Calf Muscle Pump Research Articles

Venous ulcers: A reappraisal analyzing the effects of neuropathy, muscle involvement, and range of motion upon gait and calf muscle function

Chronic venous insufficiency is a complex disease that can result in severe sequelae including venous ulceration. Though the exact progression from chronic venous insufficiency to venous ulcer remains unclear, the high cost and burden of this disease on patients and society is quite clear. Sustained ambulatory venous pressures or venous hypertension plays an integral role in the development of venous ulceration and involves the failure of the calf muscle pump system. Standard of care involves compression therapy to assist the calf muscle pump. However, several cofactors may contribute to or exacerbate this disease and understanding their impact may provide insight into new treatment modalities. Nerve involvement, which may result in neuropathic pain and muscle dysfunction, alterations in mobility and a decrease in range of motion may lead to gait alterations all affecting calf muscle pump function. In this paper, we analyze these cofactors and discuss possible treatment options to target them. Physicians treating this disease should be aware of the numerous factors involved in its development. Exploring new treatment options may 1 day lessen the burden and suffering caused by venous insufficiency.

Leg ulcer treatment

Venous ulcers continue to cause problems for patients and health care systems. These are painful and unpleasant for the patient and expensive for health care providers to treat. This brief review highlights effective methods of management. There is level 1 evidence of the efficacy of compression (bandaging or stockings) in healing ulcers as well as maintaining healing. Patients with superficial saphenous reflux, with or without perforating and deep vein incompetence, benefit from superficial venous surgery. This does not speed ulcer healing but is effective at preventing recurrence after healing with compression. Minimally invasive methods of managing incompetence of superficial saphenous trunks, including endovenous laser ablation, radiofrequency ablation, and foam sclerotherapy are probably also effective in treating patients with venous leg ulcers. Perforating vein ligation is commonly combined with superficial venous surgery for leg ulcer patients, but no systematic data are available to define the role of this treatment. Some centers use deep vein reconstruction to restore competence to deep vein valves. Insufficient data have been published to allow any general recommendation to be made for this treatment. A limited number of drugs have efficacy in promoting leg ulcer healing. They may be used in combination with compression treatment in patients with ulcers refractory to other methods of management. No particular ulcer dressing has been shown to be effective in speeding ulcer healing.

Risk factors related to the failure of venous leg ulcers to heal with compression treatment

Compression therapy is the most widely used treatment for venous leg ulcers and it was used in different forms for more than 400 years. Published healing rates of venous ulcers obtained with compression therapy vary widely from 40-95%. According to numerous studies, it has been suggested that the application of external pressure to the calf muscle raises the interstitial pressure resulting in improved venous return and reduction in the venous hypertension. Several risk factors have been identified to be correlated with the failure of venous leg ulcers to heal with compression therapy (longer ulcer duration; large surface area; fibrinous deposition present on >50% of the wound surface and an Ankle Brachial Pressure Index (ABPI) of <0.85. An open prospective single-center study was performed in order to determine possible risk factors associated with the failure of venous ulcers to heal when treated with multi-layer high compression bandaging system for 52 weeks. In the study, 189 patients (101 women, 88 men; mean age 61 years) with venous leg ulcers (ulcer surface >5 cm(2); duration >3 months) were included. The study excluded patients with arterial disease (ABPI <0.8), heart insufficiency with ejection fraction (EF) <35, pregnancy, cancer disease, rheumatoid arthritis, and diabetes. Based on clinical opinion and available literature, the following were considered as potential risk factors: sex, age, ulceration surface, time since ulcer onset, previous operations, history of deep vein thrombosis, body mass index (BMI), reduction in calf circumference >3 cm during the first 50 days of treatment, walking distance during the day <200 meters, calf:ankle circumference ratio <1.3, fixed ankle joint, history of surgical wound debridement, >50% of wound covered with fibrin, depth of the wound >2 cm. Within 52 weeks of limb-compression therapy, 24 (12.7%) venous ulcers had failed to heal. A small ulceration surface (<20 cm(2)), the duration of the venous ulcer <12 months, a decrease in calf circumference of more than 3 cm, and emergence of new skin islets on >10% of wound surface during the first 50 days of treatment were favorable prognostic factors for ulcer healing. A large BMI (>33 kg/m(2)), short walking distance during the day (<200 m), a history of wound debridement, and ulcers with deepest presentation (>2 cm) were indicators of slow healing. Calf:ankle circumference ratio <1.3, fixed ankle joint, and reduced ankle range of motion were the only independent parameters associated with non-healing (P < .001). The results obtained in this study suggest that non-healing venous ulcers are related to the impairment of the calf muscle pump.

