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: A Tangled Road to Definitive Diagnosis of Non-Hodgkin Lymphoma.

BACKGROUND Chylothorax is a rare condition caused by the leak of chyle into the pleural cavity. Malignancy, especially advanced lymphomas, are the most common non-traumatic causes of chylothorax. When thoracentesis and the following pleural effusion studies reveal the fluid to be a chyle, it is important to look at the patients' history and understand the possible etiological factors, as the appropriate management can differ. In some instances, the true reason behind the chylothorax can be a diagnostic challenge, as presented in this case. CASE REPORT We report a case of a patient in her 70s presenting with progressive dyspnea at rest and non-productive cough. A chest X-ray showed subtotal right pleural effusion that was revealed to be a chylothorax. A CT scan was performed and revealed mediastinal, abdominal, and retroperitoneal lymphadenopathy, that, compared to the CT results 6 years ago, when for the first time enlarged lymph nodes were discovered by thyroid ultrasound, was without any progression. Initial diagnostic tests were inconclusive, and the goal was to rule out other differential diagnoses while maintaining a minimally invasive diagnostic approach. A video-assisted thoracoscopic surgery with mediastinal lymph node dissection and biopsy led to a diagnosis of follicular lymphoma. CONCLUSIONS This clinical case highlights not only an uncommon follicular lymphoma complication but also is an example of a diagnostic challenge due to certain clinical features being misleading from the true cause of the chylothorax. After a wide variety of investigations were applied, the patient was finally diagnosed with non-Hodgkin lymphoma. Successful treatment led to a full metabolic remission.

Catheter-related superior vena cava obstruction: A rare cause of chylothorax in maintenance hemodialysis patients.

With the extensive use of dialysis catheters in patients undergoing hemodialysis, superior vena cava (SVC) syndrome has gradually attracted attention in recent years. Chylothorax caused by SVC syndrome is rarely reported. In this paper, we report a case of chylothorax secondary to superior vena cava obstruction (SVCO) in a maintenance hemodialysis patient after multiple dialysis catheter placements. Relieving the SVCO through intravascular intervention could effectively treat chylothorax. In the past fourteen months, no recurrence of symptoms has been observed.

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.

Clinical features and outcomes of congenital chylothorax: a single tertiary medical center experience in China.

OBJECTIVE: Congenital chylothorax (CC) is an uncommon congenital disease. The objective of this study was to analyze the clinical features, treatment, and outcome of infants with CC in a Chinese tertiary medical center. METHODS: CC was defined as a non-traumatic pleural effusion with ≥ 80% lymphocytes detected before birth or within 28 days after birth. Clinical data were collected in CC infants discharged from June 2017 to March 2021. RESULTS: A total of 24 CC infants were discharged during the study period, accounting for 67% of congenital pleural effusions. The median gestational age at birth was 36+4 weeks (range 29+5-41 weeks) and the birth weight was 3025 g (range 1850-4250 g). Twenty-one infants were diagnosed antenatally. The median gestational age at the time of diagnosis was 30+3 weeks (range 24-36+6 weeks). Nine infants presented with hydrops fetalis; 18 were bilateral. Prenatal interventions were performed in 13 fetuses. Nine infants (38%) had birth asphyxia. Compared with the infants without hydrops fetalis, the infants with CC and hydrops fetalis had lower Apgar scores at 1 and 5 min (P < 0.05) and a lower gestational age at birth (P < 0.05). Postnatally, 17 infants required continuous pleural drainage for 10 days (range 2-30 days). Analysis of the pleural effusion showed a higher cell count, lymphocyte fraction, and protein content after enteral feeding (P < 0.05). Fifteen infants required mechanical ventilation; 9 did not require any respiratory support. Ten infants received a delayed feeding strategy and 17 received a medium-chain triglyceride (MCT) formula. Only 1 infant received octreotide therapy. Twenty-one infants survived and 3 died. The main cause of death was pulmonary dysplasia. The duration of hospital stay in survivors was 21.5 days (range 10-43) days. For infants with CC and hydrops fetalis, prenatal therapy shortened the duration of pleural drainage and the length of hospital stay (P < 0.05). CONCLUSION: CC is the most common cause of congenital pleural effusions. The poor prognosis is mainly associated with prematurity, hydrops fetalis, and pulmonary dysplasia. Prenatal intervention may improve the outcome of infants with hydrops fetalis.

Pulmonary lymphangiectasia in myotubular myopathy: a novel unrecognized association?

