{"title":"Chylous ascites in the neonate: A narrative review.","authors":"G Rocha","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Chylous ascites (CA), also called chyloperitoneum, is a rare form of ascites in the neonate. It results from the leakage of lymph into the peritoneal cavity. There are congenital and acquired forms of CA. CA may occur during fetal life, and the prognosis will depend on its volume, gestational age at the onset, and the association with other anomalies. Lymphangiectasia is the most common congenital cause, and acquired forms are mainly traumatic and/or post-operative. This review aims to gather the most current information on CA and addresses important aspects regarding etiology, pathophysiology, clinic, diagnostic tools, and treatment.</p>","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 3","pages":"117-128"},"PeriodicalIF":2.5,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10536182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Centrifugal Versus Centripetal Origin(s) of the Lymphatic System: Controversy (Mostly) Resolved?","authors":"M H Witte, R P Erickson","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>New findings reopen the controversy about centrifugal vs. centripetal origin of the lymphatic system and support that the latter may be the predominant source of lymphatic endothelial cells from mesenchymal lymphangioblasts.</p>","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"55 4","pages":"141-143"},"PeriodicalIF":2.5,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9960176","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-11-18DOI: 10.2458/lymph.4728
A. Tuğral, U. Eliiyi, K. Özdemir, G. Ergin, Y. Bakar
{"title":"A new era of seeking knowledge for #lymphedema on social media: A detailed Instagram hashtag analysis.","authors":"A. Tuğral, U. Eliiyi, K. Özdemir, G. Ergin, Y. Bakar","doi":"10.2458/lymph.4728","DOIUrl":"https://doi.org/10.2458/lymph.4728","url":null,"abstract":"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.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 2 1","pages":"68-77"},"PeriodicalIF":2.5,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46393821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-11-18DOI: 10.2458/lymph.4730
N. Sampathirao, M. Indirani, G. Manokaran, A. Jaykanth, A. Patel, S. Simon
{"title":"Assessment of lymphedema with lymphoscintigraphy: Can nodal quantification help?","authors":"N. Sampathirao, M. Indirani, G. Manokaran, A. Jaykanth, A. Patel, S. Simon","doi":"10.2458/lymph.4730","DOIUrl":"https://doi.org/10.2458/lymph.4730","url":null,"abstract":"Lymphoscintigraphy with combined qualitative and quantitative analysis is reported to be a more sensitive approach to diagnose lymphedema in comparison with the conventional clinical analysis. Our study seeks to evaluate the diagnostic performance of lower limb lymphoscintigraphy with amalgamation of qualitative and quantitative analysis by measuring the ilio-inguinal nodal uptake. This prospective observational study was comprised of 86 patients (172 limbs) diagnosed with lower limb lymphedema. After a thorough clinical grading of edema, radionuclide lymphoscintigraphy was performed as per a dedicated institutional protocol. Ilio-inguinal nodal quantification of tracer uptake was computed along with the visual study of the scans. Additionally, the corresponding mean nodal uptake percentage for each grade of lymphedema was assessed and a cut off nodal uptake percentage to differentiate between normal and abnormal limbs was defined. Although quantitative analysis with nodal uptake percentage provides objective criteria to diagnose lymphedema, it can only act as an adjunct to qualitative method without replacing it. Finally, standardization of procedure for quantitative lymphoscintigraphy is needed including the potential for combining both rate of clearance of tracer from injection site and nodal uptake for quantification.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 2 1","pages":"92-105"},"PeriodicalIF":2.5,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47944481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-11-18DOI: 10.2458/lymph.4729
D. Mustacich, R. Kylat, M. Bernas, R. Myles, J. A. Jones, J. Kanady, A. Simon, T. Georgieva, M. Witte, R. Erickson, P. Pires
{"title":"Abnormal lymphatic phenotype in a CRISPR mouse model of the human lymphedema-causing Connexin47 R260C point mutation.","authors":"D. Mustacich, R. Kylat, M. Bernas, R. Myles, J. A. Jones, J. Kanady, A. Simon, T. Georgieva, M. Witte, R. Erickson, P. Pires","doi":"10.2458/lymph.4729","DOIUrl":"https://doi.org/10.2458/lymph.4729","url":null,"abstract":"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.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 2 1","pages":"78-91"},"PeriodicalIF":2.5,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49259153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-11-18DOI: 10.2458/lymph.4727
F. Khorshidi, B. Majdalany, G. Peters, A. Tran, J. Shaikh, R. Liddell, J. C. Perez Lozada, N. Kokabi, N. Nezami