Effect of the leg muscle pump on the rise in muscle perfusion during muscle work in humans

The transient and steady-state effects of the calf muscle pump on the rise in muscle perfusion during rhythmic plantarflexions were investigated in 20 volunteers. Because a large hydrostatic column would increase the effect of a muscle pump, exercise in the supine and head-up tilted positions was compared. Within approximately 15 s of the start of muscle work, femoral artery flow (ultrasound Doppler) rose 0.37 L/min above rest in the supine and 0.5 L/min above rest in the tilted position. The latter is a significantly larger rise (P < 0.05). After 80 s of muscle work, femoral flow was stable at 0.38 and 0.39 L/min above rest in the supine and tilted positions, respectively. We conclude that the muscle pump contributes to muscle perfusion during the initial phase of muscle work, but that metabolic vasodilation is a more important determinant of muscle perfusion during steady-state muscle work.

Enhancing Hemodialysis Efficacy through Neuromuscular Stimulation

Beat-to-beat blood pressure monitoring was used to assess the efficacy of reflex-mediated, calf muscle pump stimulation to enhance cardiovascular refilling in subjects in the supine and seated positions, with extension of this stimulation technology to dialysis evaluated in 2 dialysis patients. Micromechanical stimulation (50 μm at 45 Hz) of the plantar surface was found to significantly increase both the rate and volume of cardiovascular refilling relative to that observed for subjects in the supine position. During hemodialysis, calf muscle pump stimulation permitted significantly increased ultrafiltrate removal, specifically from 78 to 96% of clinical goal, while serving to maintain both blood pressure and blood volume.

Avaliação do volume de fluxo venoso da bomba sural por ultra-sonografia Doppler durante cinesioterapia ativa e passiva: um estudo piloto

CONTEXTO: O fisioterapeuta na unidade hospitalar atua sobre os efeitos da hipoatividade ou inatividade do paciente acamado. Na prática diária, a contração do músculo da panturrilha é difundida entre os profissionais de saúde no ambiente hospitalar, principalmente nos períodos de pré e pós-operatório, como forma de diminuir a estase venosa e os riscos de trombose venosa profunda nos membros inferiores. OBJETIVO: Avaliar o volume de fluxo venoso na bomba sural, através de ultra-sonografia doppler, durante cinesioterapia ativa e passiva (flexão plantar do tornozelo). MÉTODOS: A amostra foi constituída por 30 indivíduos escolhidos aleatoriamente e submetidos a ultra-sonografia doppler da veia poplítea direita, visando mensurar o volume de fluxo sanguíneo em quatro momentos: repouso, compressão manual da panturrilha, movimentação passiva e ativa do tornozelo em flexão plantar. Na análise dos resultados, utilizou-se o teste t, sendo utilizado um valor de p &lt; 0,05 como índice de significância estatística. RESULTADOS: Na amostra constituída, 16 eram do sexo feminino e 14 do sexo masculino, apresentando as seguintes médias: idade (31,57 anos), altura (1,68 m), peso (68,25 kg) e índice de massa corporal (24,16). Na análise estatística, a flexão plantar do tornozelo realizada de forma passiva, quando comparada ao valor basal, é significante (p &lt; 0,000056) em relação à ativação da bomba sural, embora não tanto quanto o exercício ativo (p &lt; 0,0000016). Também mostrou significância a compressão manual do músculo tríceps sural em relação ao exercício passivo (p &lt; 0,000000081). CONCLUSÃO: Neste estudo, a flexão plantar do tornozelo de forma ativa mostrou-se estatisticamente mais eficaz do que a passiva na ativação da bomba sural, aumentando o volume do fluxo de sangue na veia poplítea e diminuindo a estase venosa nos membros inferiores.

