References

Aarthun A, Øymar KA, Akerjordet K. How health professionals facilitate parents' involvement in decision-making at the hospital: A parental perspective. Journal of Child Health Care. 2018; 22:(1)108-121 https://doi.org/10.1177/1367493517744279

Brent AJ Sepsis. Medicine. 2017; 45:(10)649-653

British Lymphology Society. Population Needs Assessment. 2016. https://www.thebls.com/documents-library/population-needs-assessment-july-2016 (accessed 15 March 2021)

Burian EA, Karlsmark T, Franks PJ, Keeley V, Quéré I, Moffatt CJ. Cellulitis in chronic oedema of the lower leg: an international cross-sectional study. Br J Dermatol. 2021; 185:(1)110-118 https://doi.org/10.1111/bjd.19803

Clayton JA, Tannenbaum C. Reporting Sex, Gender, or Both in Clinical Research?. JAMA. 2016; 316:(18)1863-1864 https://doi.org/10.1001/jama.2016.16405

Colver A, Pearse R, Watson RM How well do services for young people with long term conditions deliver features proposed to improve transition?. BMC Health Serv Res. 2018; 18:(1) https://doi.org/10.1186/s12913-018-3168-9

Cooper G, Bagnall A. Prevalence of lymphoedema in the UK: focus on the southwest and West Midlands. Br J Community Nurs. 2016; S6-S14 https://doi.org/10.12968/bjcn.2016.21.Sup4.S6

Cucchi F, Rossmeislova L, Simonsen L, Jensen MR, Bülow J. A vicious circle in chronic lymphoedema pathophysiology? An adipocentric view. Obes Rev. 2017; 18:(10)1159-1169 https://doi.org/10.1111/obr.12565

Frank AP, de Souza Santos R, Palmer BF, Clegg DJ. Determinants of body fat distribution in humans may provide insight about obesity-related health risks. J Lipid Res. 2019; 60:(10)1710-1719 https://doi.org/10.1194/jlr.R086975

Freedman DS, Goodman AB, King RJ, Blanck HM. Tracking of obesity among 2-to 9-year-olds in an electronic heath record database from 2006 to 2018. Obes Sci Pract. 2020; 6:(3)300-306 https://doi.org/10.1002/osp4.407

Gabe-Walters M, Thomas M. Development of the Lymphoedema Patient Reported Outcome Measure (LYMPROM). Br J Nurs. 2021; 30:(10)592-598 https://doi.org/10.12968/bjon.2021.30.10.592

Gabriel P, McManus M, Rogers K, White P. Outcome Evidence for Structured Pediatric to Adult Health Care Transition Interventions: A Systematic Review. J Pediatr. 2017; 188:263-269.e15 https://doi.org/10.1016/j.jpeds.2017.05.066

Gordon K, Varney R, Keeley V Update and audit of the St George's classification algorithm of primary lymphatic anomalies: a clinical and molecular approach to diagnosis. J Med Genet. 2020; 57:(10)653-659 https://doi.org/10.1136/jmedgenet-2019-106084

Hanson CS, Newsom J, Singh-Grewal D, Henschke N, Patterson M, Tong A. Children and adolescents' experiences of primary lymphoedema: semistructured interview study. Arch Dis Child. 2018; 103:(7)675-682 https://doi.org/10.1136/archdischild-2017-313856

International Lymphoedema Framework. Care of Children with Lymphoedema. 2010. https://www.lympho.org/wp-content/uploads/2021/09/Care-of-Children-with-Lymphoedema.pdf (accessed 30 March 2022)

The diagnosis and treatment of peripheral lymphedema: 2013 consensus document of the International Society of Lymphology. Lymphology. 2013; 46:1-11

Mikhailovich K, Morrison P. Discussing childhood overweight and obesity with parents: a health communication dilemma. J Child Health Care. 2007; 11:(4)311-322 https://doi.org/10.1177/1367493507082757

Moffatt CJ, Murray SG. The experience of children and families with lymphoedema--a journey within a journey. Int Wound J. 2010; 7:(1)14-26 https://doi.org/10.1111/j.1742-481X.2010.00657.x

Moffatt C, Aubeeluck A, Stasi E A Study to Explore the Professional Conceptualization and Challenges of Self-Management in Children and Adolescents with Lymphedema. Lymphat Res Biol. 2019; 17:(2)221-230 https://doi.org/10.1089/lrb.2018.0076

Morgan PA, Franks PJ, Moffatt CJ. Health-related quality of life with lymphoedema: a review of the literature. Int Wound J. 2005; 2:(1)47-62 https://doi.org/10.1111/j.1742-4801.2005.00066.x

Phillips JJ, Gordon SJ. Conservative management of lymphoedema in children: a systematic review. J Pediatr Rehabil Med. 2014; 7:(4)361-372 https://doi.org/10.3233/PRM-140306

