Surgical creation of lymphocutaneous fistulas for the management of infants with central lymphatic obstruction.

PURPOSE: Central lymphatic obstructions are associated with anasarca and high mortality. We hypothesized that opening dilated cutaneous lymphatic channels by creating a lymphocutaneous fistula (LCF) would decompress the lymphatic circulation and improve anasarca. METHODS: We reviewed all patients that had at least one LCF created between 9/2019 and 12/2022. LCF efficacy was determined by changes in weight, urine/diuresis, ventilation, and clinical status. RESULTS: We created eleven LCFs in four infants. LCFs initially drained 108 cc/kg/d (IQR68-265 cc/kg/d). Weights significantly decreased after LCF creation (6.9 [IQR6.1-8.1] kg vs. 6.1 [IQR 4.9-7.6] kg, P = 0.042). Ventilatory support decreased significantly in all patients after at least one LCF was created, and 3/4 patients (75%) had significantly lower peak inspiratory pressures (28 [IQR 25-31] cmH2O vs. 22 [IQR 22-24] cmH2O, P = 0.005; 36 [IQR36-38] cmH2O vs. 33 [IQR 33-35] cmH2O, P = 0.002; 36 [IQR 34-47] cmH2O vs. 28 [28-31] cmH2O, P = 0.002). LCFs remained patent for 29d (IQR 16-49d). LCFs contracted over time, and 6/11 (54.5%) were eventually revised. There were no complications. Two patients died from overwhelming disease, one died from unrelated causes, and one remains alive 29 months after their initial LCF. CONCLUSION: LCFs provide safe and effective temporary lymphatic decompression in patients with central lymphatic obstruction. While LCFs are not a cure, they can serve as a bridge to more definitive therapies or spontaneous lymphatic remodeling. LEVEL OF EVIDENCE: IV.

The bright future of nanotechnology in lymphatic system imaging and imaging-guided surgery.

Lymphatic system is identified the second vascular system after the blood circulation in mammalian species, however the research on lymphatic system has long been hampered by the lack of comprehensive imaging modality. Nanomaterials have shown the potential to enhance the quality of lymphatic imaging due to the unparalleled advantages such as the specific passive targeting and efficient co-delivery of cocktail to peripheral lymphatic system, ease molecular engineering for precise active targeting and prolonged retention in the lymphatic system of interest. Multimodal lymphatic imaging based on nanotechnology provides a complementary means to understand the kinetics of lymphoid tissues and quantify its function. In this review, we introduce the established approaches of lymphatic imaging used in clinic and summarize their strengths and weaknesses, and list the critical influence factors on lymphatic imaging. Meanwhile, the recent developments in the field of pre-clinical lymphatic imaging are discussed to shed new lights on the design of new imaging agents, the improvement of delivery methods and imaging-guided surgery strategies.

A Systematic Stepwise Method to Perform a Supermicrosurgical Lymphovenous Anastomosis.

BACKGROUND: Lymphovenous anastomosis (LVA) has become an increasingly common treatment for patients with extremity lymphedema. In this article, we present our current strategy for patient selection, preoperative planning, and a series of intraoperative clues that may help to perform a supermicrosurgical LVA. Technical considerations are presented using a systematic step-by-step method to make this procedure more reproducible and straightforward. PATIENTS AND METHODS: We conducted a review of patients operated between January 2015 and June 2018 using the aforementioned approach. Data were collected prospectively, and all procedures were performed by the senior author. Preoperative assessment included lymphoscintigraphy, indocyanine green lymphography, noncontrast magnetic resonance lymphography and high-frequency ultrasonography. Lymphovenous anastomosis was decomposed into a sequential 6-step approach considering the main aspects that determine a successful anastomosis. RESULTS: Lymphovenous anastomosis was performed in 229 patients, including 677 anastomoses. Median follow-up was 33 months (range, 13-51 months). A median of 3.1 (range, 1-7) LVA were performed on 2.7 (range, 1-6) incision sites per patient. Median time for dissection of lymphatic(s) and vein(s) was 8.7 minutes (1-18 minutes) with a median time of 27.2 minutes (range, 13-51 minutes) for a complete LVA. Lymphatic detection rate was 100% (677 of 677) and vein detection rate was 99.7% (675 of 677), with 31.0% (210 of 677) of reflux-free veins. For upper-extremity lymphedema (47 of 229; 20.6%), volume reduction was achieved in 100% (47 of 47) of the cases, with a median volume reduction rate of 67% (range, 7-93%). In lower-extremity lymphedema (182 of 229; 79.4%), volume reduction was achieved in 86.8% (158 of 182) of the cases, with a median volume reduction rate of 41% (range, 7-81%). Cellulitis episodes decreased from 2.1 to 0.2 episodes/year after LVA (P < 0.05). CONCLUSIONS: Acceptable success rates were obtained using a sequential strategy for planning and execution of supermicrosurgical LVA for secondary extremity lymphedema. We believe including a stepwise approach may help to simplify this procedure, especially for surgeons in their early practice.

