Diagnosis and treatment of venous lymphedema.

Jan 2012

Raju S, Furrh JB 4th, Neglén P.

Source

The Rane Center, Flowood, MS. USA. rajumd@earthlink.net

Abstract

BACKGROUND:

Chronic venous disease (CVD) is a common cause of secondary lymphedema. Venous lymphedema is sometimes misdiagnosed as primary lymphedema and does not receive optimal treatment. We have routinely used intravascular ultrasound (IVUS) imaging in all cases of limb swelling. The aim of this study is to show that (1) routine use of IVUS can detect venous obstruction missed by traditional venous testing, and (2) iliac-caval venous stenting can yield satisfactory clinical relief and can sometimes reverse abnormal lymphangiographic findings.

METHODS:

The study comprised CVD patients who underwent iliac vein stenting. Lymphangiography was abnormal in 72 of 443 CEAP C(3) limbs, with leg swelling as the primary complaint (abnormal lymphangiography group). Clinical features and stent outcome were compared with a control group of 205 of 443 with normal lymphangiography (normal lymphangiographic group).

RESULTS:

Clinical features were a poor guide to the diagnosis of lymphedema. Isotope lymphangiography was not helpful in differentiating primary from secondary lymphedema. Venography had 61% sensitivity to the diagnosis of venous obstruction. IVUS had a sensitivity of 88% for significant (50% area stenosis) venous obstruction. At 40 months, cumulative secondary stent patency was similar for the abnormal (100%) and normal lymphangiographic (95%) groups. Swelling improved significantly after stent placement in the abnormal lymphangiographic group (mean [standard deviation] swelling grade improvement 0.8 ± 1.1) but was less  than in the control group. 

Complete swelling relief was 16% and 44% and partial improvement  was 45% and 66%  in the abnormal and normal lymphangiographic groups, respectively. 

Associated pain was present in 50% and 36% of the swollen limbs in the abnormal and normal lymphangiographic groups. Pain relief at 40 months was 87% and 83%, respectively (P = .3), with 65% and 71%, experiencing complete pain relief. Quality of life criteria improved after stent placement in both groups but to a better extent in the normal lymphangiographic group. Abnormal lymphangiography improved or normalized in 9 of 36 (25%) of those tested after stent correction.

CONCLUSIONS:

Prevailing practice patterns and diagnostic deficiencies probably result in the misdiagnosis of many cases of venous lymphedema as "primary" lymphedema. IVUS is recommended to rule out venous obstruction as the associated or initiating cause of lymphedema. Iliac venous stenting to correct the obstruction has excellent long-term patency and good clinical outcome, although results are not as good as in those with normal lymphatic function.

Elsevier

Primary lymphedema complicated by weeping chylous vesicles in the leg and scrotum: report of a case.

May 2012

Division of Vascular Surgery, Department of Surgery and Clinical Science, Yamaguchi University Graduate School of Medicine, 1-1-1 Minamikogushi, Ube, Yamaguchi, 755-8505, Japan, ksuehiro-circ@umin.ac.jp.

Abstract

We report a case of primary lymphedema complicated by leaking chylous vesicles in the toe and scrotum, caused by lymphangiectasia, which was eventually managed with lymphaticovenular anastomoses after conservative treatments proved ineffective. The patient was a 25-year-old man with a 5-year history of massive swelling with chylous weeping of his right legand scrotum. Lymphangioscintigraphy (LAS) showed dilated iliac lymph trunks causing lymph reflux. Although he was instructed in standard methods of complex therapy, it did not alleviate his symptoms. Because of the increasing frequency of cellulitis, lymphatic surgery was finally indicated. The operation consisted of lymphaticovenous anastomoses (LVA) in the ankle and groin, using a super-micro-surgical technique. After surgery, his symptoms resolved and have been controlled by self-care. Thus, early LAS to confirm the dilated iliac lymph trunks causing lymph reflux, followed by LVA might be beneficial for the management of this disease.

Springerlink

High resolution unenhanced computed tomography in patients with swollen legs.

