Invited papers. CEAP classification and implications for investigations. B. Eklöf Helsingborg, Sweden.

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1 Invited papers Acta chir belg, 2006, 106, CEAP classification and implications for investigations B. Eklöf Helsingborg, Sweden. Key words. Chronic venous disorders ; venous disease ; reporting standards ; classification. Abstract. Classification of diseases is a basic instrument for uniform diagnosis and meaningful communication about the disease. The credo of the American Venous Forum (AVF) is that The cornerstone for management of chronic venous disorders (CVD) is a proper diagnosis and accurate classification of the underlying venous problem, which creates the base for correctly directed treatment. In CVD reliance for too long has been placed on the clinical appearance of the superficial effects of CVD, such as spider veins, varicose veins, swelling, skin changes, and ulcerations, without requiring accurate objective testing of the venous system to substantiate the diagnosis. This practice has caused errors of diagnosis and has been largely responsible for the poor correlation of results between treatment methods. There have been several classifications in the past that have added to our understanding of CVD, but all lack the completeness and objectivity needed for scientific accuracy (1). The creation of the CEAP classification At the fifth annual meeting of the American Venous Forum (AVF) in 1993, John Porter suggested using the same approach as TNM for cancer to develop a classification system for venous diseases. Following a year of intense discussions a consensus conference was held at the sixth annual meeting of AVF in February 1994 on the island of Maui, Hawaii, at which an international ad hoc committee, chaired by Andrew Nicolaides, and with representatives from Australia, Europe, as well as the United States, developed the first CEAP consensus document (2). It contained two parts, a classification of CVD and a scoring system of the severity of CVD. The classification was based on clinical manifestations (C), etiologic factors (E), anatomic distribution of disease (A), and the underlying pathophysiologic findings (P), thus the name CEAP. The severity scoring system was based on three elements : the number of anatomic segments affected, grading of symptoms and signs, and disability. The CEAP consensus statement was published in 26 journals and books in nine languages, truly a universal document for CVD. It was endorsed by the Joint Councils of the SVS and the North American Chapter of the ISCVS, and its basic elements were incorporated into venous reporting standards (3). Today most published clinical papers on CVD use all or portions of the CEAP classification. Revision of CEAP Diagnosis and treatment of CVD was developed rapidly in the 1990s and the need for an update of the classification logically followed. In April 2002, an ad hoc committee on CEAP was appointed by AVF to review the classification and make recommendations for change by 2004, 10 years after its introduction. An International ad hoc committee also was established to assure continued universal utilization. The following passages summarize the results of these deliberations, by describing the new aspects of the revised CEAP (4). The recommended changes include additions to or refinements of several definitions used in describing CVD, refinement of the C-classes of CEAP, addition of the descriptor n (no venous abnormality identified), incorporation of the date of classification and level of clinical investigation, and the description of Basic CEAP, introduced as a simpler alternative to the full (advanced) CEAP classification. For details see ref. 4. Terminology and new definitions The CEAP classification deals with all forms of chronic venous disorders. The term chronic venous disorder (CVD) includes the full spectrum of morphological and functional abnormalities of the venous system from telangiectasias to venous ulcers. Some of these, like telangiectasias, are highly prevalent in the normal adult population, and in many cases the use of the term disease is not appropriate. The term chronic venous insufficiency (CVI) implies a functional abnormality of the venous system and usually is reserved for patients with more advanced disease including those with edema (C3), skin changes (C4), or venous ulcers (C5-6).

