Impact of early enteral feed composition on the rate of chyle leak post-esophagectomy.

Patients undergoing esophagectomy are at risk of malnutrition and benefit from perioperative enteral feeding. Esophagectomy carries a risk of chyle leak, and this risk may be influenced by early enteral feed composition. We evaluated the impact of early enteral medium-chain triglyceride-rich feed on the prevalence and severity of chyle leak post-esophagectomy, length of stay, and postoperative weight change. This retrospective study included consecutive patients undergoing esophagectomy at a single center between January 2015 and December 2022. Patients received enteral feed on postoperative days 1-5 with Nutrison Energy or Protein Plus Energy ('standard') (January 2015- June 2021) or Nutrison Peptisorb Plus High Energy High Protein ('HEHP') enteral feed (June 2021 to December 2022). All patients transitioned to 'standard' supplemental jejunal feeding on postoperative day 6 onwards and were discharged on oral IDDSI level 4 diet. Patients who did not commence early enteral feeding were excluded from analysis. A total of 329 patients were included. Patients who received early HEHP feed had fewer chyle leaks (5/52; 9.6%) compared with patients who received standard feed (68/277; 24.5%, P = 0.017). The HEHP group had a shorter total length of hospital stay (P = 0.011). Weight change from preoperative baseline was equivalent in both groups at 6 weeks (P = 0.066) and 3 months (P = 0.400). In the context of routine jejunostomy use and early enteral feeding post-esophagectomy, HEHP feed on postoperative days 1-5 was associated with significantly fewer chyle leaks and shorter length of stay compared with standard feed. No difference was noted in postoperative weight change between groups.

Thoracic Duct Visualization in Esophageal Resection: A Pilot Trial.

INTRODUCTION: Inadvertent thoracic duct injury is common during esophagectomy and may result in postoperative chylothorax. This study's objective was to investigate utility of patent blue injection as a modality for intraoperative thoracic duct visualization. METHODS: A prospective, single-arm, interventional study of patients undergoing minimally invasive esophagectomy was performed. Patients were injected with patent blue dye into both groins prior to thoracic stage of surgery and assessed for duct visualization. Control group was formed by propensity score matching using retrospectively collected data regarding patients who underwent esophagectomy. RESULTS: A total of 25 patients were included in analysis, compared to a control of 50 patients after matching. Thoracic duct was visualized in 60% of patients in the study group (15/25 patients). Significant differences were found between study and control groups (p < 0.05) with regards to median operative time (422 vs. 285 min, respectively), overall complications (16 vs. 34%, respectively), and median postoperative length of stay (13.5 vs. 10 days, respectively). There was a difference in rate of chyle leak between study and control groups; however, this was not significant (0 vs. 12%, respectively, p = 0.17). CONCLUSION: Patent blue injection represents a simple method for thoracic duct visualization during minimally invasive esophagectomy which may improve surgical outcomes.

Use of a Postoperative Care Management Pathway Reduces the Incidence of Chylothorax Post-Fontan Palliation.

Pleural effusions and chylothorax are challenging morbidities post-Fontan palliation. We sought to evaluate the efficacy of our Fontan Care Pathway (FCP) in reducing the incidence of post-operative chylothorax and Time to Chest Tube Removal (TTCTR), and to determine risk factors associated with longer TTCTR. Between 2016 and 2022 our institutional approach to post-Fontan care fell into three categories: Group 1 (n = 36): no standardized approach; Group 2 (n = 30): a prophylactic chylothorax diet (fat content < 5%); Group 3 (n = 57): the FCP (a chylothorax diet, fluid restriction, supplemental O2 and aggressive diuresis). The incidence of chylothorax and TTCTR was compared between groups. Predictors of TTCTR were analyzed using linear regression modelling, adjusting for covariates. Chylothorax rate decreased in Group 3 compared to Groups 1 and 2 (9% vs. 28% and 33% respectively, p = 0.011), without alteration in TTCTR. Univariate factors associated with median TTCTR included chylothorax (+ 13.7 days, p = 0.001), additional procedures at time of Fontan (+ 2.4 days per procedure p = 0.017), Fontan revision or takedown (+ 11.7 days, p = 0.018) and minor/major complications (+ 5.1, p = 0.01 and + 15.8, p < 0.001, respectively). On multivariable analysis, chylothorax (+ 6.5 days, p = 0.005) and major complications (+ 15.8 days, p = 0.001) were associated with increased TTCTR. When chylothorax was excluded from multivariable analysis, the FCP showed a significant decrease in TTCTR (- 3.3 days, p = 0.034). A bundled therapy approach was associated with reduced laboratory confirmed chylothorax post-Fontan, whereas diet change alone was not. Additional studies in this area, with larger sample sizes are warranted.

