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1 Introduction

Seventeen years after a paradigm shift in the treatment of lipoedema, the operative method (carried out as “lymphological liposculpture”) has gained increasingly more acceptance. An estimated incidence of the disease lipohyperplasia dolorosa [1] with 11 % of the female population would represent more than four million women affected by this disease in the legs and arms. Well over 80 % of patients are affected on all four extremities. Despite this significant frequency in the population, there is not enough knowledge about the lymphological disease pattern of lipoedema, including in specialist circles.

2 Clinical Picture

In contrast to previous publications, it must be noted that the frequency of the lipoedema on the arms is surely within the 80–90 % range (Fig. 79.1).

Fig. 79.1
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Lipoedema of the arms

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Examination of the arms is not usually performed, as patients are primarily examined for venous diseases due to pain in the leg and suspected venous disorders. As a result, they see a phlebotomist and the arms are not examined. Lipoedema patients with suspected lipoedema of the legs must always be examined on the arms as well, in order to establish the full clinical picture (Fig. 79.2).

Fig. 79.2
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(a) Lipoedema stage 1. (b) Lipoedema stage 2. (c) Lipoedema stage 3

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Although first described by Allen and Hines [2] as far back as the 1940s, the distribution of the fatty tissue is often not taken seriously as a clinical diagnosis – the cardinal symptoms are deliberately overlooked and patients are given the stigma of obesity. However, medical history regularly shows that experiments with diet and sport fail to reduce the disproportional enlargement of the fatty tissue, especially that of the extremities. This urges patients to make futile attempts that offer everything from ineffective diets to high-performance sports. They are still unsuccessful [2].

Since patients usually seek medical help because of their leg problems, the lipoedema should always be taken into consideration with a differential diagnostic whenever leg complaints occur symmetrically: the feet are slim and unaffected from the ankle joint onward, and there is no pitting or even a Stemmer’s sign on the feet or pitting of the ankles in the area of the aching legs. When this becomes clear to the physician, it is necessary to look at the patient’s whole body (nearly unclothed) and pay special attention to the arms. As a rule, the symmetric distribution of the fatty tissue is also pronounced from the wrists to the shoulder, as well as from the ankle to the groin. The simple medical history question as to whether fat distribution on the trunk can be reduced via diet will normally be affirmed; a simple pressure test (performed as gentle, paradoxical pinching on the outer sides of the legs and arms) will be confirmed as painful. If the same pressure test is entirely normal during manual examination of the abdomen, the painfulness of the fat on the extremities compared to the painless fat on the trunk is clinically evident. Beyond these clinical examinations and a correctly performed history, further diagnostic methods are not helpful any more, even under medical guideline aspects [3]. A sonography of the fatty tissue may outline the difference between lymphoedema and lipoedema, but the decision regarding therapy will not be amended.

The pathophysiological moment of high-volume transport insufficiency, as researched by Brauer [4] and set out as a model in the Marsch Thesis [5], is the goal of conventional therapeutic approaches. Complex decongestive therapy is responsible for removing the imbalance between the production and removal rate caused by increased lymph formation, in totally normal lymph vessels in the arms and legs.

In lipoedema, the lymph vessels are intact but fail in their transport capacity in the long run – this constitutes a dynamic deterioration of the clinical picture. It is important to interrupt this dynamic, which is why early therapies are indicated, whether with conventional or curative surgical methods [6].

3 Pathophysiology

The fat on lipoedema patients’ extremities is of a particular quality, as it is a type of tissue that does not increase in volume, but rather in the number of cells. Due to the pain of the shift, the lipoedema is to be called lipohyperplasia dolorosa and not lipohypertrophy [7]. The diagnosis “lipohyperplasia dolorosa” requires obligatory proof of:

  1. 1.

    The disruption to symmetrical fat distribution on the extremities in relation to the torso in the case of asymptomatic hands and feet

  2. 2.

    The dietary and exercise-related resistance of fat due to genetic disproportion

  3. 3.

    Painfulness

  4. 4.

    Increased vascular fragility

In almost no case will the physician find pitting in the feet area or a positive Kaposi-Stemmer’s sign in the toe region. The absence of this clinical symptom is an undoubted indication that a lipo-lymphoedema does not exist. It is amazing, however, to see how often this referral-diagnosis is put forward by patients. The aim of these therapeutic options is to treat the high-volume transport insufficiency of the lipohyperplasia dolorosa, as this form of insufficiency in the lymphatic vessels of the extremities leads to a backflow of lymph between the fat cells, and therefore to increased pressure in the tissue of completely sound but disproportional lymphatic vessels that transport well (Fig. 79.3).

