Abstract
Background:
Lymphedema is a chronic and progressive disease whose diagnosis involves determination of clinical and demographic characteristics. The aim of this retrospective study was to analyze the clinical characteristics of patients with lymphedema and their various diagnoses. We studied patients who were referred for physiotherapy services at any point during the years 2009 through 2019.
Methods:
Retrospective data were collected from the files of 430 lymphedema patients. The type, cause, localization, stage, and severity of lymphedema and physiotherapy needs were analyzed and reported.
Results:
Primary and secondary lymphedema were observed in 18 (4.2%) and 412 (95.8%) patients, respectively. The patients' mean body mass index score was 30.66 kg/m2. The data indicated that the most common cause of secondary lymphedema was breast cancer and its treatments (n = 196, 47.6%). Other causes were chronic venous insufficiency (CVI) (n = 140, 34%), lipolymphedema (n = 11, 2.7%), and other types of cancers (n = 65, 15.7%). According to the affected body regions, 416 patients had unilateral/bilateral upper and lower extremity lymphedema and 14 had head and neck lymphedema. The patients were followed with a home-based physiotherapy program (n = 353, 82.1%) or they underwent treatments through an outpatient program (n = 77, 17.9%).
Conclusions:
Most patients admitted to the clinic had a diagnosis of breast cancer and CVI. The severity and stages of lymphedema were variable. The data indicated that most patients were followed through a home-based physiotherapy program. These results may set a frame for understanding the treatment and care needs of patients with lymphedema.
Introduction
Lymphedema is classified as a disease in the International Classification of Diseases report by The World Health Organization 1 and it is known to be a chronic and potentially progressive condition. 2 In cases of lymphedema, swelling accompanies an obstruction, impairment, or other deficit in the lymphatics, resulting in the excessive accumulation of protein-rich fluid in interstitial spaces.3,4 There are two types of lymphedema: primary and secondary. With primary lymphedema, there is a developmental failure of the lymphatic system due to a congenital disorder 5 related to familial or genetic factors.6,7 Primary lymphedema is classified according to age: Congenital lymphedema, known as Milroy's disease (<1 year), is the first type; lymphedema praecox (ages 1–35 years) is the second type; and lymphedema tarda (>35 years) 8 is the third. Secondary lymphedema can occur due to damage of the lymphatic system 5 as the result of cancer and its treatment,9–12 as well as chronic venous insufficiency (CVI), obesity, infection, trauma,6,7 and other causes.
There are various methods for evaluating lymphedema. Advanced imaging methods such as magnetic resonance imaging, computerized tomography, and Doppler ultrasound flows can be used to assess the swelling of soft tissue. In addition, tonometry can be used to measure fibrosis 13 ; a perometer, water displacement method, and circumferential measurements can be used to assess volume of affected extremity/region. 14 Circumferential measurement is a practical and useful method for detecting changes in limb volume15,16 and for comparing the volume of limbs and the localization of the disease. 17
There is no specific medical treatment for lymphedema; however, it can be properly managed with physiotherapy.18,19 The gold standard treatment involves complex decongestive physiotherapy (CDP), which consists of two phases. Phase I includes skin care, manual lymph drainage (MLD), the use of compression bandages, and exercise. Phase II involves skin care, self-drainage, the use of a compression garment, and exercise.20–23
In the literature, both protective and remediating effects of physiotherapy have been reported in patients with lymphedema. It has been suggested that the risk of lymphedema can be reduced with early physiotherapy interventions such as MLD, therapeutic exercise, and patient education.19,24 The authors of various studies have demonstrated that CDP reduces extremity volume,21,25,26 decreases pain, 27 increases joint mobility, 28 and improves the quality of life.25,29
This study was conducted to address the gaps in the literature regarding the lack of information on the therapeutic needs of patients with lymphedema and the feasibility of treatments, as well as the limited number of descriptive studies to demonstrate the clinical and demographic characteristics of patients with lymphedema. More specifically, this study was designed with the aim of analyzing the clinical characteristics of patients with lymphedema and various diagnoses who were referred for physiotherapy services, including CDP and home-based physiotherapy program services, from 2009 to 2019.