Lymphoscintigraphy Findings are Associated with Outcome in Children with Chylothorax After Cardiac Surgery.

Postoperative chylothorax in patients with congenital heart diseases (CHD) results in poor outcomes if anatomical and functional abnormalities of the lymphatic system are present. While these abnormalities are typically diagnosed by intranodal lymphangiography and dynamic contrast magnetic resonance lymphangiography, the usefulness of lymphoscintigraphy in these patients has not been evaluated. Between January 2019 and December 2021, 28 lymphoscintigraphies were performed in our institution for investigating prolonged pleural effusion after cardiac surgery. The images were assessed by three board-certified pediatric cardiologists retrospectively to determine the likelihood of a central lymphatic flow disorder. The likelihood was scored (range 1-3) based on structural abnormalities and congestive flow in the lymphatic system. Those scores were summed and the likelihood was categorized as low to intermediate (< 8 points) or high (8 or 9 points). Median age at lymphoscintigraphy was 129 days (IQR, 41-412 days), it was performed at a median of 22 days (IQR, 17-43) after surgery, and median score was 6 points (IQR, 4-7.5). Kendall's coefficient of concordance (0.867; p < 0.05) indicated high inter-rater reliability. Overall survival at 6 months after surgery was 92.5% in the low-to-intermediate group but 68.6% in the high group (p < 0.05), and duration of postoperative thoracic drainage was 27 and 58 days, respectively (p < 0.05). Lymphatic abnormalities detected by lymphoscintigraphy were associated with poorer outcomes. Lymphoscintigraphy was thought to be useful in assessing anatomic and functional lymphatic abnormalities, despite its minimal invasiveness.

Successful oral midodrine therapy for treatment of refractory postoperative chylothorax in an infant.

Refractory chylothorax, a postoperative complication of CHD, is difficult to manage and sometimes fatal. Herein, we report the case of a 10-month-old infant with 22-mosaic trisomy and a coarctation complex, who developed refractory chylothorax after cardiac repairs and was successfully treated with midodrine, an oral alpha-1-adrenoreceptor agonist. Midodrine may be used as adjunctive therapy for postoperative refractory chylothorax.

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.

Ultrasound analysis of cervical thoracic duct for patients with constrictive pericarditis and chylothorax.

PURPOSE: To analyze ultrasound features of cervical thoracic duct for patients with constrictive pericarditis and chylothorax. METHODS: Patients were retrospectively assessed. The patients were divided into a non-pleural effusion (PE) group (n = 54), a chylothorax group (n = 23), and non-chylothorax group (n = 28). Conventional ultrasound was used to obtain the maximum inner diameter and collapse of the inferior vena cava, the inner diameter of left cervical thoracic duct, and the frequency of opening of the valve at the end of the left thoracic duct. Contrast ultrasonography was used to score the reverse flow of the thoracic tube. RESULTS: The percentage of PE was 48.5%, and the percentage of chylothorax was 21.9%. The three groups had significant differences in five parameters. The inner diameter of left cervical thoracic duct was correlated with the degree of central venous pressure. Contrast ultrasonography was effective in quantitative assessment of the degree of intravenous-thoracic cord reverse flow which correlated with all parameters of central venous pressure. CONCLUSION: Thoracic duct dilation and regurgitation secondary to central venous pressure can lead to chyloreflux disorder and may be the mechanism of chylothorax occurrence in constrictive pericarditis.