Prevalence and Etiology of Delayed Orthostatic Hypotension in Adult Women

Madhavan G, Goddard AA, McLeod KJ. Prevalence and etiology of delayed orthostatic hypotension in adult women. Objective To evaluate the contributing roles of venous status, microvascular filtration, and calf muscle pump activity in the etiology of delayed orthostatic hypotension (OH). Design Unblinded within-subjects trial. Setting Academic clinical research center. Participants Convenience sample of healthy adult women (N=30) with an age range of 30 to 65 years. Intervention Plantar micromechanical stimulation applied at a 45-Hz frequency and a 50-μm amplitude for a duration of 30 minutes during upright sitting. Main Outcome Measure Diastolic blood pressure (DBP). Results White women (mean age, 51.8±1.3y) were recruited and screened for delayed OH. About one quarter (9/33) of the screened subjects showed delayed OH as determined by a significant decrease in blood pressure after at least 15 minutes of quiet sitting. Air plethysmographic assessment provided no evidence of venous insufficiency (venous filling index, >2.5mL/s; venous volume, >80mL) or excessive microvascular filtration in the affected subjects, whereas activation of the calf muscle pump (CMP) through plantar-based micromechanical stimulation consistently resulted in a significant increase in systolic blood pressure (SBP) (ΔSBP=22.8±3.9mmHg, P=.003) and DBP (ΔDBP=20.9±3.3mmHg, P=.002). Conclusions About 25% of the adult women studied showed delayed OH during quiet sitting and the proximate cause appears to be neuromuscular in origin, specifically inadequate calf muscle tone, because venous and microvascular filtration status is normative in the delayed OH subpopulation and CMP stimulation reverses the hypotension.

The application of inertial and magnetic sensors to the monitoring of calf muscle pump activity

On long distance journeys passengers at high risk from deep vein thrombosis (DVT) are recommended to exercise on a regular basis to contract the calf muscle pump and encourage venous return. If a passenger fails to complete an exercise program that induces active contraction of the calf muscle pump they will remain at increased risk of DVT. This paper presents a novel inertial and magnetic sensor-based technique for monitoring calf muscle pump activity. The technique could be implemented into a system for monitoring the level of calf muscle pump activity in persons with limited mobility. Such a system could be used to provide a reminder to the user that there is a need to exercise should they have forgotten to exercise, failed to exercise sufficiently or exercised incorrectly. The proposed technique was evaluated by comparison with calf muscle pump activity measured using an electromyography (EMG) sensor. Results show that the technique can be used to monitor calf muscle pump activity over a wide range of leg exercises.

Reversal of Lower Limb Edema by Calf Muscle Pump Stimulation

Peripheral edema (PE) is commonly coupled with heart failure, restrictive cardiomyopathy, nephrotic syndrome, renal failure, and hypoproteinemia. Diuretics and/or limb elevation, although commonly prescribed to treat PE, are often insufficient to remove sufficient fluid to prevent complications. We assessed the ability of the calf muscle pump (CMP) stimulation to reverse PE. Fluid volume was evaluated by air plethysmography in the right legs of 54 adult women (mean age 46.7 +/- 1.5 years) following venous status assessment. Change in calf volume was assessed during 30 minutes of quiet sitting, followed by 30 minutes of sitting with CMP stimulation via micromechanical stimulation of the plantar surface. Leg volume changes demonstrated a bimodal distribution. Leg volume decreased during quiet sitting in 56% of the study group, whereas in 44% of the group, significant lower leg fluid pooling was evident (increase in calf volume of 14.0 +/- 0.3 mL/h). CMP stimulation reversed the fluid pooling in the edematous group (-2.7 +/- 0.1 mL/h) and was able to accelerate fluid removal in the nonedematous group. Approximately two fifths of adult women experience substantial pooling when their lower limbs are maintained in a dependent position. Lower-extremity edema exhibited by these women may primarily be due to inadequate calf muscle tone because exogenous stimulation of the CMP was sufficient to halt and reverse fluid pooling. Whether CMP stimulation would provide a means to treat PE in individuals with edema-related health complications, such as congestive heart failure, merits further investigation.

The Tromped: A Solution for Flight-Related Deep Vein Thrombosis?

