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Published on: November 2, 2019
Paediatric lymphoedema: A retrospective chart review of 86 cases
Hilary Watt1, Davinder Singh-Grewal2,3,4, Orli Wargon1,2
1School of Women's and Children's Health, University of New South Wales, Sydney, New South Wales, Australia.
Insights
Pediatric lymphoedema is typically primary, affecting lower limbs in infancy. Diagnosis is often delayed, but magnetic resonance imaging (MRI) aids effective assessment and management.
Area of Science:
- Pediatric Medicine
- Vascular Medicine
- Genetics
Background:
- Lymphoedema, a chronic condition involving fluid accumulation due to lymphatic system dysfunction, affects children, necessitating a clear understanding of its characteristics.
- Primary lymphoedema, often congenital, and secondary lymphoedema, resulting from other conditions, present unique challenges in pediatric care.
Purpose of the Study:
- To delineate the clinical features, diagnostic methods, treatment strategies, and outcomes of lymphoedema in a pediatric population.
- To identify patterns in the onset, location, and complications of pediatric lymphoedema.
- To evaluate the effectiveness of various diagnostic investigations and management approaches.
Main Methods:
- A retrospective chart review was conducted on 86 children diagnosed with lymphoedema at two tertiary pediatric hospitals.
- Data collected included demographics, clinical presentation, symptoms, complications, and treatments received.
- Telephone interviews supplemented missing information, and diagnostic investigations like lymphoscintigraphy, ultrasound, and MRI were analyzed.
Main Results:
- The cohort comprised 86 patients, with 93% having primary lymphoedema, predominantly affecting the lower limbs (94%) and presenting in infancy (60% of primary cases).
- Complications were frequent (73%), and diagnosis was often delayed (average 9 months).
- Magnetic resonance imaging (MRI) proved effective for diagnosis, especially when lymphoscintigraphy yielded inconclusive results.
Conclusions:
- Primary lymphoedema is more prevalent in children than secondary forms, typically manifesting in infancy with lower limb involvement.
- While diagnosis can be delayed, clinical assessment and MRI are effective tools.
- Management commonly involves conservative measures like compression garments and manual lymph drainage, with surgery reserved for specific cases.
Aim:
To define the clinical characteristics, investigations, management and outcomes of lymphoedema in a paediatric cohort.
Methods:
A retrospective chart review of children with lymphoedema seen at two tertiary paediatric hospitals since 1998. Telephone interviews with parents were performed when information was missing. Information recorded included demographic data, features of diagnosis and clinical presentation, symptoms, complications and treatment.
Results:
A total of 86 patients with lymphoedema were identified. Eighty cases (93%) were primary and six cases (7%) were secondary. Most were female (60%). Location of swelling was most commonly the lower limbs (94%). There were 13 cases (15%) of genital involvement. Swelling presented in the first 12 months of life in 60% of primary lymphoedema patients. Complications of lymphoedema occurred in 73% of patients. Lymphoscintigraphy was the most common investigation used (65%), followed by ultrasound (57%) and magnetic resonance imaging (MRI) (35%). Eight of the 48 (17%) lymphoscintigraphs produced a false negative result or were inconclusive with a correct diagnosis subsequently made clinically and using MRI. Average time to diagnosis was 9 months. Lymphoedema was managed with compression garments (99%), manual lymph drainage (97%) and multilayered bandaging (68%). Eight patients had an operative procedure as a part of management.
Conclusions:
Primary lymphoedema is more common than secondary lymphoedema in children. Onset tends to be during infancy for both males and females, and the lower limb is typically involved. Causes of secondary lymphoedema are diverse and rare. Diagnosis in children is often delayed but is possible based on history and physical examination alone and when further investigation is necessary MRI is effective.