Thoracoscopic Management of Chylothorax by Application of a Fibrin Sealant Patch.

Thoracic duct ligation is a demanding procedure when the chyle leak and/or the duct itself are not identified. This report describes a new procedure using thoracoscopic closure of the chyle leak by application of a fibrin sealant patch. This strategy was successfully applied for closing (i) a small fistula due to a postoperative lesion of the lymphatic tributary vessels in one case, and (ii) a large fistula due to idiopathic rupture of the thoracic duct in another case.

Persistent chylothorax associated with lymphatic malformation type 6 due to biallelic pathogenic variants in PIEZO1.

PIEZO1 is required for lymphatic valve formation, and several lymphatic abnormalities have been reported to be associated with autosomal recessive PIEZO1 pathogenic variants including neonatal hydrops, lymphedema involving various body regions, and chylothorax. Persistent or recurrent chylothorax has been infrequently described in association with pathogenic variants in the PIEZO1 gene. We present a 4-year-old female with bilateral pleural effusions detected prenatally, who was diagnosed with bilateral chylothoraces post-partum. She subsequently had recurrent pleural effusions involving both pleural cavities, which tended to improve with restriction of her fat intake, and, one occasion, subcutaneous octreotide. She also had bilateral calf, and intermittent cheek swelling. Genetic testing revealed two deleterious variants in PIEZO1: c.2330-2_2330-1del and c.3860G > A (p.Trp1287*), both of which were classified as likely pathogenic. This supported a diagnosis of Lymphatic Malformation Type 6 (OMIM 616843), also known as Hereditary Lymphedema Type III. Hereditary Lymphedema type III can be associated with persistent chylothorax that can vary in size over time.

Chylothorax due to hepatic alveolar echinococcosis with infiltration of diaphragm and left pleura: a case report.

BACKGROUND: Alveolar echinococcosis (AE) is an endemic parasitic zoonosis in Germany. In most cases, the liver is the primary organ affected. CASE PRESENTATION: A 59-year old female patient presented with increasing exertional dyspnea and unintentional weight loss. A computed tomography (CT) scan showed a left-sided chylous pleural effusion and multiple intrahepatic masses with infiltration of the diaphragm and the pleura. The findings were initially misinterpreted as hepatocellular carcinoma (HCC) with infiltrating growth. Liver biopsy of one of the masses showed no evidence of malignancy, but an amorphous necrosis of unclear origin. HCC was further ruled out by magnetic resonance imaging (MRI). However, MRI findings were highly suspicious for hepatothoracic dissemination and complications due to AE. Typical histologic findings in a repeated and more specific examination of the liver tissue and a positive serology for echinococcosis confirmed the diagnosis of AE. As the hepatic and pulmonary manifestations were considered inoperable in a curative matter, an anti-parasitic treatment with albendazole was initiated. A video-assisted thoracoscopic surgery (VATS) with removal of the chylous effusion as well as a talc pleurodesis was performed to relieve the patient from dyspnea. Two months later, the patient was asymptomatic and a positron emission tomography (PET)-CT-scan with [18 F] fluoro-2-deoxy-d-glucose (FDG) showed a remarkable diminution of the hepatic manifestation. CONCLUSIONS: This case demonstrates a rare presentation of alveolar echinococcosis with a focus on pulmonary symptoms, emphasizing the importance of evaluation for pulmonary involvement in patients with AE and respiratory symptoms.

Chylothorax in newborns after cardiac surgery: a rare complication?

