lymphedema
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Etiology
- primary
- congenital: Milroy's disease
- lymphedema praecox:
- Meige's disease
- onset at puberty
- lymphedema tarda: begins after age 35
- associated disorders
- secondary
- obesity[2]
- recurrent lymphangitis
- most frequently due to Streptococci
- common
- filariasis: most common cause world wide
- tuberculosis
- neoplasm
- surgery
- upper extremity lymphedema after mastectomy
- gynecologic surgery for gynecologic cancer (20-40%)[3]
- radiation
- infrequent causes
- tuberculosis
- contact dermatitis
- lymphogranuloma venereum
- rheumatoid arthritis
- pregnancy
- factitious - following application of tourniquets
Epidemiology
- primary lymphedema:
- prevalence: 1/10,000
- women affected more frequently than men
Pathology
- primary:
- agenesis, hypoplasia or obstruction of lymph vessels
- secondary:
- obstruction of previously normal lymphatic channels
- progressive fibrosis of cutaneous & subcutaneous tissue (lipodermatosclerosis)
- hyperkeratosis
- papillomatosis
Clinical manifestations
- generally painless
- edema of extremity of cosmetic concern
- edema may begin in foot & progress superiorly to involve the entire leg
- initially pitting edema
- tissues become indurated & fibrotic with chronic lymphedema & edema may become non-pitting
- chronic, dull heavy sensation of the leg
Radiology
- abdominal & pelvic ultrasound to detect obstructing lesions
- CT of the abdomen to detect obstructing lesions
- lymphoscintigraphy or lymphangiography
- rarely indicated
- in primary lymphedema, lymphatic channels are absent, hypoplastic or ectatic
- 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]
Differential diagnosis
Management
- foot care to prevent recurrent lymphangitis
- skin hygiene
- emollients to prevent dryness
- treat fungal infections of the foot aggressively
- prophylactic antibiotics are often helpful
- encourage physical activity
- frequent elevation of legs
- graduated compression hose
- intermittent pneumatic compression devices
- diuretics are contraindicated because they may cause intravascular volume depletion & electrolyte imbalances & will do little for the lymphedema
- microsurgical lymph-venous anastomotic procedures
- surgical resection of localized mass of lymphedema (abdomen, thigh, upper arms) may be of some success[2]
More general terms
More specific terms
- hereditary lymphedema
- hereditary lymphedema 2 (Meige lymphedema)
- lymphedema & ptosis
- lymphedema-distichiasis syndrome
Additional terms
- deep vein thrombosis (DVT)
- hypotrichosis-lymphedema-telangiectasia syndrome
- lymphatic system
- lymphedema-hypoparathyroidism syndrome
- venous stasis; venous hypertension
References
- ↑ Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 1405-1406
- ↑ 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 15, American College of Physicians, Philadelphia 2009
- ↑ 3.0 3.1 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. - ↑ 4.0 4.1 4.2 4.3 Anand NC et al. Association of lower extremity lymphedema and nonmelanoma skin cancers. Mayo Clin Proc 2023 Nov; 98:1653 PMID: https://www.ncbi.nlm.nih.gov/pubmed/37923522 https://www.mayoclinicproceedings.org/article/S0025-6196(23)00117-9/fulltext
- ↑ Lymphedema (PDQ) http://www.nci.nih.gov/cancertopics/pdq/supportivecare/lymphedema/HealthProfessional
- ↑ MedlinePlus: Lymphedema https://medlineplus.gov/lymphedema.html