Single-Center Experience With Dynamic Contrast-Enhanced Magnetic Resonance Lymphangiography for Diagnosing Lymphatic Disorders and Guiding Percutaneous Embolization.

BACKGROUND: The pragmatic role of dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) needs to be evaluated and compared across distinct lymphatic disorders. We aimed to evaluate the performance of DCMRL for identifying the underlying causes of lymphatic disorders and to define the potential benefit of DCMRL for planning lymphatic interventions. METHODS: Patients who underwent DCMRL between August 2017 and July 2022 were included in this retrospective analysis. DCMRL was performed with intranodal injection of a gadolinium-based contrast medium through inguinal lymph nodes under local anesthesia. Technical success of DCMRL and feasibility of percutaneous embolization were assessed based on the lymphatic anatomy visualized by DCMRL. Based on the underlying causes, clinical outcomes were evaluated and compared. RESULTS: Seventy consecutive patients were included. The indications were traumatic chylothorax (n = 42), traumatic chylous ascites (n = 11), and nontraumatic lymphatic leak (n = 17). The technical success rate of DCMRL was the highest in association with nontraumatic lymphatic disorders (94.1% [16/17]), followed by traumatic chylothorax (92.9% [39/42]) and traumatic chylous ascites (81.8% [9/11]). Thirty-one (47.7%) patients among 65 patients who underwent technically successful DCMRL had feasible anatomy for intervention. Clinical success was achieved in 90.3% (28/31) of patients with feasible anatomy for radiologic intervention, while 62.5% (10/16) of patients with anatomical challenges showed improvement. Most patients with traumatic chylothorax showed improvement (92.9% [39/42]), whereas only 23.5% (4/17) of patients with nontraumatic lymphatic disorders showed clinical improvement. CONCLUSION: DCMRL can help identify the underlying causes of lymphatic disorders. The performance of DCMRL and clinical outcomes vary based on the underlying cause. The feasibility of lymphatic intervention can be determined using DCMRL, which can help in predicting clinical outcomes.

Management of Chyle Leak in Pediatric Surgical Oncology: A Systematic Review.

INTRODUCTION: Chyle leak, a rare complication, arises from damage to primary lymphatic vessels due to congenital factors or medical interventions, leading to conditions such as chylothorax and chylous ascites. Managing chyle leaks is challenging, especially in pediatric surgical oncology, often arising as postoperative complications. Treatment options range from conservative dietary adjustments to surgical interventions, depending on leak severity and patient condition. This systematic review examines the management of chyle leaks in pediatric surgical oncology, emphasizing both conservative and surgical approaches. METHODS: This systematic review involved extensive database searches (EMBASE, Web of Science, and PubMed) to identify relevant studies on chyle leak management in the pediatric population. The review included studies from 1982 to 2023 and focused on pediatric and adolescent patients, assessing various treatment approaches and outcomes. Nine articles composed of 163 patients (study population size ranging from 2 to 82 patients). Independent reviewers evaluated the selected studies for inclusion. RESULTS: Among 9 articles analyzed, 98.8% of pediatric patients initially received conservative management for chyle leaks, with 11.7% eventually requiring surgical intervention due to persistent leaks (8, 10, and 16 to 22). Neuroblastoma resection is associated with 20% to 40% rate of chyle leak, and the extent of lymphadenectomy has been identified as a risk factor for chyle leak. The study highlighted variability in clinical success rates based on conservative management approaches. DISCUSSION: Chyle leak, while rare, presents a complex challenge, especially in pediatric surgical oncology. Various causes and treatment options exist, with a preference for conservative management initially and surgical intervention in specific circumstances. Factors such as leak severity and patient condition guide the choice between approaches. However, the scarcity of comparative data and randomized trials in the pediatric population necessitates further research to establish optimal management strategies for chyle leaks. CONCLUSIONS: Conservative management of chyle leaks has proven to be the preferred approach in early stages of treatment, whereas surgical management could be the preferred choice in certain situations. Larger prospective studies are needed to further evaluate these results.