The aim of this study was to analyze patients diagnosed with chylothorax after congenital heart disease surgery among a cohort of neonatal patients, comparing the evolution, complications, and prognosis after surgery of patients who were and were not diagnosed with chylothorax, and to analyze possible risk factors that may predict the appearance of chylothorax in this population. Retrospective and observational study included all neonates (less than 30 days since birth) who underwent congenital heart disease surgery in a level III neonatal intensive care department. We included infants born between January 2014 and December 2019. We excluded those infants who were born before 34 weeks of gestational age or whose birth weight was less than 1800 g. We also excluded catheter lab procedures and patent ductus arteriosus closure surgeries. Included patients were divided into two groups depending on whether they were diagnosed with chylothorax or not after surgery, and both groups were compared in terms of perinatal-obstetrical information, surgical data, and NICU course after surgery. We included 149 neonates with congenital heart disease surgery. Thirty-one patients (20.8%) developed chylothorax, and in ten patients (32.3%), it was considered large volume chylothorax. Regarding the evolution of these patients, 22 infants responded to general dietetic measures, a catheter procedure was performed in 9, and 5 of them finally required pleurodesis. Cardiopulmonary bypass, median sternotomy, and delayed sternal closure were the surgical variables associated with higher risks of chylothorax. Patients with chylothorax had a longer duration of inotropic support and mechanical ventilation and took longer to reach full enteral feeds. As complications, they had higher rates of cholestasis, catheter-related sepsis, and venous thrombosis. Although there were no differences in neonatal mortality, patients with chylothorax had a higher rate of mortality after the neonatal period. In a multiple linear regression model, thrombosis and cardiopulmonary bypass multiplied by 10.0 and 5.1, respectively, the risk of chylothorax and have an umbilical vein catheter decreases risk. CONCLUSION: We have found a high incidence of chylothorax after neonatal cardiac surgery, which prolongs the average stay and causes significant morbidity and mortality. We suggested that chylothorax could be an underestimated complication of congenital heart disease surgery during the neonatal period. WHAT IS KNOWN: • Acquired chylothorax in the neonatal period usually appears as a complication of congenital heart disease surgery, being the incidence quite variable among the different patient series (2.5-16.8%). The appearance of chylothorax as a complication of a cardiac surgery increases both mortality and morbidity in these patients, which makes it a quality improvement target in the postsurgical management of this population. WHAT IS NEW: •Most of the published studies include pediatric patients of all ages, from newborns to teenagers, and there is a lack of studies focusing on neonatal populations. The main strength of our study is that it reports, to the best of our knowledge, one of the largest series of neonatal patients receiving surgery for congenital heart disease in the first 30 days after birth. We have found a high incidence of chylothorax after cardiac surgery during the neonatal period compared to other studies. We suggested that chylothorax could be an underestimated complication of congenital heart disease surgery during this period of life.

Impact of fluorescent thoracic duct lymphography via intranodal approach in minimal access esophageal cancer surgery.

PURPOSE: Chyle leak resulting from thoracic duct (TD) injury poses significant morbidity and mortality challenges. We assessed the feasibility of using near-infrared (NIR) indocyanine green (ICG) imaging for intraoperative fluorescence TD lymphography during minimal access esophagectomy (MAE) in a semiprone position with inguinal nodal injection of ICG dye. METHODS: Ninety-nine patients with esophageal or gastroesophageal junctional cancer undergoing MAE received inguinal node injections of 2.5 mg ICG dye (total 5 mg) under sonographic guidance during anesthesia induction. Stryker's 1688 AIM HD system was used in 76 cases, Karl Storz OPAL 1 S in 20, and in three cases the Karl Storz Rubina. RESULTS: In 93 patients (94%), the TD was clearly delineated along its entire length; it was not visualized in 6 patients (6%). Fluorescence guidance facilitated TD ligation in 16 cases, while 3 cases required clipping of duct tributaries for oncological considerations. Twenty-eight patients exhibited minor duct variations. Fluorescence was sustained throughout surgery (median observation time 60 min post-injection; range 30-330). No patient experienced any chyle leak within 30 days post-surgery and no adverse reactions to ICG was evident. CONCLUSIONS: Intraoperative fluorescence TD lymphography using ICG during MAE in a semiprone position with inguinal nodal injection proved safe, feasible, and effective, allowing clear visualization of the TD in almost all cases. This approach aids safe ligation and reduces chyle leak risk. It offers real-time imaging of TD anatomy and variations, providing valuable feedback to surgeons for managing TD injuries during MAE procedures and represents an excellent educational tool.

Refractory systemic lupus erythematosus with chylous effusion successfully treated with sirolimus: a case report and literature review.