Primary Lymphedema: Update on Genetic Basis and Management.

Significance: Primary lymphedema is a chronic condition without a cure. The lower extremities are more commonly affected than the arms or genitalia. The disease can be syndromic. Morbidity includes decreased self-esteem, infections, and reduced function of the area. Recent Advances: Several mutations can cause lymphedema, and new variants continue to be elucidated. A critical determinant that predicts the natural history and morbidity of lymphedema is the patient's body mass index (BMI). Individuals who maintain an active lifestyle with a normal BMI generally have less severe disease compared to subjects who are obese. Because other causes of lower extremity enlargement can be confused with lymphedema, definitive diagnosis requires lymphoscintigraphy. Critical Issues: Most patients with primary lymphedema are satisfactorily managed with compression regimens, exercise, and maintenance of a normal body weight. Suction-assisted lipectomy is our preferred operative intervention for symptomatic patients who have failed conservative therapy. Suction-assisted lipectomy effectively removes excess subcutaneous fibro-adipose tissue and can improve underlying lymphatic function. Future Directions: Many patients with primary lymphedema do not have an identifiable mutation and thus novel variants will be identified. The mechanisms by which mutations cause lymphedema continue to be studied. In the future, drug therapy for the disease may be developed.

The Lymphatic System in the Fontan Patient-Pathophysiology, Imaging, and Interventions: What the Anesthesiologist Should Know.

The Fontan surgery was developed as a palliative intervention for congenital heart disease (CHD) patients with single-ventricle physiology who are not candidates for a biventricular repair. Improvements in the surgery and medical management of these patients have increased survival, yet this population remains at risk for complications and end-organ dysfunction due to Fontan failure. Lymphatic vessels maintain a fluid balance within the extracellular space, participate in fat reabsorption from the small intestine, and play an important role in the body's immune response. Altered Starling forces at the capillary level, capillary leak, and lymphatic obstruction contribute to lymphatic dysfunction in patients with Fontan physiology. These lymphatic complications include edema, pleural effusions, plastic bronchitis (PB), and protein-losing enteropathy (PLE). Over the past decade, there have been innovations in lymphatic imaging. These new imaging techniques include noncontrast magnetic resonance (MR) lymphangiography, intranodal lymphangiography (IL), dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL), and liver lymphangiography. These imaging techniques help in delineating anatomy and guiding the appropriate therapeutic approach. Lymphatic interventions then may be performed to decompress the lymphatic system or to identify and occlude abnormal lymphatic vessels and drainage pathways. The anesthesiologist should have an understanding of the effects of lymphatic disorders on the Fontan circulation and apply appropriate management techniques for the associated interventions. The Fontan surgery was developed as a palliative intervention for CHD patients with single-ventricle physiology who are not candidates for a biventricular repair. The surgery creates a series systemic and pulmonary circulation with the energy necessary to provide gradient-driven pulmonary blood flow generated by the ventricle.1 In the past decades, improvements in the surgery and medical management of these patients have increased survival, with 30-year survival rates close to 85%.2 Despite these improvements, this population remains at risk for complications and end-organ dysfunction due to Fontan failure, which is characterized by elevated systemic venous pressures and low cardiac output. These complications include arrhythmias, cardiac dysfunction, ascites, liver fibrosis/cirrhosis, renal dysfunction, pulmonary failure, and lymphatic complications such as edema, pleural effusions, PB, and PLE. Complications ultimately contribute to increased risk for hospitalization, death, and need for heart transplantation.3,4 For this reason, there has been increasing interest in the role of abnormal lymphatic circulation in the genesis of Fontan failure. The authors characterize the lymphatic pathophysiology associated with Fontan physiology and review the imaging and interventional strategies used to treat these patients.

