Primary Lower Extremity Lymphedema Research Articles

The value of the dermal rim sign on nonenhanced magnetic resonance imaging for predicting dermal backflow in patients with primary lower extremity lymphedema

PurposeThe dermal rim sign (DRS) on nonenhanced magnetic resonance imaging has been shown to predict dermal backflow (DBF) in patients with secondary upper limb lymphedema. However, whether the DRS has the same effects on primary lower extremity lymphedema (PLEL) has not been clearly reported. Therefore, this study aimed to explore whether the DRS can be used to diagnose DBF on lymphoscintigraphy in patients with PLEL. MethodsA total of 94 patients who were diagnosed with PLEL were recruited for this retrospective study from January 2022 to December 2023. All the patients were divided into two groups according to the lymphoscintigraphy findings: no DBF and DBF. The magnetic resonance imaging data of the two groups were recorded and statistically compared for the following indicators: range of lymphedema involvement (left, right, whole lower limbs, only thigh, only calf and ankle), signs of lymphedema (notable thickening of skin, parallel line sign, grid sign, honeycomb sign, band sign, lymph lake sign, crescent sign, DRS), and lymphedema measurement (skin thickness, band width). The DRS is characterized by notable thickening of the skin plus the grid sign and/or honeycomb sign, plus the band sign. ResultsThe following statistically significant differences in the following indicators were found between the two groups (P < .05): notable skin thickening, parallel line sign, grid sign, honeycomb sign, band sign, DRS, skin thickness, and band width. The sensitivity, specificity, and accuracy for predicting for DBF with the DRS was 82%, 64%, and 77%, respectively. ConclusionsThis study confirmed good consistency between the DRS and DBF from the perspective of imaging. This tool is suitable for children, adolescents, and patients with contraindications to lymphoscintigraphy. The DRS has important value in assessing the severity of PLEL. The DRS is suggested for the clinical use of combined surgical treatment of PLEL.

Quantitative evaluation of primary lower extremity lymphedema staging using MRI: a preliminary study.

The staging of primary lower extremity lymphedema (LEL) is difficult yet vital in clinical work, and magnetic resonance imaging (MRI) can be used for quantitative assessment of primary LEL due to its high resolution for soft tissues. In this study, we evaluated the value of MRI-based soft tissue area measurements for staging primary LEL. A total of 90 consecutive patients with clinically diagnosed primary lower limb lymphoedema from January 2017 to December 2019 in Beijing Shijitan Hospital were enrolled retrospectively. Short time inversion recovery (STIR) sequence was applied to measure the total, muscle, bone, and subcutaneous areas in the upper 1/3 level of the bilateral lower calf. The difference between the affected and unaffected calf regarding the subcutaneous area was obtained, and (subcutaneous area)/(bone area) and (subcutaneous area)/(muscle area) were calculated. According to the International Society of Lymphology (ISL) clinical staging standard established in 2020, all patients were divided into stages I, II, and III, accordingly. Statistical analysis was performed to determine the validity of MRI measurements in staging LEL. There were 33 patients classified as stage I clinically, 44 patients as stage II, and 13 patients as stage III. There were significant differences in total, subcutaneous, the difference in subcutaneous area of limbs, subcutaneous/bone (S/B), and subcutaneous/muscle (S/M) between stage I and II as well as between stage I and III (P<0.001), but not between stage II and III (P=0.706, 0.329, and 0.229, respectively). A positive correlation was detected between the clinical stage and difference in subcutaneous area of limbs (rho =0.752, P<0.001), S/B (rho =0.747, P<0.001), S/M (rho =0.709, P<0.001), and subcutaneous (rho =0.723, P<0.001). For staging primary LEL, receiver operating characteristic (ROC) curves indicated that the difference in subcutaneous area of limbs had the best discrimination ability among parameters [area under the ROC curve (AUC) =0.950; 95% confidence interval (CI): 0.875-0.987; sensitivity: 95.45%; specificity: 84.85%], followed by S/B (AUC =0.930; 95% CI: 0.848-0.975; sensitivity: 77.27%; specificity: 93.94%) and S/M (AUC =0.895; 95% CI: 0.804-0.953; sensitivity: 77.27%; specificity: 90.91%). The ROC curves indicated that subcutaneous area (AUC =0.927; 95% CI: 0.844-0.974; sensitivity: 84.09%, specificity: 90.91%) and total (AUC =0.852; 95% CI: 0.753-0.923; sensitivity: 70.45%; specificity: 90.91%) also had discrimination ability between stage I and II. The measurement of the soft tissue area of the calf may be used as an auxiliary method for staging primary LEL. For patients with unilateral primary LEL, the difference in subcutaneous area of limbs could be a specific indicator to distinguish clinical stage I from II.

