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Journal articles on the topic 'Primary lymphoedema'

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Author: Grafiati

Published: 10 December 2022

Last updated: 28 January 2023

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1

Ali Al-Chalabi, Hussein, and Kathem Kassim AL-Rubiay. "Lymphoedema Praecox: A Case Report of Primary Lymphoedema." Basrah Journal of Surgery 9, no. 1 (June 28, 2003): 110–12. http://dx.doi.org/10.33762/bsurg.2003.55291.

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2

Vignes, S., M. Arrault, A. Yannoutsos, and M. Blanchard. "Primary upper-limb lymphoedema." British Journal of Dermatology 168, no. 2 (November 20, 2012): 272–76. http://dx.doi.org/10.1111/bjd.12024.

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3

Tabel, Yilmaz, Ilke Mungan, Ahmet Sigirci, and Serdal Gungor. "Primary Lymphoedema at an Unusual Location Triggered by Nephrotic Syndrome." Annals of the Academy of Medicine, Singapore 38, no. 7 (July 15, 2009): 636. http://dx.doi.org/10.47102/annals-acadmedsg.v38n7p636.

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Introduction: Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fluid in the interstitial space, most commonly in the extremities. Primary lymphoedema, a developmental abnormality of the lymphatic system, may become evident later in life when a triggering event exceeds the capacity of normal lymphatic flow. Clinical Picture: We present a 3-year-old nephrotic syndrome patient with an unusual localisation for primary lymphoedema. Treatment and Outcome: The patient was treated with conservative approach and she was cured. Conclusion: In this particular case, lymphoedema developed at an unusual localisation, which has not been recorded before. Introduction: Lymphoedema results from impaired lymphatic transport leading to the pathologic accumulation of protein-rich lymphatic fluid in the interstitial space, most commonly in the extremities. Primary lymphoedema, a developmental abnormality of the lymphatic system, may become evident later in life when a triggering event exceeds the capacity of normal lymphatic flow. Clinical Picture: We present a 3-year-old nephrotic syndrome patient with an unusual localisation for primary lymphoedema. Treatment and Outcome: The patient was treated with conservative approach and she was cured. Conclusion: In this particular case, lymphoedema developed at an unusual localisation, which has not been recorded before.

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4

Bolcal, C., H. Iyem, M. Sargin, I. Mataraci, S. Doganci, S. Kilic, O. Karacalioglu, M. A. Sahin, U. Demirkilic, and H. Tatar. "Primary and secondary lymphoedema in male patients with oedema in lower limbs." Phlebology: The Journal of Venous Disease 21, no. 3 (September 15, 2006): 127–31. http://dx.doi.org/10.1258/026835506778253328.

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Objective: The purpose of this prospective study was to evaluate patients with clinically diagnosed lymphoedema of the lower extremities. The proportions of primary and secondary lymphoedema, the possible aetiologic factors and the concomitance of chronic venous diseases and lymphoedema were focused on. Method: The male patients who attended our outpatient clinic during 2000 and 2004 were evaluated. In all, 160 male patients with 5 cm circumference difference at calf level between two lower extremities or with clinically diagnosed bilateral leg oedema were enrolled. All patients underwent duplex venous ultrasonography and lymphoscintigraphy. Venography was performed in 12 patients with normal lymphoscintigraphy and ultrasonography. Results: The age distribution was between 20 and 54 years (mean ± SD; 22.9 ± 4.3). Among 160 patients, 70.0% had lymphoedema, while 7.5% had chronic venous insufficiency, 3.75% chronic deep venous thrombosis, 7.5% concomitant venous disease and lymphatic obstruction, and 7.5% idiopathic oedema. In the last 3.75% the pathology was Klippel–Trenaunay syndrome. Of the primary lymphoedema patients (16.25%), 18 were praecox, six tarda and two were congenital types. Conclusion: In young male patients, the causes of secondary lymphoedema are lymphadenectomy, neoplastic metastasis, cellulitis, lymphangitis, etc. Further techniques confirmed the clinical diagnosis of lymphoedema in 77.5% (sum of lymphoedema and concomitant disease) of all patients. With these findings lymphoedema can be diagnosed clinically, and further diagnostic techniques can be reserved unless treatment is effective.

