[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.

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.

Lymphatic Interventions in Congenital Heart Disease.

Lymphatic disorders in congenital heart disease can be broadly classified into chest compartment, abdominal compartment, or multicompartment disorders. Heavily T2-weighted noninvasive lymphatic imaging (for anatomy) and invasive dynamic contrast magnetic resonance lymphangiography (for flow) have become the main diagnostic modalities of choice to identify the cause of lymphatic disorders. Selective lymphatic duct embolization (SLDE) has largely replaced total thoracic duct embolization as the main lymphatic therapeutic procedure. Recurrence of symptoms needing repeat interventions is more common in patients who underwent SLDE. Novel surgical and transcatheter thoracic duct decompression strategies are promising, but long-term follow-up is critical and eagerly awaited.

State-of-the-art imaging for children with central lymphatic disorders.

Lymphatic imaging plays a crucial role in novel lymphatic interventions, offering valuable insights into central lymphatic drainage. Lymphatic system abnormalities may appear in various pediatric disorders, and accurate imaging is crucial for effective diagnosis and tailored therapeutic interventions. Traditional imaging modalities have offered valuable insights, but the demand for non-invasive, high-resolution techniques has fueled the development of innovative lymphatic imaging methods. In this review, we explore the state of the art in lymphatic imaging specifically within the context of pediatric surgery.

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.

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.

Anesthesia-induced Lymphatic Dysfunction.

General anesthetics adversely alters the distribution of infused fluid between the plasma compartment and the extravascular space. This maldistribution occurs largely from the effects of anesthetic agents on lymphatic pumping, which can be demonstrated by macroscopic fluid kinetics studies in awake versus anesthetized patients. The magnitude of this effect can be appreciated as follows: a 30% reduction in lymph flow may result in a fivefold increase of fluid-induced volume expansion of the interstitial space relative to plasma volume. Anesthesia-induced lymphatic dysfunction is a key factor why anesthetized patients require greater than expected fluid administration than can be accounted for by blood loss, urine output, and insensible losses. Anesthesia also blunts the transvascular refill response to bleeding, an important compensatory mechanism during hemorrhagic hypovolemia, in part through lymphatic inhibition. Last, this study addresses how catecholamines and hypertonic and hyperoncotic fluids may mobilize interstitial fluid to mitigate anesthesia-induced lymphatic dysfunction.

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.

Bilateral axillary web syndrome in a patient with primary lymphoedema of upper limbs and non-Hodgkin lymphoma.

Primary lymphoedema, axillary web syndrome (AWS) and yellow nail syndrome may be related. Mr B is a 66-year-old gentleman with genital lymphoedema and lymphoedema of all four extremities. In 2023, he was diagnosed with non-Hodgkin lymphoma and also underwent cardiac surgery. In November 2023, he completed an inpatient rehabilitation at the Földi clinic in Germany, where he received intensive treatment for his lymphoedema and was also diagnosed with bilateral AWS. The presence of AWS in a patient with primary lymphoedema and no history of axillary surgery is unique. Although AWS typically presents after axillary surgery, this case highlights that it can also occur in patients without lymph node surgery. While the precise cause of this presentation of AWS is not known, it may be connected to yellow nail syndrome or potentially the recent chemotherapy treatment. This article will describe the clinical case, highlighting the need for further research on AWS present in primary lymphoedema.

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.

The use of superficial circumflex iliac perforator (SCIP) pedicle vein for lymphovenous anastomosis to treat inguinal lymphatic fistula: A case report.

The management of lymphatic fistulas following surgical procedures, in particular after inguinal lymphadenectomy, represents a significant clinical challenge. The current case report shows the novel use of the superficial circumflex iliac perforator (SCIP) pedicle vein for lymphovenous anastomosis (LVA) to treat a chronic inguinal lymphatic fistula in a 58-year-old male patient. This patient had developed a persistent lymphorrhea and wound dehiscence after a right inguinal lymph node biopsy performed for oncological reasons 1.5 months before. Pre-operative assessment with indocyanine green (ICG) lymphography confirmed a substantial lymphatic contribution to the wound discharge, thus guiding the surgical strategy. During the procedure, a pedicled tissue segment containing the SCIV was dissected and utilized to fill the wound's dead space and facilitate LVA with the leaking lymphatic vessel. Notably, a coupler device was employed for the anastomosis due to the large caliber of the lymphatic vessel involved, a technique not commonly reported in lymphatic surgeries. The result of the procedure was successful, with intra-operative ICG imaging confirming the patency of the anastomosis. After surgery the wound healed without complications. This case illustrates the potential of SCIV employment in lymphatic fistula repair in the inguinal region. While further research is needed to validate these findings, this report provides an unconventional approach to a relatively common problem in clinical practice.

Genetic causes of lymphatic disorders: recent updates on the clinical and molecular aspects of lymphatic disease.