Chylous effusion is a rare manifestation of systemic lupus erythematosus (SLE). When it does occur in SLE, it is generally well treated with standard pharmacologic or surgical measures. We present a decade of management in a case of SLE with lung affliction and development of refractory bilateral chylous effusion and pulmonary arterial hypertension (PAH). In the first years, the patient was treated under a Sjogren syndrome diagnose. After few years, her respiratory condition worsened due to chylous effusion and PAH. Immunosuppression therapy (methylprednisolone) was reintroduced, and vasodilator therapy commenced. With this, her cardiac function remained stable, but respiratory function continuously worsened despite several therapy trials with different combinations of immunosuppressant (glucocorticoids, resochin, cyclophosphamide and mycophenolate mofetil). On top of pleural effusion worsening, the patient developed ascites and severe hypoalbuminaemia. Even though albumin loss was stabilized with monthly octreotide applications, the patient remained respiratory insufficient and in need of continuous oxygen therapy. At that point, we decided to introduce sirolimus on top of glucocorticoids and mycophenolate mofetil therapy. Her clinical status, radiological finding, and lung function gradually improved and she became respiratory sufficient at rest. The patient remains in our follow-up and has been stable on given therapy for over 3 years despite overcoming a severe COVID-19 pneumonia in 2021. This case adds to the body of evidence of sirolimus effectiveness in patients with refractory systemic lupus and is, to our best knowledge, the first case to report its successful application in a patient with SLE and refractory chylous effusion.

Cough-induced chylothorax in a two-year-old boy - case report and review of the literature.

BACKGROUND: Chylothorax is a very rare form of pleural effusion in children, especially after the neonatal period, and predominantly occurs secondary to cardiothoracic surgery. It can lead to significant respiratory distress, immunodeficiency, and malnutrition. Effective treatment strategies are therefore required to reduce morbidity. CASE PRESENTATION: A previously healthy two-year old boy was admitted with history of heavy coughing followed by progressive dyspnea. The chest X-ray showed an extensive opacification of the right lung. Ultrasound studies revealed a large pleural effusion of the right hemithorax. Pleural fluid analysis delivered the unusual diagnosis of chylothorax, most likely induced by preceded excessive coughing. After an unsuccessful treatment attempt with a fat-free diet and continuous pleural drainage for two weeks, therapy with octreotide was initiated. This led to complete and permanent resolution of his pleural effusion within 15 days, without any side effects. CONCLUSIONS: Severe cough may be a rare cause of chylothorax in young children. Octreotide seems to be an effective and safe treatment of spontaneous or traumatic chylothorax in children. There is, however, a lack of comprehensive studies for chylothorax in children and many issues concerning diagnostic strategies and treatment algorithms remain.

Clinical implications of chyle leakage following esophagectomy.

The clinical consequences of chyle leakage following esophagectomy are underexposed. The aim of this study was to investigate the clinical implications of chyle leakage following esophagectomy. This retrospective study of prospectively collected data included patients who underwent transthoracic esophagectomy in 2017-2020. Routinely, the thoracic duct was resected en bloc as part of the mediastinal lymphadenectomy. Chyle leakage was defined as milky drain fluid for which specific treatment was initiated and/or a triglyceride level in drain fluid of ≥1.13 mmol/L, according to the Esophagectomy Complications Consensus Group (ECCG) classification. Primary endpoints were the clinical characteristics of chyle leakage (type, severity and treatment). Secondary endpoints were the impact of chyle leakage on duration of thoracic drainage and hospital stay. Chyle leakage was present in 43/314 patients (14%), of whom 24 (56%) were classified as severity A and 19 (44%) as severity B. All patients were successfully treated with either medium chain triglyceride diet (98%) or total parental nutrition (2%). There were no re-interventions for chyle leakage during initial admission, although one patient needed additional pleural drainage during readmission. Patients with chyle leakage had 3 days longer duration of thoracic drainage (bias corrected accelerated (BCa) 95%CI:0.46-0.76) and 3 days longer hospital stay (BCa 95%CI:0.07-0.36), independently of the presence of other complications. Chyle leakage is a relatively frequent complication following esophagectomy. Postoperative chyle leakage was associated with a significant longer duration of thoracic drainage and hospital admission. Nonsurgical treatment was successful in all patients with chyle leakage.

