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.

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.

Factors Contributing to Successful Transvenous Retrograde Thoracic Duct Cannulation.

PURPOSE: To identify key factors for successful transvenous retrograde cannulation (TVRC) of the thoracic duct. MATERIALS AND METHODS: A total of 47 consecutive patients (62.1 ± 13.2 years; 32 men) who underwent attempted TVRC between July 2016 and July 2021 were included. Reasons for interventions were chylous leakage from the chest (n = 36), abdomen (n = 6), and other sites (n = 5). Patient age, sex, access vein (femoral vs brachial), anatomic classification (presence of dominant channel vs plexiform) of the terminal thoracic duct, and engagement of a diagnostic catheter into the jugulovenous junction were included in the analyses. Anatomic details were evaluated according to catheter-based high-pressure lymphangiography and conventional intranodal lymphangiography. The Firth bias-reduced penalized-likelihood logistic regression model was used to analyze prognostic factors. RESULTS: TVRC was successful in 33 of the 47 patients (70%). In univariate analysis, femoral access, diagnostic catheter engagement, and presence of dominant channel were significant positive prognostic factors (P <.05). In multivariate analysis, diagnostic catheter engagement and presence of dominant channel were significant prognostic factors (P <.05). Diagnostic catheter engagement showed the highest prognostic performance (accuracy = 0.872), followed by presence of a dominant channel. High-pressure catheter-based lymphangiographic findings showed better performance (accuracy, 0.844 vs 0.727) than intranodal lymphangiography to delineate the anatomy of the terminal thoracic duct. CONCLUSIONS: A secure selection of the jugulovenous junction and the presence of a dominant channel in the terminal portion of the thoracic duct were significant prognostic factors for successful TVRC.

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.

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.

Intranodal cone-beam computed tomographic lymphangiography with water-soluble iodinated contrast agent for evaluating chylothorax in infants - preliminary experience at a single institution.

This brief report demonstrates the diagnostic utility of cone-beam CT lymphangiography (CBCTL) with intranodal injection of water-soluble iodinated contrast agent for assessing lymphatic disorders in two infants who were contraindicated for MRI and oil-based contrast agent. Both infants had dextro-transposition of the great arteries (d-TGA) and presented with high-output chylothoraces that were recalcitrant to conservative medical therapy. Both infants were diagnosed with central lymphatic flow disorder based on the CBCTL findings.

Nutritional management of postoperative chylothorax in children with CHD.

INTRODUCTION: Chylothorax after congenital cardiac surgery is associated with increased risk of malnutrition. Nutritional management following chylothorax diagnosis varies across sites and patient populations, and a standardised approach has not been disseminated. The aim of this review article is to provide contemporary recommendations related to nutritional management of chylothorax to minimise risk of malnutrition. METHODS: The management guidelines were developed by consensus across four dietitians, one nurse practitioner, and two physicians with a cumulative 52 years of experience caring for children with CHD. A PubMed database search for relevant literature included the terms chylothorax, paediatric, postoperative, CHD, chylothorax management, growth failure, and malnutrition. RESULTS: Fat-modified diets and nil per os therapies for all paediatric patients (<18 years of age) following cardiac surgery are highlighted in this review. Specific emphasis on strategies for treatment, duration of therapies, optimisation of nutrition including nutrition-focused lab monitoring, and supplementation strategies are provided. CONCLUSIONS: Our deliverable is a clinically useful guide for the nutritional management of chylothorax following paediatric cardiac surgery.

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.

Thoracic duct embolization in treating postoperative chylothorax: does bail-out retrograde access improve outcomes?

