Postmastectomy lymphedema

Disturbing lymphedema of the related arm occurs in 20 to 30 per cent of patients after conventional mastectomy.Infection, obesity and radiation therapy are the most important contributing factors in the development of swollen arm. Continuous suction drainage of the wound and the use of antibiotics will reduce the incidence of infection. Lymphangiosarcoma is a rare fatal complication of postmastectomy lymphedema.     

Effect of lymphangiography on lymphedema

Lymphangiography, using oily radiopaque Lipiodol Ultra Fluid has been shown to increase lymphedema in one-third of the patients with obstructive lymphedema and to cause lymphatic obliteration (demonstrated histologically). No effective element has been observed, although subclinical infection could not be ruled out. Allergy is another possible contributor, but the evidence from this series suggests that Lipiodol may act as a direct irritant when it is not rapidly cleared, as in the case of obstructive lymphedema, and proceed to obliteration of the residual lymphatics. On the basis of these findings, elective routine preoperative lymphangiography for lymphedema is considered strongly inadvisable.     

Staged treatment of lymphedema praecox

Lymphedema of the lower extremities poses a challenging problem in management. Gross deformities may be encountered in patients with filarial infestation. This degree of involvement is rare in native North Americans suffering from primary lymphedema.A case is presented of a patient with changes similar to those seen in filariasis, due to several episodes of acute lymphangitis over a period of years. The involved tissue was excised and the defects skin-grafted, employing a modified Charles procedure. The magnitude of the excision was such that it was carried out in three widely spaced stages. The result was satisfactory from a functional viewpoint, and also represented a marked cosmetic improvement.     

Candidate gene analysis in primary lymphedema

BACKGROUND: Primary lymphedema, the accumulation of protein-rich fluid in the interstitial space, is the clinical manifestation of mutations involved in lymphatic development and function. Mutations in three genes, VEGFR3, FOXC2, and SOX18, cause primary lymphedema. However, mutations in these three genes only account for a fraction of primary lymphedema. To identify other genes mutated in primary lymphedema, we resequenced twenty-five biologically plausible candidate genes for lymphedema in a large collection of primary lymphedema families. METHODS AND RESULTS: Candidate genes were selected on the basis of gene expression in lymphatic endothelial cells, differential antigenic expression in lymphatics, and mouse studies of lymphatic development. The gene sequence was downloaded from GenBank and sequence primers designed to amplify 1 Kb of the 5' sequence, exons and flanking intron-exon boundaries, and 500 bp of the UTR of each gene. No common causative mutations were observed among the 25 genes screened. Single mutations were observed in elastin microfibril interfacer (EMILIN1), lymphocyte cytosolic protein 2 (LCP2), fatty acid binding protein 4 (FABP4), protein tyrosine kinase SYK (SYK), neuropilin-2 (NRP2), SpSRY-box 17 (SOX17), vascular cell adhesion molecule 1 (VCAM1), ROR orphan receptor C (RORC), and vascular endothelial growth factor B (VEGFB). Among these, the mutations in EMILIN1, RORC, LCP2, SYK, and VEGFB failed to segregate with lymphedema. The mutations in FABP4 (2), NRP2, SOX17, and VACM1 are consistent with being causative mutations, but occur in families too small to convincingly confirm cosegregation of mutation and phenotype. CONCLUSION: We excluded mutation in 21 biological candidate genes as a common cause of primary lymphedema. Mutations in FABP4, NRP2, SOX17 and VCAM1 are consistent with causality and follow up of these four genes are warranted. The evidence for FABP4 harboring lymphedema mutations is discussed.     

Creation of distal canine limb lymphedema

A canine model of distal limb lymphedema was established in order to study the treatment of this condition by lymph node transfer. This model was more difficult to establish than whole-limb lymphedema. Significant edema was achieved by a combination of preoperative irradiation and circumferential removal of skin from the irradiated areas followed by removal of the contents of the popliteal fossa. Despite these measures, it was not possible to produce lymphedema in every case, possibly because of the presence of lymphaticovenous shunts and panvascular compensation mechanisms.     