Near-infrared fluorescence imaging of thoracic duct in minimally invasive esophagectomy.

Chylothorax is a serious complication after esophagectomy and there are unmet needs for new intraoperative navigation tools to reduce its incidence. The aim of this study is to explore the feasibility and effectiveness of near-infrared fluorescence imaging (NIR-FI) with indocyanine green (ICG) to identify thoracic ducts (TDs) and chyle leakage during video-assisted thoracoscopic esophagectomy. We recruited 41 patients who underwent thoraco-laparoscopic minimally invasive esophagectomy (MIE) for esophageal cancer in this prospective, open-label, single-arm clinical trial. ICG was injected into the right inguinal region before operations, after which TD anatomy and potential chyle leakage were checked under the near-infrared fluorescence intraoperatively. In 38 of 41 patients (92.7%) using NIR-FI, TDs were visible in high contrast. The mean signal-to-background ratio (SBR) value of all fluorescent TDs was 3.05 ± 1.56. Fluorescence imaging of TDs could be detected 0.5 hours after ICG injection and last up to 3 hours with an acceptable SBR value. The optimal observation time window is from about 1 to 2 hours after ICG injection. Under the guidance of real-time NIR-FI, three patients were found to have chylous leakage and the selective TD ligations were performed intraoperatively. No patient had postoperative chylothorax. NIR-FI with ICG can provide highly sensitive and real-time assessment of TDs as well as determine the source of chyle leakage, which might help reduce TD injury and direct selective TD ligation. It could be a promising navigation tool to reduce the incidence of chylothorax after minimally invasive esophagectomy.

Pre-operative oil ingestion reduces the probability of thoracic duct trunk ligation during esophagectomy.

Chylothorax is an important complication after esophagectomy. Ligation of the injured thoracic duct is the main method to prevent chylothorax after esophagectomy, but may be associated with adverse effects. Whether ligation of the injured tributary alone, keeping the main trunk intact, may suffice to prevent post-operative chylothorax is not well known. Since March 2017, 40 mL of olive oil was administered to patients posted for esophagectomy. We compared patients admitted between March 2017 and December 2019 with patients admitted between July 2014 and February 2017, who had not received pre-operative oil. The outcome measures were the need for thoracic duct main trunk or tributary ligation, development of chylothorax and missed ligation. There were 371 patients in the oil ingestion group and 308 patients in the standard control group. Chylothorax in the oil ingestion group was significantly lower than that in the standard control group (1.3% vs. 4.5%, P = 0.012). Chyle leak from thoracic duct tributaries was diagnosed in a significantly higher percentage (5.7% vs. 0.0%, P < 0.001) and missed ligation of the injured thoracic duct was significantly lower (0.3% vs. 3.9%, P = 0.002) in the oil ingestion group compared with the standard control group. The incidence of post-operative chylothorax was not statistically different (6.3% vs. 10.0%, P = 1.000) between the tributary and the trunk ligation group. Pre-operative oil ingestion can help visualize the thoracic duct trunk and its tributaries during esophagectomy. Thus, non-selected thoracic duct trunk ligation and missed ligation during esophagectomy can be reduced. Precise ligation of the injured tributary while the main trunk is intact can also prevent post-operative chylothorax.

Spectrum of lymphatic anomalies in patients with RASA1-related CM-AVM.