2 CEAP Classification and Implications for Investigations 655 Refinement of C-classes in CEAP The essential change here is the division of class C4 into two subgroups that reflect different severity of disease, and carry a different prognosis in terms of risk of ulceration : C0 : No visible or palpable signs of venous disease C1 : Telangiectasies or reticular veins C2 : Varicose veins distinguished from reticular veins by a diameter of 3 mm or more C3 : Edema C4 : Changes in the skin and subcutaneous tissue secondary to CVD (now divided into two subclasses to better define the differing severity of venous disease) : C4a : Pigmentation and/or eczema C4b : Lipodermatosclerosis and/or atrophie blanche C5 : Healed venous ulcer C6 : Active venous ulcer Each clinical class is further characterized by a subscript for the presence of symptoms (S, symptomatic) or absence of symptoms (A, asymptomatic). Refinement of E, A, and P in CEAP To improve the assignment of designations under the E, A, and P, a new descriptor n is now recommended for use where no venous abnormality is identified. This n could be added to E (En : no venous etiology identified), A (An : no venous location identified), and P (Pn :no venous pathophysiology identified). Observer variability in assigning designations in the past may have been contributed to by the lack of a normal option. Date of classification CEAP is not a static classification ; the patient can be reclassified at any point in time. Classification starts with the initial visit, but can be better defined after further investigations. A final classification may not be complete until after surgery and histopathologic assessment. We therefore recommend that any CEAP classification be followed by the date ; for example, C4b,S,Ep,As,p,Pr ( ). Level of investigation A precise diagnosis is the basis for correct classification of the venous problem. The diagnostic evaluation of the patient with CVD can be logically organized into one or more of three levels of testing, depending on the severity of the disease : Level I : The office visit with history and clinical examination, which may include use of a hand-held Doppler Level II : The noninvasive vascular laboratory, which now routinely includes duplex color scanning, with some plethysmographic method added as desired Level III : Invasive investigations or more complex imaging studies including varicography, ascending and descending venography, venous pressure measurements, IVUS, spiral CT scan or MRV. We recommend that the level of investigation (L) should also be added to the classification, for example, C2,4b,S,Ep,As,p,Pr ( ,L II). Basic CEAP A new basic CEAP is offered here. Use of all components of CEAP is still encouraged but unfortunately many physicians merely use only the C-classification, which is just a modest advance beyond the previous classifications and is based solely on the clinical appearance. Venous disease is complex, but can be described by use of well-defined categorical descriptions. For the practicing physician, CEAP can be a valuable instrument for correct diagnosis to guide treatment and assess prognosis. In modern phlebological practice the vast majority of patients will have a duplex scan of the venous system of the leg, which largely will define the E, A, and P categories. Nevertheless, it is recognized that the merits of using the full (advanced) CEAP classification system hold primarily for the researcher and for standardized reporting in scientific journals. It allows grouping of patients so that the same types of patients can be analyzed together, and such subgroup analysis allows their treatments to be more accurately assessed. Furthermore, reports using CEAP can be compared with one another with much greater certainty. This more complex classification, for example, also allows any of the 18 named venous segments to be identified as the location of venous pathology. Take a patient with pain, varicose veins, and lipodermatosclerosis where duplex scan confirms primary reflux of the GSV and incompetent perforators in the calf. The classification here would be C2,4b,S, Ep, As,p, Pr2,3,18. Although the detailed elaboration of venous disease in this form may seem unnecessarily complex, even intimidating, to some clinicians, it provides universal understandable descriptions that may be essential to investigators in the field. To serve the needs of both, the full CEAP classification, is retained as advanced CEAP, and the following simplified form is offered as basic CEAP. In essence, Basic CEAP applies two simplifications : 1) In basic CEAP, the single highest descriptor can be used for clinical classification. For example, a patient