OBJECTIVES: To evaluate clinical outcomes of thoracic duct embolization (TDE) for the management of postoperative chylothorax with the aid of the bail-out retrograde approach for thoracic duct cannulation (TDC). MATERIALS AND METHODS: Forty-five patients with postoperative chylothorax underwent Lipiodol lymphangiography (LLG) between February 2016 and November 2019. If targetable central lymphatic vessels were identified in LLG, TDC, a prerequisite for TDE, was attempted. While the conventional antegrade transabdominal approach was the standard TDC method, the retrograde approach was applied as a bail-out method. Embolization, the last step of TDE, was performed after confirming leakages in the trans-TDC catheter lymphangiography. Technical and clinical success rates were determined retrospectively. RESULTS: TDC was attempted in 40 among 45 patients based on LLG findings. The technical success rate of TDC with the conventional antegrade approach was 78% (31/40). In addition, six more patients were cannulated using the bail-out retrograde approach, which raised the technical success rate to 93% (37/40). While 35 patients underwent embolization (TDE group), ten patients did not (non-TDE group) for the following reasons: (1) lack of targetable lymphatics for TDC in LLG (n = 5), (2) technical failure of TDC (n = 3), and (3) lack of discernible leakages in the transcatheter lymphangiography (n = 2). The clinical success of the TDE group was 89% (31/35), compared with 50% (5/10) of the non-TDE group. One major procedure-related complication was bile peritonitis caused by the needle passage of the distended gallbladder. CONCLUSIONS: Bail-out retrograde approach for TDC could improve the overall technical success of TDC significantly. KEY POINTS: • Bail-out retrograde thoracic duct access may improve the overall technical success of thoracic duct access, thus improving the clinical success of thoracic duct embolization.

Pragmatic role of noncontrast magnetic resonance lymphangiography in postoperative chylothorax or cervical chylous leakage as a diagnostic and preprocedural planning tool.

OBJECTIVES: To define the roles of noncontrast magnetic resonance lymphangiography (MRL) in the management of postoperative chylothorax or cervical chylous leakage. METHODS: A total of 50 consecutive patients underwent noncontrast MRL, intranodal lymphangiography, and thoracic duct embolization between May 2016 and April 2020. Their mean age was 62.6 years ± 10.3 (SD) years, and 35 of the participants were men. Conventional lymphangiographic images were sufficient in quality as a reference for the evaluation of diagnostic accuracy of leakage and location in 35 patients (70%) and for evaluation of anatomic details of the thoracic duct and jugulovenous junction in 34 patients (68%). RESULTS: MRL showed that the sensitivity, specificity, and positive and negative predictive values for leakage detection were 100%, 97.1%, 100%, and 100%, respectively, and the concordance rate was 97.14% (95% confidence interval [CI], 85.08-99.93%; p < .001). Leakage location was concordant between MRL and conventional lymphangiography in 27 patients (77.1%, 27/35). Regarding anatomical details of the thoracic duct, variation of the thoracic duct was missed in 11.7% of patients (4/34). The jugulovenous junction was observed in 91.1% (31/34), and its opening into the central vein was depicted in 76.4% (26/34). The concordance rate was between 76.47 and 91.18. CONCLUSIONS: Noncontrast MRL has a high sensitivity for the detection of postoperative thoracic and cervical chylous leakage but is suboptimal for the localization of the leak and depiction of anatomical details of the thoracic duct. This method is worthy of consideration as either a decision-making or planning tool for subsequent interventions. KEY POINTS: • Noncontrast MRL provides limited resolution images of CLS but has a high sensitivity for the detection of postoperative chylous leakage in the thoracic and neck regions. • Noncontrast MRL is suboptimal for depicting anatomic details in the thoracic duct and jugulovenous junction but can play a role as a decision-making and a planning tool for subsequent lymphatic interventions.

Thoracic Duct Embolization Using Transabdominal Antegrade and Transcervical Retrograde Accesses.

PURPOSE: To evaluate the technical success and clinical outcomes of thoracic duct embolization (TDE) using transabdominal antegrade and transcervical retrograde accesses to treat patients with chyle leak. MATERIALS AND METHODS: This study was a retrospective, nonblinded, single-institution chart review of all patients aged 18 years or older over a 6-year time frame who underwent lymphangiography with attempted TDE for iatrogenic or spontaneous chyle leaks using transabdominal antegrade and/or transcervical retrograde accesses. RESULTS: Ninety-nine patients underwent 113 procedures. Eighty-five patients underwent 1 procedure, and 14 patients required 2 procedures. The technical success rate of TDE was 68% (72/106) with transabdominal antegrade access and 44% (15/34) with transcervical retrograde access. The overall technical success rate of TDE, including both the access methods, was 77% (87/113). The most common reasons for transabdominal access failure were small caliber of the cisterna chyli and thoracic duct (TD) occlusion. Five patients were lost to follow-up. Overall clinical success, defined as resolution of the chyle leak, was achieved in 83% (78/94) of the patients. There were 6 Society of Interventional Radiology (SIR) level 1 adverse events (AEs), 5 SIR level 2 AEs, and 2 SIR level 3 AEs. Nontarget embolization occurred in 2 patients. CONCLUSIONS: Although transcervical retrograde TDE is a challenging procedure, with a lower technical success rate than transabdominal antegrade access, retrograde access improved the technical and clinical success rates of the treatment of chyle leaks in cases of thoracic duct occlusion, small cisterna chyli, and leaks located in the abdomen.

