title: Congenital chylothorax in newborn with trisomy 21.
abstract: Neonatal chylothorax results from the accumulation of chyle in the pleural space and may be either congenital or an acquired condition. Congenital chylothorax is most likely due to abnormal development or obstruction of the lymphatic system. It is often associated with hydrops fetalis. It can be idiopathic or may be associated with various chromosomal anomalies including Trisomy 21, Turner syndrome, Noonan syndrome, and other genetic abnormalities. Congenital pulmonary lymphangiectasia and generalized lymphangiomatosis have also been reported to be associated with congenital chylothorax. Several case reports indicate that congenital chylothorax can recur in subsequent offspring, suggesting a possible underlying genetic etiology. It is important to identify infants with chylothorax, as there are specific issues that need to be addressed in the management of these patients. We present a case of newborn with trysomy 21 (trisomy 21 was diagnosed antenatally by amniocentesis with support of Association "Perinatology"), who developed moderate Respiratory Distress Syndrome, chest X-ray and US reveal pleural effusion on right side rapid intervention was made before deterioration, requiring intensive life-saving measures. In the neonate, chylous effusion is not a common cause of pleural effusions. It is characterized as an exudate because of the high protein and lipid content once the infant is fed. The fluid will be clear/yellow to slightly cloudy in the unfed state and will quickly become milky following feeding, as chylomicrons appear in the fluid. Lymphocytes predominate in the differential cell count of chyle. The volume of fluid output can be high, and management can be challenging. We review the common manifestations of congenital chylotoraxes and emphasize the importance of early diagnosis and intervention in preventing devastating outcomes from this condition.
authors.affiliation: Tbilisi State Medical University, G. Zhvania Pediatric Clinic, Department of Neonatal Intensive Care Unit, Georgia.
authors.forename: Kh
authors.initials: Kh
authors.lastname: Lomauri
mesh_terms: D002916:Chylothorax / Q000151:congenital* / Q000175:diagnosis / Q000503:physiopathology; D004314:Down Syndrome / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D007231:Infant, Newborn; D008171:Lung Diseases / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology; D008200:Lymphangiectasis / Q000151:congenital / Q000175:diagnosis / Q000503:physiopathology; D008297:Male; D010996:Pleural Effusion; D012127:Respiratory Distress Syndrome, Newborn / Q000175:diagnosis / Q000503:physiopathology*