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.

Indocyanine green fluorescence imaging technology for the treatment of chylothorax after oesophageal cancer: A case report.

Chylothorax is a serious postoperative complication of oesophageal cancer, and to date, there is no standardized and effective intraoperative diagnostic tool that can be used to identify the thoracic duct and determine the location of lymphatic fistulas. A 50-year-old patient with oesophageal squamous cell carcinoma developed chylothorax after thoracolaparoscopy combined with radical resection of oesophageal cancer. Twelve hours after surgery, 1200 mL of clear fluid was drained from the thoracic drainage tube, and a chyle test was sent. A thoracothoracic duct ligation procedure was performed on the first day after surgery. Although fluid accumulating in the posterior mediastinum was observed, the location of the lymphatic fistula could not be determined. During the surgery, indocyanine green (ICG) was injected into the bilateral inguinal lymph nodes, and a fluorescent lens was used to determine the location of the lymphatic fistula so the surgeon could ligate the thoracic duct. ICG fluorescence imaging technology can help surgeons effectively manage chylothorax after oesophageal cancer surgery. To our knowledge, this is the first report to describe the use of ICG fluorescence imaging technology to treat postoperative chylothorax in patients with oesophageal cancer in China.

A clinical consensus guideline for nutrition in infants with congenital diaphragmatic hernia from birth through discharge.

OBJECTIVE: To develop a consensus guideline to meet nutritional challenges faced by infants with congenital diaphragmatic hernia (CDH). STUDY DESIGN: The CDH Focus Group utilized a modified Delphi method to develop these clinical consensus guidelines (CCG). Topic leaders drafted recommendations after literature review and group discussion. Each recommendation was sent to focus group members via a REDCap survey tool, and members scored on a Likert scale of 0-100. A score of > 85 with no more than 25% outliers was designated a priori as demonstrating consensus among the group. RESULTS: In the first survey 24/25 recommendations received a median score > 90 and after discussion and second round of surveys all 25 recommendations received a median score of 100. CONCLUSIONS: We present a consensus evidence-based framework for managing parenteral and enteral nutrition, somatic growth, gastroesophageal reflux disease, chylothorax, and long-term follow-up of infants with CDH.

Efficacy of nil per oral, total parenteral nutrition, milrinone and non-suction chest tube drainage-based management for chylothorax following pediatric cardiac surgery.

OBJECTIVE: A single centre experience with chylothorax in post cardiac surgical patients. METHODS: Retrospective review. RESULTS: Chylothorax developed in 55 out of 873 operated patients (6.3%). Median age of the chylothorax cohort was 95 days (range 1-995). Neonates constituted 36% and 49% were infants. Group-1(35 patients-treated during the years 2011-2015) included those who were managed with low fat diet initially with other standard measures including steroid, octreotide, pleurodesis, lymphangiogram or thoracic duct ligation whenever required.Group-2 (20 patients, treated between year 2016-2018) were managed with nil per oral, total parenteral nutrition, extended use of milrinone and no use of chest tube suction with other above standard measures when required.Group-1 and group-2 were comparable in terms of their age and weight (p > 0.05).We observed lower volume of chest drainage, shorter intubation time, length of intensive care stay and hospital stay in group-2 compared to group-1 though they were statistically not significant (p > 0.05). Occurrence of massive chylothorax (>20 ml/kg/day) in group-1 was significantly higher [18 patients (51%) in group-1 vs 4 patients in group-2 (20%) (Chi-square 5.25, p = 0.02)]. In hospital mortality in group-1 was higher compared to group-2 (5/35 = 14.5% vs 1/20 = 5%), however, it was statistically not significant [risk ratio 2.86; 95% CI 0.36, 22.77; p = 0.59)]. Acute kidney injury was observed in about 25% of patients who had chylothorax. A higher mortality was observed in patients with chylothorax who had acute kidney injury [5/14 (35%)] compared to those who did not have acute kidney injury [1/41 (2.4%)] (Chi-square 11.89, p = 0.001)]. SUMMARY: In a heterogenous cohort of post-cardiac surgical patients who developed chylothorax, our suggested new regime (nil per oral, parenteral nutrition, extended use of milrinone and no suction applied to the chest drains) contributed to reduce the frequency of massive chylothorax occurrence significantly.