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5

Chaudhry, H. L., P. S. Mortimer, J. E. Evans, and J. A. Dormandy. "The Prevalence of Venous Disease in Primary Lymphoedema." Phlebology: The Journal of Venous Disease 12, no. 1 (March 1997): 31–35. http://dx.doi.org/10.1177/026835559701200106.

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Objective: To assess the prevalence of venous incompetence in primary lymphoedema. Design: Retrospective study. Setting: Outpatients attending St George's Hospital, London, UK. Patients: Forty-six patients (61 limbs) with primary lymphoedema extracted from a consecutive series of patients presenting with swollen legs. Only those with clinical and lymphoscintigraphic evidence of lymphoedema were included. Patients with any clinical evidence of venous disease were excluded. Controls: Seventeen subjects (33 limbs) with no history or clinical evidence of either lymphoedema or venous disease. Methods: Light reflective rheography (LRR) and quantitative isotopic lymphoscintigraphy. Main outcome measures: Venous refilling times (RT) with and without tourniquet control. Statistical analysis: Prevalence of venous incompetence in lymphoedema patients compared with normal controls and literature values. Results: In the patients, seven of 61 limbs (11.5%) had a venous RT of less than 25 s; eight limbs (13.1%) had a venous RT between 25 and 40 s. An above-knee tourniquet corrected the RT in all 15 limbs indicating superficial venous incompetence. In the controls, one of 33 limbs (3%) had a venous RT between 25 and 40 s. An above-knee tourniquet corrected the RT indicating superficial venous incompetence. Conclusion: Covert venous insufficiency may complicate primary lymphoedema. This has implications for both the pathophysiology of the oedema and its management.

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6

Tatnall, F. M., and I. Sarkany. "Primary Facial Lymphoedema with Xanthomas." Journal of the Royal Society of Medicine 81, no. 2 (February 1988): 113–14. http://dx.doi.org/10.1177/014107688808100224.

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7

Dale, R. F. "The inheritance of primary lymphoedema." Journal of Medical Genetics 22, no. 4 (August 1, 1985): 274–78. http://dx.doi.org/10.1136/jmg.22.4.274.

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8

Jacobsen, J. O. G., and C. Eggers. "Primary lymphoedema in a kitten." Journal of Small Animal Practice 38, no. 1 (January 1997): 18–20. http://dx.doi.org/10.1111/j.1748-5827.1997.tb02980.x.

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9

Gajraj, H., S. G. E. Barker, K. G. Burnand, and N. L. Browse. "Lymphangiosarcoma complicating chronic primary lymphoedema." British Journal of Surgery 74, no. 12 (December 1987): 1180. http://dx.doi.org/10.1002/bjs.1800741232.

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10

Yüksel, Ahmet, Orçun Gürbüz, Yusuf Velioğlu, Gencehan Kumtepe, and Sefa Şenol. "Management of lymphoedema." Vasa 45, no. 4 (July 2016): 283–91. http://dx.doi.org/10.1024/0301-1526/a000539.

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Abstract. Lymphoedema is a common and progressive disease which causes deterioration of the quality of life of patients. It is divided into two groups: primary and secondary lymphoedema. Nowadays, the majority of patients with lymphoedema are associated with a malignancy or its treatment modalities, such as cancer surgery and radiation therapy. Accurate diagnosis and effective treatment are crucial for alleviating the symptoms, preventing progression and reducing the potential risks of lymphoedema. This report provides an overview of the management of lymphoedema.

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11

Gordon, Kristiana, Ruth Varney, Vaughan Keeley, Katie Riches, Steve Jeffery, Malou Van Zanten, Peter Mortimer, Pia Ostergaard, and Sahar Mansour. "Update and audit of the St George’s classification algorithm of primary lymphatic anomalies: a clinical and molecular approach to diagnosis." Journal of Medical Genetics 57, no. 10 (May 14, 2020): 653–59. http://dx.doi.org/10.1136/jmedgenet-2019-106084.

