Clinical,Aetiological,Anatomical and Pathological Classification (Ceap): Gold Standard and Limits

Abstract

The first CEAP (clinical, aetiological, anatomical and pathological elements) consensus document was published after a consensus conference of the American Venous Forum, held at the sixth annual meeting of the AVF in February 1994 in Maui, Hawaii. In the following years the CEAP classification was published in many international journals and books which has led to widespread international use of the CEAP classification since 1995. The aim of this paper is to review the benefits and limits of CEAP from the available literature. In an actual Medline analysis with the keywords ‘CEAP’ and ‘venous insufficiency’, 266 publications using the CEAP classification in venous diseases are available. The CEAP classification was accepted in the venous community and used in scientific publications, but in most of the cases only the clinical classification was used. Limitations of the first version including a lack of clear definition of clinical signs led to a revised version. The CEAP classification is the gold standard of classification of chronic venous disorders today. Nevertheless for proper use some facts have to be taken into account: the CEAP classification is not a severity classification, C2 summarizes all kinds of varicose veins, in C3 it may be difficult to separate venous and other reasons for oedema, and corona phlebectatica is not included in the classification. Further revisions of the CEAP classification may help to overcome the still-existing deficits.

Keywords

CEAP classification CVD CVI VCSS

The CEAP Consensus Document consisted of two parts: a classification of chronic venous diseases and a scoring system of the severity of chronic venous disorder (CVD).³ The CEAP classification included clinical manifestations (C), aetiological factors (E), anatomic distribution of disease (A) and underlying pathophysiological findings³ (P) (Table 1). The severity score system included the number of anatomical segments affected, the grading of the symptoms and signs of CVD and a disability score^1,3 (Tables 2 -4).

Table 1

CEAP classification

C	For Clinical signs (Grade-6), supplemented by (A) for asymptomatic and (S) for symptomatic presentation
E	For Etiological classification (congenital, primary, secondary)
A	For Anatomical distribution (superficial, deep or perforator, alone or in combination)
P	For Pathophysiological dysfunction (reflux or obstruction, alone or in combination)

In the following years the CEAP classification was published in many international journals and books, which has led to widespread international use of the CEAP classification since 1995.³ In an actual Medline analysis with the keywords ‘CEAP’ and ‘venous insufficiency’, 266 publications using the CEAP classification in venous diseases are available.

Early criticism of the CEAP classification

From the beginning the new CEAP classification was accepted in the venous community and used in scientific publications. However, in most of the cases only the clinical classification with the C-classes was used. The complete CEAP and also the severity score system were only used occasionally.

From the beginning the lack of clear definitions of the clinical signs and symptoms of CVD was criticized. Antignani et al.⁴ sent a questionnaire concerning the clinical part of the CEAP classification to phlebologists worldwide which was answered by 206 colleagues form 67 countries As a result there were a wide variety of opinions returned, thus demonstrating that the same term was used with various meanings by different physicians. It was also criticized on the grounds that there is a lack of vascular history no varicose vein score and corona phlebectatica was not included in the classification.⁵ It could be shown that corona phlebectatica, which was initially described by Van der Molen as an initial sign of chronic venous insufficiency, has a high clinical and haemodynamic significance in chronic venous diseases.^6,7 In a further paper the reproducibility of the C-Classes was studied. It showed good intra-observer reproducibility with 85% but a poor inter-observer reproducibility with 47%.⁸ In recent clinical practice guidelines of the Society of Vascular Surgery and the American Venous Forum, the use of the CEAP classification is recommended for patients with chronic venous diseases.⁹

Table 2

Anatomic score

Segment No.
	Superficial veins (As)
1	Telangiectases/reticular veins
	Great saphenous (GSV)
2	Above knee
3	Below knee
4	Short saphenous (SSV)
5	Non-saphenous
	Deep veins (A_D)
6	Inferior vena cava
	Iliac
7	Common
8	Internal
9	External
10	Pelvic-gonadal, broad ligament, other Femoral
11	Common
12	Deep
13	Superficial
14	Popliteal
15	Crural-anterior tibial, posterior tibial, peroneal (all paired)
16	Muscular-gastrocnemial, soleal, other
	Perforating veins (Ap):
17	Thigh
18	Calf
	No venous location identified (An)

