Bilateral axillary web syndrome in a patient with primary lymphoedema of upper limbs and non-Hodgkin lymphoma.

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.

Overcoming lymphorrhoea: a step to achieve a better life with lymphoedema.

Lymphorrhoea-otherwise known as 'wet' or 'leaky' legs-is an increasingly common condition that many community nurses will encounter in their daily practice; however, until relatively recently, evidence-based literature on its management was limited, and treatment modalities only tackled the symptoms instead of addressing the cause. In this article, Francesca Ramadan highlights the devastating impact of this condition, and the resources available to community practitioners to assist their patients to live well with lymphoedema.

Compression therapy in dermatology.

Compression therapy is a conservative therapy that can be used in many patients with dermatological conditions, especially those associated with edema. In addition to its well-established use in venous and lymphatic disorders, there is increasing evidence that compression therapy supports the healing of inflammatory dermatoses. The presence of edema, regardless of its etiology, is an indication for the use of compression therapy. Nowadays, a variety of materials and treatment options are available for compression therapy, each with their own advantages and disadvantages. Often, compression therapy with low resting pressures is sufficient for effective therapy and is better tolerated by patients. The main contraindications to compression therapy are advanced peripheral arterial disease and decompensated heart failure. Individual factors and economic considerations should be taken into account when deciding on compression therapy with the patient. Patient self-management should be encouraged whenever possible. This requires education and support tools.

Efficacy of octreotide to reduce lymphorrhea and prevent lymphocele after pelvic lymph node excision in gynecological malignancies.

AIM: To study the efficacy of octreotide to reduce lymphorrhea and prevent lymphocele after pelvic lymph node excision in gynecological malignancies. METHODS: Patients with more than 200 mL of lymph drained per day until postoperative day 3 after pelvic lymph node excision were enrolled. Of the 75 patients, 36 were managed by conservative methods without the injection of octreotide, and the other 39 patients were treated with the injection of octreotide. The treated group was injected with 0.1 mg octreotide q8h for 5 days, starting on postoperative day 3. The drainage tube was removed when the amount of drained lymph decreased to 100 mL per day. The age, BMI, operation time, removed lymph nodes, amount of lymph, duration of drain placement, proportion of patients with lymphocele and complications between these two group were compared. RESULTS: The total and mean daily amount of lymph produced per patient was significantly lower in the octreotide-treated group than in the untreated group. The duration of drain placement was shorter in the octreotide group than in the untreated group. The proportion of patients with lymphocele in the treatment group was lower than that in the untreated group. CONCLUSIONS: The injection of octreotide is effective to reduce lymphorrhea and prevent lymphocele after pelvic lymph node excision in gynecological malignancies.

Case 311: Generalized Lymphatic Anomaly.

HISTORY: A 14-year-old boy presented with asthenia, low back pain, and abdominal distention. The onset of symptoms was slow and progressive over a few months. The patient had no contributing past medical history. At physical examination, all vital signs were normal. Only pallor and positive fluid wave test results were noted; there was no lower limb edema, mucocutaneous lesions, or palpable lymph node enlargement. Laboratory work-up revealed a decreased hemoglobin concentration of 9.3 g/dL (normal range, 12-16 g/dL) and a decreased hematocrit level of 29.8% (normal range, 37%-45%), but all other laboratory values were normal. Contrast-enhanced CT of the chest, abdomen, and pelvis was performed.

Exploring disease interrelationships in patients with lymphatic disorders: A single center retrospective experience.

BACKGROUND: The lymphatic contribution to the circulation is of paramount importance in regulating fluid homeostasis, immune cell trafficking/activation and lipid metabolism. In comparison to the blood vasculature, the impact of the lymphatics has been underappreciated, both in health and disease, likely due to a less well-delineated anatomy and function. Emerging data suggest that lymphatic dysfunction can be pivotal in the initiation and development of a variety of diseases across broad organ systems. Understanding the clinical associations between lymphatic dysfunction and non-lymphatic morbidity provides valuable evidence for future investigations and may foster the discovery of novel biomarkers and therapies. METHODS: We retrospectively analysed the electronic medical records of 724 patients referred to the Stanford Center for Lymphatic and Venous Disorders. Patients with an established lymphatic diagnosis were assigned to groups of secondary lymphoedema, lipoedema or primary lymphovascular disease. Individuals found to have no lymphatic disorder were served as the non-lymphatic controls. The prevalence of comorbid conditions was enumerated. Pairwise co-occurrence pattern analyses, validated by Jaccard similarity tests, was utilised to investigate disease-disease interrelationships. RESULTS: Comorbidity analyses underscored the expected relationship between the presence of secondary lymphoedema and those diseases that damage the lymphatics. Cardiovascular conditions were common in all lymphatic subgroups. Additionally, statistically significant alteration of disease-disease interrelationships was noted in all three lymphatic categories when compared to the control population. CONCLUSIONS: The presence or absence of a lymphatic disease significantly influences disease interrelationships in the study cohorts. As a physiologic substrate, the lymphatic circulation may be an underappreciated participant in disease pathogenesis. These relationships warrant further, prospective scrutiny and study.