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Primary lymphatic anomalies may present in a myriad of ways and are highly heterogenous. Careful consideration of the presentation can lead to an accurate clinical and/or molecular diagnosis which will assist with management. The most common presentation is lymphoedema, swelling resulting from failure of the peripheral lymphatic system. However, there may be internal lymphatic dysfunction, for example, chylous reflux, or lymphatic malformations, including the thorax or abdomen. A number of causal germline or postzygotic gene mutations have been discovered. Some through careful phenotyping and categorisation of the patients based on the St George’s classification pathway/algorithm. The St George’s classification algorithm is aimed at providing an accurate diagnosis for patients with lymphoedema based on age of onset, areas affected by swelling and associated clinical features. This has enabled the identification of new causative genes. This update brings the classification of primary lymphatic disorders in line with the International Society for the Study of Vascular Anomalies 2018 classification for vascular anomalies. The St George’s algorithm considers combined vascular malformations and primary lymphatic anomalies. It divides the types of primary lymphatic anomalies into lymphatic malformations and primary lymphoedema. It further divides the primary lymphoedema into syndromic, generalised lymphatic dysplasia with internal/systemic involvement, congenital-onset lymphoedema and late-onset lymphoedema. An audit and update of the algorithm has revealed where new genes have been discovered and that a molecular diagnosis was possible in 26% of all patients overall and 41% of those tested.

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12

Gulati, Shrea, Sheragaru Hanumanthappa Chandrashekhara, Sandeep Bhoriwal, and Snehal Ishwar Kose. "Chronic lymphoedema: a nidus for squamous cell carcinoma." BMJ Case Reports 15, no. 5 (May 2022): e248543. http://dx.doi.org/10.1136/bcr-2021-248543.

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Lymphoedema is a chronic debilitating condition characterised by diffuse swelling caused by lymphatic obstruction. The secondary form of lymphoedema is more common than the primary form. Untreated filariasis remains an important cause of lymphoedema in developing countries. The most common complication of chronic lymphoedema is cellulitis. It is also a risk factor for the development of neoplasms such as lymphangiosarcoma, squamous cell carcinoma, melanoma, lymphoma and malignant fibrous histiocytoma. We report a case of a woman in her 60s who developed squamous cell carcinoma in the background of chronic lymphoedema.

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13

Garge, Saurabh, Anupama Negi, VishalK Jain, and Sangram Singh. "Saxophone penis due to primary lymphoedema." Journal of Indian Association of Pediatric Surgeons 14, no. 4 (2009): 230. http://dx.doi.org/10.4103/0971-9261.59611.

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14

Tatnall, F. M., and I. Sarkany. "(34) Primary facial lymphoedema with xanthomas." British Journal of Dermatology 119, s33 (July 1988): 90. http://dx.doi.org/10.1111/j.1365-2133.1988.tb05437.x.

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15

Ozyurt, Abdullah, Eylem Sevinc, Ali Baykan, Duran Arslan, Mustafa Argun, Ozge Pamukcu, and Kazim Uzum. "Variable clinical presentation in primary lymphoedema." Clinical Dysmorphology 23, no. 3 (July 2014): 83–87. http://dx.doi.org/10.1097/mcd.0000000000000036.

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16

Wright, N. B., and H. M. Carty. "The swollen leg and primary lymphoedema." Archives of Disease in Childhood 71, no. 1 (July 1, 1994): 44–49. http://dx.doi.org/10.1136/adc.71.1.44.

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17

Barral Mena, Estefanía, María Soriano-Ramos, Maria Rosa Pavo García, Lucía Llorente Otones, and Jaime de Inocencio Arocena. "Primary lymphoedema outside the neonatal period." Anales de Pediatría (English Edition) 85, no. 1 (July 2016): 47–49. http://dx.doi.org/10.1016/j.anpede.2016.01.005.

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18

Dai, Ting, Bohan Li, Bo He, Liwei Yan, Liqiang Gu, Xiaolin Liu, Jian Qi, Ping Li, and Xiang Zhou. "A novel mutation in the conserved sequence of vascular endothelial growth factor receptor 3 leads to primary lymphoedema." Journal of International Medical Research 46, no. 8 (June 13, 2018): 3162–71. http://dx.doi.org/10.1177/0300060518773264.

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Objective To investigate whether lymphoedema in a Chinese family showed the hereditary and clinical characteristics of Milroy disease, an autosomal dominant form of congenital lymphoedema, typically characterized by chronic lower limb tissue swelling due to abnormal lymphatic vasculature development, and to perform mutational analyses of vascular endothelial growth factor receptor ( VEGFR)3. Methods Individuals from a three-generation family affected by congenital lymphoedema were clinically assessed for Milroy disease. Mutation analysis of VEGFR3 was performed using DNA from family members and healthy controls. Results Out of 20 family members, eight were diagnosed with hereditary lymphoedema. Mutation analyses revealed a novel mutation site for c.3163 G>A, resulting in a p.1055D>N mutation in the second tyrosine kinase domain of VEGFR3, which was present in affected individuals only (absent in all unaffected family members and 130 healthy controls). Computed functional analyses showed the mutation may lead to structural alterations with a probability of 0.99999 of being disease causing. Conclusion A novel mutation associated with Milroy disease was identified in a Chinese family, expanding our knowledge of VEGFR3 gene function and providing a potential molecular target for treating hereditary lymphoedema.

