Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.

ABSTRACT: Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs).A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS. At 60 years of age, she developed chyluria (urine albumin, 2224 µg/mL) and hematuria. Lymphoscintigraphy showed a lymphatic-ureteral fistula near the ureterovesical junction. Conservative treatment was ineffective. She also developed left lower extremity lymphedema, which gradually worsened. Leg cellulitis and purulent pericarditis developed because of hypoalbuminemia (minimum serum albumin level, 1.3 g/dL).We performed 14 LVAs in 2 surgeries to reduce lymphatic fluid flow through the lymphatic-ureteral fistula. The chyluria and hematuria resolved soon after the second operation, and the urine albumin level decreased (3 µg/mL). After 28 months, she had no chyluria or hematuria recurrence and her serum albumin level improved (3.9 g/dL). Multiple LVAs can definitively treat chyluria caused by a lymphatic-ureteral fistula in patients with KTS.

Yellow Nail Syndrome: Analysis of 23 Consecutive Patients and Effect of Combined Fluconazole-Vitamin-E Treatment.

INTRODUCTION: Yellow nail syndrome (YNS), a very rare disorder of unknown etiology, is characterized by a triad associating yellow nails, respiratory manifestations, and lymphedema. YNS treatment remains non-codified. METHOD: This retrospective study was conducted from January 2008 to December 2022 in a single tertiary department exclusively dedicated to lymphatic diseases. All consecutive patients with YNS were included. RESULTS: Thirteen men and 10 women were included. Three patients had yellow nails at birth or during childhood. For the other 20 patients, median (Q1-Q3) age at first sign was 50.8 (43-61) years, with first-YNS-sign-to-diagnosis interval of 17 (10-56) months. For 4 patients, YNS was associated with primary intestinal lymphangiectasia. The first YNS sign was chronic cough (45.5%), followed by yellow nails (27.3%), chronic sinusitis (18.2%), and lymphedema (9.1%). At first consultation for all patients, 69.6% had the complete triad, all had yellow nails and cough, 82.6% had chronic sinusitis, and 69.6% had lymphedema. Twelve patients' lymphedema involved only the lower limb(s), 2 the lower and upper limbs, and 2 the lower and upper limbs and face. Nineteen (82.6%) patients were prescribed fluconazole (100 mg/day [n = 8] or 300 mg/week [n = 11]) combined with vitamin E (1,000 mg/day) for a median of 13 months. Responses were complete for 4 (21.1%) patients, partial for 8 (42.1%), and therapeutic failures for 7 (36.8%). CONCLUSIONS: YNS is a rare disease that almost always starts with a chronic cough. Despite inconstant efficacy, fluconazole-vitamin E in combination can be prescribed to treat yellow nails.

Genital elephantiasis: Surgical treatment and reconstruction.

Genital elephantiasis is a severe form of lymphedema of the groin. It is characterized by progressive enlargement and distortion of the genitals, presenting significant physical, psychological, and social challenges to the affected individuals. Although pharmacological treatment of filariasis is well-established in the medical field, the surgical management of genital elephantiasis can be varied and confusing. This review article provides an in-depth analysis of the etiology, classification, severity grading, and various effective surgical treatment and reconstructive modalities commonly employed by surgeons since the early twentieth century. We also discuss how a combination approach of ablation, soft tissue coverage, and lymphatic reconstruction is viable for treating genital elephantiasis. By examining the literature, we hope to provide insights into how surgery plays a role in the holistic management of genital elephantiasis.

Value of the short time inversion recovery sequence of magnetic resonance imaging in the staging of Klippel-Trenaunay syndrome complicated with lymphedema.

