[A Case of Lymphorrhea after Radical Cystectomy Treated by Ultrasound-Guided Inguinal Intranodal Lymphangiography].

A 76-year-old woman was diagnosed with invasive bladder cancer and underwent cystectomy, bilateral external iliac, internal iliac and obturator lymph node dissection, and bilateral cutaneous ureterostomy. Pathological findings showed no lymph node metastasis ; however, the patient had lower abdominal pain and fever from the 14th postoperative day, and computed tomography (CT) revealed fluid retention in the pelvis. Retrograde pyelography showed no leakage from the urinary tract, and a drain was placed after percutaneous puncture of the pelvic cavity. There was copious drainage fluid and its nature and composition suggested lymphorrhea. Ultrasound-guided intranodal lymphangiography revealed contrast material leakage from the bilateral lymph node dissection sites. After lymphangiography, drainage from the drain decreased. Despite the drainage being minimal yet persistent, sclerotherapy was performed, the drain was removed and the patient was discharged. After discharge, there was leakage from the site of urethral extraction, and CT revealed recurrent lymph leakage. The patient was readmitted, and a second lymphangiography was performed. The leakage from the site of urethral extraction gradually decreased, and the patient was discharged on the 59th postoperative day. CT after discharge confirmed that the lymphorrhea had shrunk in size, and there has been no recurrence since then. Lymphangiography is a promising treatment option for lymphorrhea after pelvic surgery.

Congenital Vascular and Lymphatic Diseases.

Over the past several centuries, the integration of contemporary medical techniques and innovative technologies, like genetic sequencing, have played a pivotal role in enhancing our comprehension of congenital vascular and lymphatic disorders. Nonetheless, the uncommon and complex characteristics of these disorders, especially considering their formation during the intrauterine stage, present significant obstacles in diagnosis and treatment. Here, we review the intricacies of these congenital abnormalities, offering an in-depth examination of key diagnostic approaches, genetic factors, and therapeutic methods.

Lymphatic interventions in congenital heart disease.

Congenital heart disease affects 1/100 live births and is one of the most common congenital abnormalities. The relationship between congenital heart disease and lymphatic abnormalities and/or dysfunction is well documented and can be grossly divided into syndromic and non-syndromic etiologies. In patients with genetic syndromes (as examples listed above), there are known primary abnormal lymphatic development leading to a large pleiotropic manifestation of lymphatic dysfunction. Non-syndromic patients, or those without clear genetic etiologies for their lymphatic dysfunction, are often thought to be secondary to physiologic abnormalities as sequelae of congenital heart disease and palliative surgeries. Patients with congenital heart disease and lymphatic dysfunction have a wide variety of clinical manifestations for which there were not many therapeutic interventions available. The development of new imaging techniques allows us to understand better the pathophysiology of these problems and to develop different percutaneous interventions aiming to restore normal lymphatic function.

Percutaneous procedures for central lymphatic conduction disorders.

Percutaneous endovascular techniques established in interventional cardiology and radiology are well-suited for managing lymphatic conduction disorders. In this article, we provide a synopsis of technical aspects of these procedures, including access of the thoracic duct, selective lymphatic embolization, and management of thoracic duct obstruction. In aggregate, these techniques have developed into an integral component of multidisciplinary management of these complex diseases.

Neonatal perspective on central lymphatic disorders.

Lymphatic disorders presenting in the first year of life are difficult to identify and manage given the broad range of underlying etiologies. Neonatal lymphatic disease arising from congenital or acquired conditions results in the abnormal accumulation of lymph fluid in the pleura (chylothorax), peritoneum (chylous ascites) and skin (edema/anasarca). There is also increasing recognition of lymphatic losses through the intestine resulting in protein-losing enteropathy (PLE). While the incidence of lymphatic disorders in neonates is unclear, advances in genetic testing and lymphatic imaging are improving our understanding of the underlying pathophysiology. Despite these advancements, medical management of neonatal lymphatic disorders remains challenging and variable among clinicians.