Public Health Wales NHS Trust. The case for action on obesity in Wales. 2018. http://www.wales.nhs.uk/sitesplus/documents/888/150119%20The%20case%20for%20action%20on%20obesity%20in%20Wales%200ae%20FINAL1.pdf (accessed 5 March 2021)

Quéré I, Stasi E, Mestre S, Roessler J, Roccatello D, Moffatt C. International Camps for Children with Lymphedema and Lymphatic Anomalies: When Education Links with Psychosocial Research. Lymphat Res Biol. 2021; 19:(1)36-40 https://doi.org/10.1089/lrb.2020.0095

Rodriguez JR, Hsieh F, Huang CT, Tsai TJ, Chen C, Cheng MH. Clinical features, microbiological epidemiology and recommendations for management of cellulitis in extremity lymphedema. J Surg Oncol. 2020; 121:(1)25-36 https://doi.org/10.1002/jso.25525

Sawyer SM, McNeil R, Francis KL The age of paediatrics. Lancet Child Adolesc Health. 2019; 3:(11)822-830 https://doi.org/10.1016/S2352-4642(19)30266-4

Schook CC, Mulliken JB, Fishman SJ, Grant FD, Zurakowski D, Greene AK. Primary lymphedema: clinical features and management in 138 pediatric patients. Plast Reconstr Surg. 2011; 127:(6)2419-2431 https://doi.org/10.1097/PRS.0b013e318213a218

Smeltzer DM, Stickler GB, Schirger A. Primary lymphedema in children and adolescents: a follow-up study and review. Pediatrics. 1985; 76:(2)206-218

StatsWales. Population estimates by local health boards and year. 2020. https://statswales.gov.wales/Catalogue/Population-and-Migration/Population/Estimates/Local-Health-Boards/populationestimates-by-lhb-age (accessed 4 March 2021)

Thomas MJ, Morgan K. The development of Lymphoedema Network Wales to improve care. Br J Nurs. 2017; 26:(13)740-750 https://doi.org/10.12968/bjon.2017.26.13.740

Todd J, Craig G, Todd M Audit of childhood lymphoedema in the United Kingdom undertaken by members of the Children's Lymphoedema Special Interest Group. Journal of Lymphoedema. 2014; 9:(2)14-19

Vignes S, Albuisson J, Champion L Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins). Orphanet J Rare Dis. 2021; 16:(1) https://doi.org/10.1186/s13023-020-01652-w

Welsh Government. Managing the transition from children's to adults' healthcare services. January 2020. 2020. https://gov.wales/sites/default/files/consultations/2020-01/consultation-document_1.pdf (accessed 21 February 2022)

Audit to strategy: development of a national children and young people lymphoedema service

02 April 2022
Volume 3 | British Journal of Child health · Issue 2

Abstract

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.

Lymphoedema in children and young people (CYP) can affect physical, psychological and social wellbeing and cause significant impact on daily life (Moffatt and Murray, 2010). Lymphoedema results from the failure of the lymphatic system to drain lymph fluid from the interstitial spaces (International Lymphoedema Framework [ILF], 2010). The term encompasses a range of symptoms including swelling, pain, decreased mobility and skin conditions (Morgan et al, 2005). CYP with lymphoedema have experienced issues with bullying, difficulty finding fashionable clothes and shoes that fit, altered personal relationships with family and in school (Hanson et al, 2018). Complicating factors include the risk of cellulitis and the psychosocial issues from having a visible but rare condition (Quéré et al, 2021).

As with adults, lymphoedema can be secondary to trauma or other pathologies but the majority in childhood are due to primary malformation and/or dysfunction of the lymphatic system (Gordon et al, 2020). Despite advances in the possibility of molecular and genetic diagnosis of primary lymphoedema (e.g. Milroy disease), diagnosis for many, particularly late-onset (e.g. Meige) or syndromic and systemic types (e.g. generalised lymphatic dysplasia), diagnosis for many, particularly late-onset or syndromic and systemic types, has been delayed by years (Gordon et al, 2020). True prevalence in CYP is unknown but for almost four decades, it has been based on an estimated average annual incidence of 1.15 per 100 000 (Smeltzer et al, 1985). Local variance may depend on study methods and regional service provision. For example, a regional comparison of overall prevalence (adults and children) in the West Midlands and Southwest of England found regional differences in prevalence (3.58 per 1 000 and 2.29 per 1 000 respectively) but in both regions children represented only 1% of the overall caseload (Cooper and Bagnall, 2016). However, as children services develop and accrue data, figures suggest that true incidence may be much higher (Todd et al, 2014).

Register now to continue reading

Thank you for visiting Journal of Child Health and reading some of our peer-reviewed resources for children’s health professionals. To read more, please register today. You’ll enjoy the following great benefits:

What's included

  • Limited access to our clinical or professional articles

  • New content and clinical newsletter updates each month