[Primary lymphedema in childhood]. / Les lymphœdèmes primaires de l'enfant.

Lymphedema results from impaired lymphatic transport with increased limb volume and is divided into primary and secondary forms. In children, primary lymphedema is the most frequent, with a sporadic, rarely familial form or associated with complex malformative or genetic disorders. Diagnosis of lymphedema is mainly clinical and lymphoscintigraphy is useful to assess the lymphatic function of both limbs precisely. The main differential diagnosis is overgrowth syndrome. Erysipelas (cellulitis) is the main complication, but psychological or functional discomfort may occur throughout the course of lymphedema. Lymphedema management is based on multilayer low-stretch bandage, skin care, and eventually manual lymph drainage. The objective of treatment is to reduce lymphedema volume and then stabilize it. Multilayer low-stretch bandage and elastic compression are the cornerstone of treatment. Parent's motivation, including self-management, is required to ensure the child's compliance and improve quality of life.

Complications of autologous lymph-node transplantation for limb lymphoedema.

OBJECTIVE: This study aims to assess potential complications of autologous lymph-node transplantation (ALNT) to treat limb lymphoedema. DESIGN: Prospective, observational study. METHOD: All limb-lymphoedema patients, followed up in a single lymphology department, who decided to undergo ALNT (January 2004-June 2012) independently of our medical team, were included. RESULTS: Among the 26 patients (22 females, four males) included, 14 had secondary upper-limb lymphoedema after breast-cancer treatment and seven had secondary and five primary lower-limb lymphoedema. Median (interquartile range, IQR) ages at primary lower-limb lymphoedema and secondary lymphoedema onset were 18.5 (13-30) and 47.4 (35-58) years, respectively. Median body mass index (BMI) was 25.9 (22.9-29.3) kg m⁻². For all patients, median pre-surgery lymphoedema duration was 37 (24-90) months. Thirty-four ALNs were transplanted into the 26 patients, combined with liposuction in four lower-limb-lymphoedema patients. Ten (38%) patients developed 15 complications: six, chronic lymphoedema (four upper limb, two lower limb), defined as ≥2-cm difference versus the contralateral side, in the limb on the donor lymph-node-site territory, persisting for a median of 40 months post-ALNT; four, post-surgical lymphocoeles; one testicular hydrocoele requiring surgery; and four with persistent donor-site pain. Median (IQR) pre- and post-surgical lymphoedema volumes, calculated using the formula for a truncated cone, were, respectively, 1023 (633-1375) ml (median: 3 (1-6) months) and 1058 (666-1506) ml (median: 40 (14-72) months; P = 0.73). CONCLUSION: ALNT may engender severe, chronic complications, particularly persistent iatrogenic lymphoedema. Further investigations are required to evaluate and clearly determine its indications.

Primary upper-limb lymphoedema.

BACKGROUND: Lymphoedema is a general term used to designate pathological, regional accumulation of protein-rich fluid. It can be either primary or secondary, and mainly occurs after cancer treatment. OBJECTIVES: To analyse the clinical and lymphoscintigraphic characteristics of primary upper-limb lymphoedema (ULL). METHODS: All of the patients with ULL were recruited at a single Department of Lymphology between January 2007 and December 2011. RESULTS: In total, 60 patients (33 female, 27 male) were enrolled. For the 54 noncongenital lymphoedemas, the mean age at onset was 38·5 (range 3-82) years. Lymphoedema was unilateral in 51 patients (85%). It always affected the hand, and less often the forearm (55%) or upper arm (23%). Eleven patients (18%) developed cellulitis after onset of lymphoedema, and 21 patients (35%) had associated lower-limb lymphoedema (LLL). Forty-six patients (with 49 lymphoedematous limbs) underwent lymphoscintigraphy: axillary lymph node uptake was diminished in 18 (37%), absent in 24 (49%) and normal in seven limbs (14%). Among the 43 patients with unilateral lymphoedema and lymphoscintigraphy, 28 had epitrochlear node visualization, suggesting a rerouting through the deep lymphatic system, with 15 only on the lymphoedematous limb and 22 on the contralateral nonlymphoedematous limb. The median follow-up period was 103 months, and 57/60 patients (95%) considered their lymphoedema to be stable. CONCLUSIONS: Primary ULL appears later in life than LLL, without predominance in either sex. Infectious complications are rare and patients considered the lymphoedema volume stable throughout life.

[Inflammatory bowel disease and lower limb lymphedema: a fortuitous association?]. / Maladie inflammatoire chronique de l'intestin et lymphœdème primaire des membres inférieurs: une association fortuite ?

INTRODUCTION: Extra-intestinal manifestations of chronic inflammatory bowel disease (CIBD) are various. Cases of genital lymphedema has previously been reported in Crohn's disease. CASE REPORTS: We report two women aged 57 and 68 years who presented with a lower limb lymphedema 8 and 20 years after a diagnosis of CIBD (Crohn's disease and ulcerative colitis), respectively. At the time of diagnosis of lymphedema, CIBD was asymptomatic. CONCLUSION: Pathophysiological mechanisms of this rare manifestation are unclear and lymphedema outcome is unrelated to CIBD activity.

[Adverse effects of compression in treatment of limb lymphedema]. / Effets indésirables de la compression/contention dans le traitement des lymphoedèmes des membres.

