Search Results (46)

Background: Novel robotic surgical techniques have substantially improved the safety and outcomes of Ivor Lewis esophagectomy, offering greater precision, reduced surgical trauma, and more radical lymphadenectomy compared to conventional approaches. While perioperative results are increasingly promising, the adoption of robotic technology appears to be accompanied by an emerging set of procedure-specific complications not previously encountered—or encountered with different frequency—in open surgery. Understanding this evolving complication profile is essential to fully realize the oncological potential of robotic esophagectomy. Methods: This retrospective single-center study compared 407 consecutive patients undergoing Ivor Lewis esophagectomy at a high-volume center (OPE n = 163; HRB n = 75; FRB n = 169; 2012–2023) regarding three pathophysiologically motivated primary endpoints within 12 months: paraconduit herniation, chylothorax, and neo-esophagus–airway fistula. Results: One-year survival was 71.8%, 74.7%, and 82.2% (p = 0.073). Chylothorax was significantly more frequent in FRB (12.4%) than in OPE (2.5%) or HRB (2.7%) (p < 0.001), with surgical approach as sole independent predictor. Lymphangiography in three FRB patients demonstrated thoracic duct integrity in all; leakage originated from the celiac lymphadenectomy field ascending transhiatally—suggesting a distinct mechanism potentially linked to surgical radicality. All cases resolved conservatively. Neo-esophagus–airway fistula occured significantly less frequently with robotic approaches (FRB 0.6% vs. OPE 4.9%; p = 0.031). Paraconduit herniation did not differ significantly within 12 months (p = 0.272). Conclusions: The complication profile of robotic Ivor Lewis esophagectomy reflects its oncological ambition: elevated chylothorax rates may correlate with radical lymphadenectomy and represent an acceptable trade-off within a multimodal treatment strategy. Fistula risk is meaningfully reduced. These findings support robotic esophagectomy as a safe and effective approach in experienced centers. Full article

Introduction: Identification of the thoracic duct (TD) is essential during esophageal surgery to reduce the risk of complications such as chylothorax. The clinical significance of the white plane, or Morosow’s ligament—a consistent anatomical landmark along the esophagus—remains poorly defined. Methods: A total of 166 patients undergoing robot-assisted minimally invasive esophagectomy (RAMIE) were analyzed. Intraoperative visualization of the white plane was documented. Patient demographics, tumor characteristics, postoperative complications, management strategies, hospital length of stay, and overall survival were assessed. Complication severity was graded using the Clavien–Dindo classification. The Kaplan–Meier and multivariable Cox regression analyses were used to evaluate prognostic factors, including BMI, ASA score, pneumonia, pT status, pN status, neoadjuvant and adjuvant therapy, and white plane visualization. Results: The white plane was visualized in 154 patients (92.8%). Postoperative complications, management strategies, hospital length of stay, and 30-/90-day in-hospital mortality did not differ between groups with visualized and not visualized white planes. Median overall survival was significantly longer in patients with a visible white plane (43.1 vs. 13.1 months; p = 0.0079). The multivariable analysis identified ASA classification, pT stage, pN stage, and adjuvant therapy as independent predictors of overall survival, whereas lymph node stage and adjuvant therapy were independent predictors of recurrence-free survival. Conclusions: The white plane is a distinct intraoperative anatomical structure that can be visualized in most RAMIE procedures. Its identification may assist in TD recognition and provides a framework for describing mediastinal anatomy, but further studies are needed to determine its impact on surgical standardization and patient outcomes. Full article

In human medicine, the 2025 International Society for the Study of Vascular Anomalies (ISSVA) classification distinguishes localized lymphatic malformations from systemic disorders referred to as complex lymphatic anomalies (CLAs), including generalized lymphatic anomaly (GLA) and central conducting lymphatic anomaly (CCLA). GLA is characterized by multifocal proliferation of dilated lymphatic channels infiltrating soft tissues and visceral organs, whereas CCLA involves structural or functional abnormalities of the central lymphatic pathways such as the thoracic duct commonly resulting in chylous effusion. Reports of systemic lymphatic disease in veterinary species are exceedingly rare. A five-month-old intact male domestic shorthair cat presented with progressive dyspnea and was diagnosed with chylothorax and chylous ascites. Computed tomography (CT) revealed contrast leakage from both the thoracic duct and aortic hiatus, along with diffuse subcutaneous edema, indicating a systemic lymphatic disorder. Histopathology demonstrated proliferative lymphatic channels within the subcutaneous tissues, consistent with GLA, while imaging findings suggested concurrent CCLA, supporting classification as an overlapping CLA phenotype. Despite medical and surgical interventions, the cat developed progressive edema and recurrent pleural effusion and ultimately died. This case demonstrates that cats can develop systemic lymphatic disorders analogous to human CLAs and highlights the diagnostic value of CT lymphangiography. Furthermore, it underscores the utility of applying the ISSVA classification to enhance comparative understanding of lymphatic diseases in veterinary medicine. Full article