Results of home-based modified combined decongestive therapy in patients with lower extremity lymphedema

Background/aim The aim of this study is to present the results of modified combined decongestive therapy (CDT) in patients with lower extremity lymphedema (LEL). Materials and methods We retrospectively reviewed 95 patients aged 55.84 ± 15.70 years who had been diagnosed with LEL between May 2015 and May 2017. The patients were treated for 4 weeks with modified CDT, including self-manual lymphatic drainage, self-bandaging, decongestive exercises, and skin care.Results The mean reduction amounts of edema volume before and after treatment were 296.05, 784.92, and 1038.50 mL for stages 1, 2, and 3 respectively (P = 0.001). There were significant differences between the values before and after treatment in excess extremity volume (EEV) at all stages (P = 0.001). The EEV percentages of the secondary LEL patients were higher than those of the primary LEL patients (P = 0.04). There was no correlation between BMI and treatment response in terms of EEV percentages (r = –0.99; P = 0.36).Conclusion Our results revealed that home-based modified CDT is more effective in reducing extremity edema volume in secondary LEL than primary LEL. It should be an available method for self-management of LEL at all stages.

Lymphoscintigraphic Evaluation of Systemic Tracer Uptake in Patients With Primary Lymphedema.

Lymphoscintigraphy is used to confirm the diagnosis of lymphedema. One end point for the test is to ensure a patent thoracic duct by uptake of tracer in the organs. The purpose of this study was to evaluate transit of radiolabeled colloid to the organs to gain insight into the etiopathophysiology of primary lymphedema. Patients treated in our Lymphedema Program between 2009 and 2018 were reviewed. Only subjects with bilateral lower extremity primary lymphedema were included (individuals with unilateral leg lymphedema were excluded because the tracer will reach the venous circulation and organs through the normal extremity). Disease severity and lymphoscintigraphy findings were documented. Sixty-one patients were included. Ten subjects had no radiolabeled tracer transit to the inguinal lymph nodes on lymphoscintigraphy. However, 8 of these individuals had tracer uptake to the liver, kidney, and/or bladder, illustrating clearance of tracer into the systemic venous circulation. All 8 patients had infant-onset primary lymphedema and mild disease. The 2 patients who did not have clearance of tracer to the systemic venous circulation developed lymphedema in adolescence and had clinically moderate or severe disease. Patients with primary lower extremity lymphedema often have pathways for lymph fluid to reach the venous circulation other than through the inguinal nodes and thoracic duct. Documentation of systemic tracer uptake during lymphoscintigraphy to confirm a patent thoracic duct has limited clinical significance in subjects with primary disease of the legs.

Improvement of the efficacy of vascularized lymph node transfer for lower-extremity lymphedema via a prefabricated lympho-venous shunt through lymphaticovenular anastomosis between the efferent lymphatic vessel and small vein in the elevated vascularized lymph node.

