in particular, complex decongestive therapy (CDT) has been recognized as an effective nonoperative technique for these patients. But CDT therapy for secondary lower extremity lymphedema remains a problem in China because this technique and its effectiveness have not achieved widespread use and popularity. Our goal was to assess effects of CDT in patients with secondary lower limb lymphedema after treatment for gynecological cancers. The retrospective study consisted of 60 patients who were treated with 20 sessions of CDT. Assessments included objective changes in limb circumference, degree of LE, imaging features, and incidence of erysipelas before and after CDT treatment. We found that CDT can effectively improve lymph stasis and promote backflow, and decrease circumference, interstitial fluid content, and incidence of erysipelas of lymphedematous lower limb. Our results demonstrate that CDT is an effective treatment method for patients with secondary lower limb lymphedema following treatment for gynecologic cancers. This technique should be more widely utilized and popularized in China to improve the quality of life of millions of patients with secondary lower limb lymphedema.

We have created a human chromosomal map of the location of known and candidate genes involved in primary lymphedema (PLE). This should facilitate further discovery and provide a basis for understanding microdeletions which cause lymphedema.

Gorham-Stout Disease (GSD), also named vanishing bone disease, is an ultrarare condition characterized by progressive osteolysis with intraosseous lymphatic vessel proliferation and bone cortical loss. So far, about 300 cases have been reported. It may occur at any age but more commonly affects children and young adults. The aim of this study is to retrospectively review our internal patient series and to hypothesize a diagnostic-therapeutic protocol for earlier diagnosis and treatment. Clinical datasets from our center were examined to identify all GSD patients for collection and analysis. We identified 9 pediatric cases and performed a retrospective case-series review to examine and document both diagnosis and treatment. We found that delay in diagnosis after first symptoms played a critical role in determining morbidity and that multidisciplinary care is key for proper diagnosis and treatment. Our study provides additional insight to improve the critical challenge of early diagnosis and highlights a multidisciplinary treatment approach for the most appropriate management of patients with rare GSD disease. Although GSD is an ultrarare disease, physicians should keep in mind the main clinical features since neglected cases may result in potentially fatal complications.

Surgical intervention and subsequent wound healing process are known to induce neo-lymphangiogenesis, but few studies have been reported to utilize this mechanism for lymphedema treatment. The aim of this study was to evaluate feasibility of subdermal dissection for neo-lymphangiogenesis induction (SDN) to treat lower extremity lymphedema (LEL). Medical records of secondary LEL patients who had undergone ICG lymphography and SDN procedure were reviewed. SDN was performed by dissecting fat tissues just below the dermis from the most proximal area showing dermal backflow through abdominal-toaxillary lymphatic pathways. Perioperative lymphedematous conditions were evaluated with lymphedema quality of life score (LeQOLiS) and LEL index. Seventeen female patients were included. SDN could be performed in 10 minutes on average without postoperative complication. Postoperative ICG lymphography showed new lymphatic pathways in 6 (35.3%) cases. Postoperative LeQOLiS ranged from 9 to 66, which was statistically lower than preoperative LeQOLiS (32.9 ± 19.2 vs. 36.6 ± 19.3, p = 0.048), whereas there was no statistically significant difference between pre- and post-operative LEL index (275.2 ± 23.3 vs. 270.5 ± 20.8, P = 0.073). Subdermal dissection, although its probability is not high, has a potential to induce neo-lymphangiogenesis. Further studies are required to improve and demonstrate efficacy of the procedure for new lymphatic pathway creation.