Fig. 79.3
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Age-adapted uptake rate by Brauer

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4 Extragenital Impact of Sex Hormones

Estrogen promotes proliferation, dilates the vessels, and increases extracellular fluid. The formation of edemas is encouraged by cycle-dependent hormone shifts and during stages of estrogen dominance (puberty, climax). Progesterone (corpus luteum hormone) promotes tissue differentiation and increases energy metabolism (in order to supply energy in pregnancy).

Together, these two hormones encourage the development of the typical gynoid fat distribution (particularly buttocks and thighs): for the first time in puberty and particularly during pregnancy [8].

The physiological effects of female sex hormones thus indicate causes for the clinical observation of the first occurrence of lipoedema in puberty, a “maturation” of preclinical lipoedema in pregnancy and also increased health problems during the premenopause phase (estrogen dominance).

These indications of pathophysiological links were brilliantly worked on at the end of last century, resulting in the development of therapeutic options beyond complex decongestive therapy, i.e., a surgical treatment that prioritizes the irreversible removal of fatty tissue from a medical indication [9–11].

5 Therapeutic Options

The task was to normalize the imbalance ratio of lymph production and removal, to interrupt and halt the dynamic deterioration of the disease, and to achieve a healing procedure (namely surgical). Although it is not fully understood how lymph formation is disturbed, it is undoubtedly clinically evident that healing can be achieved by means of surgery – operations which have been successfully performed for 17 years.

Following the introduction of tumescence local anesthesia by Klein in the 1980s and liposuction in Germany by Sattler in the early 1990s for cosmetic indications, a secure set of tools became available [12]. Using such tools, the first tentative attempts in applying medically indicated liposuction on lipohyperplasia dolorosa began in Düsseldorf in 1997.

6 Lymphological Liposculpture

Despite clear clinical findings, the “pioneer patients” were all subjected to preoperative indirect lymphangiography and dynamic functional lymphoscintigraphy on the arms and legs. This was to gain a clear preoperative picture of lymph flow and to demonstrate that the medically indicated removal of fatty tissue, i.e., lymphological liposculpture on lipohyperplasia dolorosa would have no adverse side effects on the lymphatic vessels. None of these patients developed a lymphoedema resulting from the treatment. Long-term studies were completed after 7 years in 2004 (Fig. 79.4).

Fig. 79.4
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Lipoedema study 2004

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Once again, the operated patients had to undergo a clinical examination in which no lymphoedemas were detected. In addition, they also underwent a dynamic functional lymphoscintigraphy and an indirect lymphangiography [13]. The clinical aspect was proven: none of the operated patients had developed a lymphoedema caused by the operation. These findings were histologically supported in studies by Rapprich [14] and Schmeller [15] as well as negative evidence of lymphocytes in the extracted tissue.

The efficiency of the method was also substantiated numerous times by other surgeons [6, 14, 15]. From a clinical point of view, it is absolutely clear that lymphological liposculpture procedures – if applied on lipohyperplasia dolorosa and if the fatty tissue of the extremities is fully removed (and this is conditio sine qua non) – will result in healing of the clinical picture. Moreover, the surgical procedure requires mandatory postoperative management in order to achieve the excellent results. “Lymphological liposculpture” is not a plastic surgery procedure for removing fatty tissue; it means consistent operative, conventional treatment of a lymphological disease [10, 16].

In 2004, i.e., 7 years after the paradigm shift, the quality of the procedure was researched for the first time in a large clinical monocentric study using 140 patients [17]. None of the patients had suffered from a postoperative lymphoedema. Five percent of the operated patients occasionally underwent complex decongestive therapy. The healing rate was therefore 95 %.

7 Cologne Lipoedema Study 2012

Fifteen years after the introduction of the lymphological liposculpture method, the authors reviewed the data on a significantly greater number of patients in order to establish the quality of the method and its effect on healing. Throughout the entire Cologne Lipoedema Study in 2012, 3,140 patients suffering from a lipoedema were examined at the surgery. Nineteen percent of these patients were operated on. The indication for surgery had been set as follow: all patients who did not exhibit internistic (clinical) contraindications and weighed a maximum of 125 kg. Since the surgical treatment is private and must be paid for by the patient, a further limitation arose, namely, those willing and able to undergo this operation. In addition, a number of patients were familiar with conventional therapy and did not want to go without it.

Of the total number of lipoedema patients, 19 % were operated on, i.e., 592 patients. These patients were then asked to answer a questionnaire. The questions were as follows (Fig. 79.5):

Fig. 79.5
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Cologne Lipoedema Study 2012: questionnaire

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  1. 1.

    How long had you been suffering from symptoms until the correct diagnosis was made?

  2. 2.

    For how long did you experience postoperative discomfort?

  3. 3.

    Do you still have pain?

  4. 4.

    Do you still undergo complex decongestive therapy?

  5. 5.

    What do you think of the cosmetic result?

  6. 6.