Following vascularized lymph node (VLN) transfer (VLNT), the VLN may be at a risk for sclerosis because of efferent lymphatic vessel obstruction. We developed a new technique to prevent VLN sclerosis via a prefabricated lympho-venous (LV) shunt. This study compared the treatment outcomes of single VLNT with prefabricated LV shunt, conventional multiple VLNTs, and conventional single VLNT. Overall, 47 limbs of 45 patients that underwent VLNT for lower-extremity lymphedema (LEL) in late clinical stage II/III were divided into 3 groups: conventional single VLNT group (control; 21 limbs where 4 had primary LEL), multiple VLNTs group (13 limbs where 3 had primary LEL), and prefabricated LV shunt group (13 limbs where 4 had primary LEL). In the prefabricated LV shunt group, lymphaticovenular anastomosis between the efferent lymphatic vessel and small vein in the elevated VLN were performed simultaneously with VLNT. Although venous thrombosis at the anastomosis site was observed in 1 case, it was salvaged by re-anastomosis, and all VLNs survived. No other complications were observed. The LEL index significantly improved in the prefabricated LV shunt group compared with that in the control group (28.0 ± 1.7 vs 20.9 ± 1.5, P = 0.02). In the prefabricated LV shunt group, all VLNs survived functionally, and the average size of the transferred lymph nodes was significantly larger than that of the control group (5.7 ± 0.1 vs 4.3 ± 0.2 mm, P < 0.01). Prefabricated LV shunt may improve the efficacy of VLNT.

Complete lymph flow reconstruction: A free vascularized lymph node true perforator flap transfer with efferent lymphaticolymphatic anastomosis

Treatment of primary lower extremity lymphedema (LEL) is challenging, and lymph node transfer (LNT) can be a choice of treatment for progressive LEL. However, LNT has a risk of donor site lymphedema and possible lymph node (LN) sclerosis due to efferent lymphatic vessel (ELV) obstruction. Here, we report the first case of complete lymph flow reconstruction with true perforator LNT with efferent lymphaticolymphatic anastomosis (ELLA) for a patient with primary LEL and severe lymphosclerosis. A 49-year-old female suffered from primary progressive unilateral left LEL refractory to conservative treatments with frequent episodes of cellulitis. A true perforator LN flap was selectively harvested from the left lateral thoracic region under indocyanine green (ICG) lymphography navigation and transferred to the left groin with perforator-to-perforator anastomosis. The ELV of the transplanted LN was supermicrosurgically anastomosed to the contralateral iliac lymphatic vessel that was subcutaneously transferred to the left groin. Postoperatively, the patient experienced no episode of cellulitis with reduced degree of compression treatment, and lymphedematous volume decreased from 306 to 264 in terms of LEL index. Postoperative ICG lymphography showed evidence of reconstructed lymph flow from the left foot to the left groin and to the right inguinal LN through the transplanted LN flap and the ELLA. There were no subjective or objective findings of donor site lymphedema of the left arm or the right back and the lower extremity. True perforator LN flap with ELLA is a safe and effective treatment and has the potential to be a useful therapeutic option for primary unilateral LEL.

Effectiveness of Lymphatic Microsurgical Procedures in the Treatment of Primary Lymphedema

INTRODUCTION: Vascularized lymph node transfer (VLNT) and lymphovenous bypass (LVB) procedures represent physiologic treatment options for symptomatic lymphedema. Secondary causes related to oncologic surgery and/or radiation have been successfully treated using these surgical procedures. Primary lymphedema represents a poorly understood lymphedematous condition with equally poor understanding of the benefits of microsurgical intervention. The purpose of this study was to review our experience with this patient population to better understand the effectiveness of microsurgical procedures. METHODS: A retrospective review of a prospectively maintained database of patients who received microsurgical treatment for primary lymphedema was reviewed. Both LVB and VLNT procedures were used in this patient cohort. Outcomes related to demographics, circumference differences, and symptoms, and quality of life (QoL) changes were evaluated. A validated questionnaire, the LYMQOL, was used to assess QoL outcomes. RESULTS: Thirteen patients were identified and met inclusion criteria. All patients had primary lower extremity lymphedema. Average age and symptom duration was 37.8 years and 162 months, respectively. The average lymphedema stage was classified as Stage II in 66.7% of patients. Average follow-up was 12.2 months. VLNT was used in most cases (69.2%) while LVB was used in the remainder of patients. The average overall circumference reduction was 3.6 cm with more improvement seen in patients who received VLNT as compared to LVB (4.2cm v. 1.9cm). Improvements in body weight and cellulitis occurrence was significantly improved in the VLNT cohort (p<0.05). In addition, patient-reported QoL domains related to function, appearance, symptoms, and mood were significantly improved following VLNT (p<0.05 in all domains) as compared to LVB (p>0.05 in all domains). CONCLUSION: Lymphatic microsurgical procedures are valuable treatment options for patients with primary lymphedema. Vascularized lymph node transfer appears to result in improved overall outcomes as compared to lymphovenous bypass procedures in this specific patient population. Improvements in objective clinical measures (limb circumference, body weight, and cellulitis occurrence) correlate well with improved patient-reported quality of life parameters.