Precise intraoperative identification of the canine thoracic duct remains challenging due to anatomical variability and limited visualization. This exploratory cadaveric feasibility study aimed to describe the technical applicability of fluorescence-guided thoracic duct mapping using video-assisted thoracoscopy (VATS) and robot-assisted thoracoscopy (Versius™ system). Four adult Beagle cadavers underwent bilateral thoracoscopic exploration after intranodal injection of indocyanine green (ICG, Verdye^®, 0.05 mg/kg; 0.5 mL). Near-infrared (NIR) fluorescence imaging enabled real-time visualization of the thoracic duct and its branches. Fluorescence quality was quantitatively characterized using signal-to-noise ratio (SNR), contrast-to-noise ratio (CNR), and contrast resolution (CR) calculated from standardized image frames. Both approaches achieved successful duct identification in all cadavers. VATS provided brighter overall fluorescence, whereas the robotic-assisted approach offered stable imaging, enhanced instrument dexterity, and improved duct-to-background discrimination. These findings confirm the feasibility of fluorescence-guided thoracic duct identification using both minimally invasive modalities in canine cadavers. The standardized assessment of optical parameters proposed here may support future in vivo studies to optimize imaging protocols and evaluate the clinical impact of fluorescence-guided thoracic duct surgery in dogs. Full article

The expanded newborn screening (NBS) program in the Russian Federation, launched in 2023, includes the detection of severe forms of T- and B-cell immunodeficiencies via TREC/KREC quantification. We report a rare case of a male infant having multiple congenital anomalies and lymphopenia identified through this program. Genetic testing revealed a 25.8 Mb terminal deletion spanning 13q31.2–qter, consistent with 13q deletion syndrome. Initial NBS revealed reduced TREC levels, prompting further evaluation. The patient exhibited a complex phenotype, including central nervous system malformation (alobar holoprosencephaly), severe congenital heart disease, renal hypoplasia, limb and genitourinary anomalies, and facial dysmorphism. Postnatal complications included pneumonia, pleuritis, and chylothorax. Flow cytometry demonstrated mild T- and B-cell lymphopenia. The genomic defect was characterized using long-read third-generation sequencing, enabling precise breakpoint identification and accurate mapping of deleted genes. The deletion was confirmed via subtelomeric FISH analysis. The patient died at 7 months of age due to the progression of underlying congenital anomalies and associated complications. Our findings broaden the clinical characterization of distal 13q deletion syndrome and demonstrate the value of long-read sequencing in structural chromosomal analysis. They further highlight the difficulties of caring for neonates having complex malformations and immune dysfunction. Given the potential for both primary and secondary immune disturbances, comprehensive immunological evaluation should be considered in patients having 13q deletion syndrome to improve diagnostic accuracy and inform appropriate clinical management. Full article

Recent diagnostic advances reveal that lymphatic disease in Noonan syndrome (NS) and other NS-like RASopathies often stems from central conducting lymphatic anomalies (CCLAs). The RAS/MAPK-ERK pathway plays a central role in lymphangiogenesis. Targeting this pathway with MEK-inhibitor trametinib has emerged as a promising therapeutic strategy for managing CCLAs in patients with NS-like RASopathies. This case series assessed the clinical outcomes of trametinib therapy in eight patients with NS-like RASopathies and CCLA, each offering unique insights into the therapeutic efficacy of MEK inhibition. In infants, a lower dose of 0.01 mg/kg/day and earlier discontinuation of trametinib therapy effectively alleviated the symptoms of congenital chylothorax and rescued the lymphatic phenotype, compared to similar published cases. Moreover, four patients aged >11 y showed a slower response and did not achieve complete symptomatic recovery. In conclusion, it is advised to consider trametinib therapy for patients with severe, therapy-refractory CCLA in patients with NS-like RASopathies. However, individual responses to trametinib therapy may vary, with some patients demonstrating more favorable outcomes than others. Further investigation into potential enhancers and suppressors of the lymphatic phenotype is necessary for more accurate treatment predictions. While these factors are likely genetic, we cannot rule out other intrinsic or physiological factors. Full article

