Abstracts LymphForsch 1/2014

Surgical aspects of liposuction in lipedema treatment: Twelve questions – twelve answers

W. Schmeller, A. Baumgartner
LymphForsch 2014; 18(1):6-12

While conservative therapy (complete physical decongestive therapy) has been an established standard treatment for lipedema for over 50 years, surgical treatment (liposuction) has been used for only the past 12 years. Many aspects of surgical lipedema treatment are still unclear. The article answers 12 questions about important surgical aspects.

Key words: lipedema, liposuction, surgical aspects

Reducing lymphostatic fibroslerosis in different skin layers during CDT: Results of a randomized controlled trial with 88 patients

S. Noori, R. Petereit, J. Rindermann, H. W. Jungkunz
LymphForsch 2014; 18(1):13-21

High-resolution color duplex sonography (18 MHz probe) combined with a patient history, physical findings, inspection and manual exploration fulfilled all requirements for ultrasound examinations to diagnose and follow lymphostatic fibrosclerosis of the skin (LFSS) if anatomical reference points were taken into account. A higher echographic density and more extensive dense echographic patterns compared to the normal skin corresponded to pronounced LFSS, which could be seen both in surgical in vivo findings and in histological findings. With respect to different skin layers, we were able to use these methods to differentiate between papillary, reticular and subcutaneous LFSS and LFSS of muscle fascia. In most cases, we found LFSS in more than 1 dermal layer. According to the guidelines, the ultrasound examinations should be performed before invasive and cost-intensive examinations are considered.
We used high-resolution color duplex sonography and histological specimens to examine LFSS reduction in 2 randomized groups of 50 patients each with primary hereditary lymphedema. All patients were undergoing phase II (optimization) of combined decongestivetherapy (CDT). Exclusion criteria included any comorbidity causing other skin lesions. Over a period of 4 months, the patients underwent CDT at lymphedema clinics complying with lymphedema management guidelines. The treatment included patient education, manual lymph drainage with multilayer compression bandages worn until the next morning 2 days per week, and flat-knitted seamed compression stockings worn on the other 5 days. The intervention group wore compression stockings with special beveled polyurethane compression inlays with a ribbed surface and defined density and hardness, which were individually constructed according to a sonographically controlled draft prepared by a physician.
The control group wore compression stockings without these special devices. The compression inlays were placed at defined skin areas. After 4 months, we compared the defined skin areas in the intervention group to those of the control group. Forty-four patients in each group finished the study. In the intervention group, we proved a 72% reduction in LFSS, while the control group had only an approximately 35% reduction in LFSS. The reduction in LFSS correlated with the reduction in cost-related secondary complications.
Based on these results, we state that if patients undergo phase II CDT combined with deep topographical compression with compression profile inlays designed by a standardized procedure, LFSS is significantly reduced compared to treatment with CDT alone.

Keywords: papillary, reticular and subcutaneous lymphostatic fibrosclerosis of the skin, topographical deep compression, compression profile inlays, high-resolution color duplex sonography.

Challenges associated with diagnostic lymph node biopsies of enlarged inguinal lymph nodes: Case report of a patient with Castleman‘s disease

C. Königshofer, W. Döller
LymphForsch 2014; 18(1):22-25

The difficulties associated with the decision to take biopsies of enlarged lymph nodes in general and in the inguinal region in particular are well known and hotly debated. While lymph node biopsies are sometimes performed for no apparent reason, they are also often not performed when indicated. The reasons for local or generalized lymph node swelling are complex. This article presents a case report of lymph node biopsy that demonstratesthe importance of this examination for diagnosing Castleman‘s disease.

Keywords: regional enlarged lymph nodes, Castleman‘s disease, lymph node biopsy

The genetics of primary lymphedema

J. Wilting
LymphForsch 2014; 18(1):26-30

Although primary lymphedema constitutes only approximately 1 % of all lymphedemacases worldwide, these cases can teach us a great deal about lymphatic development andfunction. In this review, we will discuss primary lymphedema and lymphedema-associated syndromes for which the genetic basis and gene mutations have been characterized. The affected genes code receptors for growth factors, transcription factors, motor proteins, intercellular contacts, extracellular matrix and regulators of intracellular signal transduction.
While the extensive variety of molecules underlines the complexity of lymphangiogenesis and lymph transport, it may also point to potential therapeutic interventions.

Keywords: Nonne-Milroy, Emberger, Hennekam, Norman-Roberts, VEGFR-3, GJC2, FOXC2, PTPN14, KIF11, SOX18, GATA2, Reelin, CCBE1, PTPN11

Initial lymphatics – myths and truths

E. Brenner
LymphForsch 2014; 18(1):31-40

This paper critically examines many untruths and half-truths about the initial lymphatics that have accumulated over time. Initial lymphatics are present in nearly all organs, with the exception of the central nervous system, bone, cornea and the lens of the eye. Initial lymphatics usually begin as a 2- or 3-dimensional networks, except in the intestinal villi and the hairless skin of the hands and feet, where finger-shaped blindly commencing initial lymphatics are found. In the skin, too with the exception of the hairless skin – initial lymphatics form 2- and 3-dimensional networks that clearly vary according to region. Initial lymphatics vary in width, with data ranging from 10 μm to 100 μm depending on the location and condition of filling. Initial lymphatics also have a basement membrane, although it is clearly distinguished from a conventional basement membrane. In addition, the initial lymphatics are anchored in the surrounding connective tissue by distinct anchoring filaments. The endothelial cells of initial lymph vessels are very large, with an average area of about 600 μm2. Apart from the perinuclear region, the lymphatic endothelial cells are also very thin. Meandering cell borders towards neighboring cells are characteristic; adjacent cells can also overlap to a small extent. In the area of such an overlap, interendothelial openings are also present. At maximum expansion of the initial lymphatics, these openings can have a diameter of up to 5 μm.

Keywords: initial lymphatics, lymphatic vascular networks, lymphatic endothelial cells, basement membrane, interendothelial openings