Chylothorax has been reported in rare cases of X-linked myotubular myopathy, but the pathophysiology of this association is not fully understood. We report a case of a neonate presenting prenatally with hydrops and chylothorax. The patient died at 17 days of life due to respiratory failure secondary to severe pulmonary hypertension. Comprehensive genetic testing identified a de novo hemizygous frameshift mutation in the MTM1 gene (c.142-143del, p.Glu48Serfs*12) with subsequent autopsy confirming the diagnosis of X-linked myotubular myopathy. Lung microscopy demonstrated primary pulmonary lymphangiectasia as the cause for the massive chylothorax. To the best of our knowledge, this is the first reported case of molecularly confirmed X-linked myotubular myopathy with pulmonary lymphangiectasia with prenatal findings of hydrops, chylothorax and postnatal severe pulmonary hypertension.

Gorham Stout disease: 3 additional cases with 2 very rare polyostotic diseases.

Gorham Stout disease is a very rare monostotic or polyostotic osteolysis and physiopathology of the osteolysis is not yet fully understood. Three new cases are reported with their evolution and treatment. Among these 3 cases, two are very rare cases of polyostotic involvement. One patient finally deceased from respiratory complications despite limb amputation. The two others are alive. Both needed final reconstruction with massive bone allograft for one and with a prosthesis for the other. Monostotic osteolysis is the most frequent presentation of Gorham Stout disease and extensive polyostotic osteolysis is very rare. Treatment methods vary from one clinic to another, from drug treatment to surgical treatment with or without radiotherapy. Sometimes, as a last solution, an amputation of the affected limb is performed. The prognosis depends on the affected region and the reponse to various treatments. Chylothorax seems to be a factor of poor prognosis.

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.

CD56-positive Angioimmunoblastic T-cell Lymphoma Complicated by Chylothorax.

A 77-year-old woman presented with systemic lymphadenopathy and bilateral pleural effusion. Angioimmunoblastic T-cell lymphoma (AITL) was diagnosed based on the results of a lymph node biopsy. AITL cells expressed the aberrant antigen of CD56. The bilateral pleural effusion was attributed to chylothorax, not the infiltration of lymphoma cells into the pleura, as determined by the pleural fluid analysis. We therefore diagnosed her with CD56-positive AITL complicated by chylothorax. She achieved complete remission by multidrug chemotherapy. AITL is commonly complicated by pleural effusion, but rarely by chylothorax. This is the first case of CD56-positive AITL complicated by chylothorax.

Chylothorax as a complication of congenital myotonic dystrophy: A retrospective cohort study.

BACKGROUND: The association between congenital myotonic dystrophy (CMD) and chylothorax is poorly recognized. AIMS: To evaluate the proportion of chylothorax in infants with CMD compared to its prevalence in infants without CMD. STUDY DESIGN: Single-center, retrospective, cohort study. SUBJECTS: Infants managed at a neonatal intensive care unit from 2003 to 2019. OUTCOME MEASURES: CMD was diagnosed using Southern blot for the quantification of CTG repeats in the dystrophia myotonica protein kinase (DMPK) gene. Prenatal and postnatal data of eligible infants with CMD were collected from the patients' medical records. The primary outcome was the proportion of chylothorax. RESULTS: While the proportion of chylothorax in eligible infants without CMD was 0.25% (27/10714), that in infants with CMD was 50% (5/10; odds ratio (OR), 386.3; 95% confidence interval (CI), 84.4-1850.8; P < 0.001). The variables that were significantly different between infants with CMD with chylothorax and those without chylothorax were as follows: maternal age (33.0 vs. 24.8; P = 0.005), polyhydramnios (5/5 vs 1/5; P = 0.034), number of CTG repeats (1980 vs. 1500; P = 0.038), duration of invasive mechanical ventilation (220 vs. 2, P = 0.009), and duration of hospitalization (217.2 vs 68.0; P = 0.014). The mortality rate of all eligible infants with CMD was 5/10 (50%). No variables were significantly associated with mortality. CONCLUSIONS: The proportion of chylothorax in infants with CMD was significantly higher than that in infants without CMD. Furthermore, the proportion was higher than that reported in previous studies on congenital chylothorax. This suggests that chylothorax may be frequently experienced in the clinical course of CMD.

Midodrine, an Oral Alpha-1 Adrenoreceptor Agonist, Successfully Treated Refractory Congenital Chylous Pleural Effusion and Ascites in a Neonate.

A trisomy 21 neonate presented with congenital chylous pleural effusion and ascites that was refractory to conventional pharmacotherapy. Midodrine, an oral alpha-1-adrenoreceptor agonist, achieved remission of chylous effusion without any adverse effects. To the best of our knowledge, this is the first neonatal case of successful management of congenital chylous pleural effusion and ascites with midodrine.

Non-immune hydrops fetalis secondary to congenital chylothorax with diffuse interstitial lung disease: a diagnostic conundrum.