Table 3

Clinical score

Segment No.
Pain	0 = none; 1 = moderate, not requiring analgesics; 2 = severe, requiring analgesics
Oedema	0 = none; 1 = mild/moderate; 2 = severe
Venous claudication	0 = none; 1 = mild/moderate; 2 = severe
Pigmentation	0 = none; 1 = localized; 2 = extensive
Lipodermatosclerosis	0 = none; 1 = localized; 2 = extensive
Ulcer size (largest ulcer)	0 = none; 1 = <2 cm diameter; 2 = >2 cm diameter
Ulcer duration	0 = none; 1 = <3 months; 2 = >3 months
Ulcer recurrence	0 = none; 1 = once; 2 = more than once
Ulcer number	0 = none; 1 = single; 2 = multiple

Nevertheless there is still heterogeneity of reporting standards even in randomized clinical trials. Thakur et al.¹⁰ reviewed clinical trials of endovenous interventions for varicose veins and found that the CEAP classification was used in 17/28 randomized trials.

During the 40th World Congress of the Union Internationale de Phlebologie (UIP) in Rome 2001, a conference of experts suggested definitions and refinements of the clinical classification of CEAP.¹¹

As the clinical score in the CEAP classification was not used in many studies an ad hoc committee of the American Venous Forum presented a new venous severity score system in 2000.¹² In the meantime this venous clinical severity score (VCSS) has been revised.¹³

Revision of the CEAP classification

In April 2002 an ad hoc committee on CEAP was appointed by the AVE It reviewed the classification and made recommendations for change. This was added by an international ad hoc committee which was established to ensure continued universal use.³ In 2004 a revised CEAP classification was published.³ It was stated that the CEAP classification deals with all forms of CVDs. CVDs are defined as a full spectrum of morphological and functional abnormalities of the venous systems from teleangiectases to venous ulcers. The term chronic venous insufficiency implies a functional abnormality of the venous system and is usually reserved for more severe diseases including oedema, skin changes or venous ulcers (C3–C6). The following definitions for clinical signs in the C-classes of CEAP were added to the classification (quoted from³):

Table 4

Disability score

0	Asymptomatic
1	Symptomatic, can function without support device
2	Can work 8-hour day only with support device
3	Unable to work even with support device

•

Atrophie blanche (white atrophy): Localized, often circular whitish and atrophic skin areas surrounded by dilated capillaries and sometimes hyperpigmentation. Sign of severe CVD, and not to be confused with healed ulcer scars. Scars of healed ulceration may also exhibit atrophic skin with pigment changes, but are distinguishable by history of ulceration and appearance from atrophie blanche, and are excluded from this definition;

•

Corona phlebectatica: Fan-shaped pattern of numerous small intradermal veins on medial or lateral aspects of ankle and foot. Commonly thought to be an early sign of advanced venous disease. Synonyms include malleolar flare and ankle flare;

•

Eczema: Erythematous dermatitis, which may progress to blistering, weeping or scaling eruption of skin of leg. Most often located near varicose veins, but may be located anywhere in the leg. Usually seen in uncontrolled CVD, but may reflect sensitization to local therapy;

•

Oedema: Perceptible increase in volume of fluid in skin and subcutaneous tissue, characteristically indented with pressure. Venous oedema usually occurs in ankle region, but may extend to leg and foot;

•

Lipodermatosclerosis: (LDS) Localized chronic inflammation and fibrosis of skin and subcutaneous tissues of lower leg, sometimes associated with scarring or contracture of Achilles tendon. LDS is sometimes preceded by diffuse inflammatory oedema of the skin, which may be painful and which often is referred to as hypodermitis. LDS must be differentiated from lymphangitis, erysipelas or cellulitis by their characteristically different local signs and systemic features. LDS is a sign of severe CVD;

•

Pigmentation: Brownish darkening of skin, resulting from extravasated blood. Usually occurs in ankle region, but may extend to leg and foot;

•

Reticular vein: Dilated bluish subdermal vein, usually 1 mm to less than 3 mm in diameter. Usually tortuous. Excludes normal visible veins in persons with thin, transparent skin. Synonyms include blue veins, subdermal varices and venulectasies;

•

Teleangiectases: Confluence of dilated intradermal venules less than 1 mm in calibre. Synonyms include spider veins, hyphen webs and thread veins;

•

Varicose vein: Subcutaneous dilated vein 3 mm in diameter or larger, measured in upright position. May involve saphenous veins, saphenous tributaries or non-saphenous superficial leg veins. Varicose veins are usually tortuous, but tubular saphenous veins with demonstrated reflux may be classified as varicose veins. Synonyms include varix, varices and varicosities;

•

Venous ulcer: Full-thickness defect of skin, most frequently in ankle region, that fails to heal spontaneously and is sustained by CVD.

