A 10-year-old female came to clinic for her health maintenance examination. She was a survivor of childhood leukemia and it was 3 years after her last chemotherapy. She was followed by her oncologist and did not have any obvious side effects of her treatment other than some mild lymphedema. It had begun about 15 months after a lymph node biopsy in her left inguinal area and was relatively mild. She was using a compression garment most days and was followed by the pediatric surgeons. She was current on all of her immunizations. The review of systems was negative.
The pertinent physical exam showed a healthy female with normal vital signs and growth parameters were 50-75%. She had no obvious lymphadenopathy, masses or hepatosplenomegaly. She had well-healed scars in her upper right chest from a port placement, and a scar in her left inguinal area. Her left foot and calf were mildly enlarged compared to her right (3 cm difference in calf size) with a symmetric outline of the anatomical structures. There was no pitting edema and pulses were strong and symmetric. The rest of her examination was normal.
The diagnosis of a healthy girl who was a cancer survivor with mild lymphedema was made. The pediatrician reiterated that it was important to continue to use the compression garments all or most of the time to help with the lymphedema. He also reminded the girl to make sure she wore shoes to protect her feet.
Lymphedema occurs because of abnormal development or damage to the lymphatic structures. It is a chronic often progressive swelling of tissues starting distally and advancing more proximally. Extremities are the most common sites followed by genitalia. Fluid accumulation in the interstitial, superficial spaces causes adipose deposition and fibrosis. This causes the lymphedematous tissue to then enlarge. Edema is pitting early on and later is non-pitting.
Causes of lymphedema can be primary (~1%) or secondary (~99%). Primary lymphedema has been associated with several genetic mutations. Milroy disease is a primary lower extremity lymphedema that occurs in infants with a positive family history or who have a positive mutation for VEGFR3. Familial lymphedema with onset in the adolescent age group is called Meige disease and its mutation is not known at this time.
Secondary lymphedema is most common after trauma (e.g. penetrating trauma, lymph node excision, radiation) or infectious disease (especially the round worms of Filarioidea family).
The term “lymphedema” is often generically used for causes of extremity overgrowth or misdiagnosed for other vascular anomalies. For a review of overgrowth syndromes click here. A review of lymphatic malformations can also be found here.
In addition to history and physical examination, genetic studies can be done and lymphoscintigraphy is considered the standard for lymphatic function evaluation.
Complications of lymphedema include an increased risk of infections, skin changes (often thickening) and potentially but rarely malignancy. Patients with lymphedema do not have skin ulceration but do have an increased risk of skin infections often with Streptococcus species.
Lymphedema can’t be cured but can be treated. Treatment is conservative if at all possible. The affected area should have good skin hygiene of washing/drying and moisturizing to help prevent infections. Protective clothing is also helpful, especially wearing long pants/sleeves and foot wear or gloves. Compression garments (usually custom made) are a main treatment modality and can help to reduce the extremity volume and help to slow the disease progression. Patients need enough garments so they can be laundered regularly and they need to be replaced when worn (usually every 6 months). Other compression treatments can be offered including using pneumatic pumps. Regular exercise to maintain conditioning and normal body mass also helps treatment. Muscle contraction is the primary way that lymph fluid is transported centrally to the body. Elevation of the extremity when non weight bearing and when convenient to the patient can also help. Specific massage techniques from a trained professional can also provide some relief but can be time consuming and expensive.
Surgical treatments are used when the lymphedema is causing significant morbidity. They include lipectomy to remove adipose tissue and skin/subcutaneous resections. Microsurgical techniques can also be used to help reconstruct or reconnect lymphatic drainage.
Questions for Further Discussion
1. How is lymphedema different than generalized edema?
2. What are complications of cancer treatment that survivors need to be monitored for?
3. What are indications for surgical consultations?
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.
Information prescriptions for patients can be found at MedlinePlus for this topic: Lymphedema
To view current news articles on this topic check Google News.
To view images related to this topic check Google Images.
To view videos related to this topic check YouTube Videos.
Schook CC, Mulliken JB, Fishman SJ, Alomari AI, Grant FD, Greene AK. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg. 2011 Apr;127(4):1571-81.
Blatt J, Powell CM, Burkhart CN, Stavas J, Aylsworth AS. Genetics of hemangiomas, vascular malformations, and primary lymphedema. J Pediatr Hematol Oncol. 2014 Nov;36(8):587-93.
Maclellan RA, Greene AK. Lymphedema. Semin Pediatr Surg. 2014 Aug;23(4):191-7.
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa Children’s Hospital