Background: The aim of this study was to describe our centre experience in the use of pulmonary vasodilator therapy in Fontan patients. Methods: We retrospectively enrolled patients that underwent Fontan operation between 2000 and 2024, reporting demographic and operative data and noting complications and the use of pulmonary vasodilators. Results: A total of 117 patients were followed for a median time of 150 months (90–207). In total, 36.7% were female, and the median age during the intervention was 50 months (37–64), and 53% had a single left ventricle physiology. In 20 of these 117 patients (17.1%), at least one pulmonary vasodilator drug was used during their life for the following reasons: 6 elevated pressures in the circuit, 3 low oxygen saturation, 2 plastic bronchitis, 2 pleural effusion, 1 chylothorax, 1 persistent pericardial effusion, 1 haemoptysis, 1 protein losing enteropathy, 1 poor exercise tolerance, 1 pulmonary arterial hypertension present since birth and 1 diastolic dysfunction. They had a significantly higher prevalence of single right ventricle physiology (65% vs. 37%, p = 0.03), pulmonary hypertension (60% vs. 0, p = 0.0001), plastic bronchitis (10% vs. 0, p = 0.03) and declivous oedema in the follow-up period (10% vs. 0, p = 0.03), with a higher assumption of warfarin (35% vs. 6.2%, p = 0.001). Conclusions: We found that in the absence of a standardise protocol, we usually use pulmonary vasodilator therapy in Fontan patients, as it is guided by clinical aspects and hemodynamic conditions, which lead us to start and stop this therapy. Full article

The central conducting lymphatics (CCL) and mesenteric lymphatic systems are responsible for lipid absorption, fluid regulation, and protein delivery into the bloodstream. Disruptions in these systems can result in debilitating conditions such as chylothorax, plastic bronchitis, post-operative lymphocele, protein-losing enteropathy (PLE), and chylous ascites. Advances in imaging techniques, including magnetic resonance lymphangiography (MRL), computed tomography lymphangiography (CTL), and fluoroscopic lymphangiography, allow for detailed anatomic and functional evaluation of the lymphatic system, facilitating accurate diagnosis and intervention by interventional radiologists. This review explores the embryology, anatomy, and pathophysiology of the lymphatic system and discusses imaging modalities and interventional techniques employed to manage disorders of the conducting lymphatics in the chest and abdomen. Thoracic duct embolization (TDE), percutaneous transhepatic lymphatic embolization (PTLE), and sclerotherapy are highlighted as effective, minimally invasive approaches to treat lymphatic leaks and obstructions and have shown high success rates in reducing symptoms and improving patient outcomes, particularly when medical management fails. This review seeks to demonstrate how anatomical imaging can facilitate minimally invasive procedures to rectify disorders of lymphatic flow. Full article

Purpose: To investigate the impact of paratracheal lymphadenectomy on survival in patients undergoing an esophagectomy for cancer. The secondary objective was to assess the effect on short-term outcomes. Methods: Between 2011–2017, patients with an esophageal or gastroesophageal junction carcinoma treated with elective transthoracic esophagectomy with two-field lymphadenectomy were included from the Dutch Upper Gastro-intestinal Cancer Audit registry. After 1:1 propensity score matching of patients with and without paratracheal lymphadenectomy within histologic subgroups, short-term outcomes and overall survival were compared between the two groups. Results: A total of 1154 patients with adenocarcinoma and 294 patients with squamous cell carcinoma were matched. Lymph node yield was significantly higher (22 versus 19 nodes, p < 0.001) in patients with paratracheal lymphadenectomy for both tumor types. Paratracheal lymphadenectomy was associated with more recurrent laryngeal nerve injury (10% versus 5%, p = 0.002) and chylothorax in patients with adenocarcinoma (10% versus 5%, p = 0.010) and with more anastomotic leakage in patients with squamous cell carcinoma (42% versus 27%, p = 0.014). The 3- and 5-year survival in patients with and without a paratracheal lymphadenectomy were for adenocarcinoma, respectively, 58% versus 56% and 48% in both groups (log rank: p = 0.578) and for patients with a squamous cell carcinoma, 62% in both groups and 57% versus 54% (log rank: p = 0.668). Conclusions: The addition of paratracheal lymphadenectomy significantly increases lymph node yield in transthoracic esophagectomy but did not result in improved survival for esophageal cancer patients in the current dataset. However, there was an increase in postoperative morbidity in patients who underwent a paratracheal lymphadenectomy. Full article