Multidisciplinary approach to patients with lymphatic conduction disorders.

Patients with lymphatic disorders are remarkably complex and require a wide variety of medical and surgical services. Establishing a multidisciplinary program improves the efficiency of the patients' hospital experience minimizing the compartmentalization of their care. Offering a clear intake process guarantees that patients will be seen promptly by all the required teams. Additionally, having regular multidisciplinary meetings allows all participating teams to learn from each other and gain experience in the care of a population that is extraordinarily heterogeneous. Additionally, establishing a solid program allows for long-term data collection, research and education.

Critical care management of patients with lymphatic conduction disorders.

Lymphatic dysfunction in critical illness is complex. Primary complex lymphatic anomalies can lead to profound organ dysfunction, particularly respiratory failure and shock. Critical illness, the complications of critical illness, and the procedures and therapies used to treat critical illness, can lead to secondary lymphatic dysfunction. This is most often seen with congenital and acquired cardiovascular disease and respiratory disease. The critical care management of these patients requires an expert multidisciplinary team.

Feasibility and safety of direct percutaneous embolization of lymphopseudoaneurysm for postoperative lymphatic leakage.

The treatment strategy for refractory postoperative lymphatic leakage is controversial. While the utility of direct percutaneous embolization of lymphopseudoaneurysm has been investigated, only a few reports on this topic exist. The aim of this study was to evaluate the technical and clinical feasibility and safety of direct percutaneous embolization of lymphopseudoaneurysm for refractory postoperative lymphatic leakage. This case series included six consecutive patients who underwent direct percutaneous embolization of lymphopseudoaneurysm for refractory postoperative lymphatic leakage. Technical success (successful percutaneous approach and injection of NBCA glue to the lymphopseudoaneurysm), clinical success (unnecessity of percutaneous drainage tube of the lymphatic leakage), treatment duration (the duration from the treatment to the achievement of clinical success) and procedure-related complications were mainly evaluated. Direct percutaneous embolization of the lymphopseudoaneurysm using NBCA glue was successfully performed in all cases. Clinical success was achieved in five of the six cases (83%). The mean treatment period was 9 days for the cases with clinical success. No major complications occurred postoperatively. In conclusion, direct percutaneous embolization of lymphopseudoaneurysm may become a feasible and safe treatment option for cases of refractory postoperative lymphatic leakage.

Anesthetic challenges in patients with multicompartmental lymphatic failure after Fontan palliation undergoing transcatheter thoracic duct decompression.

Lymphatic flow abnormalities are central to the development of protein losing enteropathy, plastic bronchitis, ascites and pleural effusions in patients palliated to the Fontan circulation. These complications can occur in isolation or multicompartmental (two or more). The treatment of multicompartmental lymphatic failure aims at improving thoracic duct drainage. Re-routing the innominate vein to the pulmonary venous atrium decompresses the thoracic duct, as atrial pressure is lower than systemic venous pressure in Fontan circulation. Transcatheter thoracic duct decompression is a new minimally invasive procedure that involves placing covered stents from the innominate vein to the atrium. Patients undergoing this procedure require multiple general anesthetics, presenting challenges in managing the sequelae of disordered lymphatic flow superimposed on Fontan physiology. We reviewed the first 20 patients at the Center for Lymphatic Imaging and Intervention at a tertiary care children's hospital presenting for transcatheter thoracic duct decompression between March 2018 and February 2023. The patients ranged in age from 3 to 26 years. The majority had failed prior catheter-based lymphatic intervention, including selective embolization of abnormal lympho-intestinal and lympho-bronchial connections to treat lymphatic failure in a single compartment. Fourteen had failure in three lymphatic compartments. Patients were functionally impaired (ASA 3-5) with significant comorbidities. Concurrent with thoracic duct decompression, three patients required fenestration closure for the resultant decrease in oxygen saturation. Ten patients had improvement in symptoms, seven had no changes and three have limited follow up. Five (25%) of these patients were deceased as of January 2024 due to non-lymphatic complications from Fontan failure.

