Newborn With 21 Trysomy and Chilous pleural Effusion

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 and often associated with hydropsfetalis. It can be idiopathic or may be associated with various chromosomal and other genetic abnormalities. It is important to identify infants with chylothorax, as there are specific issues that need to be addressed in the management of these patients. In the neonate, chylous effusion is a common cause of pleural effusions and 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 present a case of newborn with 21 trisomy who developed moderate RDS, chest X-ray and US reveal pleural effusion on right side, rapid intervention was made before deterioration, requiring intensive life-saving measures. We review the common manifestations of congenital chylotoraxes and emphasize the importance of early diagnosis and intervention in preventing devastating outcomes from this condition.


Case Presentation Prenatal and Birth Histories
Mom was a 35yo G2P2 who came into L&D at 37+5/7wks in active labor. She did not receive antenatal steroids or antibiotics. Serology: A+, RPR neg, Hep B neg, HIV neg, GBS status -unknown. It was spontaneous vaginal delivery of a male infant with trisomy 21, which was diagnosed antenatally. The pediatric team was called to a "code blue" for fetusbradycardia. At delivery, the infant had a HR>100/min, but no respiratory effort, and was limp and blue. He required PPV (& Oxygen) with Ambu Bag and color/tone improved. Apgar Scores were 2 and 7 at 1 and 5minutes, respectively. BW was 2800grams.

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 and often associated with hydropsfetalis. It can be idiopathic or may be associated with various chromosomal and other genetic abnormalities. It is important to identify infants with chylothorax, as there are specific issues that need to be addressed in the management of these patients. In the neonate, chylous effusion is a common cause of pleural effusions and 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 present a case of newborn with 21 trisomy who developed moderate RDS, chest X-ray and US reveal pleural effusion on right side, rapid intervention was made before deterioration, requiring intensive life-saving measures. We review the common manifestations of congenital chylotoraxes and emphasize the importance of early diagnosis and intervention in preventing devastating outcomes from this condition.

After 8hrs
Respiratory status remains unchanged: tachypnea, mild sternal retraction. X-ray: with no improvement; Chest US: effusion on right pleural space, covering almost all right lung; Chest tube was inserted; CT scan: no malignancy in mediastinum;

Conclusion
Congenital Chylothorax is a rare condition with multiple etiologies. Pleural fluid analysis can identify this condition when clinical suspicion exists. Conservative management is generally recommended in most cases with surgery being reserved for those that have a large or persistent leak or in those who become immunologically challenged or malnourished.