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19

Pike, Cheryl, Melanie J. Thomas, Rhian Noble-Jones, Elizabeth Coveney, and Karl Hocking. "Audit to strategy: development of a national children and young people lymphoedema service." British Journal of Child Health 3, no. 2 (April 2, 2022): 67–75. http://dx.doi.org/10.12968/chhe.2022.3.2.67.

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Lymphoedema in children and young people (CYP) can cause significant impact affecting physical, psychological and social wellbeing. This audit of 286 CYP with Lymphoedema (2015–2018) is the first national cohort reported and provides new information on patient reported outcome (PROM) changes over time. Conservative therapy produced statistically significant change in outcome measures relating to swelling, infection, appearance and compression garments. Almost half of the children had primary lymphoedema of varying types. An overall prevalence of 31 per 100 000 CYP with lymphoedema was found among a population aged 0–25 over a 3-year period. This finding suggests a higher occurrence of lymphoedema in children and young people than previously reported and is important for service planning and health professionals' education.

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20

Fassiadis, N., and N. Law. "Unilateral Congenital Foot Lymphoedema with Hypoplastic Toes." Phlebology: The Journal of Venous Disease 17, no. 2 (June 2002): 70–71. http://dx.doi.org/10.1177/026835550201700207.

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Congenital lymphoedema causes limb swelling from impaired lymph drainage and it can predispose patients to infection and rarely to cancer. We report a case of unilateral primary congenital lymphoedema of the foot associated with hypoplastic toes, which has not been documented in the literature before.

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21

Alexander, Maggie. "Primary care trusts should bolster lymphoedema services." Nursing Standard 23, no. 46 (July 22, 2009): 33. http://dx.doi.org/10.7748/ns.23.46.33.s46.

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22

Zenciroglu, Aysegül, Aysin Uckun, Cigdem Akincioglu, Mustafa ÜnlÜ, and Tahsin Tezic. "Primary congenital lymphoedema with unilateral arm involvement." European Journal of Pediatrics 160, no. 6 (May 25, 2001): 402–3. http://dx.doi.org/10.1007/s004310100736.

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23

Planinsek Rucigaj, Tanja, Matija Rijavec, Jovan Miljkovic, Julij Selb, and Peter Korosec. "A novel mutation in the FOXC2 gene: a heterozygous insertion of adenosine (c.867insA) in a family with lymphoedema of lower limbs without distichiasis." Radiology and Oncology 51, no. 3 (July 6, 2017): 363–68. http://dx.doi.org/10.1515/raon-2017-0026.

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Abstract Background Primary lymphoedema is a rare genetic disorder characterized by swelling of different parts of the body and highly heterogenic clinical presentation. Mutations in several causative genes characterize specific forms of the disease. FOXC2 mutations are associated with lymphoedema of lower extremities, usually distichiasis and late onset. Patients and methods Subjects from three generations of a family with lymphoedema of lower limbs without distichiasis were searched for mutations in the FOXC2 gene. Results All affected family members with lymphoedema of lower limbs without distichiasis, and still asymptomatic six years old girl from the same family, carried the same previously unreported insertion of adenosine (c.867insA) in FOXC2. Conclusions Identification of a novel mutation in the FOXC2 gene in affected family members of three generations with lymphoedema of lower limbs without distichiasis, highlights the high phenotypic variability caused by FOXC2 mutations.

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24

Mouaddine, Khadija, Mouna Sabib, Laila Benbella, Najat Lamalmi, and Nezha Mouane. "Waldmann’s Disease Revealed by Chronic Diarrhea and Lymphedema: Case Report." Archives of Medical Case Reports and Case Study 5, no. 2 (January 10, 2022): 01–04. http://dx.doi.org/10.31579/2692-9392/085.