Flight-related deep vein thrombosis (DVT) is well recognized. Reduced venous return occurs during immobility. This alteration in venous hemodynamics may contribute to DVT development. A prototype design of an in-flight exercise device to stimulate ambulatory bloodflow while seated has been developed, consisting of a foot pedal attached to a base by a hinge mechanism. Four devices of differing resistance were evaluated. Calf muscle pump function was assessed by air plethysmography in 10 healthy volunteers. Ejection volume fraction and RVF were determined in the standing position (control values) and were compared with those achieved by depression of the 4 devices while seated. Similar EVF and RVF values were achieved by the control and 2 of the devices. Plantar flexion against a predetermined resistance can effectively activate the calf muscle pump while seated and may reduce the incidence of flight-related DVT.

Successful Iliac Vein and Inferior Vena Cava Stenting Ameliorates Venous Claudication and Improves Venous Outflow, Calf Muscle Pump Function, and Clinical Status in Post-Thrombotic Syndrome

Successful Iliac Vein and Inferior Vena Cava Stenting Ameliorates Venous Claudication and Improves Venous Outflow, Calf Muscle Pump Function, and Clinical Status in Post-Thrombotic Syndrome

Injection Drug Use

Injection drug use has devastating effects on the veins, skin, muscles, and joints of the lower extremities, thus increasing the risk of chronic venous disease (CVD). We examined the following risk factors for CVD in persons who injected drugs: health and drug use history, ankle mobility, pain, and skin and wound assessment. Because of deep venous thrombosis and injury and immobility to the calf muscle pump from injected drugs, CVD occurs at a young age. Decreased ankle joint movement, decreased walking, and increased pain are associated with worsening CVD clinical classification. Associated venous ulcers tend to be multiple and large by the time wound care is sought. Cellulitis and abscesses may also be present. Injection drug users serve as a model for the multifactorial nature of CVD including vein damage, diminished ankle range of motion, and decreased calf muscle strength. Persons who inject drugs need to have their lower extremities assessed for CVD on a routine basis.

Métodos de diagnóstico não-invasivos para avaliação da insuficiência venosa dos membros inferiores

A avaliação clínica dos membros inferiores na insuficiência venosa por si só não identifica os sistemas envolvidos ou os níveis anatômicos, sendo necessários exames complementares. Esses exames podem ser invasivos ou não-invasivos. Os invasivos, como flebografia e pressão venosa ambulatória, apesar de terem boa acurácia, trazem desconforto e complicações. Dentre os não-invasivos, destacam-se: Doppler ultra-som de ondas contínuas, fotopletismografia, pletismografia a ar e mapeamento dúplex. O Doppler ultra-som avalia a velocidade do fluxo sangüíneo de maneira indireta. A fotopletismografia avalia o tempo de reenchimento venoso, fornecendo um parâmetro objetivo de quantificação do refluxo venoso. A pletismografia a ar permite quantificar a redução ou não da capacitância, o refluxo e o desempenho da bomba muscular da panturrilha. O dúplex é considerado padrão-ouro dentre os não-invasivos, porque permite uma avaliação quantitativa e qualitativa, fornecendo informações anatômicas e funcionais, dando avaliação mais completa e detalhada dos sistemas venosos profundo e superficial.

Calf muscle pump impairment and delayed healing of venous leg ulcers: Air plethysmographic findings

There is a need for a diagnostic tool to predict clinical outcome of venous leg ulcer patients, as the prognosis of healing based on clinical data alone has not appeared to be satisfactory. Air plethysmographic assessment of calf muscle pump was performed in the supine and upright position in 129 patients with active ulcers on their legs. All patients were managed in a specialized leg ulcer clinic. Results of air plethysmography were compared to clinical data and time of healing of ulcers. Muscle pump failure was found in 42.6% of extremities (supine position, 33.3%; upright, 22.5%; both, 12.4%). Patients with insufficient pump were older, and their ulcers were larger. Failure of pump was found more often in patients who began the treatment after long, unsuccessful, non-specialized care. Healing time of ulcers was prolonged in cases with insufficient pump. Regarding the subgroups with good clinical prognosis (patients with small ulcers or with a short history of ulceration), it was found that insufficiency of muscle pump correlated with delayed healing. It could be summarized that venous leg ulcers associated with calf muscle failure were larger, long-standing, and that their healing even after specialized treatment was delayed. Impaired muscle pump function revealed in plethysmographic examination can be a prognostic factor of delayed healing of leg ulcer. Ulcers with poor prognosis according to plethysmographic findings, and no quick recovery after standard management, should be considered for advanced therapies.