INTRODUCTION: Limb lymphedema, whether primary or secondary, is a chronic disease. Compression is the cornerstone of therapy and includes multilayer low-stretch bandages and elastic garments. Compression is usually well-tolerated. The aim of our study was to identify all the different types of adverse effects of compression. MATERIALS AND METHODS: Since January 2005, we have recorded all adverse events occurring in outpatients and inpatients consulting in a single lymphology department, spontaneously reported by patient during consultations or physical examinations, and noted the type of compression material used. RESULTS: Adverse effects were secondary to poor choice of therapeutic material, excessive pressure or contact dermatitis. For the arms, an elastic garment stopping at the wrist can be responsible for lymphedema of the hand and fingers. Rubbing of sleeve seams may cause pain and even ulcers between the thumb and forefinger. Open-toed elastic stockings may exacerbate digital lymphedema, leading to the formation of oozing lymph vesicles. Hyperpressure may cause severe pain localized to the first and fifth toes, overlapping toes, interdigital corns and/or ingrown toenails. Silicone-banded soft-fit elastic garments may cause painful phlyctena, urticaria or eczematiform lesions. Elastic bandages may induce pain or purpuric lesions. CONCLUSION: Compression can be responsible for adverse effects, sometimes severe, requiring treatment change or withdrawal. Further studies are needed to precisely determine their frequency to improve prescriptions and currently available products.

[Puffy hand syndrome]. / Le syndrome des grosses mains.

Puffy hand syndrome is an unrecognized complication of intravenous drug abuse. This painless syndrome appears during or after a long period of drug addiction. It involves the hands and sometimes the forearms, and may cause functional, aesthetic and social disturbances when the hand volume is important. Physiopathological mechanisms of the puffy hand syndrome are unclear and include venous and lymphatic insufficiencies, infectious complications and direct toxicity of injected drugs and their adulterants. Low-stretch bandage and elastic garment, usually used in lymphedema treatment, are proposed to treat the puffy hand syndrome.

[Upper limb lymphedema revealing breast cancer]. / Lymphoedème du membre supérieur révélant un cancer du sein.

INTRODUCTION: Upper limb lymphedema occurs in 15 to 20% of patients after breast cancer treatment. Upper limb lymphedema without any history of neoplasia is an unusual situation. In this situation, breast cancer should be suspected. EXEGESIS: We reported two women, 53 and 67 years old, who developed upper limb lymphedema, 18 and 8 months before the diagnosis of breast cancer. In the two cases, clinical examination (breast and axillary palpation) was normal. In one case, mammography led to the diagnosis and in the other breast MRI was required to confirm the cancer. DISCUSSION: Upper limb lymphedema may be the presenting clinical feature of breast cancer. Breast cancer should be actively sought despite normal clinical and radiological findings. Breast MRI is required in this situation.

[Poor influence of surgery on upper limb lymphedema volume in patients after breast cancer treatment]. / Effet délétère d'un acte chirurgical sur le volume d'un lymphoedème du membre supérieur après cancer du sein.

OBJECTIVES: Secondary upper lymphedema occurs in 15 to 20% of patients after breast cancer treatment. Surgery may be required on lymphedematous limb. The aim of our study was to analyze the effects of surgery on lymphedema volume. METHODS: Nine women with upper limb lymphedema after breast cancer treatment were recruited. Mean age at time of breast cancer was 45.6 +/- 8 years. Breast cancer was localized at right side and was treated with mammectomy (n=4), radiotherapy (n=8), chemotherapy (n=4) and antiestrogen (n=5). Mean duration of lymphedema before upper limb surgery was 10 years. Six patients reported one or more previous cellulitis. Surgery was indicated for carpal tunnel syndrome (n=6), forearm (n=2) or humeral (n=1) fracture. Lymphedema volume, calculated by the difference of volume between the lymphedematous and the contralateral arm, was compared before and after surgery. RESULTS: Six patients had carpal tunnel release by transecting the transverse carpal ligament under local anesthesia with short total time tourniquet. Humeral and forearm fractures were treated with osteosynthesis. Before surgery, lymphedema volume of upper limb was 747 +/- 315 ml. Lymphedema management included low stretch bandages, elastic sleeve, manual lymph drainage. After 8 months follow up, lymphedema volume was significantly higher, 858 +/- 293 ml (p=0.012). Mean absolute variation of lymphedema volume was 111 ml (CI 95%: 32 to 109 ml), i.e. 15% of pre surgery lymphedema volume. CONCLUSION: Surgery of carpal tunnel syndrome or osteosynthesis for fractures may lead to increased lymphedema volume in patients previously treated for breast cancer despite compressive therapy.

[Puffy hand syndrome in drug addiction treated by low-stretch bandages]. / Syndrome des "grosses mains" des toxicomanes: intérêt des bandages peu élastiques.

BACKGROUND: Puffy hand syndrome is a complication of intravenous drug abuse, which has no current available treatment. Arm and forearm edema are voluminous and cause functional and aesthetic disturbances. We report two cases successfully treated by low-stretch bandages. OBSERVATIONS: A 40-year-old man and a 34-year-old woman, both intravenous drug users, with puffy hand syndrome were hospitalized for 11 days. Treatment included daily multilayer bandaging. Lymphedema volumes calculated by utilizing the formula for a truncated cone decreased by 16% on the left side and 12% on the right side for the first patient and 31 and 17% for the second. Hand circumference decreased 4.3 cm on the left side and 3.2 cm on the right side in case 1, and 2.5 cm and 1.9 cm respectively for case 2. The patients were taught self-bandaging techniques during their hospital stays. Elastic gloves were fitted at the end of treatment. Reduction of lymphedema volume remained stable after 18 months in one patient while for the second patient further treatment and hospitalization were required due to poor compliance. DISCUSSION: The pathogenesis of this edema is probably multifactorial: venous, lymphatic insufficiency and the direct toxicity of injected drugs. Lymphedema treatment currently consists of low-stretch bandaging and wearing elastic garments, which is effective in decreasing the volume of puffy hand syndrome.