The Lymphatic System in the Fontan Patient-Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should Know.

The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle.1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%.2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation.3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients.

Ultrasound Monitoring of Thymus Involution in Septic Mice.

Thymus involution is characterized by a progressive regression of thymus size and contributes to immunosuppression in sepsis. High-frequency ultrasonography is a non-invasive monitoring system in multiple organs, including the thymus, in mice. However, thymus involution has not been studied using ultrasonography in septic mice. This study reports ultrasound approaches to monitoring septic thymus involution in mice. Sepsis was induced by cecum ligation and puncture (CLP). Mice were euthanized at three time points: baseline and days 3 and 10 after CLP. Thymus areas and volumes were measured using 2-D and 3-D ultrasound approaches. Thymus weights were measured ex vivo. Compared with values at baseline, both thymus area and volume decreased significantly at days 3 and 10. In addition, thymus areas and volumes correlated positively with thymus weights. In conclusion, ultrasonography provides reliable thymus measurements and is an optimal technique for monitoring thymus involution in septic mice.

[Management of patients with lymphatic diseases and lipoedema during the COVID-19 pandemic. Recommendations of the Spanish Group of Lymphology]. / La atención al paciente con patología linfática y lipedema en la pandemia COVID-19. Recomendaciones del Grupo Español de Linfología (GEL).

The COVID-19 pandemic poses a challenge to the management of non-COVID pathologies such as lymphatic diseases and lipoedema. The use of telemedicine can prevent the spread of the disease. A system is needed to help determine the clinical priority and selection of face-to-face or telemedicine options for each patient and how to carry them out during the pandemic. The Spanish Lymphology Group has drafted a consensus document with recommendations based on the literature and clinical experience, as clinical practice guidelines for the management of lymphatic abnormalities and lipoedema during the COVID-19 pandemic. These recommendations must be adapted to the characteristics of each patient, the local conditions of the centres, and the decisions of health care professionals. The document contains minimum criteria, subject to modifications according to the evolution of the pandemic, scientific knowledge and instructions from health authorities.

Axillary web syndrome in women after breast cancer surgery referred to an Oncological Rehabilitation Unit: which are the main risk factors? A retrospective case-control study.

OBJECTIVE: Axillary web syndrome (AWS) is a complication of surgical procedures in breast cancer (BC) patients. This condition with poorly understood incidence and etiology is characterized by the locoregional development of scar tissue, leading to subcutaneous cording, motion impairment and pain. The early identification of patients at risk for AWS would improve their clinical management. Here, we sought to characterize the prevalence of and the risk factors associated with AWS in BC women after surgery. PATIENTS AND METHODS: All patients with BC that underwent axillary surgery referred to an Outpatient Service for Oncological Rehabilitation were retrospectively collected. These women were assessed two weeks after the surgical procedure for their clinicopathologic features, type of therapeutic interventions, and AWS presence, laterality, pain, localization, cords type, and number of cords. RESULTS: Altogether, 177 patients (mean aged 60.65 ± 12.26 years) were included and divided into two groups: AWSPOS (n=52; 29.4%) and AWSNEG (n=125; 70.6%). Patients with tumor N ≥1 (OR=3.7; p<0.001), subjected to mastectomy, axillary lymph node dissection (ALND) and chemotherapy showed significant correlations with AWS onset (p<0.05). The range of shoulder motion limitation (OR=11.2; p<0.001) and the presence of breast cancer related lymphedema (OR=3.5; p=0.020) were associated with AWS. CONCLUSIONS: Mastectomy, ALND, chemotherapy, low staging tumors, shoulder range of motion limitations, and BCRL represent risk factors for AWS onset. Realizing new strategies for assessing the individual risk of AWS is a crucial clinical need to improve the health-related quality of life of BC survivors.

Linfadenopatía proteinácea asociada a rotura esplénica / Proteinaceous lymphadenopathy associated with splenic rupture

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Single Gland, Ectopic Location: Adenomas are Common Causes of Primary Hyperparathyroidism in Children and Adolescents.

BACKGROUND: Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Data-driven guidelines for management in pediatric patients are limited. METHODS: We performed a retrospective cohort analysis of all patients (1997-2017) with PHPT ≤ 21 years of age who underwent parathyroidectomy at three institutions. Clinical and demographic variables were analyzed. Primary operative outcome was cure (normocalcemia > 6 months after surgery); secondary outcome was operative success (intraoperative parathyroid hormone decrease of ≥ 50%). RESULTS: We identified 86 patients with a median age of 17 years (IQR: 14, 19); 64% (n = 55) were female. The mean preoperative serum calcium was 11.7 mg/dL, median parathyroid hormone (PTH) was 110 pg/mL, and median urine calcium was 4.1 mg/kg/24 h. Preoperatively, sestamibi scan localized in 41/71 patients (58%); neck ultrasound localized in 19/44 (43%). The most common pathology at surgery was a single ectopic parathyroid adenoma in 71% (n = 61). A high incidence of ectopic adenomas (25%, n = 22) was observed, most commonly intrathymic (n = 13), followed by tracheoesophageal groove (n = 5), carotid sheath (n = 2), and intrathyroidal (n = 2). Of 56 patients with retrievable data > 6 months postoperatively, cure was achieved in 55 of 56 patients (98%). One patient who presented to us with parathyromatosis require subsequent reoperation. CONCLUSION: In this multi-institutional series of PHPT in children and adolescents, the majority were sporadic PHPT and were due to a single adenoma. We observed a high incidence of ectopic parathyroid adenomas, most commonly intrathymic. Given the high risk for ectopic adenoma in pediatric patients, parathyroid surgery in children and adolescents should be performed by experienced surgeons.