A Chinese male infant was born at 35 weeks weighing 2935 g to a mother with polyhydramnios and prenatal hydrops fetalis. He developed marked respiratory distress secondary to bilateral congenital chylothorax and required pleural drainage, high frequency oscillation and inhaled nitric oxide therapy. He was extubated to non-invasive ventilation by day 14. There was no bacterial or intrauterine infection, haematologic, chromosomal or cardiac disorder. He was exclusively fed medium-chain triglyceride formula. High-resolution CT showed diffuse interstitial lung disease. He received a dexamethasone course for chronic lung disease to facilitate supplemental oxygen weaning. A multidisciplinary team comprising neonatology, pulmonology, haematology, interventional radiology and thoracic surgery considered congenital pulmonary lymphangiectasia as the most likely diagnosis and advised open lung biopsy, lymphangiography or scintigraphy for diagnostic confirmation should symptoms of chylothorax recur. Fortunately, he was weaned off oxygen at 5 months of life, and tolerated human milk challenge at 6 months of life and grew well.

Nutritórax: una complicación infrecuente de la nutrición parenteral / Nutrithorax: an uncommon complication of parenteral nutrition

INTRODUCCIÓN: la nutrición parenteral (NP) es una modalidad de soporte nutricional con posibles complicaciones, en parte asociadas al catéter venoso central (CVC). El quilotórax consiste en el derrame de líquido linfático de origen intestinal en el espacio pleural. CASO CLÍNICO: varón de 57 años ingresado para colecistectomía. Presenta un postoperatorio complicado que requiere reposo digestivo y NP. Posteriormente presenta disnea y dolor torácico con derrame pleural bilateral y pericárdico. Inicialmente se interpretó como un quilotórax, por su aspecto lechoso y su contenido en triglicéridos. La TC confirmó la malposición del CVC con salida de NP a nivel del tronco venoso innominado. Fue intervenido quirúrgicamente, realizándose un lavado del mediastino anterior y la reparación de la perforación. La evolución posterior fue favorable. DISCUSIÓN: la extravasación de la NP al espacio pleural es una complicación infrecuente pero posible de la administración de NP por vía central. Por tanto, debe tenerse en cuenta en el diagnóstico diferencial

INTRODUCTION: parenteral nutrition (PN) is commonly used as a nutritional support option. It may cause complications, partly due to a central venous access. Chylothorax is an accumulation of lymphatic fluid in the pleural space. CASE REPORT: a 57-year-old man was admitted for cholecystectomy. A complicated postoperative period required PN. Cardiorespiratory symptoms started while receiving PN, and a bilateral pleural and pericardial effusion was identified. It was initially interpreted as chylothorax due to its milky appearance and high triglyceride content. A CT scan confirmed a malposition of the CVC with PN leakage at the level of the innominate venous trunk. It was surgically repaired. DISCUSSION: parenteral nutrition leakage is an unusual complication of PN. It should be included in the differential diagnosis of pleural effusion

Leche materna descremada en recién nacidos con quilotórax: opciones con una unidad de nutrición personalizada / Skimmed breast milk in newborns with chylothorax: Options with a personalised nutrition unit

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Successful thoracic duct embolisation in a child with recurrent massive pericardial effusion diagnosed as a lymphatic anomaly.

A 29-month-old girl had idiopathic massive pericardial effusion for over 6 months. Lymphangiography was performed for chronic and recurrent pericardial effusion and pulmonary lymphangiectasia, suspected based on CT findings. Magnetic resonance lymphangiography revealed chylolymphatic reflux from a tortuously dilated thoracic duct in the mediastinum to the pericardial space, suggesting primary chylopericardium with lymphangiectasia. Pericardial effusion resolved immediately after thoracic duct embolisation at the lower thoracic level. However, pericardial effusion recurred after 5 months, which resolved after additional embolisation of the abnormal lymphatic collateral vessels from the remnant upper thoracic duct. Here, we report an unusual case with chylous massive pericardial effusion diagnosed by magnetic resonance lymphangiography and treated with percutaneous embolisation.

Primary Mediastinal Large B-Cell Lymphoma in a Child Presenting With Superior Mediastinal Syndrome and Chylous Pleural and Pericardial Effusion.

Primary mediastinal (thymic) large B-cell lymphoma is an aggressive B-cell lymphoma. It comprises <3% of all pediatric non-Hodgkin lymphomas (NHLs). Primary mediastinal (thymic) large B-cell lymphoma usually presents with serous pleural effusion, but presentation with chylous pleural and pericardial effusions is rare. We present a child who presented with features of a superior mediastinal syndrome. Biopsy of the mediastinal mass confirmed the diagnosis of large B-cell lymphoma. In view of nonimprovement of respiratory distress with chemotherapy and persistence of features of superior mediastinal syndrome, the child was evaluated and found to have massive pleural and pericardial effusion on imaging. Therapeutic thoracentesis and pericardiocentesis revealed chylous nature of the fluid.