An experimental model for chronic lymphedema

Although a multitude of operations exist for the treatment of lymphedema, none is highly successful. An experimental model that reliably and easily produces chronic lymphedema in an extremity would be useful to study treatments in a controlled and comparative manner and would enhance our understanding of the physiology and treatment of lymphedema. Many models that simulate clinical lymphedema have been described, but they suffer from cumbersome protocols, high laboratory costs, and an inconsistent yield of permanent lymphedema. We describe an experimental model for chronic lymphedema in the lower extremity of the rat that creates a lymphatic block in the groin induced by radiation treatment and one operation--surgical division of the superficial and deep lymphatics. All animals develop stable chronic lymphedema of the lower extremity within days of operation, with swelling that persists for at least 9 months. A mortality rate of 8 percent was associated with this technique. Methods for quantification of limb swelling are described, as is analysis of the lymphatic block by lymphoscintigraphic imaging of lymph channels and nodes. This model has the advantages of simplicity of technique, cost-effective use of rodent subjects, reproducibility of lymphedema, and quantification of results.     

Hereditary congenital lymphedema with pseudosexual ambiguity

A case of uncommon genital lymphedema in a newborn girl like a pseudo sexual ambiguity is reported. The karyotype was 46, XX. Lymphedema of the lower limbs in the patient and in the mother's family confirmed a None-Milroy disease. Different considerations about genetic counseling in hereditary lymphedema, isolated or associated with others anomalies, are developed.     

A new surgical approach in genital lymphedema

A new surgical approach has been used in a case of genital lymphedema. After resection of the lymphedematous mass, U-shaped flaps were made from the suprapubic region anteriorly and the posterior scrotal skin posteriorly. The denuded penis was transposed to its original place by passing it through a buttonhole incision made on the anterior flap. The testicles were placed and fixed in pouches prepared between the anterior and posterior flaps. The patient had an acceptable postoperative outcome both in testicular function and habitual sexual activities.     

Overview of surgical treatments for breast cancer-related lymphedema

Breast cancer-related upper extremity lymphedema is an unsolved iatrogenic complication with a reported incidence ranging from 9 to 41 percent. The increase in volume and recurrent cellulitis of the affected limb cause both physical and mental distress to many breast cancer survivors. However, postmastectomy lymphedema has received little attention, and no curative treatment is available. Conservative treatment with decongestive therapy has been the primary choice for lymphedema treatment, but it is cumbersome and has limited benefits. To date, there is no consensus on surgical procedure and protocol. However, refinements in microsurgical techniques and improved examination devices may lead to the establishment of a standard surgical treatment for lymphedema. This review of surgical procedures for the treatment of postmastectomy lymphedema focuses on microsurgical lymphovenous shunt operations and discusses current issues in surgical treatment and the need for uniform treatment standards.     

Proteomic profiling of lymphedema development in mouse model

The lymphatic vascular system plays an important role in tissue fluid homeostasis. Lymphedema is a chronic, progressive, and incurable condition that leads to lymphatic fluid retention; it may be primary (heritable) or secondary (acquired) in nature. Although there is a growing understanding of lymphedema, methods for the prevention and treatment of lymphedema are still limited. In this study, we investigated differential protein expressions in sham‐operated and lymphedema‐operated mice for 3 days, using two‐dimensional gel electrophoresis (2‐DE) and mass spectrometry analysis. Male improved methodology for culturing noninbred (ICR) mice developed lymphedema in the right hindlimb. Twenty functional proteins were found to be differentially expressed between lymphedema induced‐right leg tissue and normal left leg tissue. Out of these proteins, the protein levels of apolipoprotein A‐1 preprotein, alpha‐actinin‐3, mCG21744, parkinson disease, serum amyloid P‐component precursor, annexin A8, mKIAA0098 protein, and fibrinogen beta chain precursor were differentially upregulated in the lymphedema mice compared with the sham‐operated group. Western blotting analysis was used to validate the proteomics results. Our results showing differential up‐regulation of serum amyloid P‐component precursor, parkinson disease, and apolipoprotein A‐1 preprotein in lymphedema model over sham‐operated model suggest important insights into pathophysiological target for lymphedema. Copyright © 2016 John Wiley & Sons, Ltd.