Primary lymphedema French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins).

Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 ( https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf ). Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer's sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major, deteriorating the patient's quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.

Impact of breast cancer-related lymphedema on working women.

BACKGROUND: The professional impact of upper limb lymphedema, which affects 15-20% of women after breast cancer treatment, has been poorly evaluated. OBJECTIVE: To analyze lymphedema characteristics and global lymphedema- and/or sleeve-attributed impact (mildly inconvenient to severely debilitating) on professional activities, workplace relationships, and workstation ergonomics. METHODS: Patients received a standardized, anonymous, self-administered questionnaire at consultation/hospitalization for treatment in a specialized lymphedema management center. RESULTS: All 134 consecutive women (March/2015-March/2017; median age 54), with 53-month median lymphedema duration and 34% median excess volume, were included; 35% considered global impact (arm-use impairment) high. For high vs. low global impact during occupational activities, univariate analyses identified global impairment as being associated with the low (23.8%), intermediate (60%), or high (63.2%) (p < 0.01) arm-use level, while multivariate analyses retained intermediate (OR 6.9 [95% CI 1.1-118.1], p < 0.01) and high (OR 4.5 [95% CI 1.5-37.3], p < 0.05) vs. low arm-use level. Lymphedema affected the careers of 70 (52.2%) patients, mostly those with severely impaired arm movement (53.8% vs. 10.2, p < 0.001), without modifying their relationships with colleagues and superiors for 84 (62.7%). Highly impaired women reported changed relationships with colleagues (45% vs. 20%, p < 0.01) and superiors (43.6% vs. 16.9%, p < 0.01). Only 10 women's (7.5%) job changes reflected lymphedema or its treatment. Workplace adaptations (53% ergonomic) were made for 36 (26.9%) patients, mostly those with greater arm-movement impairment (43.6% vs. 25.3%, p < 0.05), who were highly satisfied (86%). CONCLUSION: Upper limb lymphedema can significantly impact work, sometimes upending careers. The rare workstation adaptations were beneficial. Occupational physicians should assess lymphedema-attributed difficulties to improve working conditions.

Specialized consultations in a hospital-based referral center for patients suspected of having limb lymphedema: Impact on diagnosis.

Lymphedema, a chronic debilitating disease, is not always easily diagnosed. A total of 254 new patients ((217 women, 37 men), median (Q1-Q3) age 61 (46-72) years) were referred for suspected limb lymphedema to an exclusively lymphedema-dedicated department for a first consultation (January - March 2015) were included; among 118 with upper limb involvement, 100 (84.7%) were diagnosed with post-breast cancer therapy and four with primary lymphedemas; among 136 with lower limb involvement, 31 (22.8%) were diagnosed with primary lymphedemas and 35 (25.7%) with post-cancer lymphedemas. The main alternative diagnoses were: 32 (45.7%) lipedemas/lipo-lymphedemas and 21 (30%) chronic venous insufficiencies. Age at symptom onset, body mass index, referral origins and first-symptom-to-specialized-consultation intervals differed between primary, post-cancer lymphedema and alternative diagnosis patients. Among the entire cohort, 57 (22.4%) had cellulitis. For all 135 (53.1%) upper or lower limb post-cancer lymphedemas and the 119 (46.9%) others, the median (Q1-Q3) first-symptom-to-specialized-consultation intervals were 1.4 (0.7-3.8) and 4 (1.1-18.8) years, respectively ( p < 0.0001). Specialized consultations confirmed primary and post-cancer lymphedema diagnoses and identified others, especially for patients with suspected lower limb lymphedema.

Impact of carpal tunnel syndrome surgery on women with breast cancer-related lymphedema.

Carpal tunnel syndrome may occur in women with ipsilateral lymphedema after breast cancer treatment. Surgery on the lymphedematous arm is classically feared. Thirty-two consecutive women (mean age at cancer treatment 49 years, interquartile range (Q1;Q3) 43;56) with upper limb lymphedema after breast cancer treatment, followed in a single lymphology unit, and symptomatic carpal tunnel syndrome (electromyographically confirmed) requiring surgery were included. Lymphedema volume was calculated using the truncated cone formula, recorded before and after carpal tunnel syndrome surgery, and at each follow-up visit. Median time to lymphedema onset after cancer treatment was 19 (interquartile range (Q1;Q3) 5;73) months. Median lymphedema volume was 497 (Q1;Q3 355;793) mL before (median 4 months) and 582 (Q1;Q3 388;930) mL after carpal tunnel syndrome surgery (median 5 months) (P = 0.004). At the last follow-up post-carpal tunnel syndrome surgery (median 33 months), lymphedema volume was 447 (Q1;Q3 260;733) mL (non-significant, compared to pre-surgery volume). Regular lymphedema treatment included elastic sleeve (n = 31), low-stretch bandage (n = 20), and/or manual lymph drainage (n = 20), with no change before and after carpal tunnel syndrome surgery. All carpal tunnel syndrome clinical manifestations disappeared after surgery and none of the patients experienced local complications. Carpal tunnel syndrome may be treated surgically in women with ipsilateral upper limb lymphedema after breast cancer treatment. Although lymphedema volume increased transiently, it remained stable over long-term follow-up, with no local complications.