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Intestinal lymphangiesctasia (IL) is a rare disease characterized by the dilation of intestinal lymphatics. It can be classified as primary or secondary depending on the underlying etiology. Usually seen in childhood or adolescence, chronic diarrhea and diffuse edema are the main clinical manifestations of the disease. True lymphoedemas can also be present and affect the lower and upper limbs, they are concomitant with the diagnosis or occur during the course. The diagnosis is based on the visualization of duodenal lymphangiesctasia. The primitive nature of the disease being affirmed by the elimination of the diseases causing secondary lymphangiectasias. Treatment is based on a strict hypo lipid diet enriched with medium chain triglycerides (MCTs). We present through this work the observation of a 12-month-old infant who presented with primary intestinal lymphangiesctasia revealed by chronic diarrhea with lymphoedema of the left upper limb.

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25

Vemulakonda, Sri Hari Priya, Naveen Kumar Gaur, Oseen Hajilal Shaikh, and Uday Shamrao Kumbhar. "Lymphoedema praecox in a young woman: a rare disease." BMJ Case Reports 14, no. 9 (September 2021): e245353. http://dx.doi.org/10.1136/bcr-2021-245353.

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Primary lymphoedema is a rare disorder. Often presents at a young age with asymptomatic limb oedema with gradual progression. We present a 16-year-old woman who presented with a history of swelling of the left lower limb for 6 years. There was the presence of isolated left lower limb oedema, which was a non-pitting type. The patient underwent imaging studies and was diagnosed to have primary lymphoedema. The patient was managed conservatively as the patient did not have any other problems other than the left lower limb oedema.

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26

Vilaça, Beatriz, João Pazello, Adria Farias, Leilane Oliveira, Miguel Andrade, Andre Dias, Miguel Agulham, and Camila Fachin. "Congenital chylothorax and primary lymphoedema: a case report." Residência Pediátrica 9, no. 3 (2019): 344–46. http://dx.doi.org/10.25060/residpediatr-2019.v9n3-32.

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27

Bailey, Jed. "A personal history of living with primary lymphoedema." British Journal of Community Nursing 13, Sup5 (October 2008): S28—S29. http://dx.doi.org/10.12968/bjcn.2008.13.sup5.31194.

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28

Harmer, Victoria. "Breast cancer-related lymphoedema: implications for primary care." British Journal of Community Nursing 14, Sup5 (October 2009): S15—S19. http://dx.doi.org/10.12968/bjcn.2009.14.sup5.44505.

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29

Harding, Jane E. "The experiences of young people with primary lymphoedema." British Journal of Community Nursing 17, Sup10 (October 2012): S4—S11. http://dx.doi.org/10.12968/bjcn.2012.17.sup10.s4.

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30

Illsley's, Joanne. "Patient experience: raising a child with primary lymphoedema." British Journal of Nursing 27, no. 12 (June 28, 2018): 678–80. http://dx.doi.org/10.12968/bjon.2018.27.12.678.

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31

Sigstad, H., Ø. Aagenaes, R. W. Bjørn-Hansen, and K. Rootwelt. "PRIMARY LYMPHOEDEMA COMBINED WITH HEREDITARY RECURRENT INTRAHEPATIC CHOLESTASIS." Acta Medica Scandinavica 188, no. 1-6 (April 24, 2009): 213–19. http://dx.doi.org/10.1111/j.0954-6820.1970.tb08028.x.

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32

Mullinger, K., R. S. Rampaul, S. Holmes, J. Cid, R. D. Macmillan, and R. W. Blamey. "Lymphoedema rate following treatment for primary breast cancer." European Journal of Cancer 37 (September 2001): 14–15. http://dx.doi.org/10.1016/s0959-8049(01)80074-5.

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33

Costello, Mary, Zena Moore, Pinar Avsar, Linda Nugent, Tom O'Connor, and Declan Patton. "Non-cancer-related lower limb lymphoedema in complex decongestive therapy: the patient experience." Journal of Wound Care 30, no. 3 (March 2, 2021): 225–33. http://dx.doi.org/10.12968/jowc.2021.30.3.225.