Successful iliac vein and inferior vena cava stenting ameliorates venous claudication and improves venous outflow, calf muscle pump function, and clinical status in postthrombotic syndrome

Conclusion: Successful stenting of iliac-femoral (I-F) veins or the inferior vena cava (IVC), or both, improves venous claudication and some measures of venous function. Summary: The purpose of the study was to determine effects of successful stenting of patients with chronic venous disease. Consecutive patients were analyzed. The patients had previously failed conservative treatment with compression or wound care for at least 12 months. Patients had moderate-to-advanced venous disease (CEAP classifications 3 to 6), with or without venous claudication. Venous claudication was determined by the judgement of at least three independent examiners. Plethysmography was used to determine venous filling index (VFI) as a measure of venous reflux, ejection fraction (EF) as a measure of calf muscle pump function, and residual volume fraction (RVF) as a measure of ambulatory venous hypertension. Outflow fractions at 1 and 4 seconds were also determined. The study assessed 23 limbs in 16 patients. Control limbs (n = 9) were those limbs without infrainguinal clots on duplex scanning and without prior I-F thrombosis. Patients were excluded if they had malignancy, stent occlusion or stenosis, peripheral arterial disease, symptomatic cardiac disease, or unrelated causes of walking impairment. Venous outflow and calf muscle function was reduced and RVF was increased in the limbs with I-F ± IVC thrombosis compared with the control group (P < .05 for all comparisons). At 8.4 months, successful I-F ± IVC stenting improved venous outflow, calf muscle pump function, and RVF (P < .001 for all comparisons). CEAP class also improved after intervention (P < .05). Patients with successful I-F ± IVC stenting did have worsening of VFI by 24% (P = .002). At 8.4 months, venous outflow, calf muscle pump function, and RVF in the control limbs did not differ from the stented limbs. Venous reflux as measured by VFI was worse in the stented limbs (P < .0025). Before treatment, 15 of 23 limbs (10 of 16 patients) had incapacitating venous claudication. After successful stenting, venous claudication was eliminated in all the affected limbs (P < .001). Comment: The study suggests that patients with venous claudication due to an I-F venous obstruction may be improved by stenting. This study, however, has several weaknesses: it is essentially a retrospective analysis, the follow-up is short, there was no objective measurement of venous claudication, and only successfully treated patients were analyzed, making it impossible to determine the true efficacy of I-F ± IVC stenting in patients with chronic venous disease.

The Effect Of Graded Compression Socks On Maximal Exercise Capacity And Recovery In Runners

The application of compresion socks in the clinical setting is well known, i.e. for the treatment of edema, lymph edema, phlebitis, varicose veins, spider veins and deep vein thrombosis. However, renowned international athletes have occasionaly worn compression socks during races, claiming that it improves their performance and reduce their recovery time. The rationale behind graded compression socks is that it may enhance venous return to the heart through a more efficient calf muscle pump, leading to increased endurance capacity. It is also claimed that muscles are kept more compact (causing less muscle damage), thereby minimizing muscle fatigue. None of the above claims have been studied in an athletic population. PURPOSE: To determine whether athletes improve their maximal exercise capacity and recovery when running with graded compression socks. METHODS: Ten healthy male students (age = 23 ± 1.9 years, height = 182 ±6.3 cm, body mass = 82 ± 11.4 kg) volunteered to participate in this study. All subjects completed two identical maximal exercise protocols, with and without compression socks, in random order and within seven days. Blood lactate concentrations were measured at rest, and at 2, 10 and 30 min after the exercise tests. Maximal exercise capacity was determined on a h/p/cosmos saturn treadmill using a standardized, incremental protocol to exhaustion. Physiological responses were measured with the Cosmed Quark b2 metabolic system. RESULTS: There were no statisticaly significant differences in maximal oxygen consumption, heart rate or minute ventilation between the two exercise tests (p > 0.05). Subjects did, however, show a faster lactate recovery rate after exercise when wearing the compression socks. CONCLUSIONS: Our study does not support the claims of some distributors of compression socks that it would enhance short-term, maximal exercise performance. The possibility that compression socks may improve recovery after strenuous exercise seems promising and warrants further investigation.