Large-volume sirolimus-induced upper limb lymphedema after renal transplantation ipsilateral to the arteriovenous fistula.

OBJECTIVE: To analyze upper-limb lymphedema characteristics of renal transplant recipients taking sirolimus, an mTOR inhibitor. METHOD: Cross-sectional study of sirolimus-treated upper-limb lymphedema patients (01/2009-12/2013). RESULTS: Three men and two women, whose mean age at transplantation was 60 (range: 49-76) years, were included. Sirolimus (1-2.5 mg/day) had been taken for 27.5 ± 21 (range: 7-58) months before left (n=4) or right (n=1), whole limb (n=4), or hand and forearm (n=1) upper-limb lymphedema onset, always ipsilateral to the functional arteriovenous fistula. Ultrasonography or fistulography excluded venous thrombosis in all patients. At the time lymphedema appeared, all five arteriovenous fistulas were functional. Mean upper-limb lymphedema volume, calculated with the truncated-cone formula, was 774 ± 162 [range: 594-1035] mL, (i.e. 44%± 11% [range: 36%-64%] excess volume compared to the contralateral limb. One patient also had ipsilateral breast lymphedema. The three lymphoscintigraphies obtained showed total absence of ipsilateral axillary-region tracer uptake. Sirolimus was maintained in all cases. Upper-limb lymphedema treatment included low-stretch bandages (n=4) and elastic sleeve (20-36 mm Hg) (n=5) without fistula complications. Two patients had their fistulas closed without any impact on lymphedema volume. CONCLUSION: Sirolimus may be implicated in large-volume upper-limb lymphedema in kidney-transplant recipients, ipsilateral to the arteriovenous fistula, and requires compression-based therapy.

Intensive complete decongestive physiotherapy for cancer-related upper-limb lymphedema: 11 days achieved greater volume reduction than 4.

BACKGROUND: Upper-limb lymphedema (ULL) occurs in 15-20% of women after breast-cancer treatment. Its intensive therapy relies on complete (complex) decongestive physiotherapy (CDP), whose duration is not well-established. OBJECTIVE: Determine optimal intensive-phase CDP duration for lymphedema-volume reduction and factors predicting its success, with the hope of halving it from 11 to 4 days. METHODS: All patients with ULL (08/2011-06/2012) after breast-cancer treatment referred to our Department of Lymphology in a rehabilitation facility for 11 days of CDP were eligible. Lymphedema volume was calculated using the truncated-cone formula. Volume reduction considered clinically relevant after 4 days was defined as ≥ 75% of the total reduction obtained after 11 days. RESULTS: We included 129 women (median age: 64 (range: 42-88) years). Mean (sd) lymphedema volume was 907 (558) ml at CDP onset, decreased to 712 (428) ml after 4 days (vs. onset, P<.0001) and 606 (341) ml after 11 days (vs. 4, P<.0001), corresponding to 33% total lymphedema-volume reduction. For all patients, 4 days of CDP achieved 63% (sd 40%) of that total reduction, with ≥ 75% for 50 (39%) patients. Surgery-to-lymphedema-onset interval >2 years was the only factor significantly associated with 4 days achieving ≥ 75% of the total lymphedema-volume reduction. CONCLUSION: Intensive phase CDP for 11 days obtained significantly more volume reduction of breast cancer-related ULL than 4.

Positive impact of delayed breast reconstruction on breast-cancer treatment-related arm lymphoedema.

BACKGROUND: The study aims to determine the impact of delayed breast reconstruction on women with breast-cancer treatment-related lymphoedema after mastectomy. METHODS: Twenty women with breast-cancer treatment-related arm lymphoedema prospectively requesting breast reconstruction were included between January 2002 and June 2009. Lymphoedema volume was calculated for each 5-cm segment using the formula for a truncated cone. RESULTS: Three patients underwent transverse rectus abdominis myocutaneous flap reconstruction, five received pedicled latissimus dorsi myocutaneous flaps associated with a breast implant and 12 a breast implant (silicone) alone. Median lymphoedema duration at the time of breast reconstruction was 21 (interquartile range (IQR): 17-34) months. Breast reconstruction was done a median of 30 (IQR: 23-56) months after mastectomy. The median lymphoedema volume at medians of 5 (244 ml, IQR: 159-435) and 22 months (235 ml, IQR: 146-361) of follow-up after reconstruction compared to that measured 6 months before breast reconstruction of 378 ml (IQR: 261-459) were significantly lower (p<0.02 for both). Specific lymphoedema treatment, unchanged during the study, included manual lymph drainage for all women, elastic sleeves for 19 and low-stretch bandages for 14. CONCLUSION: Delayed breast reconstruction is feasible for women who have developed lymphoedema after mastectomy for breast cancer. Further studies are needed to compare the different breast-reconstruction techniques and to determine their ideal timing (immediate or delayed).

