Quality of life following surgical management of lymphedema. Evaluating important outcome measures.

Quality of life following surgical management of lymphedema

Evaluating important outcome measures

By Alex Munnoch

Lymphedema

affects about 250 million globally, the majority as a result of filariasis. In Western societies, most cases are either primary in origin, or secondary to cancer treatment, trauma or recurrent cellulitis. Much work has been done looking at the impact of lymphedema on patients’ quality of life (QoL), particularly in relation to breast cancer related lymphedema of the arm. Less has been published about lower limb and genital lymphedema. One author recently suggested that primary lower limb lymphedema, compared to secondary, had little impact on quality of life (Huggenberger), while another has suggested that lymphedema severity does not appear to impact QoL (Lee). Initial studies tended to use generic questionnaires, such as the Hospital Anxiety Depression Score (HADS), Short-Form-36 and EuroQol, but over the past 10 years or so, there have been a number of validated questionnaires introduced in Germany, UK, Sweden, USA and The Netherlands, which specifically look at upper and lower limb lymphedema (FLQA-I, LYMQoL, LyQLI, LSIDS, Lymph-ICF). These all have common features, separating questions into a range of domains covering physical, practical and psychological aspects of QoL, using either a visual analogue scale or a 4-point Likert scale to record responses. The main issues for most patients relate to compression garments, limb bulk, mobility, cellulitis, and understanding of the condition.

However, the perception of quality of life and how much it is impaired is subjective and individual to each patient and the underlying aetiology of their condition.

Improvement in quality of life is now seen as an important outcome measure when looking at treatment options. Numerous authors have reviewed the impact of conservative treatments, such as manual lymphatic drainage, bandaging, compression garments, pneumatic compression, and exercise on patients’ quality of life.

Surgical management of lymphedema, popular in the 60’s & 70’s, diminished thereafter due to poor techniques, poor outcomes

Dr. Alex Munnoch FRCS Ed (Plast) is a consultant plastic surgeon at Ninewells Hospital, Dundee, Scotland providing surgical treatment for lymphedema patients and has presented his results at numerous national and international conferences. Dr. Munnoch helped develop the surgical lymphedema service at Macquarrie University Hospital in Sydney, where he holds the position of honorary professor.

and much better conservative therapies being offered by an increasing number of lymphedema therapists. In the last decade, there has been a resurgence of surgical techniques due to improved awareness, better surgical skills and patient selection.

These techniques fall into two broad categories: those designed to improve lymphatic drainage (reconstructive) and those that reduce excess tissue (debulking). Many papers have been published describing individual surgeons’ experiences with these procedures, often with small cohorts of patients, where the prime outcome measures are a reduction in limb circumference or volume and a reduction in the incidence of cellulitis. There are only a few papers reporting quality of life outcomes using validated questionnaires.

Reconstructive surgical techniques

Lymphatico-venous anastomosis (LVA) is a technique whereby functioning subdermal, or deeper collecting lymphatics are identified

Figure 1a: Primary lymphedema pre-op, 15166 mls excess volume, LyQLI score 49.

Figure 1b: 1 year postop, 1046 mls excess, LyQLI score 1.

FIGURE 1a

FIGURE 1b

and anastomosed to adjacent veins to improve the drainage of the limb. This utilises supermicrosurgical techniques, as these vessels are often less than 1mm in diameter. Multiple anastomoses are usually performed throughout the length of the limb, commonly under local anaesthetic. Modest improvements in limb volume have been reported, but the long-term effectiveness is still to be confirmed, with some authors reporting a reduction in patency after one year. Cornelissen reported significant improvements in QoL using the Lymph-ICF in a cohort of 20 women with early arm lymphedema following LVA surgery.

Lymphatic grafting has not found universal acceptance and is only performed in a few centres in Germany. Functioning lymphatic vessels are harvested from a thigh and either tunnelled to the contralateral lymphedematous leg and anastomosed to collecting lymphatics, or removed as a graft and placed between a lymphedematous arm and lymphatics in the neck. In a longterm follow-up, excellent reduction

of limb volume was achieved and significant improvement in QoL noted in a cohort of 212 patients (Springer).