Indocyanine green lymphography in the congenital chylothorax and chylous ascites.

BACKGROUND: The prognosis of congenital chylothorax and ascites ranges from spontaneous resolution to death, but no established examination exists to predict the prognosis. We aimed to develop a clinically useful method to evaluate lymphatic abnormalities using indocyanine green (ICG) lymphography in infants with congenital chylothorax and ascites. METHODS: We retrospectively evaluated infants with congenital chylothorax and chylous ascites who underwent ICG lymphography in our hospital between 2012 and 2022. The ICG lymphography findings was evaluated. We defined the dermal backflow in the trunk as the lymphatic flow from the end of the limb back through the lymphatic vessels on the surface of the trunk. The association between the dermal backflow in the trunk and clinical outcomes, as follows, are investigated: the duration of the drainage period, the duration of endotracheal intubation, and the length of hospital stay. RESULTS: Twenty infants had a dermal backflow in the trunk, and ten did not. Clinical outcomes in infants with and without dermal backflow in the trunk were as follows (median): the duration of the drainage period (20 vs. 0 days, p = 0.001), the duration of endotracheal intubation (12 vs. 2 days, p = 0.04), and the length of hospital stay (62 vs. 41 days, p = 0.04), respectively. In multivariate linear regression analysis adjusted for gestational age, the duration of the drainage period was correlated with the dermal backflow in the trunk [exp(B) = 2.62; p = 0.003]. CONCLUSIONS: The dermal backflow in the trunk in ICG lymphography was useful in predicting the clinical course of congenital chylothorax and ascites.

Advances in the Treatment of Chylothorax.

Idiopathic chylothorax is a challenging clinical condition historically associated with poor resolution rates following surgical intervention. Recent advances in imaging and surgical techniques have revolutionized the treatment of this disease process. Computed tomographic lymphangiography has facilitated improved surgical planning and postoperative assessment, while intraoperative use of near-infrared fluorescence imaging aids in highly accurate intraoperative thoracic duct identification. Utilizing these advancements, minimally invasive surgical techniques have been successfully developed and have been associated with considerable improvements in surgical outcomes.

Interventional treatment of refractory non-traumatic chylous effusions in patients with lymphoproliferative disorders.

To report results of interventional treatment of refractory non-traumatic abdomino-thoracic chylous effusions in patients with lymphoproliferative disorders. 17 patients (10 male; mean age 66.7 years) with lymphoproliferative disorders suffered from non-traumatic chylous effusions (chylothorax n = 11, chylous ascites n = 3, combined abdomino-thoracic effusion n = 3) refractory to chemotherapy and conservative therapy. All underwent x-ray lymphangiography with iodized-oil to evaluate for and at the same time treat lymphatic abnormalities (leakage, chylo-lymphatic reflux with/without obstruction of central drainage). In patients with identifiable active leakage additional lymph-vessel embolization was performed. Resolution of effusions was deemed as clinical success. Lymphangiography showed reflux in 8/17 (47%), leakage in 2/17 (11.8%), combined leakage and reflux in 3/17 (17.6%), lymphatic obstruction in 2/17 (11.8%) and normal findings in 2/17 cases (11.8%). 12/17 patients (70.6%) were treated by lymphangiography alone; 5/17 (29.4%) with leakage received additional embolization (all technically successful). Effusions resolved in 15/17 cases (88.2%); 10/12 (83.3%) resolved after lymphangiography alone and in 5/5 patients (100%) after embolization. Time-to-resolution of leakage was significantly shorter after embolization (within one day in all cases) than lymphangiography (median 9 [range 4-30] days; p = 0.001). There was no recurrence of symptoms or post-interventional complications during follow-up (median 445 [40-1555] days). Interventional-radiological treatment of refractory, non-traumatic lymphoma-induced chylous effusions is safe and effective. Lymphangiography identifies lymphatic abnormalities in the majority of patients and leads to resolution of effusions in > 80% of cases. Active leakage is found in only a third of patients and can be managed by additional embolization.