Background: Esophageal cancer, the seventh most common malignancy globally, requires esophagectomy for curative treatment. However, esophagectomy is associated with high postoperative morbidity and mortality, highlighting the need for minimally invasive approaches. Robotic-assisted surgery has emerged as a promising alternative to traditional open and minimally invasive esophagectomy (MIE), offering potential benefits in improving clinical and oncological outcomes. This review aims to assess the postoperative morbidity and outcomes of robotic surgery. Methods: A comprehensive review of the current literature was conducted, focusing on studies evaluating the role of robotic-assisted surgery in esophagectomy. Data were synthesized on the clinical outcomes, including postoperative complications, survival rates, and recovery time, as well as technological advancements in robotic surgery platforms. Studies comparing robotic-assisted esophagectomy with traditional approaches were analyzed to determine the potential advantages of robotic systems in improving surgical precision and patient outcomes. Results: Robotic-assisted esophagectomy (RAMIE) has shown significant improvements in clinical outcomes compared to open surgery and MIE, including reduced postoperative pain, less blood loss, and faster recovery. RAMIE offers enhanced thoracic access, with fewer complications than thoracotomy. The RACE technique has improved patient recovery and reduced morbidity. Fluorescence-guided technologies, including near-infrared fluorescence (NIRF), have proven valuable for sentinel node biopsy, lymphatic mapping, and angiography, helping identify critical structures and minimizing complications like anastomotic leakage and chylothorax. Despite these benefits, challenges such as the high cost of robotic systems and limited long-term data hinder broader adoption. Hybrid approaches, combining robotic and open techniques, remain common in clinical practice. Conclusions: Robotic-assisted esophagectomy offers promising advantages, including enhanced precision, reduced complications, and faster recovery, but challenges related to cost, accessibility, and evidence gaps must be addressed. The hybrid approach remains a valuable option in select clinical scenarios. Continued research, including large-scale randomized controlled trials, is necessary to further establish the role of robotic surgery as the standard treatment for resectable esophageal cancer. Full article

Background and Clinical Significance: The use of point-of-care ultrasound (POCUS) in emergency departments is rapidly growing due to its ability to provide immediate and accurate diagnostic information at the bedside. Furthermore, it can provide precise and rapid information on the location of multidistrict effusions in patients with suspected lymphatic decompensation. Case Presentation: This unique clinical case report describes a patient who presented with massive, multidistrict chylous effusion secondary to acute lymphatic insufficiency, a rare and challenging condition. Due to a recent diagnosis of celiac disease, the patient had started a gluten-free diet ten days before the onset of symptoms, suggesting a possible causal link. Through comprehensive thoracoabdominal POCUS, the diagnosis was made promptly, avoiding delays in treatment and enabling timely decision-making. Conclusions: This case emphasizes the critical role of POCUS not only in expediting diagnosis but also in guiding invasive procedures, such as thoracentesis, by visualizing fluid accumulation and anatomical structures in real-time. Moreover, POCUS provides an invaluable tool for ongoing clinical ultrasound follow-up, facilitating continuous monitoring without exposing the patient to the risks of radiation, thus optimizing patient care and resource utilization. Full article

Chylothorax is a rare complication occurring after cardio-thoracic surgical procedures. This condition presents challenges for diagnosis and treatment. Operative ductal ligation is the method of choice for relapsing or refractory cases, and it can be performed through the aid of IGC injection for the identification of chylous leakage. Our report presents the use of ICG fluorescence during VATS to successfully identify and treat a left-sided post-surgical chylothorax. The patient underwent a pulmonary wedge resection for a suspect malignant lesion and developed chylous leakage in the early postoperative period. On postoperative day 7, the patient underwent a revision thoracoscopy for hemostasis and thoracic duct ligation. ICG injections were performed through bilateral inguinal lymph nodes and approximately 15 min after we performed the re-thoracoscopy with effective identification and ligation of the chyle leakage. ICG fluorescence-guided VATS is a valuable and effective method for managing postoperative chylothorax, especially for left-sided leaks. Full article