OBJECTIVE: Currently, the focus on limb lymphedema (LE) is on classification and staging. However, few scholars have conducted staging for Klippel-Trenaunay syndrome complicated LE (KTS-LE). This study aimed to investigate the value of the short time inversion recovery sequence of magnetic resonance imaging (MRI) in the staging of KTS-LE. METHODS: Forty-six patients who were diagnosed with KTS-LE were recruited for this retrospective study from July 2011 to November 2022. Referring to the clinical staging standard of lower extremity LE of the International Society of Lymphology in 2020, all patients were divided into three groups: stages I, II, and III. The MRI indicators of the three groups were recorded and statistically compared: LE range (unilateral bilateral, lower limbs, only thighs, only calves and ankles), abnormal parts (skin thickening, abnormal subcutaneous fat signal, abnormal muscle signal, muscle hypertrophy or contraction, abnormal bone signal, hyperostosis), and subcutaneous soft tissue signs (parallel line sign, grid sign, band sign, honeycomb sign, lymph lake sign, crescent sign, and nebula sign). RESULTS: There was a significant difference in the honeycomb sign among the three periods (P = .028). There was a significant difference between stage II and stage I disease (P < .05). There was a significant difference between stage II and stage III disease (P < .05). The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the honeycomb sign in diagnosing KTS-LE of stage II were 87.5%, 63.2%, 33.3%, 96.0%, and 67.4%, respectively. In contrast, the other signs were not statistically significant among the three periods. CONCLUSIONS: The short time inversion recovery sequence of MRI is of great value in KTS-LE. The honeycomb sign is an important imaging indicator for the diagnosis of stage II disease. It is necessary to evaluate the severity of edema with MRI for KTS-LE, which is very important for therapeutic options.

Elephantiasis mimicry in recurrent lower limb skin infections in a diabetic patient: a case report.

BACKGROUND: Chronic edema as a complication of systemic diseases or infections can mimic filarial lymphedema (also known as elephantiasis) and considered so. We describe a case of chronic lymphedema that mimicked elephantiasis in a diabetic man. CASE PRESENTATION: The patient was a 70-year-old black man, bed-bound at the time of admission following a diagnosis of stroke and hypertension in the previous 5 years. He had been diabetic for 20 years with poorly controlled diabetes mellitus. He suffered recurrent bilateral lower limb skin infections for 5 years prior to admission that culminated into progressive lowerlimb edema. The infections eventually complicated into skin edema, hardening, fissuring, and hyperkeratotic plaques. The physical examination revealed Tinea pedis and bilateral non-pitting edema of lowerlimbs to the level of the knees. Investigations confirmed non-filarial lymphedema-related skin changes. The absence of the classic pebbly/cobblestone skin changes ruled out elephantiasis nostra verrucosa (ENV), with a possibility of it being in the early stages of evolution. The patient's skin fissuring and infections were successfully treated with antibiotics and antifungals while compression stockings helped to relieve the edema. CONCLUSIONS: Chronic lymphedema can complicate repeated non-filarial infections of lower limbs. Its fissures are a risk factor for cellulitis, prompting early identification and management of both infections and lymphedema to halt their vicious cycle, especially in at risk populations like diabetics.

Immobilization, Lymphedema, and Obesity are Predictive Factors in the Development of Adhesive Capsulitis in Breast Cancer Patients.

OBJECTIVE: Adhesive capsulitis is a condition characterized by shoulder pain and stiffness. Breast cancer treatment has been linked to the development of this condition, but its mechanisms are still little known. This study's objective was to identify predictors factors associated with the development of adhesive capsulitis in breast cancer patients. METHODS: A case control study was performed with women undergoing treatment for breast cancer in a single center. The sampling was nonprobabilistic and consecutive. Adhesive capsulitis was defined as constant pain associated with decreased active and passive shoulder movement in anterior elevation, external rotation at 0°/90° abduction, and internal rotation at 90° abduction. The study group consisted of patients with shoulder pain and range of motion limitations, while the control group consisted of women without any shoulder abnormalities. Sociodemographic and clinical variables were collected. A univariate logistic regression was used to assess the influence of variables on the studied outcome. For p < 0.20, a multivariate logistic regression was used. The probability of null hypothesis rejection was 5%. RESULTS: A total of 145 women were assessed, with 39 (26.9%) on the study group and 106 (73.1%) on the control group. The majority was under 60 years old. In the multivariate analysis, variables correlated to the outcome under study were shoulder immobilization (OR = 3.09; 95% CI: 1.33-7.18; p = 0.009), lymphedema (OR = 5.09; 95% CI: 1.81-14.35; p = 0.002), and obesity (OR = 3.91; 95% CI: 1.27-12.01; p = 0.017). CONCLUSION: Lymphedema, postsurgery immobilization, and obesity are predictive factors for the development of adhesive capsulitis in breast cancer patients.

