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 Lymphedema

Louis M. Messina, M.D., Robert B. Smith, M.D.

I. Anatomy

1. To know the anatomy of the adult lymphatic system from the level of the terminal lymphatics to the cisterna chyli

2. To know the microscopic anatomy of the lymphatic capillaries and conducting lymph vessels and specifically how they differ from veins and arteries.

3. To understand the physiological determinants of lymph flow, including intrinsic contractility of lymph vessels, increased interstitial pressure, muscular activity, arterial pressure, respiratory pressure, and gravity.

4. To know the major differences that distinguish the physiology of the lymphatic system from the venous system.

5. To know the major purposes of the lymphatic system, including transport of interstitial fluid and macromolecular proteins lost from capillaries, bacterial and fungal infections, foreign material.

6. To know the classification of causes of lymphedema , including:

A. Primary lymphedema , Congenital (onset before one year of age)

1. Non-familial

2. Familial (Milroy's Disease)

B. Primary lymphedema , Praecox (onset 1 to 35 years of age)

1. Non-familial

2. Familial ( Meige Disease)

C. Primary lymphedema , Tarda (onset after 35 years of age)

D. Secondary lymphedema , including filariasis , lymph node excision and radiation, tumor invasion, infection, and trauma

1. To understand the functional classification of lymphedema based on the underlying lymphatic anatomy as determined by lymphangiography.

2. To understand how lymphedema develops the compensatory mechanisms that develop in response to increased interstitial pressure, and the tissue effects of chronic lymphatic obstruction including impaired immune cell trafficking, lymphatic obstruction, and chronic intestinal inflammation.

3. To understand the secondary consequences of long-standing lymphedema : infection, fibrosis, and neoplasia .

4. To understand the functional and anatomical abnormalities that cause chylous disorders.

5. To understand the consequences of the loss of chyle into body cavities or through a chylocutaneous fistula.

II. Diagnosis of Lymphedema

1. To understand classic clinical classifications of lymphedema based on etiology (primary vs secondary), genetics (familial vs sporadic), and time of onset.

2. To understand the history and physical findings which enable the clinician to identify the cause and site of lymphatic obstruction.

3. To understand pattern of pain, edema, and skin changes that distinguish lymphedema from other causes of extremity edema.

4. To understand the clinical presentation of complications of chronic lymphedema including infection (fungal and bacterial) and malignancy.

5. To understand the nutritional and immunological consequences of chronic lymphangeiectasia with protein-losing nephropathy, chylous ascites , or chylothorax .

6. To understand the accuracy and limitations of the most frequent noninvasive imaging modalities used to evaluate lymphatic disease: lymphoscintigraphy , computed tomography, and magnetic resonance imaging.

7. To understand the technique of lymphoscintography , the features of a normal lymphoscintogram and the typical scintographic findings in primary and secondary lymphedema .

8. To understand the indications, techniques, interpretation and complications of lymphangiograms .

III. Management of Chronic Lymphedema

1. To understand the techniques of non-operative management of primary and secondary lymphedema .

2. To know the mechanisms of action and effectiveness/ineffectiveness of pharmacologic agents such as diuretics, benzopyrones , and steroids in the treatment of lymphedema .

3. To understand the mechanical techniques to reduce a limb swelling

A. To understand the technique of limb elevation.

B. To understand the technique , advantages, and disadvantages of manual lymphatic drainage.

C. To understand the technique of intermittent pneumatic compression, including pressure, ratio of compression/decompression, duration of therapy.

D. To understand the technique of intermittent, non-pneumatic high pressure compression

E. To know the role of antibiotics in the treatment and prophylaxis of recurrent cellulitis in patients with chronic lymphedema .

1. To know the techniques for maintenance of limb size including elastic and non-elastic support.

2. To know the indications for surgical management of chronic lymphedema

3. To understand the technique, complication rate, and effectiveness of excisional procedures including the Charles procedure, Thompson's buried dermal flap, suction curretage , and Sistrunk procedures.

4. To know the indications, technique, complications rate, and outcome of direct lymphatic reconstruction such as lymphovenous anastomosis including lymphnodal -venous and lymphvenous procedures.

5. To know the indications, technique, complication rate, and outcome of lymphatic grafting.

6. To know indication, technique, complications rate of indirect lymphatic reconstructions such as the mesenteric bridge operation, omental flap, and autotransplantation of free lymphatic flap.

A. To know the indications, technique, complications, and outcome of procedures for primary chylous disorders.

References

1. Rudkin GH, Miller TA: Lymphatic Disease. In Ernst CB and Stanley JC ( eds ): Current Therapy in Vascular Surgery. St. Louis , Mosby : 1995, pp 973-978.

2. Goldsmith HS, Holmes W: Secondary Lymphedema . In Ernst CB and Stanley JC ( eds ): Current Therapy in Vascular Surgery. St. Louis , Mosby : 1995, pp 978-982.

3. Gloviczki P: The management of lymphatic disorders. In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1883-1888.

4. Witte CL, Witte MH: Lymphodynamics and pathophysiology of lymphedema . In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1889-1899.

5. Gloviczki P, Wahner H: Clinical diagnosis and evaluation of lymphedema . In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1899-1920.

6. Rooke TW, Gloviczki P: Nonoperative management of chronic lymphedema . In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1920 -1927.

7. Abdou MS, Ashby ER, Miller TA: Excisional operations for chronic lymphedema . In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1928-1936.00

8. Gloviczki P: Lymphatic reconstruction. In Rutherford RB (ed): Vascular Surgery. Philadelphia, W.B. Saunders Co.: 1995, pp 1936-1950.

9. Gloviczki P: Physiologic changes in lymphatic dysfunction. In White RA and Hollier LH ( eds ): Vascular Surgery. Basic Science and Clinical Correlations. Philadelphia , J.B. Lippincott : 1994, pp 293-302.

10. Gloviczki P, Calcagno D, Schirger A, et al: Non-invasive evaluation of the swollen extremity: Experiences with 190 lymphoscintigraphic examinations. J Vasc Surg 9:683, 1989.

11. Clouse ME, Wallace S: Lymphatic Imaging. Lymphography , Computed Tomography, and Scintigraphy . 2nd ed . Baltimore, Williams & Wilkins, 1985.

12. Pappas CJ, O'Donnell TF Jr : Long-term results of compression treatment for lymphedema . J Vasc Surg 16:555, 1992.

13. Gloviczki P, Fisher J, Hollier LH, et al: Microsurgical lymphevenous anastomosis for treatment of lymphedema : A critical review. J Vasc Surg 7:647, 1988.

14. O'Brien BMcC , Mellow CG, Khasanchi RK, et al: Long-term results after microlymphatico -venous anastomoses for the treatment of obstructive lymphedema . Plast Reconstr Surg 85:562, 1990.

15. Casley -smith JR, Morgan RG, Piller NB: Treatment of lymphedema of the arms and legs with 5.6-benzo-(alpha)- pyrone . N Engl J Med 329:1158, 1993.

16. Browse NL: The diagnosis and management of primary lymphedema . J Vasc Surg 3:181, 1986.

17. Thiadens SRJ: Advances in the management of lymphedema . In Goldstone J (ed): Perspectives in Vascular Surgery. St. Louis , Quality Medical Publishing, 1990, pp 125-141.

18. Kobayashi MR, Miller TA: Lymphedema . Clin Plast Surg 14:303, 1987.

Posted June 2010