BACKGROUND: Capillary malformation-arteriovenous malformation (CM-AVM) is characterized by multifocal fast-flow capillary malformations, sometimes with arteriovenous malformations/fistulas, skeletal/soft tissue overgrowth, telangiectasias, or Bier spots. Lymphatic abnormalities are infrequently reported. We describe seven patients with CM-AVM and lymphatic anomalies. METHODS: Following IRB approval, we identified patients with CM-AVM and lymphatic anomalies seen at the Vascular Anomalies Center at Boston Children's Hospital from 2003 to 2023. We retrospectively reviewed records for clinical, genetic, laboratory, and imaging findings. RESULTS: We found seven patients with CM-AVM and lymphatic abnormalities. Five patients were diagnosed prenatally: four with pleural effusions (including one suspected chylothorax) and one with ascites. Pleural effusions resolved after neonatal drainage in three patients and fetal thoracentesis in the fourth; however, fluid rapidly reaccumulated in this fetus causing hydrops. Ascites resolved after neonatal paracentesis, recurred at 2 months, and spontaneously resolved at 5 years; magnetic resonance lymphangiography for recurrence at age 19 years suggested a central conducting lymphatic anomaly (CCLA), and at age 20 years a right spermatic cord/scrotal lymphatic malformation (LM) was detected. Chylous pericardial effusion presented in a sixth patient at 2 months and disappeared after pericardiocentesis. A seventh patient was diagnosed with a left lower extremity LM at 16 months. Six patients underwent genetic testing, and all had RASA1 mutation. RASA1 variant was novel in three patients (c.1495delinsCTACC, c.434_451delinsA, c.2648del), previously reported in two (c.2603+1G>A, c.475_476del), and unavailable in another. Median follow-up age was 5.8 years (4 months-20 years). CONCLUSION: CM-AVM may be associated with lymphatic anomalies, including pericardial/pleural effusions, ascites, CCLA, and LM.

Octreotide for Acquired Chylothorax in Pediatric Patients Post-Cardiothoracic Surgery for Congenital Heart Disease: A Systematic Review.

Chylothorax is a life-threatening complication post-corrective congenital heart surgery. Octreotide is used for treatment of refractory chylothoraces, with no standardized treatment protocol and a paucity of literature describing its efficacy. Our aim was to provide an update on the safety and efficacy of octreotide for the treatment of refractory chylothoraces in neonatal and pediatric patients' post-corrective congenital heart surgery. We performed a systematic review of PubMed, Medline, CINAHL, and Cochrane Library databases. Only intravenous octreotide treatment was included. A total of 621 patients across 27 studies were included. Studies included were 11 case series, 5 case studies, and 11 retrospective cohort studies. Variation in treatment regimens were reported. Treatment efficacy was reported in 95% (23/27) of studies. Definitions of treatment efficacy were reported in 33% (9/27) of studies. No prospective or randomized control trials were available for inclusion. Octreotide efficacy is widely reported despite a lack of standardization on criteria for treatment initiation or what defines an appropriate response to therapy.Please check and confirm whether the edit made to the article title is in order.Yes.

Chylothorax After Heart Surgery in Children.

Chylothorax is a consequence of a thoracic duct injury that can occur during surgical procedures in patients with congenital heart disease. It is associated with high rates of morbimortality and increased use of clinical and hospital resources. The aim of this study was to evaluate the risk factors, distribution, manifestations, complications, and treatments for chylothorax in patients undergoing cardiac surgery in a tertiary pediatric hospital in southern Brazil. This is a retrospective, quantitative study, in which all medical records (n = 166) of patients with chylothorax after pediatric cardiac surgery between January 2014 and December of 2020 and a matched control group (n = 166) were analyzed. Over the study period, there was an increase in incidence of chylothorax from 4.5% in 2014 to 7.6% in 2020, a trend that has been reported in the literature. After multivariate analysis, the following were identified as risk factors for the diagnosis of chylothorax: genetic syndrome (OR 2.298); prolonged cardiopulmonary bypass time (greater than 120 min) (OR 2.410); fluid overload in the immediate postoperative period (OR 1.110); and SIRS (OR 2.527). Mortality was two times greater (p = 0.021) and there was a higher rate (34.4%) of infection (p < 0.001) in patients who developed chylothorax. In addition, a sensitivity analysis was performed comparing patients with low- and high-output chylothorax (> 20 mL/kg), which confirmed unfavorable outcomes for the latter group. Herein, we show that hemodynamic alterations were important factors for diagnosis. Understanding the risk factors, outcomes, and complications helps early identification and enables the reduction of morbidity and mortality.