Combined pedicled superficial circumflex iliac artery perforator (SCIP) flap with lymphatic tissue preservation and lymphovenous anastomosis (LVA) for defect reconstruction and lymphedema-lymphocele prevention in thigh sarcoma surgery: Preliminary results.

BACKGROUND: Sarcoma surgery often requires large tissue resection to be treated safely. When the tumor is localized in the groin and/or medial thigh, lymphocele and lymphedema are common complications because of the rich lymphatic network present there. The aim of this study is to share the outcome of seven patients who received defect reconstruction in this area with combined pedicled superficial circumflex artery perforator (SCIP) flap with lymphatic tissue preservation and lymphovenous anastomosis (LVA) for prevention of lymphatic complications. PATIENTS AND METHODS: Seven patients who underwent surgical resection of sarcoma in the groin and/or adductors compartment received defect reconstruction with pedicled SCIP flap combined with LVA. For a better dead space obliteration, four of them also received an additional tissue flap: two pedicled deep inferior epigastric perforator flaps and two free anterolateral thigh flaps. Indocyanine green lymphography was performed in all cases to identify the lymphatic pathway, make the preoperative marking and check the patency of the anastomoses. RESULTS: All seven patients were successfully treated reaching a good aesthetic result and a full range of motion. No immediate nor delayed complications such as lymphocele or lymphorrhea and early extremity lymphedema were observed during the follow up (range: 6-9 months; mean: 7.3) and no secondary procedures were required. CONCLUSIONS: The combination of the pedicle SCIP lymphatic tissue transfer with LVA seems to be effective in preventing the development of lymphatic sequelae after large resections in the medial thigh.

Surgical Management of Lymphedema.

Lymphedema is a chronic debilitating disease in which impaired drainage of lymphatic fluid causes accumulation of fluid in the soft tissues resulting in a swollen heavy limb. This ultimately leads to severe fibrosis, recurrent infections, non-healing wounds, and a poorly functioning limb that negatively affects a patient's quality of life. Primary lymphedema is due to abnormal development of the lymphatic system and patients can present with lymphedema at birth or later in life. Secondary lymphedema is caused by damage to the lymphatic system from infection, surgery to treat malignancies, trauma, and obesity. In the past, the only treatment was controlling the swelling to prevent progression of the disease by lymphatic therapy and various types of compression which is still currently the first line treatment. Advances in supermicrosurgery (connecting vessels less than 0.8 mm) have made way for surgical treatment options for lymphedema, including lymphovenous bypass and vascularized lymph node transplant. These new surgical treatment options combined with lymphatic therapy and compression have led to better results and improved patient's quality of life. After reading this article, the participant should be familiar with diagnosis, imaging, and surgical treatment of lymphedema.

The Charles Procedure as Part of the Modern Armamentarium Against Lymphedema.