PURPOSE OF REVIEW: The lymphatic system facilitates several key functions that limit significant morbidity and mortality. Despite the impact and burden of lymphatic disorders, there are many remaining disorders whose genetic substrate remains unknown. The purpose of this review is to provide an update on the genetic causes of lymphatic disorders, while reporting on newly proposed clinical classifications of lymphatic disease. RECENT FINDINGS: We reviewed several new mutations in genes that have been identified as potential causes of lymphatic disorders including: MDFIC, EPHB 4 , and ANGPT2. Furthermore, the traditional St. George's Classification system for primary lymphatic anomalies has been updated to reflect the use of genetic testing, both as a tool for the clinical identification of lymphatic disease and as a method through which new sub-classifications of lymphatic disorders have been established within this framework. Finally, we highlighted recent clinical studies that have explored the impact of therapies such as sirolimus, ketoprofen, and acebilustat on lymphatic disorders. SUMMARY: Despite a growing body of evidence, current literature demonstrates a persistent gap in the number of known genes responsible for lymphatic disease entities. Recent clinical classification tools have been introduced in order to integrate traditional symptom- and time-based diagnostic approaches with modern genetic classifications, as highlighted in the updated St. George's classification system. With the introduction of this novel approach, clinicians may be better equipped to recognize established disease and, potentially, to identify novel causal mutations. Further research is needed to identify additional genetic causes of disease and to optimize current clinical tools for diagnosis and treatment.

Diagnostic dilemma in maxillofacial pathologies: a case series.

BACKGROUND: Head and neck are a site of numerous pathologies with different aetiologies and presentations. Rare pathologies, although infrequent still do exist. Diagnostic dilemma in maxillofacial pathologies can be the most challenging situation. Dealing with uncertainty, although difficult, is a reality in surgical practice. Being thorough, attentive to details and clues, and maintaining an open mind are critical strategies in the approach to such a patient. CASE PRESENTATION: In our paper we are reporting a spectrum of 4 unusual variants of head and neck pathologies, whose age and sex were 52 years/ M, 37 years/F, 41 years/ F, 30 years/F respectively. All the patients were of Indian origin. The diagnosis ranged from autoimmune diseases to lymphatic cancer which posed a unique challenge both in the terms of diagnosis and management. CONCLUSION: A thorough systematic evaluation along with a multidisciplinary approach is mandatory in the diagnosis of unusual head and neck pathologies.

MR Lymphangiography in Lymphatic Disorders: Clinical Applications, Institutional Experience, and Practice Development.

Lymphatic flow and anatomy can be challenging to study, owing to variable lymphatic anatomy in patients with diverse primary or secondary lymphatic pathologic conditions and the fact that lymphatic imaging is rarely performed in healthy individuals. The primary components of the lymphatic system outside the head and neck are the peripheral, retroperitoneal, mesenteric, hepatic, and pulmonary lymphatic systems and the thoracic duct. Multiple techniques have been developed for imaging components of the lymphatic system over the past century, with trade-offs in spatial, temporal, and contrast resolution; invasiveness; exposure to ionizing radiation; and the ability to obtain information on dynamic lymphatic flow. More recently, dynamic contrast-enhanced (DCE) MR lymphangiography (MRL) has emerged as a valuable tool for imaging both lymphatic flow and anatomy in a variety of congenital and acquired primary or secondary lymphatic disorders. The authors provide a brief overview of lymphatic physiology, anatomy, and imaging techniques. Next, an overview of DCE MRL and the development of an MRL practice and workflow in a hybrid interventional MRI suite incorporating cart-based in-room US is provided, with an emphasis on multidisciplinary collaboration. The spectrum of congenital and acquired lymphatic disorders encountered early in an MRL practice is provided, with emphasis on the diversity of imaging findings and how DCE MRL can aid in diagnosis and treatment of these patients. Methods such as DCE MRL for assessing the hepatic and mesenteric lymphatic systems and emerging technologies that may further expand DCE MRL use such as three-dimensional printing are introduced. ©RSNA, 2024 Test Your Knowledge questions for this article are available in the supplemental material.

Comparing easywrap with six other adjustable wraps for compression therapy in venous and lymphatic disease by variation in pressure, stiffness and elasticity.

BACKGROUND: There is no clear guidance or comprehensive comparative evidence to help clinicians select between adjustable wraps for compression therapy. However, adjustable wraps could be compared with three metrics used for compression bandages: variation in pressure, stiffness and elasticity. AIMS: To determine whether easywrap (Haddenham Healthcare) had lower standard deviation (SD) in lying pressure, greater static stiffness index (SSI) and/or a greater mean elongation ratio than six other adjustable wraps. METHODS: In this preliminary exploratory study, seven adjustable wraps were tested in vivo 20 times each (140 tests in total), on 20 people (with a variable number of readings per participant), to give the SD in lying pressure and mean SSI. The same wraps were tested in vitro five times each to give the mean elongation ratio. FINDINGS: Of all seven adjustable wraps, easywrap had the second lowest SD in lying pressure at 5.9 mmHg, compared with 8.9 mmHg overall; the highest mean SSI at 13.2 mmHg, compared with 9.5 mmHg overall; and the highest mean elongation ratio at 137.0%, compared with 107.1% overall. CONCLUSIONS: These metrics suggest that easywrap has clinical advantages over other adjustable wraps. A lower variation in lying pressure suggests more consistent application of pressures within intended therapeutic levels. Higher SSI indicates greater resistance to stretching, which is linked to improved venous function and oedema reduction. Higher elongation ratio demonstrates greater tensile strength, which should maintain therapeutic pressure for longer without readjustment.