Materials and Methods
Retrospective physiotherapy records were used to identify patients with lymphedema over a 10-year period (2009–2019) at a physiotherapy and rehabilitation clinic of a university. A total of 430 patients with lymphedemas in different bodily regions such as the upper limb(s), lower limb(s), head, and neck areas who were referred from several medical departments were included in this study. This study was approved by (Gazi) University ethical committee (Number: 91610558-604.01.02, 23.07.2020-E.78158).
Evaluation methods
Routine clinical care data were collected. The physiotherapy record review included the following variables: age, gender, weight, height, diagnosis (cause of lymphedema), lymphedema type (primary or secondary), and affected bodily region (unilateral/bilateral upper and/or lower extremity, head and neck area). In addition, each person's body mass index (BMI) was calculated as their weight (in kilograms) divided by the square of their height (in meters). 30
Cases of lymphedema in the upper and lower extremities were divided into four stages, including latency (stage 0), reversible (stage 1), spontaneously reversible (stage 2), and elephantiasis (stage 3) according to the Foldi Lymphedema Rating Scale. 3 Head and neck lymphedema (HNL) staging was performed with the M.D. Anderson Cancer Center rating scale. 31 This scale was adapted from the Foldi Lymphedema Rating Scale with levels from 0 to 3 (0: No visible edema but patient reports heaviness; 1a: Soft visible edema, no pitting, reversible; 1b: Soft pitting edema, reversible; 2: Firm pitting edema, not reversible, no tissue changes; and 3: Irreversible, tissue changes). 3
To determine the severity of extremity lymphedema, circumference measurement records were included only for cases of unilateral upper and lower limb lymphedema, except when bilateral involvement and HNL were present. The evaluation was performed with measuring the reference points (from the ulnar styloid process to the axillary fold for the upper limbs and from the lateral malleolus to the inguinal fold for the lower limbs with 4 cm intervals). 32
Physiotherapy intervention
Before treatment, each patient individually received education that included information on the causes of lymphedema as well as its prevention and management. In addition, written information was provided in the book titled “Physiotherapy Recommendations for Individuals Living with Cancer and Lymphedema.” This consisted of 3 booklets, which included (1) “Exercise for Individuals Living with Cancer,” (2) “Lymphedema,” and (3) “Understanding Lymphedema.” 33
The appropriate treatment methods were determined and applied to the patients according to the lymphedema severity, each patient's compliance, and their other medical conditions. They were followed by professionals from two main programs (an outpatient physiotherapy program and a home-based physiotherapy program).
The outpatient physiotherapy program was started with CDP-phase I. 20 The MLD lasted 30–45 minutes and varied according to the region. After CDP-phase I, the patient began CDP-phase II. 34
The home-based physiotherapy program involved education, self-MLD, skin care, and exercises such as posture, breathing, active stretching, pumping, and aerobic exercise, according to the needs of each patient. The patients were counseled to continue following the medical advice they received and to keep their appointments so that the medical team could follow the progress of their lymphedema.
Statistical analysis
All statistical analyses were performed with SPSS version 22.0 for Windows (SPSS, Inc., Chicago, IL). Descriptive statistics (including mean, range, frequency, and percentage) were used to analyze the demographic characteristics, lymphedema type and etiology, lymphedema stage, lymphedema severity of the study sample, and recommended physiotherapy program.
Results
A total of 385 (89.5%) female and 45 (10.5%) male patients were included in the study. The mean age was 52.73 ± 13.25 years, and the mean BMI was 30.66 ± 7.37 kg/m2.
Primary lymphedema was observed in 18 (4.2%) patients, 412 (95.8%) had secondary lymphedema, and 60.5% of all patients had cancer-related lymphedema. The most common etiology for secondary lymphedema was breast cancer and its related treatments (n = 196, 47.6%). Lymphedema was detected in 140 (34%) patients with CVI, 11 (2.7%) with lipolymphedema, and 65 (15.7%) with other types of cancers.
Urogenital cancers accounted for the second-most common cancer etiology in this population and it included 18 (48.6%) cases of ovarian cancer, 11 (29.7%) cases of uterine cancer, 4 (10.8%) cases of endometrial cancer, 3 (8.1%) cases of cervical cancer, and 1 (2.7%) case of bladder cancer.
There were various types of head and neck cancers (HNC), such as 6 (42.9%) of the larynx, 2 (14.3%) of the tongue, 1 (7.1%) of the nasopharynx, 1 (7.1%) of the thyroid, 1 (7.1%) on the floor of the mouth, 1 (7.1%) in the parotid gland, 1 (7.1%) tonsillar, and 1 (7.1%) in the nose.