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

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

How we approach pediatric congenital chylous effusions and ascites.

Congenital lymphatic leak may develop in patients with maldeveloped lymphatics and result in life-threatening fluid and electrolyte imbalance, protein deficiency, and immunodeficiency. Rapid diagnosis and therapy are necessary to prevent these complications; however, the field lacks clinical trials to support standardized diagnostic treatment guidelines. We present our current multidisciplinary approach to the diagnosis and management of congenital lymphatic leak including chylous pleural effusions and ascites. Depending on the rate of lymphatic leak, therapy can range from observation with nutritional modifications to surgical and interventional procedures aimed to reduce lymphatic drainage. Modalities to image central and peripheral lymphatics have advanced considerably. Genetic variants and subsequent targets that drive lymphatic maldevelopment have expanded the repertoire of possible pharmacotherapeutic options.

Imaging Findings of Thoracic Lymphatic Abnormalities.

The lymphatic system plays an important role in balancing fluid compartments in the body. It is disrupted by various disease processes in the thorax, including injury to the thoracic lymphatic duct after surgery, as well as malignancy and heart failure. Because of the small size of lymphatic vessels, imaging of the lymphatics is relatively difficult, and effective imaging methods are still being optimized and developed. The standard of reference for lymphatic imaging has been conventional lymphangiography for several decades. Other modalities such as CT, noncontrast or contrast-enhanced MRI, and lymphoscintigraphy can also demonstrate lymphatic abnormalities and help in treatment planning. Imaging findings associated with lymphatic abnormalities can be seen in the pulmonary parenchyma, pleural space, and mediastinum. In the pulmonary parenchyma, common findings include interlobular septal thickening as well as reversal of lymphatic flow with intravasation of contrast material into pulmonary lymphatics. In the pleural space, findings include chylous pleural effusion and occasionally nonchylous pleural effusion. In the mediastinum, thoracic duct leak, plexiform thoracic duct, lymphatic malformations, and lymphangiectasis may occur. Management of chylothorax includes conservative or medical treatment, surgery, and interventional radiology procedures. The authors discuss thoracic lymphatic anatomy, imaging manifestations of lymphatic abnormalities in the various anatomic compartments, and interventional radiology treatment of chylothorax. Radiologists should be familiar with these imaging findings for diagnosis and to help guide appropriate management. ©RSNA, 2022.

Congenital Chylothorax of the Newborn: A Systematic Analysis of Published Cases between 1990 and 2018.

BACKGROUND: Congenital chylothorax (CCT) of the newborn is a rare entity but the most common cause of pleural effusion in this age-group. We aimed to find the optimal treatment strategy. MATERIAL AND METHODS: A PubMed search was performed according to the PRISMA criteria. All cases were analyzed according to prenatal, perinatal, and postnatal treatment modalities and follow-ups. RESULTS: We identified 753 cases from 157 studies published between 1990 and 2018. The all-cause mortality rate was 28%. Prematurity was present in 71%, male gender dominated 57%, mean gestational age was 34 weeks, and birth weight was 2,654 g. Seventy-nine percent of newborns had bilateral CCT, the most common associated congenital anomalies with CCT were pulmonary lymphangiectasia and pulmonary hypoplasia, and the most common chromosomal aberrations were Down, Noonan, and Turner syndromes, respectively. Mechanical ventilation was reported in 381 cases for mean 17 (range 1-120) days; pleural punctuations and drainages were performed in 32% and 64%, respectively. Forty-four percent received total parenteral nutrition (TPN) for mean 21 days, 46% medium-chain triglyceride (MCT) diet for mean 37 days, 20% octreotide, and 3% somatostatin; chemical pleurodesis was performed in 116 cases, and surgery was reported in 48 cases with a success rate of 69%. In 462 cases (68%), complete restitution was reported; in 34 of 44 cases (77%), intrauterine intervention was carried out. CONCLUSION: Respiratory support, pleural drainages, TPN, and MCT diet as octreotide remain to be the cornerstones of CCT management. Pleurodesis with OK-432 done prenatally and povidone-iodine postnatally might be discussed for use in life-threatening CCT.