OBJETIVO: Capsulite adesiva é uma afecção caracterizada por dor e limitação dos movimentos do ombro. O tratamento do câncer de mama está relacionado ao desenvolvimento dessa doença por meio de mecanismos ainda pouco conhecidos. O objetivo do estudo foi identificar os fatores associados ao desenvolvimento de capsulite adesiva em pacientes com câncer de mama. MéTODOS: Um estudo caso-controle foi realizado com mulheres em tratamento para câncer de mama em um centro único. A amostra foi consecutiva e não-probabilística. A capsulite adesiva foi pré-definida como dor constante e diminuição da amplitude de movimentos em elevação anterior, rotação externa em 0°/90° abdução e rotação interna em 90° abdução. O grupo caso foi constituído por pacientes com dor e limitação de todos os movimentos do ombro, enquanto o controle por pacientes sem qualquer alteração nesta articulação. Variáveis sociodemográficas e clínicas foram coletadas. Foi realizada uma análise de regressão logística univariada para avaliar a influência das variáveis em relação ao desfecho estudado. Para valores de p < 0,20, realizou-se a análise de regressão logística multivariada. A probabilidade de se rejeitar a hipótese nula foi de 5%. RESULTADOS: Foram avaliadas 145 mulheres, sendo 39 casos (26,9%) casos e 106 controles (73,1%). Na análise multivariada, as variáveis associadas ao desfecho estudado foram imobilização do ombro (OR = 3,09; 95% IC: 1,33­7,18; p = 0,009), linfedema (OR = 5,09; 95% IC: 1,81­14,35; p = 0,002) e obesidade (OR = 3,91; 95% IC: 1,27­12,01; p = 0,017). CONCLUSãO: Linfedema, imobilização pós-cirúrgica e obesidade são fatores preditores associados ao desenvolvimento de capsulite adesiva em pacientes com câncer de mama.

Reconstruction of dermal layers evaluated by high-frequency ultrasound following treatment for skin fibrosis.

Background: Lymphedema is a chronic, progressive clinical condition that evolves with intense fibrosis, the most advanced stage of which is stage III (lymphostatic fibrosclerosis). Aim: The aim of the present study was to show the possibility to reconstruct the dermal layers with the intensive treatment of fibrosis using the Godoy method. Case description: A 55-year-old patient with an eight-year history of edema of the lower limb of the leg had constant episodes of erysipelas, despite regular treatments. The edema progressed continually, associated with a change in the color of the skin and the formation of a crust. Intensive treatment (eight hours per day for three weeks) was proposed with the Godoy method. The ultrasound was performed and results revealed substantial improvement in the skin, with the onset of the reconstruction of the dermal layers. Conclusion: It is possible to reconstruct the layers of the skin in fibrotic conditions caused by lymphedema.

Postoperative swelling after elbow surgery: influence of a negative pressure application in comparison to manual lymphatic drainage-a randomized controlled trial.

