ReviewJournal of medical genetics2020
Update and audit of the St George's classification algorithm of primary lymphatic anomalies: a clinical and molecular approach to diagnosis.
Review in Journal of medical genetics, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers, 1 of them a synthesis that pooled it.
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Who cites it
40 citing papers in PubMed, 1 synthesis or guideline pooled it.
- A systematic review of indocyanine green lymphography imaging for the diagnosis of primary lymphoedema.The British journal of radiology · 2025Pooled it
- Lymphatic Endothelial Cells in Health and Disease.MedComm · 2026Review
- Insights into KIF11 pathogenesis in microcephaly-lymphedema-chorioretinopathy syndrome from a lymphatic perspective.JCI insight · 2026Article
- Further characterisation of immortalised human lymphatic endothelial cells to explore their transcriptomic profile and VEGFC response.Scientific reports · 2025Article
- Review
- PIEZO Force Sensing in Vascular Biology: An Explosion of New Knowledge, Concepts and Opportunity.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Review
- Review
- Exercise interventions for the treatment of lower limb lymphoedema after treatment for gynaecological cancers.The Cochrane database of systematic reviews · 2025Article
- Clinical Relevance of Animal Models of Lymphatic Dysfunction and Lymphedema.Microcirculation (New York, N.Y. : 1994) · 2025Review
- [Lymphatic malformations in childhood and adolescence].Radiologie (Heidelberg, Germany) · 2025Article
- Case Report: Surgical management of primary lymphedema with a novel PROX1 mutation involving upper and lower limbs.Frontiers in genetics · 2025Article
- The utility of dynamic contrast-enhanced intranodal magnetic resonance lymphangiography (MRL) in the investigation of primary lymphatic anomalies.Clinical radiology · 2024Article
- FLT4 causes developmental disorders of the cardiovascular and lymphovascular systems via pleiotropic molecular mechanisms.Cardiovascular research · 2024Article
- Etiology and treatment of cancer-related secondary lymphedema.Clinical & experimental metastasis · 2024Review
- Pathogenic variants in HGF give rise to childhood-to-late onset primary lymphoedema by loss of function.Human molecular genetics · 2024Article
- Congenital Vascular and Lymphatic Diseases.Circulation research · 2024Review
- AInternational journal of molecular sciences · 2024Article
- Lymphatic System and the Kidney: From Lymphangiogenesis to Renal Inflammation and Fibrosis Development.International journal of molecular sciences · 2024Review
- Review
- Erythematous capillary-lymphatic malformations mimicking blood vascular anomalies.JCI insight · 2023Article
Corrections and comments
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Authors and funding
9 authors.
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Abstract
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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