3 656 B. Eklöf with varicose veins, swelling, and lipodermatosclerosis would be C4b. The more comprehensive clinical description, in advanced CEAP, would be C2,3,4b. 2) In basic CEAP, where duplex scan is performed, E, A, and P should also be classified using the multiple descriptors recommended, but the complexity of applying these to the 18 possible anatomic segments is avoided in favor of applying the simple s, p, and d descriptors to denote the superficial, perforator, and deep systems. Thus, using basic CEAP, the same patient cited in a previous example (painful varicosities plus lipodermatosclerosis and duplex scan determined reflux involving the superficial and perforator systems) would be classified as C4b,S, Ep, As,p Pr (rather than C2,4b,S, Ep, As,p, Pr2,3,18). Revision of CEAP : summary Clinical Classification C0 : No visible or palpable signs of venous disease C1 : Telangiectasies or reticular veins C2 : Varicose veins C3 : Edema C4a : Pigmentation and/or eczema C4b : Lipodermatosclerosis and/or atrophie blanche C5 : Healed venous ulcer C6 : Active venous ulcer S : Symptoms including ache, pain, tightness, skin irritation, heaviness, muscle cramps, as well as other complaints attributable to venous dysfunction A : Asymptomatic Etiologic Classification Ec : Congenital Ep : Primary Es : Secondary (postthrombotic) En : No venous etiology identified Anatomic Classification As : Superficial veins Ap : Perforator veins Ad : Deep veins An : No venous location identified Pathophysiologic Classification Basic CEAP : Pr : Reflux Po : Obstruction Pr,o : Reflux and obstruction Pn : No venous pathophysiology identifiable Advanced CEAP Same as basic, with the addition that any of 18 named venous segments can be utilized as locators for venous pathology : Superficial veins : 1. Telangiectasies/reticular veins 2. Great saphenous vein (GSV) above knee 3. GSV below knee 4. Small saphenous vein 5. Nonsaphenous veins Deep veins : 6. Inferior vena cava 7. Common iliac vein 8. Internal iliac vein 9. External iliac vein 10. Pelvic : gonadal, broad ligament veins, other 11. Common femoral vein 12. Deep femoral vein 13. Femoral vein 14. Popliteal vein 15. Crural : anterior tibial, posterior tibial, peroneal veins (all paired) 16. Muscular : gastrocnemial, soleal veins, other Perforating veins : 17. Thigh 18. Calf Example : A patient presents with painful swelling of the leg and varicose veins, lipodermatosclerosis, and active ulceration. Duplex scanning on September 6, 2006 showed axial reflux of GSV above and below the knee, incompetent calf perforators, and axial reflux in the femoral and popliteal veins. No signs of postthrombotic obstruction. Classification according to basic CEAP : C6,S, Ep, As,p,d, Pr Classification according to advanced CEAP : C2,3,4b,6,S, Ep, As,p,d,Pr2,3,18,13,14 ( , LII) Clinical application of CEAP Figure 1 is showing six patients with severe skin changes of their legs. This is Bob Kistner s and my favourite illustration on the controversy of CVD. The majority of physicians would assume that these are legs with complications from previous DVT, and start conservative treatment without further investigation with Unna boot or similar compression device. Could you from this clinical picture decide if : * the etiology is primary, secondary (postthrombotic), congenital or even non-venous ; * the anatomic distribution of venous disease is superficial, perforator, deep or a combination of these ; * pathophysiology is due to reflux, obstruction or a combination of both?

