Congenital chylous ascites is a rare cause of ascites in newborn infants. Its aetiology varies from localised leaky lymphatic duct to genetic syndromes. Most of these cases have transient ascites resolving over time with conservative management but some may progress needing medical as well as surgical treatment. We describe a case of antenatally detected large fetal ascites necessitating abdominal paracentesis and amnioreduction. Marked respiratory distress at birth required urgent abdominal paracentesis to relieve symptoms. The infant initially showed a good response to medium chain triglyceride (MCT) based formula milk feeds. Feeds were discontinued for 3 weeks due to sepsis with ileus. On recovery, recommencement of feeds resulted in reaccumulation of ascites. As the response to MCT-based formula was inadequate, octreotide therapy was initiated. Ascites showed remarkable resolution over the next 2 weeks and was discharged home. Follow-up at 5 years of age revealed normal growth and neurodevelopment.
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http://dx.doi.org/10.1136/bcr-2020-235849 | DOI Listing |
J Inflamm Res
November 2024
Pediatric Urology Department, Anhui Provincial Children's Hospital, Hefei, Anhui, People's Republic of China.
Background: Chylous effusion is a rare condition characterized by the accumulation of lymphatic fluid in body cavities, often due to trauma, malignancy, or congenital lymphatic abnormalities. The association of chylous effusion with a patent processus vaginalis (PV) in pediatric patients is exceptionally uncommon, presenting unique diagnostic and therapeutic challenges.
Objective: To report a rare case of bilateral patent processus vaginalis with chylous effusion in a pediatric patient, detailing the diagnostic process, surgical management, and outcomes, while contributing to the limited literature on this condition.
Front Pediatr
September 2024
Section of Neonatology and Pediatric Intensive Care, Department of Pediatrics, School VI-School of Medicine and Health Sciences, Carl von Ossietzky Universität Oldenburg, Oldenburg, Germany.
Background: Central conducting lymphatic anomaly (CCLA) is a heterogeneous disorder characterized by structural anomalies in the main collecting lymphatic vasculature. These anomalies result in chronic chylous leaks, causing issues such as congenital hydrothorax and potentially impairing the normal immune response. Recently, mutations in the MyoD family inhibitor domain-containing () gene have been identified as a cause of CCLA.
View Article and Find Full Text PDFChest
September 2024
Division of Respiratory, Critical Care, and Occupational Pulmonary Medicine, Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, UT.
Chylothorax, which accounts for 1% to 3% of pleural effusions, typically results from either surgery (traumatic) or underlying malignancy (nontraumatic). Less common causes of nontraumatic chylothorax are numerous and include congenital lymphatic abnormalities, connective tissue diseases, cirrhosis, and infection, among others. We describe what appears to be the first reported case of chylothorax caused by chylous ascites in Crohn disease.
View Article and Find Full Text PDFInvest Radiol
January 2025
From the Department of Radiology, Seoul National University Hospital, Seoul, Republic of Korea (S.L., S.H., Y.H.C., J.-Y.H., J.-E.C.); Department of Radiology, Seoul National University College of Medicine, Seoul, Republic of Korea (S.L., S.H., Y.H.C., J.-Y.H., J.-E.C.); and Institute of Radiation Medicine, Seoul National University Medical Research Center, Seoul, Republic of Korea (J.-E.C.).
Congenital lymphatic flow disorders collectively refer to a heterogeneous group of diseases that manifest as chylothorax, chylous ascites, intestinal lymphangiectasia, protein-losing enteropathy, and peripheral extremity or genital lymphedema, all in the absence of identifiable injury to the lymphatic system. We have only recently begun to understand congenital lymphatic flow disorders through the ability to image lymph flow dynamically. Intranodal dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is a crucial technique for imaging lymphatic flow in pediatric patients with congenital lymphatic flow disorders.
View Article and Find Full Text PDFJ Soc Cardiovasc Angiogr Interv
January 2024
Department of Cardiology, Jill and Mark Fishman Center for Lymphatic Disorders, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
The lymphatic system plays a central role in some of the most devastating complications associated with congenital heart defects. Diseases like protein-losing enteropathy, plastic bronchitis, postoperative chylothorax, and chylous ascites are now proven to be lymphatic in origin. Novel imaging modalities, most notably, noncontrast magnetic resonance lymphangiography and dynamic contrast-enhanced magnetic resonance lymphangiography, can now depict lymphatic anatomy and function in all major lymphatic compartments and are essential for modern therapy planning.
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