In the clinical classification, the class C4 for skin changes was divided into two subgroups, reflecting the severity of the disease. C4a was defined as pigmentation or eczema, C4b as LDS or atrophy blanche (Table 5).

Table 5

Clinical classification

Class 0	No visible or palpable signs of venous disease
Class 1	Telangiectases or reticular veins
Class 2	Varicose veins
Class 3	Oedema
Class 4a	Pigmentation and/or eczema
Class 4b	Lipodermatosclerosis and/or atrophie blanche
Class 5	Healed venous ulcer
Class 6	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

Each clinical class can be associated to clinical symptoms and in this case marked ‘S’ – symptomatic or the absence of symptoms marked in this case ‘A’ – asymptomatic. Symptoms include aching, pain, tightness, skin irritation, heaviness, muscle cramps and other complaints attributable to venous dysfunction.³

In the E, A and P segments of the CEAP classification the Class ‘N’ was added, defined as no venous abnormality identified in this section (Tables 6 –8). As an example En would mean that no venous aetiology was identified, An that no venous location was identified and Pn that no venous pathophysiology was identified.³

Table 6

Etiological classification

Ec	Congenital
Ep	Primary
Es	Secondary
En	No venous aetiology identified

Table 7

Anatomical classification

As	Superficial veins (As)
Ad	Deep veins (A_D)
Ap	Perforating veins (Ap)
An	No venous location identified

Table 8

Pathophysiological classification

Pr	Reflux
Po	Obstruction
Pr, o	Reflux and obstruction
Pn	No venous pathophysiology identifiable

It was also recommended to document the date when the classification was done as changes may occur with time. In addition, three levels of investigation have been defined, describing the tests which were made to classify the patient³ (Table 9).

Table 9

Level of investigation

Level 1	The office visit with history and clinical examination, which may include use of a hand-held Doppler
Level 2	The non-invasive vascular laboratory, which includes duplex colour scanning and plethysmography
Level 3	Invasive investigations or more complex imaging studies, including varicography, ascending and descending venography, venous pressure measurements, IVUS, spiral CT scan or MRV

IVUS, intravascular ultrasonography; CT, computed tomography; MRV, magnetic resonance venography

The use of the complete CEAP classification with a documentation of all clinical findings and anatomical segments, the level of investigation and the date is recommended for scientific use. For use in the daily practice the Basic CEAP is recommended. In this case the single highest descriptor can be used for clinical classification. A patient with teleangiectases, varicose veins, oedema with atrophy and healed venous ulcers would be classified as C5 whereas in the full CEAP format he would be a patient classified as C1, 2, 3, 4b, 5. In addition, a simplified anatomical classification divided into superficial, deep or perforating veins is used.³

Discussion

The CEAP classification is the gold standard of classification of CVDs today. Nevertheless for proper use some facts have to be taken into account:

(1)

CEAP classification is not a severity classification but describes the clinical and functional situation of the patient. To classify the severity of the disease additional tools like the VCSS or Quality of Life scales have to be used;¹⁴

(2)

In the C1 class teleangiectases and reticular veins are summarized although they may have different implications for clinical symptoms;

(3)

In the C2 class all kinds of varicose veins are summarized. Saphenous veins in the interfacial space, accessory saphenous veins and non-saphenous tributaries may have different implications not only for the severity of the disease but also for the risk of progression of the disease, clinical symptoms and the choice of treatment. The anatomical classification may add this information. However varicose veins may have a small or a large diameter with implications for clinical symptoms and for the choice of treatment option. The diameter of the vein is not included in the CEAP classification;

(4)

The C3 class is defined as oedema in the lower legs. This includes minimal oedema, which occurs only in the evening as well as extensive oedema of the leg. In addition, oedema may not only be caused by venous insufficiency but also by other diseases like cardiac insufficiency or drug treatment. These different diseases may be combined as varicose veins are prevalent in more than 20% of the population and the main cause of the swelling may be difficult to evaluate;

(5)

Corona phlebectatica is still not a part of the clinical classification and summarized in the C1 class defined as teleangiectases and reticular veins. However teleangiectases at the foot as corona phlebectatica has a completely different implication for the venous disease compared with isolated teleangiectases in the thigh region. At least corona is now part of the revised VCSS classification;¹⁴

(6)

In the severe stages of CVD lymphatic involvement and lymphoedema is a frequent finding. This is not classified in the CEAP classification;

(7)

It has to be mentioned that also the critical class C0 can be assorted with pathological venous findings not visible or palpable. Examples are post-thrombotic changes without clinical signs, insufficient saphenous veins without varicose veins and functional venous insufficiency in obesity.