Back to the Future II-A Comprehensive Update on the Rapidly Evolving Field of Lymphatic Imaging and Interventions.

ABSTRACT: Lymphatic imaging and interventional therapies of disorders affecting the lymphatic vascular system have evolved rapidly in recent years. Although x-ray lymphangiography had been all but replaced by the advent of cross-sectional imaging and the scientific focus shifted to lymph node imaging (eg, for detection of metastatic disease), interest in lymph vessel imaging was rekindled by the introduction of lymphatic interventional treatments in the late 1990s. Although x-ray lymphangiography is still the mainstay imaging technique to guide interventional procedures, several other, often less invasive, techniques have been developed more recently to evaluate the lymphatic vascular system and associated pathologies. Especially the introduction of magnetic resonance, and even more recently computed tomography, lymphangiography with water-soluble iodinated contrast agent has furthered our understanding of complex pathophysiological backgrounds of lymphatic diseases. This has led to an improvement of treatment approaches, especially of nontraumatic disorders caused by lymphatic flow abnormalities including plastic bronchitis, protein-losing enteropathy, and nontraumatic chylolymphatic leakages. The therapeutic armamentarium has also constantly grown and diversified in recent years with the introduction of more complex catheter-based and interstitial embolization techniques, lymph vessel stenting, lymphovenous anastomoses, as well as (targeted) medical treatment options. The aim of this article is to review the relevant spectrum of lymphatic disorders with currently available radiological imaging and interventional techniques, as well as the application of these methods in specific, individual clinical situations.

Intranodal Glue Embolization for Postoperative Lymphatic Leaks in the Groin and Pelvis: Comparison with Sclerotherapy.

PURPOSE: To compare the effectiveness of and adverse events related to intranodal glue embolization (IGE) with those of intracavitary sclerotherapy for the treatment of postoperative groin and pelvic lymphatic leaks. MATERIALS AND METHODS: From November 2015 to July 2021, IGE for postoperative pelvic or groin lymphocele or lymphorrhea was performed in 33 patients. From January 2010 to July 2021, 28 patients with postoperative pelvic or groin lymphocele were treated with sclerosis alone. Clinical success was defined as resolution of drainage within 3 weeks of the last intervention performed without recurrence. Patients presenting >1 year after surgery or with <30 days of follow-up were excluded. Patients with lymphorrhea treated with IGE were not statistically compared with those in the sclerosis group because they were not eligible for sclerosis. RESULTS: Clinical success was similar between the groups (lymphocele IGE, 15/18, 83.3%, vs sclerosis, 15/23, 65.2% [P = .29]; lymphorrhea IGE, 8/9, 88.9%). The mean number of interventions performed to successfully treat a lymphocele was significantly higher in the sclerosis group (2.5 for sclerosis vs 1.3 for IGE; P = .003; lymphorrhea IGE, 1.0). The mean time to resolution was significantly longer for sclerosis than for IGE (27 vs 7 days; P = .002; 4 days for lymphorrhea IGE). There were no sclerosis-related adverse events and 2 IGE-related adverse events: (a) 1 case of mild lymphedema and (b) 1 case of nontarget embolization resulting in deep vein thrombosis. CONCLUSIONS: For treatment of postoperative pelvic and groin lymphoceles, IGE results in faster resolution with fewer interventions compared with sclerosis. IGE is also an effective treatment for postoperative groin lymphorrhea.

Interventional Management of Acquired Lymphatic Disorders.

The lymphatic system is a complex network of tissues, vessels, and channels found throughout the body that assists in fluid balance and immunologic function. When the lymphatic system is disrupted related to idiopathic, iatrogenic, or traumatic disorders, lymphatic leaks can result in substantial morbidity and/or mortality. The diagnosis and management of these leaks is challenging. Modern advances in lymphatic imaging and interventional techniques have made radiology critical in the multidisciplinary management of these disorders. The authors provide a review of conventional and clinically relevant variant lymphatic anatomy and recent advances in diagnostic techniques such as MR lymphangiography. A detailed summary of technical factors related to percutaneous lymphangiography and lymphatic intervention is presented, including transpedal and transnodal lymphangiography. Traditional transabdominal access and retrograde access to the central lymph nodes and thoracic duct embolization techniques are outlined. Newer techniques including transhepatic lymphangiography and thoracic duct stent placement are also detailed. For both diagnostic and interventional radiologists, an understanding of lymphatic anatomy and modern diagnostic and interventional techniques is vital to the appropriate treatment of patients with acquired lymphatic disorders. ©RSNA, 2022.