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Objective: Lymphoedema is a debilitating condition that results in the swelling of one or more limbs, leading to a significant impairment of mobility. From a psychosocial perspective, body image and quality of life (QoL) are also adversely affected. To date, non-cancer lymphoedema has been under-researched. The primary objectives of this study were to explore QoL and limb volume in patients with primary and secondary non-cancer-related lower limb lymphoedema during the intensive, maintenance and self-care phases of complex decongestive therapy over a 24-week period. Secondary objectives included exploring patients' experiences of living with lymphoedema and the challenges of self-care maintenance. Method: A sequential mixed methodology, using quantitative and qualitative methods, was used. The quantitative part of the study involved the use of tape measurements at 4cm intervals to ascertain limb volume alterations. Measurements were taken during the intensive phase at weeks 1, 2, 3 and 4; and during the maintenance phase at weeks 8, 16 and 24. QoL was measured using the lymphoedema QoL (LYMQOL) leg, at baseline and weeks 8 and 24. The qualitative part of the study involved the use of semi-structured interviews with patients at week 8. Interviews explored the experiences of patients living with lymphoedema and their experiences of complex decongestive therapy. Results: A total of 20 patients were recruited, two male and 18 female, with a mean age of 58.8 years (standard deviation (SD): 16 years), and all receiving complex decongestive therapy. At baseline, for all patients, the mean QoL score was 4.3 (SD: 2.4); and 8 (SD: 1.5) after 8 weeks of complex decongestive therapy. Mean difference was 4.00 (95% confidence interval: 2.76–5.24; p=0.00001) indicating a positive treatment effect. Limb volume measurements demonstrated a reduction in oedema from baseline to week 8. However, there were fluctuations in limb volume over the maintenance period of four months after treatment as the patients endeavoured to engage in self-care. Analysis of the interviews identified themes pertaining to lack of knowledge of lymphoedema among health professionals, a negative impact of living with lymphoedema and the positive therapeutic effect of complex decongestive therapy. Conclusion: Lower limb lymphoedema is a chronic condition that impacts negatively on the individual. Complex decongestive therapy improves QoL and reduces oedema. However, given the fluctuations in oedema post-treatment, individuals need greater support to maintain active engagement in effective self-care strategies.

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34

Bottyán, Krisztina, Ekaterine Paschali, Erika Varga, Irma Korom, István Németh, Réka Németh, Klára Piukovics, and Judit Oláh. "Thoracic lymphoedema caused by primary mediastinal large B-cell lymphoma." Bőrgyógyászati és Venerológiai Szemle 90, no. 4 (August 29, 2014): 169–72. http://dx.doi.org/10.7188/bvsz.2014.90.4.8.

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35

Ghalamkarpour, A., W. Holnthoner, P. Saharinen, L. M. Boon, J. B. Mulliken, K. Alitalo, and M. Vikkula. "Recessive primary congenital lymphoedema caused by a VEGFR3 mutation." Journal of Medical Genetics 46, no. 6 (March 15, 2009): 399–404. http://dx.doi.org/10.1136/jmg.2008.064469.

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36

Keeley, Vaughan. "Advances in understanding and management of lymphoedema (cancer, primary)." Current Opinion in Supportive and Palliative Care 11, no. 4 (December 2017): 355–60. http://dx.doi.org/10.1097/spc.0000000000000311.

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37

Blamey, R. W. "Morbidity from lymphoedema following treatment of primary breast cancer." European Journal of Cancer 39, no. 15 (October 2003): 2113. http://dx.doi.org/10.1016/s0959-8049(03)00424-6.

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38

Raynor, Colin R., and J. H. N. Wolfe. "The prognosis and possible cause of severe primary lymphoedema." Plastic and Reconstructive Surgery 79, no. 4 (April 1987): 677. http://dx.doi.org/10.1097/00006534-198704000-00074.

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39

Todd, Marie, John Welsh, and Deirdre Moriarty. "The experience of parents of children with primary lymphoedema." International Journal of Palliative Nursing 8, no. 9 (September 2002): 444–51. http://dx.doi.org/10.12968/ijpn.2002.8.9.10689.

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40

Wigg, Jane. "Genital lymphoedema and its hidden impact: a treatment guide." British Journal of Community Nursing 27, Sup4 (April 1, 2022): S32—S38. http://dx.doi.org/10.12968/bjcn.2022.27.sup4.s32.

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Genital lymphoedema is recognised as a rare consequence of cancer surgery or its treatment, which also occurs with primary lymphoedema. Over recent years, there has been an increased understanding of the condition; however, good assessment, early recognition and effective management are crucial to prevent the progression and improve the lives of those with this condition. Treatment needs to be individualised and the psychosocial and emotional aspects of the condition need to be carefully considered to ensure that the patient can achieve successful outcomes with realistic expectations.