Although the CEAP classes are not a severity classification, reflux prevalence increases with higher C-stages in the superficial veins.¹⁵ Patients’ reported quality of life also decreases significantly with higher C-classes.¹⁶ The clinical class of CEAP also correlates significantly with foot volumetric parameters in CVD patients.¹⁷

Further revisions of the CEAP classification may help to overcome the still-existing deficits of the classification.

Conflicts of interest: None declared.

References

Beebe

, Bergan

, Bergqvist

. Classification and grading of chronic venous disease in the lower limbs. Vasc Surg 1996; 30: 5–11

Porter

, Moneta

. Reporting standards in venous disease: an update. J Vasc Surg 1995; 21: 635–45

Eklöf

, Rutherford

, Bergan

. Revision of the CEAP classification for chronic venous disorders: consensus statement. J Vasc Surg 2004; 40: 1248–52

Antignani

, Cornu-Thenard

, Allegra

, Carpentier

, Partsch

, Uhl

. Results of a questionnaire regarding improvement of ‘C’ in the CEAP classification. Eur J Vasc Endovasc Surg 2004; 28: 177–81

Cornu-Thenard

, Uhl

, Carpentier

. Do we need a better classification than CEAP? Acta Chir Belg 2004; 104: 276–82

Uhl

, Cornu-Thenard

, Carpentier

, Widmer

, Partsch

, Antignani

. Clinical and hemodynamic significance of corona phlebectatica in chronic venous disorders. J Vasc Surg 2005; 42: 1163–8

Uhl

, Cornu-Thenard

, Satger

. Clinical analysis of the corona phlebectatica. J Vasc Surg 2012; 55: 150–3

Uhl

, Cornu-Thenard

, Carpentier

, Schadeck

, Parpex

, Chleir

. Reproducibility of the ‘C’ classes of the CEAP classification. Phlebology 2001; 1: 39–48

Gloviczki

, Comerota

, Dalsing

. The care of patients with varicose veins and associated chronic venous diseases: clinical practice guidelines of the Society for Vascular Surgery and the American Venous Forum. J Vasc Surg 2011; 53 (Suppl):2S–48S

10.

Thakur

, Shalhoub

, Hill

, Gohel

, Davies

. Heterogeneity of reporting standards in randomized clinical trials of endovenous interventions for varicose veins. Europ J Vasc Endovasc Surg 2010; 40: 528–33

11.

Allegra

, Antignani

, Bergan

. The ‘C’ of CEAP: suggested definitions and refinements: an International Union of Phlebology conference of experts. J Vasc Surg 2003; 37: 129–31

12.

Rutherford

, Padberg

, Comerota

, Kistner

, Meissner

, Moneta

. Venous severity scoring: an adjustment to venous outcome assessment. J Vasc Surg 2000; 31: 1307–12

13.

Vasquez

, Rabe

, McLafferty

. Revision of the venous clinical severity score: venous outcomes consensus statement: special communication of the American Venous Forum Ad Hoc Outcomes Working Group. J Vasc Surg 2010; 52: 1387–96

14.

Vasquez

, Munschauer

. Venous Clinical Severity Score and quality-of-life assessment tools: application to vein practice. Phlebology 2008; 23: 259–75

15.

Maurins

, Hoffmann

, Losch

, Jockel

, Rabe

, Pannier

. Distribution and prevalence of reflux in the superficial and deep venous system in the general population – results from the Bonn Vein Study, Germany J Vasc Surg 2008; 48: 680–7

16.

Kahn

, M'lan

, Lamping

, Kurz

, Berard

, Abenhaim

. Relationship between clinical classification of chronic venous disease and patient-reported quality of life: results from an international cohort study. J Vasc Surg 2004; 39: 823–8

17.

Danielsson

, Norgren

, Jungbeck

, Peterson

. Global venous function correlates better than duplex derived reflux to clinical class in the evaluation of chronic venous disease. Int Angiol 2003; 22: 177–81