Coil or Plug-Assisted Ethylene Vinyl Alcohol Copolymer (EVOH) Thoracic Duct Embolization in the Treatment of Postoperative Chylothorax.

Eleven patients (5 men, 6 women) with post-operative thoracic duct injuries and high output chylothorax were treated with thoracic duct embolization (TDE). Six patients underwent intraprocedural thoracic duct ligation at the time of original procedure. In all cases, the pleural fluid demonstrated high triglyceride levels (414 mg/dL; interquartile range [IQR], 345 mg/dL). Median daily (IQR) chest tube outputs before and after TDE were 900 mL (1,200 mL) and 325 mL (630 mL), respectively. Coil- or plug-assisted ethylene vinyl alcohol (EVOH) copolymer was used as embolic agent in all patients. Technical and clinical success rates were 100% and 82%, respectively. Nontarget venous embolization of EVOH copolymer was not identified on subsequent imaging.

Neonatal Chylothorax and Early Fluid Overload After Cardiac Surgery: Retrospective Analysis of the Neonatal and Pediatric Heart and Renal Outcomes Network Registry (2015-2018).

OBJECTIVES: To evaluate the association between postoperative cumulative fluid balance (FB) and development of chylothorax in neonates after cardiac surgery. DESIGN: Multicenter, retrospective cohort identified within the Neonatal and Pediatric Heart and Renal Outcomes Network (NEPHRON) Registry. SETTING: Twenty-two hospitals were involved with NEPHRON, from September 2015 to January 2018. PATIENTS: Neonates (< 30 d old) undergoing index cardiac operation with or without cardiopulmonary bypass (CPB) entered into the NEPHRON Registry. Postoperative chylothorax was defined in the Pediatric Cardiac Critical Care Consortium as lymphatic fluid in the pleural space secondary to a leak from the thoracic duct or its branches. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 2240 NEPHRON patients, 4% ( n = 89) were treated for chylothorax during postoperative day (POD) 2-21. Median (interquartile range [IQR]) time to diagnosis was 8 (IQR 6, 12) days. Of patients treated for chylothorax, 81 of 89 (91%) had CPB and 68 of 89 (76%) had Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery Congenital Heart Surgery 4-5 operations. On bivariate analysis, chylothorax patients had higher POD 1 FB (3.2 vs. 1.1%, p = 0.014), higher cumulative POD 2 FB (1.5 vs. -1.5%, p < 0.001), achieved negative daily FB by POD 1 less often (69% vs. 79%, p = 0.039), and had lower POD 1 urine output (1.9 vs. 3. 2 mL/kg/day, p ≤ 0.001) than those without chylothorax. We failed to identify an association between presence or absence of chylothorax and peak FB (5.2 vs. 4.9%, p = 0.9). Multivariable analysis shows that higher cumulative FB on POD 2 was associated with greater odds (odds ratio [OR], 95% CI) of chylothorax development (OR 1.5 [95% CI, 1.1-2.2]). Further multivariable analysis shows that chylothorax was independently associated with greater odds of longer durations of mechanical ventilation (OR 5.5 [95% CI, 3.7-8.0]), respiratory support (OR 4.3 [95% CI, 2.9-6.2]), use of inotropic support (OR 2.9 [95% CI, 2.0-4.3]), and longer hospital length of stay (OR 3.7 [95% CI, 2.5-5.4]). CONCLUSIONS: Chylothorax after neonatal cardiac surgery for congenital heart disease (CHD) is independently associated with greater odds of longer duration of cardiorespiratory support and hospitalization. Higher early (POD 2) cumulative FB is associated with greater odds of chylothorax. Contemporary, prospective studies are needed to assess whether early fluid mitigation strategies decrease postoperative chylothorax development.