The "Octopus" Lymphaticovenular Anastomosis: Evolving Beyond the Standard Supermicrosurgical Technique.

Supermicrosurgical lymphaticovenular anastomosis (LVA) is a promising treatment modality for lymphedema. However, its practice is restricted by the surgeon/equipment-related factors, and its effectiveness limited by technical constraints. We conducted a pilot study to evaluate the feasibility of a modified "octopus" LVA technique in addressing the above problems. Nine consecutive lymphedema patients underwent LVA procedure using the "octopus" technique. Six had the upper extremity disease; three had the lower extremity disease. Except for one patient having primary lower extremity lymphedema, all had secondary disease related to cancer treatment. Disease severity ranged from Campisi stage Ib to IV. Qualitative and quantitative assessments were performed preoperatively, at 1, 3, and 6 months. A total of 130 lymphaticovenular drainage pathways were created in 39 "octopus" LVAs. All patients experienced prompt relief of lymphedema symptoms during the 1st postoperative week and continued to improve during the study period. None had postoperative complications. All had disease regression as demonstrated by statistically significant decrease in limb measurements (p = 0.0003) and severity down-staging. The modified technique was found to be easier than the standard supermicrosurgical technique and could be performed using a standard surgical microscope. The "octopus" technique is a viable, effective technical alternative to the standard LVA technique. It may greatly simplify this technically challenging procedure.

Indocyanine Green Lymphography Findings in Primary Leg Lymphedema

Indocyanine green (ICG) lymphography has been reported to be useful for the evaluation of secondary lymphedema, but no study has reported characteristic findings of ICG lymphography in primary lymphedema. This study aimed to classify characteristic ICG lymphography patterns in primary lymphedema. The study was a retrospective observational study. Thirty one primary lower extremity lymphedema (LEL) patients with a total of 62 legs were studied. ICG lymphography patterns were categorized according to the visibility of lymphatics and dermal backflow (DB) extension. Clinical demographics were compared with categorized ICG lymphography patterns. All symptomatic legs showed abnormal patterns, and all asymptomatic legs showed normal patterns on ICG lymphography. Abnormal lymphographic patterns could be classified into proximal DB (PDB), distal DB (DDB), less enhancement (LE), and no enhancement (NE) patterns. There were significant differences between PDB (16 patients), DDB (6 patients), LE (4 patients), and NE patterns (5 patients) in age (37.3 ± 18.3 vs. 61.8 ± 19.2 vs. 50.8 ± 27.7 vs. 29.2 ± 18.0 years, p = .035), onset of edema (23.9 ± 19.4 vs. 46.8 ± 27.0 vs. 43.0 ± 31.3 vs. 6.6 ± 14.2 years, p = .020), laterality (bilateral; 18.8% vs. 66.7% vs. 75.0% vs. 0%, p ¼ .016), cellulitis history(56.3% vs. 100% vs. 25.0% vs. 0%, p ¼ .007), and LEL index (292.2 ± 32.8 vs. 254.2 ± 28.6 vs. 243.3 ± 9.4 vs. 295.2 ± 44.8, p = .016). ICG lymphography findings in primary lymphedema could be classified into four patterns withdifferent patient characteristics.