PURPOSE: Postoperative soft tissue swelling is a significant factor influencing outcomes after elbow surgery. It can crucially affect important parameters such as postoperative mobilization, pain, and subsequently the range of motion (ROM) of the affected limb. Furthermore, lymphedema is considered a significant risk factor for numerous postoperative complications. Manual lymphatic drainage is nowadays part of the standardized post-treatment concept, basing on the concept of activating the lymphatic tissue to absorb stagnated fluid from the tissue into the lymphatic system. This prospective study aims to investigate the influence of technical device-assisted negative pressure therapy (NP) on early functional outcomes after elbow surgery. NP was therefore compared to manual lymphatic drainage (MLD). Is a technical device-based NP suitable for treatment of lymphedema after elbow surgery? METHODS: A total of 50 consecutive patients undergoing elbow surgery were enrolled. The patients were randomized into 2 groups. 25 participants per group were either treated by conventional MLD or NP. The primary outcome parameter was defined as the circumference of the affected limb in cm postoperative up to seven days postoperatively. The secondary outcome parameter was a subjective perception of pain (measured via visual analogue scale, VAS). All parameters were measured on each day of postoperative inpatient care. RESULTS AND CONCLUSION: NP showed an overall equivalent influence compared to MLD in reducing upper limb swelling after surgery. Moreover, the application of NP showed a significant decrease in overall pain perception compared to manual lymphatic drainage on days 2, 4 and 5 after surgery (p < 0.05). CONCLUSION: Our findings show that NP could be a useful supplementary device in clinical routine treating postoperative swelling after elbow surgery. Its application is easy, effective and comfortable for the patient. Especially due to the shortage of healthcare workers and physical therapists, there is a need for supportive measures which NP could be.

Surgical management of acquired buried penis and scrotal lymphedema: A retrospective review.

Acquired buried penis is a condition that can have detrimental physical and psychological consequences for patients. Factors such as elevated BMI, chronic scrotal lymphedema, hidradenitis suppurativa, and chronic inflammation can lead to the condition. Surgical intervention is the treatment of choice for advanced disease. Following IRB approval, a retrospective chart review was performed for patients with a diagnosis of acquired buried penis who required surgical intervention. Details of patient history, surgical management including intraoperative and post-operative photography, and complications were reviewed. Seven patient cases were reviewed. The average age at time of surgery was 44 with a mean weight of 344 pounds and an average BMI of 48. Severe scrotal lymphedema and hidradenitis were common concurrent comorbidities. Concurrent scrotoplasty and infraumbilical panniculectomy were standard parts of the operations. Native glans skin was salvageable in all but one case. Penile shaft skin was reconstructed with skin grafts or adjacent tissue transfer. 88% of the cases had some element of wound dehiscence post-operatively. Surgical management of an acquired buried penis can be challenging. The patient demographic with the disease is frequently complicated by morbid obesity, concurrent lymphedema, or hidradenitis. Post-operative complications are expected. The surgical techniques presented can aid in simplifying the management of this challenging surgical population.

Phenotypical characterization, and antibiotics susceptibility patterns of skin bacteria found in podoconiosis patients in the North West Region of Cameroon.

BACKGROUND: Podoconiosis, a non-infectious disease originating from long-term exposure of bare feet to irritant red clay soil is a lifelong, disabling disease with no specific diagnostic tool, classified into 5 stages based on the severity of leg swelling (lymphoedema). Secondary bacterial infections have been suggested to cause acute dermatolymphangioadenitis (ADLA) attacks and drive disease progression. Although the North West Region of Cameroon has a proven history of podoconiosis endemicity, the bacterial composition of lymphoedema due to this condition has not been studied. Thus, this study investigated the leg bacterial diversity of patients who suffered from the lymphoedema and their susceptibility pattern to selected antibiotics. METHODS: A cross-sectional study was carried out in which podoconiosis affected and non-lymphoedema individuals living in the same community were purposively selected. Samples were collected by swabbing the skin between the toes and around the anklebone, then cultured and sub-cultured on nutrient agar to obtain pure isolates. The cultured isolates were then morphologically and biochemically classified using microscopy and analytic profile index test kits, respectively. The disk diffusion technique was used to determine antibiotic susceptibility. RESULTS: Thirty-three participants were recruited, and 249 bacterial isolates were characterized into 29 genera, 60 species; with 30 (50%) being gram positive rods, 19 (31.7%) gram positive cocci, and 11 (18.3%) gram negative rods. Thirteen gram positive rods, fifteen gram positive cocci, and eight gram negative rods of bacterial species were found only in podoconiosis individuals among which Cellulomonas spp / Microbacterium spp. (2.8%), Staphylococcus lentus (3.3%), and Burkholderia cepacia (4.0%) dominated. 90% (90%) of the bacterial isolates were sensitive to doxycycline, whereas ampicillin had a high level of intermediate resistance, and penicillin G had the greatest resistant profile. CONCLUSION: Our findings show that 94 (37.8%) out of 249 described bacterial isolates were exclusively found in the legs of podoconiosis individuals, and their susceptibility pattern to antibiotics was similar to that of others.