Traumatic, non-filarial infection and obesity-related lymphedema were included as the category of the others (2.9%). Table 1 shows the type, etiology, and localization of lymphedema.
Lymphedema Type and Etiology
Bold characters indicate total numbers.
CVI, chronic venous insufficiency; HNC, head and neck cancers.
A total of 416 patients with unilateral/bilateral upper and lower extremity lymphedema were staged according to the number of affected extremities (n = 601), as shown in Table 2. Two thirty-one patients had unilateral lymphedema; 199 patients had bilateral lymphedema. As a result of the analysis of HNL staging, 3 patients had stage 1a and 11 patients had stage 1b lymphedema.
Lymphedema Stages of Upper and Lower Limbs
Bold characters indicate total numbers.
The severity of lymphedema is demonstrated in Table 3 for unilateral upper and lower extremity lymphedema.
Lymphedema Severity of Upper and Lower Limbs for Unilateral Lymphedema
The patients were referred to a home-based physiotherapy program (n = 353, 82.1%) or an outpatient physiotherapy program (n = 77, 17.9%).
Discussion
This study includes data from 10 years of experience in the physiotherapy clinic that were used to set a frame for the diagnosis of lymphedema and services for its management. Analyzing data from a large cohort of patients regarding the type, cause, localization, stage, and severity of each patient's lymphedema and their affected body parts may provide the basis for determining patients' needs. According to the data collected from the years 2009 to 2019, most of the patients in this study had a diagnosis of breast cancer and CVI. The severity and stages of lymphedema were variable. It was found that most of the patients received follow-up services from a home-based physiotherapy program.
To our knowledge, there have been no studies conducted with the purpose of analyzing physiotherapy services for lymphedema patients, and there are only a limited number of studies in the literature whose authors have examined the characteristics of lymphedema patients. Onoda et al. reported on the characteristics of 33 patients with idiopathic lymphedema. In this study, the effectiveness of conservative and surgical treatments was compared, rather than patient characteristics. 35 Drivdal et al. reported on the characteristics of 17 patients with hereditary lymphedema. 36 Liao et al. reported on the characteristics of 29 patients with malignant lymphedema. In the study, a group of palliative care patients who had lymph node involvement as the result of cancer and/or metastasis were included. 37 Due to having been conducted in an inpatient clinic, it only included specific cancer patients, so their characteristics were different from those in this study. Wang and Keast reported on the characteristics of 326 lymphedema patients who were admitted to a wound care clinic. 38 Almost all the patients in that study were reported to have had lower limb lymphedema, since the patient group was mostly unrelated to cancer. In our study, patients had different diagnoses, including various types of cancer and CVI, as well as primary lymphedema and lipolymphedema. Including a large number of patients with greater levels of diversity and reporting on the characteristics of all admitted patients are among the strengths of this study.
Most of the patients admitted to our clinic were female (89.5%). This may be due to the fact that the majority of all patients also had breast cancer (47.6%) and CVI (34.0%) diagnoses.39,40
The number of survivors from HNC is increasing as a consequence of better diagnosis and treatment options. 41 Laryngeal and hypopharyngeal cancer, nasal cavity and paranasal sinus cancer, nasopharyngeal, oral and oropharyngeal cancer, and salivary gland cancer are the five main types of HNC. 42 Although they were all main types of HNC diagnosis, most of the diagnoses in this study were of laryngeal cancer.
The mean BMI of the patients in our study population was 30.66 kg/m2 (first-degree obesity). It is clearly known that obesity is an important risk factor for cancers such as breast cancer, 43 endometrial cancer, 44 and also CVI 45 ; all of these are directly associated with secondary lymphedema. In addition, obesity may increase the severity of lymphedema as the result of peri-lymphatic inflammation and/or lymphatic dysfunction. 46
According to the classifications used in our study, 4.2% of the patients had primary lymphedema and 95.8% had secondary lymphedema. Most of the patients with primary lymphedema had praecox lymphedema (72.2%) and were female (88.9%). Praecox lymphedema was the most common form and it was responsible for many of the cases (94%) in a reported series of primary lymphedema. Regarding gender, with women it is estimated that the rate of primary lymphedema due to estrogen is 1 out of 10. 47 Thus, the data obtained in current study were similar to those in the literature.
