CHAPTER 23 Clinical Staging of Lymphedema

10.1055/b-0037-143479

CHAPTER 23 Clinical Staging of Lymphedema

Sandro Michelini, Marco Cardone, Alessandro Fiorentino

KEY POINTS

There is no perfect staging system for lymphedema.
A staging system is essential to facilitate a scientific dialog and cohesive treatment planning.
In addition to staging the clinical disease, it is important to incorporate functional assessment.
Proper treatment planning can only occur with such a staging system.

There is no perfect staging system for lymphedema. This becomes patently obvious as we learn more about the condition. The present lack of a universally recognized staging system makes it difficult to compare not only diagnoses but also treatment options and outcomes in published reports. The staging of lymphedema is a long-standing question that has been discussed at consensus meetings at all national and international congresses of lymphology.

To secure universal agreement about the definitions and framing of the pathology of lymphedema, a staging system characterized by simplicity, recognizability, and worldwide use is required. Four international proposals exist; they are based on different clinical aspects and instrumental measures of the pathology but have some characteristics in common. It is essential that these be synchronized and synthesized through the work of a special world commission to obtain a scientific tool with universally recognized and accepted parameters. A common tool to stage lymphedema is necessary for more precise scientific communication and medicolegal and social reasons. Particularly in its advanced stages, lymphedema is a socially significant disease with substantial cost implications for treatment and the loss of working capacity.

International Society of Lymphology

The present staging reported in the 2013 Consensus Document of the International Society of Lymphology (ISL) ¹ includes one preclinical and three clinical stages (Table 23-1). The initial staging of three clinical stages was revised by the International Society of Lymphology, Lymphology Association of North America, and German Society of Lymphology. This revision underscored the importance of including a preclinical stage for both primary and secondary lymphedema, which was defined as stage 0. The three clinical stages differ essentially in the presence of a pitting (stage I) or nonpitting (stages II and III) edema and in the association in the third stage of complications, often with a progressive evolution in the skin and subcutaneous tissues.

**TABLE 23-1 Lymphedema Staging According to the 2013 Consensus Document of the International Society of Lymphology**
Stage	Evidence
0	Subclinical; absence of edema in “risk of development” patient
I	Presence of edema reduced by treatment (pitting edema)
II	Edema partially reduced by treatment (no pitting edema)
III	Elephantiasis with skin lesions and relapsing infections

The same stages are indicated in the Consensus Document of the International Union of Phlebology. ² Stage 0 is subclinical. Stage 1 represents an early accumulation of fluid with high-protein content that subsides with limb elevation (Fig. 23-1, A). Pitting edema is seen in this stage. In stage 2, limb elevation rarely reduces tissue swelling, and pitting is not a feature (Fig. 23-1, B). Stage 3 includes lymphostatic elephantiasis with absent pitting and trophic skin changes (acanthosis, fat deposits, and warty overgrowths) (Fig. 23-1, C). The severity of the stage is based on volume differences: minimal (less than 20% increase), moderate (20% to 40% increase), and severe (greater than 40% increase).

**FIG. 23-1** International Union of Phlebology stages. Stage 0 (is subclinical). A, Stage 1 (pitting edema); B, stage 2 (no pitting edema); C, stage 3 (complicated elephantiasis).

At the Twentieth International Congress of Lymphology in Brazil, various groups offered different proposals on lymphedema staging. A special international commission gathered all these ideas to define the new official staging according to the ISL. ²

German Staging

The German Society of Lymphology, led by Etelka Földi, included for the first time four clinical stages of lymphedema. In addition to the four stages of the ISL Consensus Document, a stage 0 was included, representing all cases of subclinical lymphedema but with significant risk of the clinical appearance of edema (for example, lymphoscintigraphic findings of lymphatic impairment) ³ (Table 23-2).

**TABLE 23-2 Lymphedema Staging According to the German Society of Lymphology**
Stage	Evidence
0	No edema but significant risk of its clinical appearance
1	Edema reduced with treatment (pitting edema)
2	Edema reduced with treatment only partially (no pitting edema)
3	Elephantiasis with skin lesions and relapsing infections