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lymphedema

Etiology: 1) primary a) congenital: Milroy's disease b) lymphedema praecox: - Meige's disease - onset at puberty c) lymphedema tarda: begins after age 35 d) associated disorders - Turner syndrome - Noonan syndrome - lymphangiectasia - lymphangiomyomatosis 2) secondary a) obesity [2] b) recurrent lymphangitis - most frequently due to Streptococci - common c) filariasis: most common cause world wide d) tuberculosis e) neoplasm - prostate cancer - lymphoma f) surgery - upper extremity lymphedema after mastectomy - gynecologic surgery for gynecologic cancer (20-40%) [3] g) radiation h) infrequent causes - tuberculosis - contact dermatitis - lymphogranuloma venereum - rheumatoid arthritis - pregnancy - factitious - following application of tourniquets Epidemiology: 1) primary lymphedema: a) prevalence: 1/10,000 b) women affected more frequently than men Pathology: 1) primary: - agenesis, hypoplasia or obstruction of lymph vessels 2) secondary: - obstruction of previously normal lymphatic channels 3) progressive fibrosis of cutaneous & subcutaneous tissue (lipodermatosclerosis) 4) hyperkeratosis 5) papillomatosis Clinical manifestations: 1) generally painless 2) edema of extremity of cosmetic concern a) edema may begin in foot & progress superiorly to involve the entire leg b) initially pitting edema c) tissues become indurated & fibrotic with chronic lymphedema & edema may become non-pitting 3) chronic, dull heavy sensation of the leg Differential diagnosis: 1) deep vein thrombosis 2) chronic venous insufficiency (venous stasis) Radiology: 1) abdominal & pelvic ultrasound to detect obstructing lesions 2) CT of the abdomen to detect obstructing lesions 3) lymphoscintigraphy or lymphangiography a) rarely indicated b) in primary lymphedema, lymphatic channels are absent, hypoplastic or ectatic c) in secondary lymphedema, lymphatic channels are generally dilated & it may be possible to determine the level of obstruction Complications: - excess risk for cutaneous angiosarcoma* with upper extremity lymphedema after mastectomy [4] - excess risk of non-melanoma skin cancer* with lower extremity lymphedema (RR=2.7) [4] * excess risk for skin cancer in affected extremity [4] Management: 1) foot care to prevent recurrent lymphangitis a) skin hygiene b) emollients to prevent dryness c) treat fungal infections of the foot aggressively 2) prophylactic antibiotics are often helpful 3) encourage physical activity 4) frequent elevation of legs 5) graduated compression hose 6) intermittent pneumatic compression devices 7) diuretics are contraindicated because they may cause intravascular volume depletion & electrolyte imbalances & will do little for the lymphedema 8) microsurgical lymph-venous anastomotic procedures 9) surgical resection of localized mass of lymphedema (abdomen, thigh, upper arms) may be of some success [2]

Related

deep vein thrombosis (DVT) hypotrichosis-lymphedema-telangiectasia syndrome lymphatic system lymphedema-hypoparathyroidism syndrome Stewart-Treves Syndrome venous stasis; venous hypertension

Specific

hereditary lymphedema hereditary lymphedema 2 (Meige lymphedema) lymphedema & ptosis lymphedema-distichiasis syndrome

General

lymphatic disease

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1405-1406
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, American College of Physicians, Philadelphia 2009
  3. Bankhead C. Lower Leg Lymphedema Common after Gyn Surgery. More than a fourth of patients affected. MedPage Today. March 29, 2018 https://www.medpagetoday.com/meetingcoverage/sgo/72069 - Carlson J, et al GOG 244, the lymphedema and gynecologic cancer (LeG) study: Incidence and risk factors in newly diagnosed patients. Society of Gynecologic Oncology (SGO) 2018; Abstract 11.
  4. Anand NC et al. Association of lower extremity lymphedema and nonmelanoma skin cancers. Mayo Clin Proc 2023 Nov; 98:1653 PMID: 37923522 https://www.mayoclinicproceedings.org/article/S0025-6196(23)00117-9/fulltext
  5. Lymphedema (PDQ) http://www.nci.nih.gov/cancertopics/pdq/supportivecare/lymphedema/HealthProfessional
  6. MedlinePlus: Lymphedema https://medlineplus.gov/lymphedema.html