Neonatal lymphatic flow disorders: central lymphatic flow disorder and isolated chylothorax, diagnosis and treatment using novel lymphatic imaging and interventions technique.

PURPOSE OF REVIEW: Neonatal lymphatic disorders (NLDs) are conditions that are relatively rare and difficult to treat. The recent development of lymphatic imaging, such as Dynamic Contrast-Enhanced MR Lymphangiography and Intranodal Lymphangiography has led to a new, better understanding of the anatomical substrate and pathophysiological mechanisms of the diseases. Consequently, this has allowed the development of new targeted therapeutic interventions as well as prognostication for this population with lymphatic flow disorders. RECENT FINDINGS: The underlying causes of all NLD is an obstruction or altered flow of the central lymphatic flow. Two types of NLD have been described: isolated neonatal chylothorax and central lymphatic flow disorder (CLFD). Isolated neonatal chylothorax can be treated successfully with oil-based contrast (lipiodol) embolization. CLFD secondary to obstruction of the thoraco-venous junction can be successfully treated with surgical thoracic duct-venous anastomosis. CLFD caused by elevated central pressure and/or thoracic duct dysplasia can be treated medically, including with new systemic therapies such as mammalian target of rapamycin inhibitors. SUMMARY: New diagnostic and interventional tools have recently allowed for classification, prognostication, and targeted interventions for neonatal patients with lymphatic flow disorders. Further research will build on these discoveries.

Lymphatic leakage after pelvic lymphadenectomy for cervical cancer: a retrospective case-control study.

BACKGROUND: The study aims to evaluate the clinical features and management of postoperative lymphatic leakage (PLL) in patients with cervical cancer who received pelvic lymphadenectomy. METHODS: This retrospective study screened consecutive patients with cervical cancer (stage Ia2-IIb). RESULTS: Among 3427 cases screened, 63 patients (1.8%) were diagnosed with PLL, which manifested as persistent abdominal drainage (42/63, 66.7%), chylous ascites (12/63, 19.0%) or vaginal drainage (9/63, 14.3%). Median time from surgery to onset of PLL was 6 days (range, 4-21 days). All cases resolved in a median 10 days (range, 3-56 days) after conservative treatment; although one case experienced recurrence of vaginal drainage after 26 days, this also resolved after conservative therapy. Multivariate analysis showed that two cycles of neoadjuvant chemotherapy (odds ratio [OR], 3.283; 95% confidence interval [95%CI], 1.289-8.360; P = 0.013), a decrease in hemoglobin level of ≥20 and < 30 g/L (OR, 6.175; 95%CI, 1.033-10.919; P = 0.046) or ≥ 30 g/L (OR, 8.467; 95%CI, 1.248-17.426; P = 0.029), and postoperative albumin level ≥ 30 and < 35 g/L (OR, 2.552; 95%CI, 1.112-5.857; P = 0.027) or < 30 g/L (OR, 5.517; 95%CI, 2.047-18.148; P = 0.012) were associated with PLL. CONCLUSION: Neoadjuvant chemotherapy, postoperative anemia and postoperative hypoproteinemia are risk factors for PLL.

Combined internal and external negative pressure wound therapy: breakthrough treatment for lymphocutaneous intractable fistula.