Lymphatic complications are becoming increasingly identified in cancer patients. Chylous ascites, chylothorax, lymphoceles, and lymphorrhea are common in cancer patients and can occur due to traumatic injury during surgeries or infiltrative effects of the tumors themselves. Recently, some anti-neoplastic medications are also thought to result in lymphatic complications. Management options range from conservative options to minimally invasive interventions, to surgical interventions with no standardized management strategy. Imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography and intranodal computed tomography or fluoroscopic lymphangiography are becoming more valuable in diagnosis and treatment planning. Minimally invasive interventions are rapidly evolving and have become the first-line intervention in most cases. Current research, however, faces limitations due to study design and variability. Standardized reporting and prospective studies are needed to advance the field. This review summarizes some of the latest literature on lymphatic interventions in cancer patients and provides reporting recommendations for future studies on lymphatic interventions. Full article

Objective: The occurrence of chylous effusion in children undergoing cardiac surgery is progressively increasing due to technical advances that have led to a rise in the number of surgeries. In this context, the objective was to describe the clinical profile of a cohort of patients at the time of chylous effusion diagnosis. Methods: A retrospective cohort analysis was conducted between January 2011 and July 2023, involving 23 patients, aged 0 to 18 years, treated at a quaternary university hospital in southeastern Brazil. Data were obtained from the follow-up records of the Multidisciplinary Nutritional Therapy Team (MNTT) for patients who received nutritional support after developing chylous effusion in the postoperative period of cardiac surgery. Results: The younger population predominated (median age of 6 months), with a high prevalence of malnutrition (60.9%). At the time of chylous effusion diagnosis, 83% had lymphopenia, and 74% had hypoalbuminemia. The longer the time elapsed after surgery for the onset of chylous effusion, the lower the HDL cholesterol, the lower the albumin levels, the greater the surgical complexity, the younger the patient, and the lower their weight. Hypocalcemia occurred in nearly half of the sample and hypophosphatemia in 26% of the analyzed cases. Conclusions: There was a notable presence of lymphopenia, hypoalbuminemia, and low HDL cholesterol, as well as a high incidence of mineral imbalances, particularly hypocalcemia and hypophosphatemia, which, if untreated, may lead to unfavorable outcomes. Therefore, clinical and laboratory monitoring of children in the postoperative period of cardiac surgery is important and can aid in the early diagnosis of chylous effusion and, consequently, in the timely initiation of treatment. Full article

RASopathies are a group of genetic syndromes caused by germline mutations in genes involved in the RAS/Mitogen-Activated Protein Kinase signaling pathway, which regulates cellular proliferation, differentiation, and angiogenesis. Despite their involvement at different levels of this pathway, RASopathies share overlapping clinical phenotypes. Noonan syndrome is the most prevalent RASopathy, with an estimated incidence of 1 in 2500 live births, and it is typically inherited in an autosomal dominant manner, with 50% of cases involving gain-of-function mutations in the PTPN11 gene. De novo mutations are common, accounting for 60% of cases. The phenotype of Noonan syndrome includes characteristic facial and physical features, congenital cardiac defects, lymphatic and cerebrovascular anomalies, renal malformations, hematological abnormalities, developmental issues, and an increased risk of cancer. Severe congenital cardiac defects and lymphatic abnormalities significantly impact prognosis, contributing to increased morbidity and mortality. Recent therapeutic advancements have introduced trametinib, an MEK1/2 inhibitor, for treating Noonan syndrome patients with severe cardiac and lymphatic complications. To assess its efficacy, here, we present a case of a newborn with Noonan syndrome who exhibited refractory chylothorax, ventricular hypertrophy, and pulmonary stenosis who was treated with trametinib. The patient demonstrated significant improvement in chylothorax and left ventricular hypertrophy, though pulmonary stenosis persisted. This case further confirms trametinib’s potential as a therapeutic option for severe Noonan syndrome complications, emphasizing the need for further clinical trials to optimize treatment protocols and evaluate long-term outcomes. Full article