Refractory thrombocytopenia could be a rare initial presentation of Noonan syndrome in newborn infants: a case report and literature review.

BACKGROUND: Noonan syndrome (NS) is a relatively rare inherited disease. Typical clinical presentation is important for the diagnosis of NS. But the initial presentation of NS could be significant variant individually which results in the difficult of working diagnosis. Here we report a rare neonatal case of NS who presented with refractory thrombocytopenia as the initial manifestation. CASE PRESENTATION: This was a preterm infant with refractory thrombocytopenia of unknown origin transferred from obstetric hospital at 6 weeks of age. During hospitalization, typical phenotypes of NS in addition to thrombocytopenia were observed, such as typical facial characteristics, short stature, atrial septal defect, cryptochidism, coagulation defect and chylothorax. Genetic testing showed a pathogenic variant at exon 2 of the PTPN11 gene with c.124A > G (p.T42A). Respiratory distress was deteriorated with progressive chylothorax. Chest tube was inserted for continuous draining. Chemical pleurodesis with erythromycin was tried twice, but barely effective. Finally, parents decided to withdraw medical care and the patient died. CONCLUSIONS: Thrombocytopenia could be the first symptom of Noonan syndrome. After ruling out other common causes of thrombocytopenia, NS should be considered as the working diagnosis.

Lymphatic anomalies in congenital heart disease.

Congenital heart disease can lead to various lymphatic complications including traumatic leaks, lymphatic overproduction, conduction abnormalities or lymphedema. Advancements in the imaging of central lymphatics and guided interventions have improved outcomes in these children. Dynamic contrast-enhanced magnetic resonance (MR) lymphangiography allows for the assessment of abnormal lymphatic drainage. This technique is preferred for evaluating lymphatic conditions such as plastic bronchitis, chylothorax, chyloptysis, chylopericardium, protein-losing enteropathy and chylous ascites, among other lymphatic disorders. In this review, we discuss lymphatic abnormalities encountered on MRI in children with congenital heart disease. We also briefly review treatment options.

Prenatal and Postnatal Management of Intrauterine Pleural Effusions Associated with Nonimmune Hydrops Fetalis.

OBJECTIVE: Nonimmune hydrops fetalis (NIHF) is defined as the accumulation of excess fluid in two or more body cavities in the fetus without blood incompatibility between mother and baby. We aimed to present our prenatal and postnatal management of intrauterine pleural effusions associated with NIHF. STUDY DESIGN: A total of 60 patients diagnosed with NIHF with intrauterine pleural effusion were analyzed retrospectively. Gestational age of delivery or fetal demise, the intrauterine treatment procedure including extrauterine intrapartum treatment (EXIT), chest tube, and medical treatment methods in fetuses with chylothorax analyzed. RESULTS: Thirty-nine patients (65%) were born alive between 26 and 38 weeks. A thoracoamniotic shunt was placed in one patient during the intrauterine period. Seven patients were placed bilaterally during the postnatal period, all without the umbilical cord being clamped during delivery. But 25 patients died within the first few days following birth. A total of four patients had chylothorax. Two patients who did not respond to medical treatment (somatostatin) were injected with thoracic local batticon and cured. A total of 14 patients were discharged with healing. CONCLUSION: Cases of progressive prenatal pleural effusions associated with NIHF have a high risk for fetal and neonatal death. We think that extreme prematurity increases postnatal mortality because it negatively affects the development of the lung and heart. A close obstetric follow-up and a multidisciplinary approach are required for the management to be selected.