In this study, the most common cause of upper extremity secondary lymphedema was found to be breast cancer (47.6%). When considering that this finding was provided from patients in a developing country, it is consistent with a report that the most common cause of secondary lymphedema is cancer and cancer-associated treatments in developed countries. 18
In the literature, the incidence of lower extremity secondary lymphedema has been less documented compared with the incidence of cases of lymphedema in the upper extremities. 48 The patients with secondary lymphedema in their lower extremities who were referred to the clinic had diagnoses of CVI (34%), urogenital cancer (9%), and/or lipolymphedema (2.6%). The available data on lower extremity lymphedema are often associated with cancer. It is known that the incidence of lymphedema is 6%–29% in patients with malignant melanomas, 5%–49% in those with gynecological cancers, and 3%–8% among those with prostate cancer. 48 However, there is only a limited amount of data demonstrating the incidence of trauma, CVI, non-filarial infections, and obesity. 49 It is expected that patients with advanced chronic venous disease, 20%–30% will have lymphatic dysfunction. 50 Thus, it can be considered that CVI may be a common cause of secondary lymphedema. The result that CVI is the most common cause of lower extremity lymphedema in the current study may be explained with many factors. These results may be due to the differences in knowledge about lymphedema, the attitudes of physicians regarding treatment cooperation, or their ideas about consulting each patient's profile for physiotherapy.
The result of this study was surprising in that the majority of patients with upper extremity lymphedema were either at stage 1 or at stage 3, with mild or severe lymphedema. The number of patients with grade 2 and moderate lymphedema was very limited. An explanation for the high number of mild lymphedemas may be increased awareness among patients and physicians; regarding severe lymphedema, it may be related to increased distress due to lymphedema and the need for professional advice.
Regarding the staging of lymphedema of the lower extremities, most patients were at stage 2. However, the severity of lymphedema was generally mild and severe. Lymphedema is usually seen bilaterally in patients with lower extremity lymphedema. Since the severity of bilateral lymphedema cannot be evaluated, the distributions of stage and severity may be differentiated. Diagnoses of CVI, lipolymphedema, and lymphedema can be confused so this may prevent patients from accessing appropriate treatment for a long time. This is especially true in patients with CVI, since edema is initially addressed with medical therapies and because it is difficult to get physiotherapy in the early stage. Among patients with unilateral lymphedema, cancer-associated lymphedema and primary lymphedema may be classified as mild and severe, for reasons similar to those related to lymphedema in the upper extremity.
Similar to upper extremity lymphedema, HNL may develop as the result of HNC treatments. Secondary HNL is a comorbidity that is largely unknown, often overlooked, and, in many cases, not handled properly. 51 According to our results from the staging of HNL, all the patients with HNC had reversible lymphedema. An increase in physicians' awareness may lead to early intervention in this patient group.
It was reported that most of the patients in this study were followed with services from a home-based physiotherapy program (82.1%). To our knowledge, there has been no study of participation in outpatient or home-based physiotherapy programs in lymphedema treatment. Although the physical needs of the patient are important, many factors may be emphasized in deciding the treatments for patients with lymphedema. Although we did not document the costs of the treatment, difficulties related to the workplace (inability to obtain treatment permit, etc.), difficulties with transportation due to residence, and problems with adapting to treatment could be demonstrated as reasons for this situation. 52 Moreover, lymphedema is a chronic and progressive condition that requires self-management and home-based physiotherapy at every stage.
The limitations of this study are that the results were garnered from the data of a single physiotherapy clinic; any specific methods and devices; and no data were collected on lymphedema in other body parts such as the abdomen, breast, trunk, and genitals.53,54 The strength of this study is that it offers valuable results on various diagnoses of patients from different areas of the whole country.
In future studies, it may be better to analyze the data of a multicenter, patient-centered, and/or inpatient clinic.
Conclusions
In conclusion, female gender and obesity may have an influence on lymphedema. Breast cancer and CVI diagnosis are the most common causes of the lymphedema according to the results of current study. Most of the patients were followed with a self-management or home-based physiotherapy program. The severity and stages of lymphedema were variable according to the body parts affected. The results from this study may establish a framework for understanding the needs of people with lymphedema, as well as their treatment and care.
Footnotes
Author Disclosure Statement
No competing financial interests exist.
Funding Information
No funding was received for this study.