An older abstract from 2002, laying the basic info foundation for understanding CT scans of leg lymphedema. Also mentioned in the article are DVTs and lipedema, and the use of the ultrasound.

The study:

High resolution unenhanced computed tomography in patients with swollen legs.

Sept 2002

Monnin-Delhom ED, Gallix BP, Achard C, Bruel JM, Janbon C.

Source

Service d'Imagerie Medicale, Saint Eloi Hospital, Montpellier, France. e-monnin@chu-montpellier.fr

Abstract

PURPOSE:

To evaluate the accuracy of computed tomography (CT) scan imaging in distinguishing lymphedema from deep venous thrombosis (DVT) and lipodystrophy (lipedema) in patients with swollen legs.

MATERIAL AND METHODS:

CT scans of the lower limbs were performed in 55 patients with 76 swollen legs (44 lymphedemas, 12 DVT and 20 lipedemas). Thirty-four normal contralateral legs were also similarly evaluated. Primarylymphedema was verified by lymphography or lymphoscintigraphy, whereas secondary lymphedema was documented by a typical clinical history. DVT was established by ultrasound Doppler imaging. The diagnosis of lipedema was made with bilateral swollen legs where lymphoscintigraphy and Doppler examination were both unremarkable. Qualitative CT analysis was based on skin thickening, subcutaneous edema accumulation with a honeycombed pattern, and muscle compartment enlargement.

RESULTS:

Sensitivity and specificity of CT scan for the diagnosis of lymphedema was 93 and 100%, respectively; for lipedema it was 95 and 100%, respectively; andfor DVT it was 91 and 99%, respectively. Skin thickening was found in 42 lymphedemas (95%), in 9 DVT (75%), and in 2 lipedemas (16%). Subcutaneous edema accumulation was demonstrated in 42 legs (95%) with lymphedema and in 5 (42%) with DVT but in none with lipedema. A honeycombed pattern was present only in lymphedema (18 legs or 41%); muscle enlargement was present in all patients with DVT, in no patient with lipedema, and in 4 (9%) with lymphedema.

CONCLUSION:

Edema accumulation is readily demonstrated with plain CT scan and is not present in lipedema. Specific CT features of the subcutaneous fat and muscle compartments allow accurate differentiation between lymphedema and DVT.

PubMed

Another older article from 2002, that discusses CT staging of leg (lower limb) lymphedema.

Doctors have long maintained that these radiography scans can be valuable in assesing the stages of lymphedema and in understand the physiology of a lymphedematous leg. However, this remain tremendously difficult for several reasons.

First, not many doctors appear to be well trained to interpret these scans in as much as lymphedema is concerned.

Secondly, even if a doctor was able to interpret a CT scan, there really is no specific diagnostic criteria for such a diagnoses or staging.

Notice too, in the study, the comments regarding the difference between the description of a primary lymphedema thigh versus a secondary one.

The abstract follows:

Lymphedema of the lower limbs: CT staging].

[Article in French]

Marotel M, Cluzan RV, Pascot M, Alliot F, Lasry JL.

Source

Hôpital Saint-Michel, 33, rue Olivier-de-Serres, 75015 Paris, France. martine.marotel@hopital-saint-michel.org

Abstract

Routinely performed, CT is useful and reliable for staging lower limb lymphedema. We describe methods we utilized. We found in frequency order: skin thickening, subcutaneous tissues area increase in regard the safe limb, perimuscular aponevrosis thickening, fat infiltration: lines parallel to the skin, edematous areas along perimuscular aponevrosis, lines perpendicular to the skin. The lowest fat density is increased on the pathologic side. Subfascial compartment is slightly fattened. We found huge differences between primary and secondary lymphedema for the thigh. Same images may be generated by old or young lymphedema. Rarely useful for positive diagnosis, CT is indispensable for secondarylymphedema staging (initial staging or after a recent increase). It seems us indispensable for any pretherapeutic staging (whole objectively disorders, exact upper limit, infraclinic bilaterality).