Neonatal Lymphatic Flow Disorder.

OBJECTIVE: To examine and discuss patients diagnosed with acquired and congenital chylothorax in the neonatal period in the light of the literature. METHODS: The files of newborns followed-up in the neonatal intensive care unit (NICU) and diagnosed with congenital and acquired chylothorax were reviewed retrospectively. Patients with isolated chylothorax were classified as Group 1 and those with multiple lymphatic flow disorders were classified as Group 2. Antenatal and clinical features were recorded and compared between the groups. RESULTS: Thirteen infants were diagnosed with chylothorax; 92.3% (n = 12) of the patients were congenital. The rate of antenatal diagnosis was 61.5% (n = 8). Eight patients (61.5%) were diagnosed with hydrops fetalis. Among the cases in Group 1 and Group 2, receiving ocreotide and the incidence of sepsis (p = 0.05) were partially significant. Seven of the patients (66.6%) responded to medium chain triglycerides (MCT), and complete resolution was seen in 6 (85.7%) of the responders. Complete resolution of chylothorax fluid was observed in 7 (77.7%) of nine patients who responded to ocreotide treatment. CONCLUSIONS: In neonatal chylothorax, the postnatal period includes a multidisciplinary approach that requires drug therapy, dietary modifications, drainage of pleural fluid, and rarely, surgery.

Chylothorax related to acute SARS-CoV-2 infection in a patient with Noonan syndrome with prior uncomplicated cardiac surgeries.

SARS-CoV-2 is a novel coronavirus that has rarely been associated with chylothorax. Patients with Noonan syndrome are at risk for developing chylothorax, especially after cardiothoracic interventions. We present the case of SARS-CoV-2 infection triggering the underlying tendency of a patient with Noonan syndrome to develop chylothorax who did not develop it even after prior cardiothoracic interventions. Patient presented in respiratory distress without hypoxia and was found, on imaging, to have a large right-sided pleural effusion, which was eventually classified as chylothorax. The patient was then started on a low-fat diet. Chest tube drainage substantially reduced the effusion in size, and it remained stable. Our report highlights that SARS-CoV-2 infection can cause the development of a chylothorax or a chylous effusion in patients with Noonan syndrome or among populations with a similar predisposition. A high index of suspicion in vulnerable patients or those not responding to traditional therapy should exist with providers, thus leading to the testing of the fluid to confirm the diagnosis.

Recurrent bilateral idiopathic chylothorax: a therapeutic challenge.

Chylothorax is a rare condition caused by pleural effusion resulting from thoracic duct injury. Recurrent chylothorax is often resistant to conservative treatment and presents a clinical conundrum in its management. Here, we report a compelling case of recurrent chylothorax that persisted despite the administration of total parenteral nutrition, octreotide and thoracic duct embolisation. The patient eventually required thoracic duct ligation and talc pleurodesis, which resulted in the resolution of the effusion. Our case is an illustrative example of the effective multidisciplinary management of recurrent bilateral idiopathic chylothorax.

Intractable chylous leak after radical esophagectomy treated with radiotherapy.