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41

Kaur, Maninder, Dinesh P. Asati, Ujjawal Khurana, and Suman Patra. "Unilateral lymphoedema of lower limb: an unusual presenting feature of hidden tuberculosis verrucosa cutis of foot." BMJ Case Reports 13, no. 11 (November 2020): e236587. http://dx.doi.org/10.1136/bcr-2020-236587.

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A 42-year-old Indian farmer presented with gradually progressive swelling of the right lower limb for the last 20 years. There were few verrucous plaques over the right foot for the same duration. Those plaques were initially ignored and mistaken as lymphoedema-induced secondary changes by primary care physicians. Histopathology of the skin lesion showed pseudoepitheliomatous hyperplasia with upper dermal granulomatous infiltrate and a diagnosis of tuberculosis verrucosa cutis was suspected. Subsequently, the lesions as well as lymphoedema improved significantly with antitubercular therapy.

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42

Stanisić, M.-G., M. Gabriel, and K. Pawlaczyk. "Intensive decongestive treatment restores ability to work in patients with advanced forms of primary and secondary lower extremity lymphoedema." Phlebology: The Journal of Venous Disease 27, no. 7 (December 12, 2011): 347–51. http://dx.doi.org/10.1258/phleb.2011.011044.

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Objective To show that adequate therapy for lymphoedema is able to restore ability to work. Materials and Methods The population of patients with primary lymphoedema registered in the university clinical centre diagnosed with primary or secondary lymphoedema and presumed by the national social institution as completely unable to work was selected for the retrospective analysis and divided into two groups. Group 1 consisted of 25 patients treated with a complex decongestive therapy programme daily for 3–6 weeks. The study population comprised 19 women and six men from 14 to 61 years of age (mean 31.5). In all 25 patients, complete inability to work was certified by the social institution before the treatment started. Group 2 consisted of 47 patients, 14 men and 33 women, aged from 26 to 71 years (mean 39 years) treated by so-called standard methods, who resigned from the proposed intensive treatment. In all 47 patients, complete inability to work was declared by the social institution before the treatment. Ability to work and oedema reduction were assessed by the treating physician. Results The intensive phase of treatment succeeded in 3870–15,330 mL oedema reduction in Group 1. After the end of therapy, 21 patients were able to work or study without any limitation and patients returned to their regular professional activity. Among four others, two were on welfare for at least 10 years, for another one welfare was their only income and one person was receiving a social pension. In none of the patients from group 2 was any significant oedema reduction observed. Every patient from group 2 maintained the social pension due to ineffective treatment. Conclusions Complex decongestive therapy is a very efficient form of treatment in advanced primary and secondary lymphoedema. It allows returning to work after a short period of temporary disability without the necessity of a social pension.

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Cooper-Stanton, Garry. "Compression therapy and heart failure: a scoping review of the existing evidence." British Journal of Community Nursing 27, no. 3 (March 2, 2022): 128–34. http://dx.doi.org/10.12968/bjcn.2022.27.3.128.

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The completion of a scoping review within the area of compression therapy and heart failure offers an insight into the present literature in this area, alongside offering the ability to connect this existing knowledge to chronic oedema/lymphoedema when both conditions co-exist. The evidence obtained included national agreed guidelines, consensus documents and existing primary/secondary research. The review identified existing evidence that suggests that the application of compression therapy in those with heart failure may be appropriate, but is dependent upon staging and stability. However, this needs to be contextualised against other co-morbidities, such as lymphoedema, which may impact upon the exact compression therapy and level applied. Further research within the area of heart failure in combination with chronic oedema/lymphoedema would expand the existing evidence base. This is set against a need for further consensus guidance to bridge the gap that exists within the literature.

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Pujol-Blaya, Vicenta, Sira Salinas-Huertas, M. Luisa Catasús, Teresa Pascual, and Roser Belmonte. "Effectiveness of a precast adjustable compression system compared to multilayered compression bandages in the treatment of breast cancer–related lymphoedema: a randomized, single-blind clinical trial." Clinical Rehabilitation 33, no. 4 (January 4, 2019): 631–41. http://dx.doi.org/10.1177/0269215518821785.