[Immune-related adverse events after immune checkpoints inhibitors in 2019: An update]. / Toxicités immunologiques induites par les inhibiteurs de checkpoint en 2019 : mise au point.

Use of checkpoint inhibitors to treat cancer was one of the most important revolution these last years and an increasing number of new types of tumors is currently under investigation with these new treatments. However, immune-related adverse events associated with these agents frequently affect various organs, mimicking auto-immune or inflammatory diseases. Some of these effects can be severe, often requiring hospitalization and specialized treatment (immunosuppression). Most known agents are ipilimumab (anti-CTLA-4 antibody) nivolumab and pembrolizumab (anti-PD-1 antibodies). New molecules are now approved or in development as anti-PD-L1 antibodies, anti-LAG-3 or anti-TIM-3 antibodies, increasing the probability and new description of immune-related adverse events. With his experience in auto-immune diseases, the immunologist/internal medicine specialist has an important role in the management of these toxicities. The goal of this review is to focus on the incidence, diagnostic assessment and recommended management of the most relevant immune-related adverse events.

[Clinical characteristics of Kaposiform lymphangiomatosis: a report of 8 cases].

Objective: To investigate the clinical symptoms, treatment intervention and prognosis of Kaposiform lymphangiomatosis(KLA). Methods: Medical information and clinical characteristics data of 8 KLA patients who were admitted to Department of Pediatric Surgery of West China Hospital of Sichuan University from January 2016 to February 2019 were retrospectively reviewed and analyzed. There were 5 males and 3 females with age of 5.8 years old (from 8 months to 29 years old). Results: The lesions in all patients were diffusely distributed. In all 8 patients, the lung and mediastinum were involved with different degrees. Three cases had lesions involving pelvic and abdominal organs. Three cases had lesions involving bones. One case simultaneously involved pelvic and abdominal organs, and 1 case was involved laryngeal and neck. The clinical characteristics were mainly respiratory symptoms. In the laboratory tests, 6 patients had different degrees of thrombocytopenia (minimum 3 × 10(9)/L), and 4 patients had severe fibrinogen reduction (minimum 0.42 g/L). Three patients had prolonged activated partial thromboplastin time (up to 64.2 seconds) and 3 patients had prolonged prothrombin time (up to 18.6 seconds). After surgery (including thoracotomy, chest tube, pericardiocentesis, splenectomy) and empiric medicine therapy (vincristine, sirolimus and corticosteroid), the symptoms improved in 1 case, 2 cases died of complications, 2 cases were stable and 3 cases progressed up to February 2019. Conclusions: KLA is a rare disease that should be differentiated from other types of vascular diseases. Currently, there is no consensus treatment guidelines exist. Accurate diagnosis in KLA can be a challenge. The situation in patients with KLA is prone to rapid deterioration and progress. Future research efforts should seek to develop target-specific drugs for KLA.

Role of the lymphatic vasculature in cardiovascular medicine.

The lymphatic vasculature has traditionally been considered important for removal of excessive fluid from the interstitial space, absorption of fat from the intestine and the immune system. Advances in molecular medicine and imaging have provided us with new tools to study the lymphatics. This has revealed that the vessels are actively involved in regulation of immune cell trafficking and inflammation. We now know much about how new lymphatic vessels are created (lymphangiogenesis) and that this is important in, for example, wound healing and tissue repair. The best characterised pathway for lymphangiogenesis is the vascular endothelial growth factor C (VEGF-C)/VEGFR3 pathway. Over recent years, there has been an increasing interest in the role of the lymphatics in cardiovascular medicine. Preclinical studies have shown that lymphangiogenesis and immune cell trafficking play a role in cardiovascular conditions such as atherosclerosis, recovery after myocardial infarction and rejection of cardiac allografts. Targeting the VEGF-C/VEGFR3 pathway can be beneficial in these conditions. The clinical spectrum of lymphatic abnormalities and lymphoedema is wide and overlaps with congenital heart disease. Important long-term complications to the Fontan circulation involves the lymphatics. New and improved imaging modalities has improved our understanding and management of these patients. Lymphatic leaks and flow abnormalities can be successfully treated, minimally invasively, with percutaneous embolisation. Future research will prove if the preclinical findings that point to a role of the lymphatics in several cardiovascular conditions will result in new treatment options.