PubMed

Computerized tomography of 150 cases of lymphedema of the leg

This is a very old abstract from 1998. What was fascinating to me personally, is the CT description of the leg tissue with lymphedema. It reminded me of the ones I have had on both legs.

In the early 1970's, I had three Thompsons procedures done on my left leg, and none done on the right. There is a noticeable difference internally for the legs. Strange too was the fact that after a week in the hospital last year, it was the left leg that experienced the greatest decrease in swelling - specifically due to the fact that according to the CT, it has a much smaller amount of subcutaneous tissue then the left.

Does that make the surgery worthwhile? Hardly, the complications, including lymphoma still make it a terrible choice for lymphedema treatment. It is also the only leg that has experienced cellulitis.

The abstract follows:

Computerized tomography of 150 cases of lymphedema of the leg

Nov 1998

[Article in French]

Marotel M, Cluzan R, Pascot M, Ghabboun S, Alliot F, Lasry JL.

Source

Hôpital Saint-Michel, Paris, France.

Abstract

The aim of this work was to evaluate the usefulness of CT imaging to stage lower limb lymphedemas. Between 1992 and 1997, we studied 150 cases of lymphedema, half idiopathic and half secondary. Methods used are described. In decreasing order of frequency, we found: skin thickening, increased subcutaneous tissue surface area compared with the healthy limb, thickening of the perimuscular aponevrosis, fat infiltration: lines parallel to the skin (parallel), edematous areas along the perimuscular aponevrosis, lines perpendicular to the skin (perpendicular). The lowest fat density was increased on the diseased side. The subfascial tissue showed some fat accumulation. These results were compared with findings reported in the literature. There were very major differences between idiopathic lymphedema and secondary lymphedema of the thigh. Similar images were generally generated by new and long-standing lymphedema. Rarely useful for positive diagnosis, CT is indispensable for establishing stage initially or after recent increase and, in our opinion, is essential for pretherapeutic assessment. The CT-scan gives objective evidence of overall disorders, the exact upper limit of the lymphedema, and sometimes reveals infraclinical bilateral involvement.

Full text arricle: EM/Consulte

Intensive decongestive treatment restores ability to work

Intensive decongestive treatment restores ability to work in patients with advanced forms of primary and secondary lower extremity lymphoedema.

Dec 2011

Stanisic MG, Gabriel M, Pawlaczyk K.

Source

Department of General and Vascular Surgery, Poznan University of Medical Sciences, ul Długa 1/2, 61-848 Poznań, Poland.

Abstract

OBJECTIVE:

To show that adequate therapy for lymphoedema is able to restore ability to work.

MATERIALS AND METHODS:

The population of patients with primary lymphoedema registered in the university clinical centre diagnosed with primary or secondary lymphoedema and presumed by the national social institution as completely unable to work was selected for the retrospective analysis and divided into two groups. Group 1 consisted of 25 patients treated with a complex decongestive therapy programme daily for 3-6 weeks. The study population comprised 19 women and six men from 14 to 61 years of age (mean 31.5). In all 25 patients, complete inability to work was certified by the social institution before the treatment started. Group 2 consisted of 47 patients, 14 men and 33 women, aged from 26 to 71 years (mean 39 years) treated by so-called standard methods, who resigned from the proposed intensive treatment. In all 47 patients, complete inability to work was declared by the social institution before the treatment. Ability to work and oedema reduction were assessed by the treating physician.

RESULTS:

The intensive phase of treatment succeeded in 3870-15,330 mL oedema reduction in Group 1. After the end of therapy, 21 patients were able to work or study without any limitation and patients returned to their regular professional activity. Among four others, two were on welfare for at least 10 years, for another one welfare was their only income and one person was receiving a social pension. In none of the patients from group 2 was any significant oedema reduction observed. Every patient from group 2 maintained the social pension due to ineffective treatment.

CONCLUSIONS:

Complex decongestive therapy is a very efficient form of treatment in advanced primary and secondarylymphoedema. It allows returning to work after a short period of temporary disability without the necessity of a social pension.

PubMed