We designed a study to compare effects of complete decongestive therapy (CDT) and kinesiology taping (KT) (with exercise and skin care) on limb circumference, lymphedema volume, grip strength, functional status, and quality of life in patients with unilateral breast cancer-related lymphedema (BCRL). Forty patients with unilateral stage 2 BCRL were randomized to either the CDT group (n=20) or the KT group (n=20). Patients in the CDT group underwent 30-min manual lymphatic drainage (MLD) and multi-layer, short-stretch bandaging once a week for four weeks. Patients in the KT group underwent taping once a week for four weeks. In addition, all patients were informed about skin care and given an exercise program throughout the treatment. Upper extremity circumference and volume differences as primary outcomes and grip strength, Quick-Disabilities of the Arm, Shoulder and Hand (Q-DASH), and Functional Assessment of Cancer Therapy-Breast (FACT-B) scores as secondary outcomes were assessed initially, after treatment (4 weeks), and at the 1st month follow-up. Limb circumference and volume differences were significantly reduced in the CDT group after the 4-week treatment compared with the KT group (p=0.012 and p=0.015, respectively), but there was no difference between the groups in the 1st month follow-up (p>0.05). There was no difference between the groups in terms of grip strength, Q-DASH, and FACT-B scores after treatment and at the 1st month follow-up (p>0.05). Our results show that both KT and CDT were found to significantly reduce limb volume and circumference individually at 4-weeks and the one-month follow-up in patients with BCRL and that CDT significantly reduced both limb volume and circumference compared to KT at the 4- week time point, but not at the follow-up. Further randomized controlled trials with patients at different stages of BCRL are needed to confirm and expand these results.

Instagram® is one of the most active social media platforms with over a billion users worldwide. Since the importance of education on lymphedema has been established due to the chronic nature of the disease, seeking knowledge attracts much attention not only clinically but also on social platforms such as Instagram ®. Our aim was to examine content by analyzing posts tagged with hashtags on Instagram ® related to lymphedema. Nine predefined hashtags related to lymphedema were used to search posts uploaded to Instagram® via the Apify tool. Retrieved public posts were classified and analyzed by four researchers for their content and post-type. We found that the vast majority of sharing on Instagram® in the context of lymphedema and its related aspects have relatively low scores for both relevancy and accuracy with a 77% irrelevancy rate. The best posts were those determined to be educational, which were found 57% relevant and correct. Medical professionals should consider that disseminating true guidance and therapy carries importance for patients with lymphedema and treatment success. The ability for patients to reach knowledge via social media might also be an important aspect in reliving suffering due to lymphedema. However, our results demonstrate that Instagram® might not be a good platform for patients to discover reliable information about lymphedema.

Connexin proteins form gap junctions controlling exchange of ions and small molecules between cells and play an important role in movement of lymph within lymphatic vessels. Connexin47 (CX47) is highly expressed in lymphatic endothelial cells and CX47 missense mutations, i.e., R260C, cosegregate with primary lymphedema in humans. However, studies utilizing CX47 knockout mice have failed to demonstrate any lymphatic anomalies. To unravel the lymphatic consequences of expressing a mutant CX47 protein, we used CRISPR technology to create a mouse carrying a Cx47 missense mutation (Cx47R259C) equivalent to the human CX47R260C missense mutation associated with human primary lymphedema. Intradermal Evans Blue dye injection identified a 2-fold increase in regional lymph nodes in homozygous Cx47R259C mice compared to wildtype, particularly in the jugular region (4.8 ± 0.4 and 2.0 ± 0.0, respectively, p<0.01). Associated lymphatic channels were increased in Cx47R259C mice and mesenteric lymph reflux occurred in homozygous Cx47R259C mice but not in wildtype. Contractility of superficial cervical lymphatics, assessed by pressure myography, was reduced in homozygous Cx47R259C mice compared to wildtype. In conclusion, our data are the first to demonstrate a role for the Cx47 protein in lymphatic anatomy and function. This phenotype is similar to that found with other valve deficient mouse mutants, e.g., in Foxc2. Of significance, this study is the first to use CRISPR technology to develop a pre-clinical model of primary lymphedema and demonstrates the importance of distinguishing between lack of and presence of mutant protein when developing clinically relevant animal models for translation of pre-clinical findings.