4 CEAP Classification and Implications for Investigations 657 Fig. 1 Upper row from left : case 1-3 ; lower row from left : case 4-6 Proper investigation of these 6 patients regarding cause of their skin changes led to the following diagnoses and treatments : Case 1 : Diagnosis : GSV incompetence ; CEAP : C2,3,4b,6,S Ep As Pr2,3 ; Treatment : GSV ablation ; Case 2 : Diagnosis : GSV + perforator incompetence ; CEAP : C2,3,4b,6,S Ep As,p Pr2,3,18 ; Treatment : GSV + perforator ablation ; Case 3 : Diagnosis : isolated perforator incompetence ; CEAP : C2,3,4b,6,S Ep As,p Pr18 ; Treatment : perforator ablation ; Case 4 : Diagnosis : primary incompetence of GSV, perforators and deep veins ; CEAP : C2,3,4b,6,S Ep As,p,d Pr2,3,11,13,14,15,18 ; Treatment : GSV + perforator ablation and deep venous valve repair ; Case 5 : Diagnosis : secondary (postthrombotic) incompetence of GSV, perforators and deep veins ; CEAP : C2,3,4b,6,S Es As,p,d Pr2,3,11,13,14,15,18 ; Treatment : GSV + perforator ablation and femoral vein transposition ; Case 6 : Diagnosis : no venous disease ; CEAP : C3,4b,6,S En An Pn ; Treatment : no surgery. The clinical class C is almost the same in all 6 cases. Level 2 investigation using duplex scanning could differentiate etiology, anatomic localization and pathophysiology. Proper diagnosis leading to accurate classification of the underlying venous problem is the base for correct treatment. A simple guide to the level of investigation in relation to CEAP clinical classes is presented ; this can be modified according to clinical circumstances and local practice : Class 0/1 : no visible or palpable signs of venous disease ; telangiectasies or reticular veins : Level I investi-

5 658 B. Eklöf gations are usually sufficient. In symptomatic patients level II may be indicated. Class 2 : varicose veins : Level II should be used in the majority of patients, and level III may be needed in special cases. Class 3 : edema : Level II is usually indicated. If the deep system is involved level III may be needed. Class 4-6 : skin changes, healed or active ulcer : Level II indicated in the majority of these patients. Patients for deep reconstruction will need level III. Level I investigations may be sufficient in some patients with irreversible calf muscle pump failure due to neurological disease or severe and noncorrectable reduction of ankle movement. Revision of CEAP An ongoing process With improvement in diagnostics and treatment there will be continued demands to adapt the CEAP classification to better serve future developments. There are several conditions that are not included in the CEAP classification but that can influence the management of the patients : Combined arterial/venous etiology Postthrombotic lymphedema Ankle ankylosis with atrophy of the calf Venous aneuryms Venous neuropathy Corona phlebectatica Pelvic congestion syndrome Morbid obesity The role of corona phlebectatica (CP) was discussed during the meetings and the Atlantic Ocean was a clear divider. In parts of Europe CP has been used as an early indicator of advanced CVD. Its scientific significance is now under investigation, particularly in France. There is a need to incorporate appropriate new features without too frequent disturbances of the stability of the classification. Acknowledgement Part of this article was previously published in EKLÖF B., RUTHERFORD R. B., BERGAN J. J., CARPENTIER P., GLOVICZKI P., KISTNER R. L. et al., for the American Venous Forum International Ad Hoc Committee for Revision of the CEAP classification. Revision of the CEAP classification for chronic venous disorders : Consensus statement. J Vasc. Surg, 2004, 40 : The author wishes to thank the Society for Vascular Surgery for permission to reproduce the relevant section. References 1. EKLÖF B. Revision of the CEAP classification 10 years after its introduction in Medicographia, 2006, 28 : BERGAN J. J., EKLOF B., KISTNER R. L., MONETA G. L., NICOLAIDES A. N., and the International ad hoc committee of the American Venous Forum. Classification and grading of chronic venous disease in the lower limbs. A consensus statement. Vasc Surg, 1996, 30 : PORTER J. M., MONETA G. L. International Consensus Committee on Chronic Venous Disease. Reporting standards in venous disease : An Update. J Vasc Surg, 1995, 21 : EKLÖF B., RUTHERFORD R. B., BERGAN J. J., CARPENTIER P., GLOVICZKI P., KISTNER R. L. et al. for the American Venous Forum International Ad Hoc Committee for Revision of the CEAP classification. Revision of the CEAP classification for chronic venous disorders : Consensus statement. J Vasc Surg, 2004, 40 : B. Eklöf, M.D., Ph.D. Past president American Venous Forum Emeritus professor of surgery University of Hawaii Batteritorget 8 SE Helsingborg, Sweden Tel. : moboek@telia.com