Background: Primary lymphedema occurs as a result of genetic abnormalities of the lymph system. Currently, complex decongestive therapy is accepted as the standard treatment of the lymphedema. In this case presentation, we described the management of bilateral primary lower extremity lymphedema and the use of complex decongestive therapy.
Case Report: A 62 years old female patient had stage III primary lymphedema on her left lower extremity and stage II primary lymphedema on her right lower extremity. The patient, who had morbid obesity, also had untreatable sleep apnea, urinary incontinence, umbilical hernia and hypertension controlled by drugs. She had stage 4 gonarthrosis according to Kellgren – Lawrence classification in her both knees. The patient received complex decongestive therapy as an outpatient.
After 27 sessions of complex decongestive therapy, edema reduced in both lower extremities. Before the treatment started, the patient couldn’t go up and down stairs, get out and had difficulty mobility in the home. But after the treatment, the patient could go up and down 16 stairs by holding the railing, get out by two walking sticks and had less difficulty mobility in the home. However, due to gonarthrosis in her knees, her pain did not diminish.
Conclusion: Complex decongestive therapy is effective in the management of bilateral primary lower extremity lymphedema, which progressed with multiple health conditions.
The lymphatic system consists of small non-contractile lymph vessels which collect fluid from the interstitial space and carry it to the major contractile lymphatic vessels with valves that maintain a unidirectional flow to the lymph nodes and is responsible for returning proteins, lipids and water from the interstitium to the venous system near the junction of the subclavian vein and internal jugular vein on both sides. Lymphedema would be the result of the failure of transport with the further accumulation of fluid rich in protein at the interstitium, especially in limbs.
Primary lymphedema, which affects 1.15 / 100000 children (especially pre-pubescent girls), not always clinically evident at birth, is a genetic disorder that represents many different subsets of phenotypes: congenital or congenital lymphangiectasia, congenital trofoedema family or Meige, congenital amniotic band and essence or idiopathic.
The most common form of presentation is due to congenital absence or abnormality of the lymphatic tissue caused by a genetic mutation of the genes responsible for the development of lymphatic vessels that is characterized by the difficulty of draining lymph vessels. This genetic alteration may be sporadic or inherited.
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