Persistent chylothorax associated with lymphatic malformation type 6 due to biallelic pathogenic variants in PIEZO1.

PIEZO1 is required for lymphatic valve formation, and several lymphatic abnormalities have been reported to be associated with autosomal recessive PIEZO1 pathogenic variants including neonatal hydrops, lymphedema involving various body regions, and chylothorax. Persistent or recurrent chylothorax has been infrequently described in association with pathogenic variants in the PIEZO1 gene. We present a 4-year-old female with bilateral pleural effusions detected prenatally, who was diagnosed with bilateral chylothoraces post-partum. She subsequently had recurrent pleural effusions involving both pleural cavities, which tended to improve with restriction of her fat intake, and, one occasion, subcutaneous octreotide. She also had bilateral calf, and intermittent cheek swelling. Genetic testing revealed two deleterious variants in PIEZO1: c.2330-2_2330-1del and c.3860G > A (p.Trp1287*), both of which were classified as likely pathogenic. This supported a diagnosis of Lymphatic Malformation Type 6 (OMIM 616843), also known as Hereditary Lymphedema Type III. Hereditary Lymphedema type III can be associated with persistent chylothorax that can vary in size over time.

The utility of computed tomography venography in the routine evaluation of patients who present to a lymphedema center with lower extremity edema.

OBJECTIVE: Computed tomography venography (CTV) is not routinely used to screen patients presenting with a presumed lower extremity lymphedema diagnosis for left iliac vein obstruction (IVO) or May-Thurner syndrome (MTS). The objective of this study is to determine the utility of routine CTV screening for these patients by evaluating the proportion presenting with clinically significant CTV-identified left IVO. METHODS: We retrospectively reviewed 121 patients who had presented to our lymphedema center with lower extremity edema between November 2020 and May 2022. Information regarding demographics, comorbidities, lymphedema characteristics, and imaging reports was collected. Cases of IVO present on CTV were reviewed by a multidisciplinary team to determine the clinical significance of the CTV findings. RESULTS: Of the patients with complete imaging studies, 49% (n = 25) had abnormal lymphoscintigraphy findings, 45% (n = 46) had reflux on ultrasound, and 11.4% (n = 9) had IVO on CTV. Seven patients (6%) had CTV findings of IVO and edema of either the isolated left (n = 4) or bilateral (n = 3) lower extremities. Cases of IVO on CTV were determined by the multidisciplinary team to be the predominant cause of lower extremity edema for three of these seven cases (43%; or 2.5% of all 121 patients). CONCLUSIONS: Six percent of patients presenting to a lymphedema center with lower extremity edema had left-sided IVO on CTV suggestive of MTS. However, the cases of IVO were determined to be clinically significant <50% of the time or for 2.5% of all patients. CTV should be reserved for patients with isolated left-sided or bilateral lower extremity edema with a greater left-sided component and a history of findings that raise clinical suspicion for MTS.

Efficacy of kinesio taping on upper limb volume reduction in patients with breast cancer-related lymphedema: a systematic review of randomized controlled trials.