Lymph node transfer has become extremely popular in the past few years, with multiple surgeons offering this procedure. A vascularised lymph node free flap is removed from a donor site (groin, axilla, neck, omentum) and transferred to the lymphedematous limb where the artery and vein are anastamosed to revascularise the tissue. No lymphatic repair is undertaken. The expectation is that lymphangiogenesis will take place, with new lymphatic channels growing from this tissue and anastomosing with lymphatics in the limb, creating new drainage pathways. Some surgeons believe in placing the nodal tissue in the old nodal basin, while others prefer to place the tissue distally on the limb. Reported outcomes are variable, depending on the measurement method for limb circumference or volume, and the stage of the lymphedema. Patel reported in 2015 on QoL outcomes following vascularised node transfer using the LYMQoL questionnaire, noting improvement

in all domains. Ciudad reported a 2.6-fold improvement in LYMQoL scores with a mean volume reduction of 36% in his cohort of 10 patients. Gratzon similarly reported improvement in LYMQoL scores in a series of 50 patients, also noting a significant reduction in the incidence of cellulitis. However, this technique is not without its risks. There is the danger of flap failure or lymphedema developing at the donor site, which may have an adverse impact on quality of life for the patient. With all reconstructive techniques it would appear that intervention at an earlier stage has better outcomes.

Release of axillary vein scarring was originally described in the 1970’s and again in the 90’s as a means to treat breast cancer related lymphedema, with up to a 60% reduction in excess limb volume being reported. This may be the underlying principle of improvement seen in some of the lymph node transfer patients. More recently, a team in Belgium has started performing scar release through small axillary incisions with harvested fat being placed around the vessel.

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They have demonstrated a reduction in limb volume postoperatively without any additional conservative therapies.

Debulking techniques

Debulking techniques were originally described over a hundred years ago and were the mainstay of treatment prior to microsurgical reconstructive techniques and compression garments. Excisional techniques, such as Charles procedure, which involved circumferential excision and skin grafting, or more limited excisional techniques, like those described by Sistrunk, Kondoloneon and Thompson, resulted in significant scarring, often limited volume reduction and further psychological distress for the patient. These techniques can, however, be very successful in the management of penoscrotal lymphedema. In 2006, Modolin published outcomes on 17 patients with penoscrotal lymphedema, reporting improved physical appearance, ambulation, hygiene, micturition and sexual function.

Full limb liposuction and 24-hour continuous compression was introduced by Brorson 20 years ago and remains an effective technique for reducing (and maintaining) excess limb volume. There is now a much greater understanding of the pathogenesis of lymphedema and the fat hypertrophy which results. Conservative therapies and reconstructive surgical techniques can reduce the lymphatic fluid element of a lymphedematous limb, but will have no impact on the adipose tissue.

Surgeons undertaking reconstructive techniques now routinely include liposuction in their treatment protocol to achieve total excess volume reduction. Brorson has demonstrated long-term limb volume reduction in the incidence of cellulitis and improved shoulder function and QoL for women with breast cancer related lymphedema.

Mean Scores

My work

I have been performing liposuction since 2005 and measuring quality of life outcomes using the HADS questionnaire. Since 2013, as part of an international collaboration, I have also been using the LyQLI and ICF-LL with all my patients pre-operatively and at 3, 6 and 12 months postoperatively. The LyQLI asks 41 questions within three domains for a maximum score of 121 (36 physical, 48 psychosocial and 39 practical). The LymphICF asks 28 questions over five domains. With all three questionnaires in use, a high score indicates poorer QoL. All patients had received the full range of conservative therapies and

were wearing compression garments prior to surgery. Liposuction was performed following Brorson’s technique and the patients were placed in compression wraps immediately, reverting to compression hosiery at two weeks. 37 lower limb lymphedema patients (3 male, 34 female) have undergone treatment in the past four years. 22 had primary lymphedema, average age was 43 years (21-67), average length of time with lymphedema was 19 years (5-38) and average excess volume was 4159 mls (848-15166). At one year follow up there was an average reduction of the original excess limb volume of 93% (62-170).

Mean scores can be seen in Table 1, demonstrating a significant improvement in QoL outcomes within all domains, particularly psychosocial, maintained at 1 year. However, at the individual level there was great variation, with some patients having full reduction of excess volume, yet still having significant QoL issues, often due to other life events (Figures 1 and 2). Similar improvements were seen with the HADS and Lymph-ICF questionnaires.

In summar y, lymphedema has an adverse impact on patients’ QoL, even when they are receiving maximal conservative therapy. Surgical interventions have been shown to help reduce limb volume, reduce the incidence of cellulitis and improve the QoL for these patients. Patient reported outcome measures need to be included when assessing the effectiveness of any treatment and should be used routinely with all patients. LP

A comprehensive set of references can be found at: www.lymphedemapathways.ca

TABLE 1

Figure 2a: Secondary lymphedema, 4979 mls excess, LyQLI score 94.

Figure 2b: 1 year postop, 248 mls excess, LyQLI score 32.

FIGURE 2a

FIGURE 2b