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Objective: To compare the effectiveness of a precast adjustable compression system with that of multilayered compression bandages in the treatment of breast cancer–related lymphoedema. Design: Multicenter, randomized, single-blind parallel-group clinical trial. Setting: The rehabilitation services of four general university hospitals. Subjects: Patients with upper limb breast cancer–related lymphoedema. Interventions: All the patients received manual lymphatic drainage, followed by a precast adjustable compression system or multilayered compression bandages, according to the group allocation. The treatment included 10 consecutive sessions over a two-week period from Monday to Friday, followed by some sessions on three alternate days per week, until the patient received a tailored compression garment. Primary measurements: The patients were evaluated just before the treatment, after 10 sessions and at three months posttreatment. The primary outcome was the change in excess lymphoedema volume. Secondary outcomes were changes in the symptoms of pain, heaviness, tightness and hardness. Analyses were performed using an intention-to-treat approach. Results: In all, 42 patients were included; there were 22 in the precast adjustable compression system group and 20 in the multilayered compression bandages group. Both groups exhibited significant decreases in excess volume and symptoms after 10 sessions and at three months. There were no significant differences regarding excess volume or symptoms between the precast adjustable compression system and multilayered compression bandages groups after 10 sessions and at the three-month follow-up exam. Conclusion: The precast adjustable compression system and the multilayered compression bandages have similar efficacy for the reduction of excess lymphoedema volume or symptoms.

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Pigott, Amanda, Bena Brown, Megan Trevethan, Sandra Porceddu, Andrew McCann, Rachael Pitt, and Jodie Nixon. "Head and neck lymphoedema—research challenges during the COVID-19 pandemic." British Journal of Community Nursing 26, Sup10 (October 1, 2021): S6—S15. http://dx.doi.org/10.12968/bjcn.2021.26.sup10.s6.

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Management of secondary head and neck lymphoedema has undergone little research investigation. Its treatment is time and labour intensive and involves multiple therapeutic modalities without a clear understanding of which is most effective. This study aimed to determine the feasibility of a randomised controlled trial comparing two therapeutic modalities to manage head and neck lymphoedema. The secondary objective was to evaluate the clinical effects of these treatments. Participants were randomised to receive treatment with manual lymphatic drainage or compression over 6 weeks, with the primary outcome—percentage tissue water—measured 12 weeks after treatment. Six participants were recruited until the study was ceased due to restrictions imposed by the COVID-19 pandemic. Some 86% of required attendances were completed. Percentage tissue water increased in all participants at 12 weeks. No consistent trends were identified between internal and external lymphoedema. The small number of people recruited to this study informs its feasibility outcomes but limits any conclusions about clinical implications.

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Perez, A., S. E. Clements, E. Benton, A. Robson, B. Bhogal, C. M. Stefanato, and D. McGibbon. "Localized bullous pemphigoid in a patient with primary lymphoedema tarda." Clinical and Experimental Dermatology 34, no. 8 (December 2009): e931-e933. http://dx.doi.org/10.1111/j.1365-2230.2009.03722.x.

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Vidal, F., M. Arrault, and S. Vignes. "Paediatric primary lymphoedema: a cohort of 155 children and newborns." British Journal of Dermatology 175, no. 3 (June 23, 2016): 628–31. http://dx.doi.org/10.1111/bjd.14556.

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Karg, E., C. Bereczki, J. Kovacs, I. Korom, A. Varkonyi, P. Megyeri, and S. Turi. "Primary lymphoedema associated with xanthomatosis, vaginal lymphorrhoea and intestinal lymphangiectasia." British Journal of Dermatology 146, no. 1 (January 2002): 134–37. http://dx.doi.org/10.1046/j.1365-2133.2002.04477.x.

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Wahab, Afthab Jameela, Anandan V, Saradha K P, and Nithya Baskar. "CONGENITAL MULTISEGMENTAL LYMPHATIC DYSPLASIA WITH SYSTEMIC INVOLVEMENT – EVALUATING PRIMARY LYMPHOEDEMA." Journal of Evolution of Medical and Dental Sciences 4, no. 100 (December 14, 2015): 16580–83. http://dx.doi.org/10.14260/jemds/2015/2469.

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Karkkainen, Marika J., Robert E. Ferrell, Elizabeth C. Lawrence, Mark A. Kimak, Kara L. Levinson, Michele A. McTigue, Kari Alitalo, and David N. Finegold. "Missense mutations interfere with VEGFR-3 signalling in primary lymphoedema." Nature Genetics 25, no. 2 (June 2000): 153–59. http://dx.doi.org/10.1038/75997.

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