INTRODUCTION: One of the most common disabling complications in breast cancer (BC) patients is breast cancer-related lymphedema (BCRL), a pathological condition affecting both physical and psychological function, with detrimental consequences on health-related quality of life (HR-QoL). Rehabilitation has a key role in the comprehensive management of this condition with several studies reporting positive results after performing complex decongestive therapies (CDT) in these women. Kinesio taping (KT) is a rather recent therapeutic approach to treat BCRL, however, evidence in literature regarding its effectiveness is far from being fully characterized. Therefore, this systematic review aimed at assessing the role of KT among the CDT to treat BCRL. EVIDENCE ACQUISITION: PubMed, Scopus, and Web of Science were systematically searched from inception until May 5th, 2022 to determine randomized control trials (RCTs) reporting patients with BCRL; KT as intervention; limb volume as outcome (PROSPERO number: CRD42022349720). EVIDENCE SYNTHESIS: Out of the documents identified, 123 were eligible for data screening, and only 7 RCTs satisfied the eligibility criteria and were included. We found that KT might have a positive effect on limb volume reduction in patients with BCRL, albeit there is little evidence for low quality of the included studies. CONCLUSIONS: Taken together, this systematic review showed that KT did not significantly reduce the upper limb volume in BCRL women, albeit it seemed to increase the flow rate during the passive exercise. Further high-quality-studies are mandatory to improve the knowledge in order to include the KT might into a multidisciplinary rehabilitative approach for the management of BC survivors affected by lymphedema.

The effect of adjuvant radiotherapy on skin biophysical properties in patients with breast cancer at risk for breast lymphedema: A prospective study.

Breast cancer (BC) is the most common type of cancer among women. Radiotherapy (RT) is one of the main and primary treatment options for BC, especially in breast-conserving surgery (BCS). BC patients who underwent RT experience a wide range of symptoms, in which breast oedema and irritation of the skin take the lion's share. Breast oedema/lymphedema, which is also a prominent side effect after RT should be well determined in earlier settings due to the chronicity of lymphedema. Therefore, this study aimed to analyze the biophysical parameters of skin on the ipsilateral (IL) and contralateral (CL) sites via Tissue dielectric constant (TDC) and Transepidermal water loss (TEWL) methods in terms of oedema and skin barrier function (SBF). The following reference points before and after the RT were measured: (R1: Pectoralis muscle, R2: Upper breast, R3: Lower breast, R4: Lateral site of the thorax). A total of 24 BC patients (mean age and BMI: 52.78 ± 9.85 years and 28.42 ± 5.64 kg/m2 ) were evaluated. In the IL site, the SBF was not found significant in R1-R3, whereas significantly lower SBF was observed in R4 after RT (t = -3.361, p = 0.003). A significant increase in TDC was observed in R2 at the 5.0 mm depth (t = -2.500, p = 0.02). We suggest that a longer period of follow-up should be carefully carried out to track changes in terms of SBF and oedema in the irradiated breast. The increased need for early detection of changes associated with breast lymphedema can be achievable via noninvasive, safe, cheap, and easily repeatable devices.

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.

BACKGROUND: stewart-treves syndrome (STS) is an angiosarcoma associated with chronic lymphedema. OBJECTIVES: This article analyses the characteristics of twenty-two patients and proposes active intervention in lymphedema and the early diagnosis of STS. METHODS: Twenty-two patients with STS were diagnosed at the centre over an 11-year period. Clinical manifestations, a series of conventional analyses, and histopathology were used to study these cases retrospectively. RESULTS: The age range of 22 patients with STS was 15 to 78 years. The main clinical manifestations included multiple skin and subcutaneous nodules and scattered red or purplish-red rashes in the lymphoedematous limbs. These patients often showed clinical symptoms such as lymphedema, weakness, emaciation, pain, mass, lymphadenopathy and so on. The positive rates of ultrasonography, MRI and radionuclide imaging were 66.7% (6/9), 92.3% (12/13) and 18.2% (2/11), respectively. The main points regarding active intervention in lymphedema and early diagnosis of STS were summarized. STUDY LIMITATIONS: Since this was a retrospective study, the main points summarized by the author need to be further quantified in clinical work to guide the diagnosis of this kind of disease more conveniently. In addition, further clinical trials are needed to evaluate the role of lymphedema in the occurrence and development of malignant tumors. CONCLUSIONS: STS can appear in lymphoedematous tissue many years after lymphedema onset. To avoid delays in the diagnosis and therapy of STS, physicians should actively look for signs or symptoms of malignant lymphedema during the follow-up period and promptly manage patients developing problems.