    How would you evaluate your quality of life?

  7. 7.

    Would you have the surgery again?

  8. 8.

    Would you recommend the operation?

The response rate was 57 % with 292 patients.

Since all patients had to undergo several operations due to the amount of fat, among other things, the total number of operations from 1997 until the beginning of the study in May 2012 for the indication lipohyperplasia dolorosa amounted to 1,654 procedures (Fig. 79.6). From medical records, we determined regular accumulation of stage II at an average age of 23–45 – the youngest patient was 15 and the oldest 83 years of age.

Fig. 79.6
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CologneLipoedema Study 2012:response rate

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The amount of removed fat varied between 1,500 and 8,000 mL per operation; in the most complex case, we removed 26.5 l of fat in several steps. This magnitude underlines the fact that the indication for this operation was by no means cosmetic [6, 10]. For nearly a decade, preoperative preparation has been done not only by a surgeon but also by an anesthesiologist. The surgery itself always takes place in a team with the anesthesiologist: tumescence local anesthesia and accompanying analgosedation. After removing such huge amounts of tissue, it is mandatory to monitor patients for 24 h in inpatient conditions. The cutting-suture time takes up to 320 min as a rule.

The question of the duration of symptoms until correct diagnosis yielded a spread of 5 months to 15 years (Fig. 79.7). Postoperative symptoms were indicated on average between 2 and 6 months (Fig. 79.8). On the question of whether complaints still existed at the time of the study, 83 % of the patients responded that they no longer had symptoms (Fig. 79.9).

Fig. 79.7
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Cologne Lipoedema Study 2012: suffering from symptoms before diagnosis

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Fig. 79.8
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Cologne Lipoedema Study 2012: length of postoperative discomfort

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Fig. 79.9
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Cologne Lipoedema Study 2012: postoperative discomfort

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The authors were particularly interested in the continuation of complex decongestive therapy. Findings showed that 3 % of patients, i.e., nine of the operated patients, still underwent decongestive therapy from time to time. Ninety-seven percent of patients reported requiring no further complex decongestive therapy.

While satisfaction with the cosmetic result was 89 %, considering stage III lipohyperplasia dolorosa, 95 % of patients stated that their quality of life had improved considerably; the recommendation rate for the operation was also 95 % (Figs. 79.2 and 79.10).

Fig. 79.10
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Cologne Lipoedema Study 2012: satisfaction cosmetic result

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In addition to the exact operation procedure, the lymphological liposculpture method also includes a crucial postoperative setting. This consists of a 1-week intensive outpatient follow-up treatment at the patient’s residence: 1 h of lymphatic drainage per day and wearing the appropriate compression garments 24 h a day. In the second, third, and fourth week of the follow-up treatment, the frequency is halved. The first week is accompanied by antibiosis as well as a heparinization in order to avoid side effects. Symptoms occurred in less than 0.2 % of patients: panniculitis, hematoma, muscle aches, wound infections, erysipelas, thrombosis, and pulmonary embolism. Despite the large amount of tissue to be operated on, the authors believe that the operation must be carried out as outpatient surgery, but carefully planned, due to the low side effect rate (Fig. 79.11).

Fig. 79.11
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Cologne Lipoedema Study 2012: improvement of life quality

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Since 97 % of the patients did not undergo further complex decongestive therapy, the authors of the 2012 Cologne Lipoedema Study allowed themselves to continue talking of “healing” the disease lipohyperplasia dolorosa by means of lymphological liposculpture (Fig. 79.12).

Fig. 79.12
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Cologne Lipoedema Study 2012: surgery recommendation

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It is reported, encouragingly, that colleagues Schmeller (in Lübeck) and Rapprich (in Darmstadt) have had similarly good results in the surgical treatment of lipoedema. Professor Schmeller [15] reported on 114 patients and Rapprich [14] on 27 cases. Regardless of the surgical procedure’s excellent results on lipoedema, it should be noted that it is not advisable to try to cure the lymphatic disorder “lipohyperplasia dolorosa” by means of lymph surgery alone. Furthermore, up to 80 % of the patients at the CG Lympha center in Cologne are treated successfully using primarily nonoperative procedures, even though the curative treatment is increasingly becoming more accepted (Fig. 79.13).

Fig. 79.13
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Cologne Lipoedema Study 2012: findings at a glance

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8 Conclusions

Surgical treatment on patients with a lymphatic illness is a special one. It should certainly not be confused with plastic surgery procedures. Both preoperative and above all postoperative patient care require qualified, conventional lymphological procedures. Patients with lipohyperplasia dolorosa should be examined carefully on the legs and arms; they should be offered both conventional and surgical therapy.

Healing rates following surgical procedures are established at 97 % in the largest sample size study with 592 patients and a follow-up term of 15 years.