Factors influencing breast cancer-related lymphedema volume after intensive decongestive physiotherapy.

BACKGROUND: Lymphedema treatment is based on an intensive decongestive physiotherapy phase of volume reduction followed by a long-term maintenance phase. Factors influencing the morbid lymphedema volume increase during maintenance were analyzed. MATERIALS AND METHODS: Among 867 consecutive women recruited and followed in a single lymphology unit, 682 were analyzed. The other 185 were not analyzed because of an initial lymphedema volume <250 ml, <20% lymphedema volume decrease during the intensive phase, or they were lost to follow-up. Lymphedema volume was recorded prior to and at the end of intensive phase, and at each follow-up visit. During follow-up, treatment failure was defined as a lymphedema volume increase of ≥50% of the total reduction obtained during the intensive phase. RESULTS: Median lymphedema volume was 936 ml before and 335 ml after intensive decongestive physiotherapy (P < 0.0001). Median follow-up was 28 months. During the maintenance phase, the risk of treatment failure at 1, 2, and 4 years was estimated to be 38.1%, 53.1%, and 64.8%, respectively. Wearing an elastic sleeve during the day and an overnight multilayer low-stretch bandage (median, four nights per week; interquartile range, 2-6) significantly decreased the risk of treatment failure [hazard ratio, 0.53, (0.34-0.82), P = 0.004], whereas manual lymph drainage adjunction to those therapeutic components did not. The risk of treatment failure was also associated with weight and body mass index at inclusion. CONCLUSION: Risk of maintenance-therapy failure after intensive decongestive physiotherapy was associated with patients characteristics (younger age, higher weight, and body mass index), while elastic sleeve and bandage were associated with better maintenance results. Paradoxical effect of manual lymph drainage is likely to be related to indication bias.

Surgical resection of vulva lymphoedema circumscriptum.

BACKGROUND: Lymphangioma circumscriptum, a rare, benign disease that can be either congenital or acquired, involves the deep dermis and subcutaneous lymphatics. OBJECTIVE: This study aims to analyse the efficacy of surgical resection of vulva lymphangioma circumscriptum (VLC). MATERIALS AND METHODS: Between January 2000 and December 2008, eight consecutive women referred to our centre and treated surgically for VLC were included in the study. VLC was responsible for recurrent lymph oozing in seven cases. All women were treated by the same plastic surgeon specialising in lymphatic diseases. RESULTS: The first surgery was performed after a median interval of 5.4 years since VLC onset. The first cutaneous resection included the labia majora of all women and labia minora of five and clitoral hood of four. Five women experienced rapidly recurrent vesicles associated with lymph oozing and underwent resection again (once: two women, twice: three women). The second resection was performed 4-6 months after the first, whereas the third took place 1-6 years after the second. Five women had moderate and transitory post-surgical lymph oozing. After a median follow-up of 53 months after the last surgery, seven of the eight women were free from symptom. CONCLUSION: Surgical resection is an effective and well-tolerated therapy for VLC in most women. Lesion recurrence is frequent but resection can be repeated several times with no adverse effects.

Subjective assessment of pregnancy impact on primary lower limb lymphedema.

OBJECTIVE: To analyze subjective influence of pregnancy on lower limb lymphedema. METHOD: Cross-sectional study on 49 affected women was conducted in a single lymphology department between January 2002 and December 2006. All women were asked whether their lymphedema had worsened during pregnancy. RESULTS: Mean age at lymphedema onset was 17 years, with no familial history of lymphedema. Lymphedema was unilateral for 30 women and bilateral for 19. Median age at the first delivery was 28 years. Eighteen women had only 1 pregnancy, 23 women had only 2, and 8 women had 3. For the first pregnancy, birth weight was 3.4 kg. Subjective lymphedema worsening was reported by 5 women after the first pregnancy compared to 44 women without worsening (P = .006) and after 10 (11%) of the 88 pregnancies (1 twin birth) involving 9 women. During the median 18 years since the first pregnancy, only a 14-year-old boy has developed bilateral lymphedema. CONCLUSION: Pregnancy did not significantly exacerbate primary lower limb lymphedema.