{"title":"Minimally invasive treatment of abdominal lymphocele: A review of contemporary options and how to approach them.","authors":"F. Khorshidi, B. Majdalany, G. Peters, A. Tran, J. Shaikh, R. Liddell, J. C. Perez Lozada, N. Kokabi, N. Nezami","doi":"10.2458/lymph.4727","DOIUrl":"https://doi.org/10.2458/lymph.4727","url":null,"abstract":"Lymphoceles are lymphatic fluid collections resulting from lymphatic vessel disruption after surgery or trauma. They are most often described following retroperitoneal surgeries such as cystectomies, prostatectomies, renal transplants, and gynecologic surgeries. Most lymphoceles are asymptomatic and resolve spontaneously without treatment. If persistent, they can become infected or exert mass effect on adjacent structures causing pain, urinary, or lower limb edema particularly for lymphoceles in the pelvis Symptomatic lymphoceles should be treated to relieve symptoms and prevent functional compromise of vital adjacent structures. Although surgery has been traditionally accepted as the gold standard treatment, advances in imaging and interventional technology allow for less invasive, percutaneous treatment. Available minimally invasive treatment options include percutaneous aspiration, catheter drainage, sclerotherapy, and lymphangiography with lymphatic embolization. A review of these treatment options and a suggested algorithm for managing lymphoceles is presented.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 2 1","pages":"56-67"},"PeriodicalIF":2.5,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45339100","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-11-18DOI: 10.2458/lymph.4731
S. Salvia, M. Amore, C. Papendieck
{"title":"Topical Tacrolimus 0.1% for treatment of cutaneous microcystic lymphatic malformations.","authors":"S. Salvia, M. Amore, C. Papendieck","doi":"10.2458/lymph.4731","DOIUrl":"https://doi.org/10.2458/lymph.4731","url":null,"abstract":"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.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 2 1","pages":"106-111"},"PeriodicalIF":2.5,"publicationDate":"2021-11-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49096613","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-09-07DOI: 10.2458/lymph.4679
T. Cox, C. Vance, S. Daley, C. Papendieck, H. McGregor, P. Kuo, M. Witte
{"title":"Imaging of lymphatic dysplasia in Noonan syndrome: Case studies and historical atlas.","authors":"T. Cox, C. Vance, S. Daley, C. Papendieck, H. McGregor, P. Kuo, M. Witte","doi":"10.2458/lymph.4679","DOIUrl":"https://doi.org/10.2458/lymph.4679","url":null,"abstract":"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.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 1 1","pages":"23-40"},"PeriodicalIF":2.5,"publicationDate":"2021-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"49128894","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
LymphologyPub Date : 2021-09-07DOI: 10.2458/lymph.4677
T. Gillespie, S. Roberts, C. Brunelle, L. Bucci, M. Bernstein, K. Daniell, G. N. Naoum, C. Miller, A. Taghian
{"title":"Comparison of perometry-based volumetric arm measurements and bioimpedance spectroscopy for early identification of lymphedema in a prospectively-screened cohort of breast cancer patients.","authors":"T. Gillespie, S. Roberts, C. Brunelle, L. Bucci, M. Bernstein, K. Daniell, G. N. Naoum, C. Miller, A. Taghian","doi":"10.2458/lymph.4677","DOIUrl":"https://doi.org/10.2458/lymph.4677","url":null,"abstract":"Breast cancer-related lymphedema (BCRL) affects more than one in five women treated for breast cancer, and women remain at lifelong risk. Screening for BCRL is recommended by several national and international organizations for women at risk of BCRL, and multiple methods of objective screening measurement exist. The goal of this study was to compare the use of perometry and bioimpedance spectroscopy (BIS) for early identification of BCRL in a cohort of 138 prospectivelyscreened patients. At each screening visit, a patient's relative volume change (RVC) from perometer measurements and change in L-Dex from baseline (ΔL-Dex) using BIS was calculated. There was a negligible correlation between RVC and ΔL-Dex (r=0.195). Multiple thresholds of BCRL were examined: RVC ≥5% and ≥10% as well as and ΔL-Dex ≥6.5 and ≥10. While some patients developed an elevated RVC and ΔL-Dex, many demonstrated elevations in only one threshold category. Moreover, the majority of patients with RVC ≥5%, ΔL-Dex ≥6.5, or ΔL-Dex ≥10 regressed to non-elevated measurements without intervention. These findings suggest a role for combining multiple screening methods for early identification of BCRL; furthermore, BCRL diagnosis must incorporate patient symptoms and clinical evaluation with objective measurements obtained from techniques such as perometry and bioimpedance spectroscopy.","PeriodicalId":51122,"journal":{"name":"Lymphology","volume":"54 1 1","pages":"1-11"},"PeriodicalIF":2.5,"publicationDate":"2021-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42325898","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}