PURPOSE: Lymphocutaneous fistula after lymph node dissection is intractable, yet there is no established treatment strategy. This study demonstrates the wound closure time achieved by a new method of combined internal and external negative pressure wound therapy (CIEN) in patients with lymphocutaneous fistula. METHODS: The subjects of this study were six consecutive patients with lymphocutaneous fistula after lymphatic surgery, who were treated with CIEN between 2018 and 2020. The CIEN technique can be summarized as follows: first, internal foam is inserted into the fistula from the opening of the fenestration. Next, a slightly larger area of external foam is applied above the fistula flap outside the external margin of the foam-filled fistula. After bridging the internal foam and external foam, negative-pressure wound therapy is carried out on this bridging foam block. RESULTS: CIEN led to rapid and complete wound healing in all six patients. Fistula flap margin ischemia developed in one patient, but adjusting the mode and pressure settings resulted in improvement. Three patients suffered contact dermatitis. There were no signs of tumor or fistula recurrence in any patients after at least 3 months of follow-up. CONCLUSION: CIEN is an effective and less invasive treatment modality than the conventional method of managing lymphocutaneous fistula.

Unusual Left Periclavicular Cutaneous Lymphatic Fistula After Port Explantation Without Lymph Vessel Injury: Imaging and Interventional Treatment.

Complex oncological treatment can be associated with lymphatic vascular injury that is burdened by considerable morbidity. Lymphatic imaging and interventional techniques offer new minimally invasive treatment options. We report the case of a 59-year-old woman with an unusual lympho-veno-cutaneous fistula, diagnosed by magnetic resonance lymphangiography and treated by minimally invasive embolization therapy and venous recanalization.

Lymphatic Interventional Treatment for Chyluria via Retrograde Thoracic Duct Access.

Chyluria is the leakage of intestinal lymph (chyle) into the urine. Novel lymphatic intervention techniques, such as interstitial lymphatic embolization, proved to be a useful treatment option for chyluria. However, one of the challenges of this approach is the difficulty in identifying connections between the lymphatic system and kidney collecting system. Here, embolization of the abnormal lymphatic connection through retrograde thoracic duct access in 3 chyluria patients is introduced.

Refractory Hepatic Lymphorrhea: Percutaneous Transhepatic Lymphangiography and Embolization with n-Butyl-2-Cyanoacrylate Glue.

Hepatic lymphorrhea is a leakage from the liver's lymphatic ducts into the abdominal cavity and an extremely rare complication associated with injury of the hepatoduodenal ligament, which can lead to refractory ascites. Hepatic lymphorrhea is constituted by non-chylous ascites and can be visualized by transhepatic lymphangiography instead of pedal or intranodal lymphangiography. To date, only a few successfully treated cases using interventional procedures have been reported. Although n-butyl-2-cyanoacrylate (NBCA) glue is widely used in various cases of vascular embolization and other lymphatic leak treatments, there have been no reports of its use for post-surgical hepatic lymphorrhea. The NBCA glue embolization described in this case report may be one of the treatment options to control the refractory ascites derived from hepatic lymphorrhea.

Research Priorities in Lymphatic Interventions: Recommendations from a Multidisciplinary Research Consensus Panel.

Recognizing the increasing importance of lymphatic interventions, the Society of Interventional Radiology Foundation brought together a multidisciplinary group of key opinion leaders in lymphatic medicine to define the priorities in lymphatic research. On February 21, 2020, SIRF convened a multidisciplinary Research Consensus Panel (RCP) of experts in the lymphatic field. During the meeting, the panel and audience discussed potential future research priorities. The panelists ranked the discussed research priorities based on clinical relevance, overall impact, and technical feasibility. The following research topics were prioritized by RCP: lymphatic decompression in patients with congestive heart failure, detoxification of thoracic duct lymph in acute illness, development of newer agents for lymphatic imaging, characterization of organ-based lymph composition, and development of lymphatic interventions to treat ascites in liver cirrhosis. The RCP priorities underscored that the lymphatic system plays an important role not only in the intrinsic lymphatic diseases but in conditions that traditionally are not considered to be lymphatic such as congestive heart failure, liver cirrhosis, and critical illness. The advancement of the research in these areas will lead the field of lymphatic interventions to the next level.