Primary Lymphedema

Say Goodbye to Compression Garments after Dr. Cheng’s Lymphedema Microsurgery

Primary lymphedema is the presence or development of lymphedema without any relation to any underlying medical condition. Primary lymphedema has a quoted incidence of approximately 1-3 births out of every 100,000 births, with a particular female preponderance to male ratio of 3.5:1 worldly. In North America, the incidence of primary lymphedema is approximately 1.15 births out of every 100,000 births.

Primary lymphedema can be classified depending on the age of onset of the patients: at infancy (birth to 1 year), during childhood (1-8 or 9 years), during adolescence (9-21 years), and lastly during adulthood (after 21 years). Mutations in VEGFR3 (Milroy disease), CCBE1 (Hennekam syndrome), SOX18 (hypotrichosis-telangiectasia-lymphedema), and FOXC2 (lymphedema distichiasis) are several eponymous conditions that present at birth and involve the development of lymphedema. Familial lymphedema of the lower extremities that presents itself during adolescence is known as Meige disease. Its underlying genetic abnormality is not known yet, but its familial nature and presentation at adolescence are characteristics of it.

Symptoms of Primary Lymphedema

Primary lymphedema often occurs at birth and for causes or by mechanisms that are unknown. When primary lymphedema becomes symptomatic in adulthood, these patients often have a long-standing history of lymphedema that is associated with the destruction of lymphatic channels. Adipogenesis or proliferation of adipose tissue coupled with dense fibrosis often results in severe lymphedema of the limb and a more severe presentation. Severe fibrosis occurs with long-standing lymphedema due to the accumulation of protein-rich fluid in the interstitial spaces coupled with inflammation repeated bouts of cellulitis.

Diagnosis of Primary Lymphedema

Pre-operative lymphoscintigraphy and indocyanine green (ICG) lymphography are used to detect the presence of any intact lymphatic channels. If a lymphatic duct is available at ICG lymphography, the patient is offered the lymphovenous anastomosis (LVA), whereas those without patent lymphatic ducts are indicated for vascularized lymph node transfers (VLNT). If the lymphoscintigraphy shown total obstruction, the patients are offered VLNT directly. Ultrasound Doppler is used to evaluate the concomitant vascular lesions at the proximal site. Single-photon emission computed tomography (SPECT) is indicated for those patients with suspected concomitant chylous ascites. MRI is prescribed for the evaluation of donor site lymph node basins of neck if the vascularized lymph node flap is indicated.

Treatment Of Primary Lymphedema

Both Vascularized lymph node transfers (VLNT) and lymphovenous anastomosis (LVA) are surgical treatments that have been proven effective in treating secondary lymphedema. VLNT involves the microsurgical transfer of lymph node-containing tissue to a lymphedematous limb, which works based on the movement of lymphatic fluid from the affected limb into the transferred lymph node and drainage via the newly anastomosed venous route.

We have reported a paper to compare the results of VLNT and LVA treatments of lymphedema. 80% of primary lymphedema required a VLNT for the functional recovery and 20% of primary lymphedema may undergo a LVA. At a mean follow-up of 20 months, mean circumferential reduction of limb circumference and episodes of cellulitis were 3.7 ± 2.9 cm and 1.9 ± 2.9 cm; 5.1 ± 2.8 times/year and 4.2 ±0.5 times/year in VLNT and LVA groups, respectively (p = 0.7). Improvements in overall score of the Lymphedema quality of life questionnaire (LYMQoL) (from 3.9 ± 1.2 to 6.4 ± 1.1, p < 0.05) in VLNT group had statistically significant difference than that (from 3.0 ± 1.4 to 5.0 ± 2.4, p=0.07) in LVA group. Both VLNT and LVA surgeries can effectively treat primary lymphedema patients. The reduction of above-knee circumference, body weight, episodes of cellulitis, and the improvement of LYMQoL was significantly greater in LVNT compared to LVA. All of the patients did not use compression garments post-operatively!


Recommended reading journal:

  1. Vascularized lymph node flap transfer and lymphovenous anastomosis for klippel-trenaunay syndrome with congenital lymphedema.
    Qiu SS, Chen HY, Cheng MH.
    Plast Reconstr Surg Glob Open. 2014 Jul 9;2(6):e167.
  2. Successful Outcomes of Vascularized Lymph Node Transfer and Lymphovenous Anastomosis for Treatment of Primary Lymphedema
    Charles, Cheng MH.
    Plast Reconstr Surg Glob Open. 2018 [Epub ahead of print]
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