Pathogenic variants in HGF give rise to childhood-to-late onset primary lymphoedema by loss of function.

Developmental and functional defects in the lymphatic system are responsible for primary lymphoedema (PL). PL is a chronic debilitating disease caused by increased accumulation of interstitial fluid, predisposing to inflammation, infections and fibrosis. There is no cure, only symptomatic treatment is available.

The VASCERN PPL working group patient pathway for primary and paediatric lymphoedema.

Lymphoedema is caused by an imbalance between fluid production and transport by the lymphatic system. This imbalance can be either caused by reduced transport capacity of the lymphatic system or too much fluid production and leads to swelling associated with tissue changes (skin thickening, fat deposition). Its main common complication is the increased risk of developing cellulitis/erysipelas in the affected area, which can worsen the lymphatic function and can be the cause of raised morbidity of the patient if not treated correctly/urgently.

Ureteropelvic junction obstruction with primary lymphoedema associated with variants.

Background: Primary lymphoedema (PL) is a chronic, debilitating disease caused by developmental and functional defects of the lymphatic system. It is marked by an accumulation of interstitial fluid, fat and tissue fibrosis. There is no cure.

Paediatric lymphoedema: An audit of patients seen by the paediatric and primary lymphoedema group of vascular European Reference Network (VASCERN).

Little is known about the overall prevalence of lymphoedema in children and the types of paediatric lymphoedema seen by specialist centres. Therefore, this study was aimed to provide a profile of children with primary or secondary lymphoedema seen by the expert centres of the paediatric and primary lymphoedema working group (PPL-WG) of VASCERN and to compare the profile between the different countries. A retrospective review of all children (aged up to 18 years) seen for the first time by the expert centres over one year (2019) was carried out.

Recent Advances in Ultrasound Technology: Ultra-High Frequency Ultrasound for Reconstructive Supermicrosurgery.

Background:  Currently, microsurgeons are in the era of supermicrosurgery and perforator flap reconstruction. As these reconstructions frequently utilize vessels that are smaller than a single millimeter, understanding of location of lymphatic vessels and perforator anatomy preoperatively is essential. To change with the times, the role of ultrasound has changed from just an adjunct to primary imaging of the choice in reconstructive supermicrosurgery.

Intraoperative Real-Time Visualization of the Lymphatic Vessels Using Microscope-Integrated Laser Tomography.

Background:  Detection and selection of the lymphatic vessels are important for maximizing therapeutic efficacy of lymphaticovenular anastomosis (LVA). Some imaging modalities have been reported to be useful for intraoperative identification of the lymphatic vessels, but they have limitations. In this article, we present new capabilities of intraoperative laser tomography, which was used to evaluate the lumen of the lymphatic vessel and to validate the patency of anastomosis.

Bedside 3D Visualization of Lymphatic Vessels with a Handheld Multispectral Optoacoustic Tomography Device.

Identification of lymphatics by Indocyanine Green (ICG) lymphography in patients with severe lymphedema is limited due to the overlying dermal backflow. Nor can the method detect deep and/or small vessels. Multispectral optoacoustic tomography (MSOT), a real-time three- dimensional (3D) imaging modality which allows exact spatial identification of absorbers in tissue such as blood and injected dyes can overcome these hurdles.

Successful treatment of breast cancer-related breast lymphedema by lymphovenous anastomosis in a male patient.

Men, as well as women may develop breast lymphedema following breast cancer treatment. Microsurgically performed lymphovenous anastomosis (LVA), an effective treatment for lymphedema of the extremities, has also been successfully applied to breast lymphedema. Here we report the first case of breast lymphedema secondary to male breast cancer, treated with supermicrosurgical LVA.

The Application of Virtual Reality for Preoperative Planning of Lymphovenous Anastomosis in a Patient with a Complex Lymphatic Malformation.

The management of lymphatic malformations (LMs) is challenging, particularly for large and complex lesions involving anatomical structures in the adjacent tissue. While lymphovenous anastomosis (LVA) has been reported as an effective treatment for lymphedema, it has hardly been described as a treatment for LM. Virtual reality has the ability to visualize human structures in three dimensions and can be used for the preoperative planning of complex cases.

Ultra High-frequency Ultrasonographic Imaging with 70 MHz Scanner for Visualization of the Lymphatic Vessels.

Background: Identification and localization of functional lymphatic vessels are important for lymphaticovenular anastomosis. Conventional high-frequency ultrasound (CHFUS) has been reported to be useful for them, but it has some disadvantages. In this article, we present new capabilities of ultra high-frequency ultrasound (UHFUS) for imaging of the lymphatic vessels, which may overcome the weakness of CHFUS.

Lymphatic supermicrosurgery for the treatment of recurrent lymphocele and severe lymphorrhea.

Background: Lymphocele and lymphorrhea are frequent complications after lymph node excision. Recurrent lymphoceles and intractable lymphorrhea are particularly difficult to treat conservatively. We describe the outcomes of four patients with recurrent lymphocele and nine patients with persistent lymphorrhea that were treated by supermicrosurgery.

Supermicrosurgical lymphaticovenous anastomosis for a patient with breast lymphedema secondary to breast cancer treatment.

Breast lymphedema (BLE) has been reported as a complication following breast cancer treatment. As for extremity lymphedema treatment, supermicrosurgical lymphaticovenous anastomosis (LVA) is considered an option for the treatment of progressive BLE refractory to conservative treatments, but no case has been reported so far. We report the first case of BLE successfully treated with supermicrosurgical LVA.