Does size matter? The impact of calf muscle volume on venous return in patients with venous leg ulcers

The calf muscle pump is recognized as an integral component of effective venous return from the lower limbs. The aim of this study was to determine if there is a correlation between calf muscle volume and venous blood flow among patients with venous leg ulcers. Nine patients with venous leg ulceration were recruited for this study. These patients underwent haemodynamic testing using duplex ultrasound to measure peak venous velocities in response to voluntary maximum plantar flexion, with and without compression bandaging. Each patient then had magnetic resonance imaging (MRI) of the lower limbs. Calf muscle volume was calculated from the MRI images using a specially designed Matlab computer program to identify and count muscle pixels. Analyses applied Pearson's correlation coefficient to determine correlation between calf muscle volume and mean peak venous velocities in response to voluntary contraction. No correlation was seen between calf muscle volume and haemodynamic venous return in response to voluntary contraction, with or without compression bandaging. The volume of calf muscle available for promoting venous return alone may not be an accurate indicator of muscle functioning capability.

Avaliação da bomba muscular da panturrilha em pacientes portadores de varizes primárias dos membros inferiores através da pletismografia a ar

OBJETIVO:Avaliar a influência do refluxo das veias musculares da panturrilha (surais e geniculares) na função da bomba muscular da panturrilha em pacientes com varizes primárias nos membros inferiores. MÉTODOS: Estudo transversal prospectivo, no qual os pacientes foram avaliados por meio de exame físico, mapeamento dúplexe pletismografia a ar. Foram selecionados 120 pacientes divididos em dois grupos (60 indivíduos em cada). O primeiro grupo apresentava refluxo das veias musculares da panturrilha e o segundo grupo apresentava ausência de refluxo. Cada grupo foi analisado com pletismografia a ar para estudo da função da bomba da panturrilha. As variáveis estudadas foram fração de ejeção, fração do volume residual, volume ejetado e índice de enchimento venoso para membro inferior direito e esquerdo. RESULTADOS: No grupo com refluxo, índices de fração de ejeção abaixo de 60% (com p &lt; 0,001) foram encontrados em 82,3% (em perna esquerda) e em 74,6% (em perna direita) dos casos. Índices de fração do volume residual acima de 60% foram identificados em 62,5% (em perna esquerda, p = 0,015) e em 86,7% (em perna direita, p = 0,014) dos casos avaliados. Não houve variação estatisticamente significativa para o índice de enchimento venoso entre os grupos com e sem refluxo, com p = 0,140 e p = 0,140 para pernas esquerda e direita, respectivamente. Foram encontrados 63,6% dos pacientes com refluxo das veias da panturrilha (em perna esquerda), e 61,8% (em perna direita) com volume ejetado acima de 150 mL (p = 0,001). CONCLUSÕES: Houve alteração da bomba muscular da panturrilha, levando a uma diminuição de sua função, em ambos os membros inferiores por conseqüência da presença do refluxo das veias musculares da panturrilha (geniculares e surais).

Mobility in Patients with Venous Leg Ulceration

To compare mobility in patients with venous leg ulcers to matched controls and determine the influence of mobility, age and ulcer size on ulcer healing. 25 leg ulcer patients, and 25 matched controls wore a mobility monitor (ActivPAL, PAL Technologies Ltd, Glasgow, Scotland)) which recorded the number of steps and amount of time spent walking, standing, sitting or lying for a one-week period. A walking index was calculated. The ulcer group were treated with compression bandaging and ulcer healing recorded over 12 weeks. There were 13 female subjects in each group. The median age was 70.5 (range 30-89) years. There was no difference in the amount of time either group spent standing, walking and resting. There was a significant reduction in the number of steps taken and in the walking index in the ulcer group compared to controls (ulcer group, median 6,685 steps/day, range 2074-17,999; control group median 8750, range 4917-16,043, p<0.05, Mann Whitney u test). Smaller ulcers and ulcers of recent onset were most likely to heal within 12 weeks (p=0.005 and p=0.011 respectively, Chi squared test). The percentage of time spent mobilising and resting did not influence ulcer healing (r(s)=-0.125; p=0.55). Mobility patterns among patients with leg ulcers are not significantly different to age matched controls. Ulcer patients take fewer steps per week compared to controls indicating they have reduced calf muscle pump function. Further studies are required to determine whether therapies which increase calf muscle activity have a role in ulcer treatment.

