The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema.

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently. The term primary lymphoedema covers a group of rare conditions caused by abnormal functioning and/or development of the lymphatic system. It covers a highly heterogeneous group of conditions. An accurate diagnosis of primary lymphoedema is crucial for the implementation of an optimal treatment plan and management, as well as to reduce the risk of worsening. Patient care is diverse across Europe, and national specialised centres and networks are not available everywhere. The European Reference Network on Rare Multisystemic Vascular Diseases (VASCERN) gathers the best expertise in Europe and provide accessible cross-border healthcare to patients with rare vascular diseases. There are six different working groups in VASCERN, which focus on arterial diseases, hereditary haemorrhagic telangiectasia, neurovascular diseases, lymphoedema and vascular anomalies. The working group Paediatric and Primary Lymphedema (PPL WG) gathers and shares knowledge and expertise in the diagnosis and management of adults and children with primary and paediatric lymphoedema. The members of PPL WG have worked together to produce this opinion statement reflecting strategies on how to approach patients with primary and paediatric lymphoedema. The objective of this patient pathway is to improve patient care by reducing the time to diagnosis, define the best management and follow-up strategies and avoid overuse of resources. Therefore, the patient pathway describes the clinical evaluation and investigations that lead to a clinical diagnosis, the genetic testing, differential diagnosis, the management and treatment options and the patient follow up at expert and local centres. Also, the importance of the patient group participation in the PPL WG is discussed.

Wounds in chronic leg oedema.

Wounds and chronic oedema are common disorders, but rarely studied together. The objective of this cross-sectional study was to investigate the point-prevalence and risk factors of wounds on the leg, in chronic leg oedema. Forty sites in nine countries were included. Of 7077 patients with chronic leg oedema, 12.70% had wounds. Independent risk factors were: peripheral arterial disease (odds ratio (OR) 4.87, 95% confidence intervals (CI) 3.63-6.52), cellulitis within the past 12 months (OR 2.69, 95% CI 2.25-3.21), secondary lymphoedema (OR 2.64, 95% CI 1.93-3.60), being male (OR 2.08, 95% CI 1.78-2.44), being over 85 years of age (OR 1.80, 95% CI 1.23-2.62), underweight (OR 1.79, 95% CI 1.14-2.79), bed bound (OR 1.79, 95% CI 1.01-3.16), chair bound (OR 1.52, 95% CI 1.18-1.97), diabetes (OR 1.47, 95% CI 1.23-1.77), and walking with aid (OR 1·41, 95% CI 1.17-1.69). 43.22% of those with wounds had clinically defined well-controlled oedema, associated with a significantly lower risk of wounds (OR 0.50, 95% CI 0.42-0.58, P < .001). Hard/fibrotic tissue (OR 1.71, 95% CI 1.19-2.48), and a positive Stemmers sign (OR 1.57, 95% CI 1.05-2.35) were associated with wounds. The study reinforces the importance of measures to control oedema, as controlled swelling was associated with a 50% lower risk of wounds.

Inter-observer and Intra-observer Variability in Volume Measurements of the Lower Extremity Using Perometer.

Background: The swelling of the extremities seen in lymphedema can be measured with many different volumetric devices; however, many methods lack important characteristics including reproducibility and independence from the subjectivity and skill of the operator. The aim of this study was to validate the use of the Perometer® as a possible standard for volumetric measurement methods based on the inter-observer and intra-observer variability when using a standard method of Perometry®. Methods and Results: Volumetric measurements were performed on 10 healthy test subjects by 5 individuals (the observers) who had been instructed in the measurement techniques to be used. The inter-observer variability was assessed by having the five observers measure all the test subjects both in the morning and in the early afternoon. The intra-observer variability was examined by having each observer measure all the 10 test subjects 4 times in a row in the aforementioned time frames. A data set was created using the measurements, allowing for the assessment of other parameters including variation of volume between the right and left leg and daily variation in swelling. Statistical measurements were performed using the Statistical Package for the Social Sciences (SPSS), from which it was determined that there was no statistically significant inter-observer (p-value 0.997) and intra-observer variation (p-value 0.995) based on a significance level of >5%. Furthermore, it was observed that a statistically significant difference in volume occurred in the leg volume during the day. Conclusion: It was concluded that the use of the Perometer provides consistent measurements of volume independent of the observer and therefore appears to provide a candidate standard for volumetric measurements.

LIMPRINT: Prevalence of Chronic Edema in Health Services in Copenhagen, Denmark.

Background: The International Lymphedema Framework developed an international study, Lymphedema Impact and Prevalence International (LIMPRINT), to estimate the prevalence and impact of chronic edema (CO) in heterogeneous populations. Methods and Results: A validation study using the LIMPRINT methodology was undertaken in Denmark. Participants with CO were identified from inpatient services and compared with those identified within a specialist lymphedema service and three primary care settings. Of 452 inpatients available for screening, CO was present in 177 (39%) and absent in 275 (61%). In addition, 723 participants were found from specialist and primary care services (LPCSs). Inpatients were significantly older and more likely to be underweight or normal weight. They were more likely to suffer from heart failure/ischaemic heart disease (44.6% vs. 23.4%, p < 0.001) and have neurological problems (18.1% vs. 10.9% p = 0.009). Patients in the inpatient group were nearly all suffering from secondary lymphedema and were less likely to have a cancer or venous diagnosis, but more likely to have immobility as the cause of CO (44.0% vs. 17.7%, p < 0.001). No inpatients had midline CO compared with 30 within LPCSs. Fewer in the inpatient group had standard CO treatment (17.1% vs. 73.5%, p < 0.001) and subjective control of swelling was worse (19.9% vs. 66.7%, p < 0.001). While the inpatient group experienced fewer acute infections, when they did so, they were more likely to be admitted to hospital for this (78.6% vs. 51.0%, p = 0.049). Conclusion: The prevalence of CO in inpatient facilities is high and those with CO have multiple comorbidities that vary according to setting. The feasibility study showed that the methodology could be adapted for use in different health systems.

Validity and interobserver agreement of lower extremity local tissue water measurements in healthy women using tissue dielectric constant.

BACKGROUND: Tissue dielectric constant (TDC) measurement may become an important tool in the clinical evaluation of chronic lower extremity swelling in women; however, several factors are known to influence TDC measurements, and comparative data on healthy lower extremities are few. METHODS: Thirty-four healthy women volunteered. Age, BMI, moisturizer use and hair removal were registered. Three blinded investigators performed TDC measurements in a randomized sequence on clearly marked locations on the foot, the ankle and the lower leg. The effective measuring depth was 2.5 mm. RESULTS: The mean TDC was 37.8 ± 5.5 (mean ± SD) on the foot, 29.0 ± 3.1 on the ankle and 30.5 ± 3.9 on the lower leg. TDC was highly dependent on measuring site (P<0.001) but did not vary significantly between investigators (P=0.127). Neither age, BMI, hair removal nor moisturizer use had any significant effect on the lower leg TDC. Intraclass correlation coefficients were 0.77 for the foot, 0.94 for the ankle and 0.94 for the lower leg. CONCLUSION: The TDC on the foot was significantly higher compared with ankle and lower leg values. Foot measurements should be interpreted cautiously because of questionable interobserver agreement. The interobserver agreement was high on lower leg and ankle measurements. Neither age, BMI, hair removal nor moisturizer use had any significant on effect on the lower leg TDC. TDC values of 35.2 for the ankle and 38.3 for the lower leg are suggested as upper normal reference limits in women.