Postoperative chylous leak after esophagectomy is a rare but potentially life-threatening complication that results in hypovolemia, electrolyte imbalance, malnutrition, and immunologic deficiency. However, the management of postoperative chylous leak remains controversial. Following a diagnosis of esophageal cancer, a 64-year-old man was treated by video-assisted thoracoscopic esophagectomy, laparoscopic gastric tube formation, prophylactically thoracic duct ligation, and reconstruction with esophagogastrostomy at the neck level. Massive postoperative drainage from the thorax and abdomen did not initially meet the diagnostic criteria for chylothorax, which was ultimately diagnosed 3 weeks after the operation. Despite various treatments including total parenteral nutrition, octreotide and midodrine, reoperation (thoracic duct ligation and mechanical pleurodesis), and thoracic duct embolization, the chylous leak persisted. Finally, low-dose radiation therapy was administered with a daily dose of 2 Gy and completed at a total dose of 14 Gy. After this, the amount of pleural effusion gradually decreased over 2 weeks, and the last drainage tube was removed. The patient was alive and well at 60 months postoperatively. Herein, we describe a patient with intractable chylous leak after esophagectomy, which persisted despite conservative treatment, thoracic duct ligation, and embolization, but was finally successfully treated with radiotherapy.

Non-Contrast MR Lymphography and Intranodal Dynamic Contrast MR Lymphangiography in Children with Congenital Heart Disease-Imaging Findings as well as Impact on Patient Management and Outcome.

Lymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities that can depict central lymphatic anatomy and flow pattern. Our objective was to describe the technical aspects and our imaging findings of central lymphatic abnormalities and their impact on patient management and outcomes: We conducted a retrospective review of 26 children with congenital heart disease who presented for lymphatic imaging between 2015 and 2020 at our institution. Eleven had postoperative chylothorax, six had plastic bronchitis, seven had protein-losing enteropathy and three had Noonan syndrome. Our lymphatic imaging demonstrated severely abnormal lymphatic flow in all of the children, but only minor abnormalities in protein-losing enteropathy. No major procedure-related complication occurred. Lymphatic interventions were performed in six patients, thoracic duct decompression in two patients and chylothorax revision in three patients. This led to symptomatic improvements in all of the patients: Lymphatic imaging is safe and essential for the diagnosis of lymphatic flow disorders and therapy planning. Our intranodal lymphangiography depicts an abnormal lymphatic flow pattern from the central lymphatics but failed to demonstrate an abnormal lymphatic flow in protein-losing enteropathy. These imaging techniques are the basis for selective lymphatic interventions, which are promising to treat lymphatic flow disorders.

Chylothorax after chimeric antigen receptor T cell therapy for relapsed and refractory diffuse large B-cell lymphoma: A case report.

RATIONALE: Anti-CD19-targeted chimeric antigen receptor (CAR) T cell therapy is effective in treating relapsed/refractory diffuse large B-cell lymphoma (DLBCL). This therapy is associated with several side effects that can be life-threatening such as cytokine release syndrome (CRS). However, chylothorax associated with CRS after CAR-T therapy has not been reported. PATIENT CONCERNS: A 23-year-old male diagnosed with DLBCL relapsing after autologous peripheral blood stem cell transplantation was treated with anti-CD19-targeted CAR-T cell therapy. After CAR-T cell transfusion, he developed grade 3 CRS includes fever, dyspnea, tachycardia and hypotension. The symptoms of CRS persisted and chest plain film revealed bilateral pleural effusion. DIAGNOSIS: Chylothorax was confirmed by the pleural effusion analysis that triglyceride level was 1061 mg/dL. Bacterial and fungal culture of pleural fluid reported no pathogen was detected. Cytological examination of pleural effusion revealed no malignant cells. INTERVENTIONS: The chylothorax resolved after treatment with intravenous administration of tocilizumab. OUTCOMES: On 30-day follow-up, the patient was in stable clinical condition with complete remission of DLBCL on whole-body positron emission tomography scan. LESSONS: We reported a rare case of CAR-T associated chylothorax in a patient with relapsed and refractory DLBCL. Grade 3 CRS with high interleukin-6 level was presented in our patient. The symptoms of CRS were improved with tocilizumab treatment and the chylothorax resolved later on. It is suggested that high interleukin-6 releases might induce chyle leakage resulting from activations of endothelium and coagulation. Our finding highlights the occurrence of chylothorax during the course of CAR-T cell therapy and the importance of proper monitoring and prompt management of this life-threatening side effect.