Factors associated with increased breast cancer-related lymphedema volume.

BACKGROUND: Upper limb lymphedema occurs in approximately 15-20% of women after breast cancer treatment. We analysed the factors associated with lymphedema volume. METHOD: Cross-sectional study of 807 patients with secondary arm lymphedema was performed in a single lymphology unit. Data collected included patient characteristics, characteristics of breast cancer treatment, past history of cellulitis, Body Mass Index, delay from cancer to onset of lymphedema and duration of lymphedema. Lymphedema volume was calculated for each 5-cm segment by utilizing the formula for a truncated cone. Univariate and multivariate regression models were fitted to study the factors associated with increased lymphedema volume. RESULTS: In univariate analysis, factors associated with lymphedema volume were duration of lymphedema, Body Mass Index, mastectomy, and past history of cellulitis. Treatment with anti-estrogen drugs was negatively associated with lymphedema volume (p = 0.02). In multivariate analysis, factors associated with lymphedema volume were duration of lymphedema (p < 0.001), Body Mass Index (p < 0.001), delay from cancer to onset of lymphedema (p = 0.002), mastectomy (p = 0.02) and past history of cellulitis (p = 0.011). CONCLUSION: Early diagnosis and management of lymphedema, weight control and advices to avoid cellulitis are the main controllable parameters in women to prevent severity of breast cancer-related lymphedema.

[Management of lymphedema of the upper extremity after treatment of breast cancer]. / Prise en charge du lymphoedème du membre supérieur après traitement du cancer du sein.

Management of breast cancer-related lymphedema is based on complex decongestive physiotherapy including two phases. The first intensive phase reduces lymphedema volume with daily low stretch bandages associated with manual lymph drainage. Treatment includes exercises and skin care. The second phase of treatment consists of stabilization of lymphedema volume with regular overnight self-bandaging, daily elastic compression garment and manual lymph drainage if needed. Lymphedema volume may be stabilized after the intensive phase if the patient is compliant to long term treatment. Practical advices such as weight control and skin care to avoid cellulitis should be given. Regular and prolonged medical follow-up is required for this chronic disease to maintain motivation of the patient.

Long-term management of breast cancer-related lymphedema after intensive decongestive physiotherapy.

BACKGROUND: Treatment of lymphedema is based on intensive decongestive physiotherapy followed by a long-term maintenance treatment. We analyzed the factors influencing lymphedema volume during maintenance treatment. METHOD: Prospective cohort of 537 patients with secondary arm lymphedema were recruited in a single lymphology unit and followed for 12 months. Lymphedema volume was recorded prior to and at the end of intensive treatment, and at month 6 and month 12 follow-up visits. Multivariate models were fitted to analyze the respective role of the three components of complete decongestive therapy, i.e. manual lymph drainage, low stretch bandage, and elastic sleeve, on lymphedema volume during the 1-year maintenance phase therapy. RESULTS: Mean volume of lymphedema was 1,054 +/- 633 ml prior and 647 +/- 351 ml after intensive decongestive physiotherapy. During the 1-year maintenance phase therapy, the mean lymphedema volume slightly increased (84 ml-95% confidence interval [CI]: 56-113). Fifty-two percent of patients had their lymphedema volume increased above 10% from their value at the end of the intensive decongestive physiotherapy treatment phase. Non-compliance to low stretch bandage and elastic sleeve were risk factors for an increased lymphedema after 1-year of maintenance treatment (RR: 1.55 [95% CI: 1.3-1.76]; P < 0.0001 and RR: 1.61 (95% CI: 1.25-1.82); P = 0.002, respectively). Non-compliance to MLD was not a risk factor (RR: 0.99 [95% CI: 0.77-1.2]; P = 0.91). CONCLUSION: During maintenance phase after intensive decongestive physiotherapy, compliance to the use of elastic sleeve and low stretch bandage should be required to stabilize lymphedema volume.

[Risk factors for developing upper limb lymphedema after breast cancer treatment]. / Facteurs de risque de développement d'un lymphoedème du membre supérieur après traitement du cancer du sein.

Secondary lymphedema occurs in 14 to 28% after breast cancer treatment. Many studies have defined risk factors of lymphedema. Number of axillary nodes removed and radiotherapy especially on axillary nodes are the main risk factors. Others factors have been described such as type of surgery (mastectomy/tumorectomy), overweight at time of cancer, weight gain after surgery, skin puncture, reduction of physical activity. Risk of lymphedema and its complications (cellulitis, psychological and aesthetic discomfort) may be reduce by improvement in radiotherapy methods, sentinel lymph node biopsy, weight control, maintain of level physical activity after treatment and avoidance of skin puncture on ipsilateral arm.