Hemodynamic impairment, venous segmental disease, and clinical severity scoring in limbs with Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) is a complex congenital anomaly featuring two or more of the following: (1) capillary malformations (port-wine stains), (2) soft tissue or bony hypertrophy (or both), and (3) varicose veins or venous malformations. With the purpose of determining the actual significance of venous impairment in patients with KTS, we quantified the venous valvular competency and calf muscle pump function and examined their effect on clinical severity. Included were patients with near-normal function of affected limb(s) and minimal/small foot hypertrophy. Excluded were those with deep venous hypoplasia, aplasia or thrombosis, lymphedema, limb length discrepancy (>2.5 cm), peripheral arterial (ankle-brachial index <1.0), or cardiac disease and walking impairment. Venous duplex scanning, ascending venography, magnetic resonance imaging, strain gauge plethysmography, and a bone scanogram were performed. We studied eight men and seven women aged 15 to 51 years (median, 24 years). The KTS involved 17 limbs (unilateral in 13 patients and bilateral in 2). Contralateral limbs in patients with unilateral KTS acted as controls (n = 13). Venous clinical severity was graded according to the CEAP and venous clinical severity score (VCSS), and reflux complexity was classified according to the venous segmental disease score. Outflow obstruction (outflow fraction at 1 and 4 seconds; OF(1) and OF(4), respectively), reflux (venous filling index), calf muscle pump function (ejection fraction), and hypertension (residual volume fraction) were determined in both limbs with strain gauge plethysmography. Data, reported as median and interquartile range, were analyzed with the Mann-Whitney test. Varicose veins or venous malformations occurred in the medial, posterior, or anterolateral limb segments of the ankle (7/17, 7/17, and 9/17), calf (10/17, 8/17, and 12/17), knee (9/17, 8/17, and 8/17), and thigh (10/17, 6/17, and 8/17, respectively). Venous malformations occupied the subcutaneous space (17/17) and extended into the subfascial space in 6 (35.3%) of 17 limbs. Abnormal reflux (>0.5 seconds) was distributed in the great (64.7%; 11/17) and small (5.9%; 1/17) saphenous veins and the common femoral (23.5%; 4/17), femoral (41.1%; 7/17), popliteal (29.4%; 5/17), perforator (70.6%; 12/17), and axial calf (35.3%; 6/17) veins. There was no difference in the OF(1) and OF(4) between the affected limbs and the controls. Limbs with KTS had a fivefold greater venous filling index (0.133-0.46 mL . 100 mL(-1) . s(-1); 0.258 mL . 100 mL(-1) . s(-1)) than the controls (0.034-0.055 mL . 100 mL(-1) . s(-1); 0.046 mL . 100 mL(-1) . s(-1); P < .0001), and this was linked to a higher venous segmental disease score (3 [2-4] vs 0 [0-1]; P < .0001). Limbs with KTS had half the ejection fraction (20.8%; 12.3%-24%) of the controls (39.3%; 30.9%-64.6%) and twice as high a residual venous fraction (77% [69.6%-84.5%] vs 40.9% [20.6%-60%]; both P < .004). Patients complained of swelling (100%; 15/15), aching (100%; 15/15), pain (93.3%; 14/15) and heaviness (100%; 15/15), tiredness (66.7%; 10/15), and tightness (33.3%; 5/15) of the limb(s) with KTS. Limbs with KTS had a worse (1) venous clinical severity by 11 VCSS points (11 [8-12] vs 0 [0-1]) and (2) clinical status by 3 CEAP classes (C3 [C3-C4] vs C0 [C0-C2]) than the control limbs (both P < .0001). Venous disease in limbs with KTS is a major source of morbidity in affected patients. Limbs with KTS are characterized by complex reflux patterns, severe valvular incompetence, calf muscle pump impairment, and venous hypertension, thus explaining the advanced clinical severity (VCSS) and CEAP grade.