BACKGROUND: Over the past 2 decades, the surgical treatment of lymphedema has advanced considerably with lymphovenous bypass and vascularized lymph node transfer. Despite these advances, some patients can only be treated with a radical debulking procedure, commonly known as Charles procedure. However, the Charles procedure is not a commonly performed procedure and can have significant risks such as fluid shifts, blood loss and wound infections. In this article, we present our experience with Charles procedure and share pearls to perform this procedure expediently while avoiding potential pitfalls. METHODS: Patients with severe lymphedema who were treated by the senior author with Charles procedure were evaluated. The medical record was queried for their pertinent medical history, including cause of lymphedema, prior treatments for the condition, medical comorbidities and preoperative and postoperative course. The surgical technique and lessons learned from each case are described. RESULTS: Three patients were identified within the study period who underwent treatment with the Charles procedure. All patients had secondary lower extremity lymphedema. All patients had single-stage direct excision and skin grafting. One patient required postoperative ICU stay due to significant fluid shifts and blood loss, another suffered from a wound infection. All 3 patients ultimately recovered to exceed their preoperative activity levels with satisfactory outcomes. CONCLUSIONS: The Charles procedure continues to have clinical utility in modern lymphedema treatment. Despite potential risks, it can be done successfully with proper planning and careful attention to technical details; it can be life changing for patients suffering from most extreme lymphedema.

[Analysis of chyle leakage after central lymph node dissection for thyroid carcinoma].

Objective: To analyze the clinical characteristics, treatment and prognosis of chyle leakage after central lymph node dissection for thyroid cancer. Methods: A retrospective analysis was made of 985 patients who underwent surgical for thyroid carcinoma plus central lymph node dissection from January 2017 to June 2018 in Renji Hospital Affiliated to Medical College of Shanghai Jiaotong University. Patients were divided into those without (group A, n=973) and with (group B, n=12) chyle leakage. Patients with chyle leakage who underwent left central lymph node dissection were divided into group B1 (n=5) and right central lymph node dissection into group B2 (n=7). Patients with chyle leakage were treated with fat-free diet and negative pressure drainage. SPSS 20.0 software was used to analyze the general condition, surgical pathology, postoperative drainage, hospitalization days, treatment and prognosis of patients in B1 and B2 groups. Results: The incidence of chyle leakage after central lymph node dissection for thyroid cancer was 1.2% (12/985). There were no significant differences in age, sex, size of primary lesion, number of lymph node dissection in central area and number of lymph node metastasis in central area between group A and group B (all P>0.05). The drainage volume on the first day after operation [((51.7±26.7)) ml] and the average hospitalization days [(3.4±0.8) d] in group A were significantly lower than those in group B ([131.3±56.0)]ml, [10.4±2.6)]d). The differences were statistically significant (t value was -5.442, -11.238, respectively, both P<0.001). There were no significant differences in age, size of primary lesion, number of lymph node dissection, number of lymph node metastasis, drainage volume on the first day after operation and average hospitalization days between group B1 and group B2 (all P>0.05). All chyle leakages in group B stopped after conservative management without surgical intervention. Conclusion: The occurrence of chyle leakage after central lymph node dissection is a rare complication. It can be cured by conservative treatment such as diet control, pressure bandaging and negative pressure drainage, and generally does not require secondary surgery.

Use of Isotope Tracers to Assess Lipid Absorption in Conscious Lymph Fistula Mice.

This protocol provides a comprehensive reference for the evolution of the lymph fistula model, the mechanism of lipid absorption, the detailed procedure for studying lipid absorption using the lymph fistula model, the interpretation of the results, and consideration of the experimental design. The lymph fistula model is an approach to assess the concentration and rate of a range of molecules transported by the lymph by cannulating lymph duct in animals. In this protocol, mice first undergo surgery with the implantation of cannulae in the duodenum and mesenteric lymph duct and are allowed to recover overnight in Bollman restraining cages housed in a temperature-regulated environment. To study in vivo lipid absorption, a lipid emulsion is prepared with labeled tracers, including [3 H]-triolein and [14 C]-cholesterol. On the day of the experiment, mice are continuously infused with lipid emulsion via the duodenum for 6 hr, and lymph is usually collected hourly. At the end of the study, gastrointestinal segments and their luminal contents are collected separately for determination of the digestion, uptake, and transport of exogenous lipids. © 2019 by John Wiley & Sons, Inc.

Direct trans-cervical endolymphatic thoracic duct stent-graft for plastic bronchitis.

Plastic bronchitis is a poorly understood and uncommon diagnosis, arising from multiple etiologies. Traditional treatment consists of steroids and vasodilators, with thoracic duct embolization emerging as a new procedural therapy. Herein, abnormal lymphatic vessels were noted on lymphangiography in an adult patient with debilitating plastic bronchitis, but anterograde lymphatic access was not feasible due to the patient's morbid obesity and non-visualization of retroperitoneal lymphatics. After trans-venous thoracic duct access could not be established, direct trans-cervical thoracic duct access was performed. A thoracic duct stent-graft was placed, excluding the abnormal bronchial lymphatics and maintaining physiologic anterograde flow through the central lymphatics. At three-month follow-up, the patient's condition had resolved.

MR lymphography in patients with upper limb lymphedema: The GPS for feasibility and surgical planning for lympho-venous bypass.

BACKGROUND AND OBJECTIVES: Lymphedema is caused by insufficient lymphatic drainage leading to abnormal accumulation of interstitial fluid within soft tissues. Lympho-venous anastomosis (LVA), as a surgical option for selected patients, is widely applied. Through preoperative localization of functional lymphatics with indocyanine green, real time visualization of functioning lymphatic vessels is possible. This examination is time consuming and operator dependant and is not suitable to differentiate the ratio of fat hypertrophy to liquid edema. We investigated whether MR lymphangiography is accurate for imaging functional lymphatics and adjacent veins in arms. Furthermore, we investigated the accuracy and predictability of preoperative mapping for the feasibility of performing LVA and the preoperative decision making in lymphedema surgery. METHODS: A prospective study was performed in which 25 patients suffering from lymphedema of the upper extremity were examined. MR lymphography with contrast agent injection in a deep dermal plane was performed. RESULTS: Precise localization of lymphatic vessels crossing a vein was achieved in 18 of 25 arms. In 16 of the 18 patients in whom functional lymphatics were localized with an adjacent vein on MRI an LVA was performed successfully. CONCLUSIONS: MR lymphangiography is an accurate and reproducible method for imaging and mapping of lymphatic channels in the lymphedemateous limb.

Lymphedema surgery: the current state of the art.

BACKGROUND/PURPOSE: Lymphedema surgery, when integrated into a comprehensive lymphedema treatment program for patients, can provide effective and long-term improvements that non-surgical management alone cannot achieve. Such a treatment program can provide significant improvement for many issues such as recurring cellulitis infections, inability to wear clothing appropriate for the rest of their body size, loss of function of arm or leg, and desire to decrease the amount of lymphedema therapy and compression garment use. METHODS: The fluid predominant portion of lymphedema may be treated effectively with surgeries that involve transplantation of lymphatic tissue, called vascularized lymph node transfer (VLNT), or involve direct connections from the lymphatic system to the veins, called lymphaticovenous anastomoses (LVA). VLNT and LVA are microsurgical procedures that can improve the patient's own physiologic drainage of the lymphatic fluid, and we have seen the complete elimination for the need of compression garments in some of our patients. These procedures tend to have better results when performed when a patient's lymphatic system has less damage. The stiff, solid-predominant swelling often found in later stages of lymphedema can be treated effectively with a surgery called suction-assisted protein lipectomy (SAPL). SAPL surgeries allow removal of lymphatic solids and fatty deposits that are otherwise poorly treated by conservative lymphedema therapy, VLNT or LVA surgeries. CONCLUSION: Overall, multiple effective surgical options for lymphedema exist. Surgical treatments should not be seen as a "quick fix", and should be pursued in the framework of continuing lymphedema therapy and treatment to optimize each patient's outcome. When performed by an experienced lymphedema surgeon as part of an integrated system with expert lymphedema therapy, safe, consistent and long-term improvements can be achieved.

Presente y futuro de la especialidad desde la perspectiva de la Comisión Nacional / Present and future of the specialty from the perspective of the National Committee

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