Congenital chylothorax: current perspectives and trends

Abstract

Congenital chylothorax (CC) is the most common cause of pleural effusion in the perinatal period. The etiology is unknown in the majority of the cases. However, in some cases, it can be associated with various syndromes and genetic conditions. CC is associated with a high mortality rate. Most of the clinical manifestations are secondary to pressure effects (pulmonary hypoplasia) and loss of protein and lymphatic fluid (hydrops, malnutrition). Conservative management in the neonatal period is effective in up to 80% of cases and includes pleural drainage, parenteral nutrition/enteral medium-chain triglyceride-based formulae, and medications such as octreotide. Surgical intervention (pleurodesis, thoracic duct ligation/embolization, pleuroperitoneal shunt) may be required in persistent cases. A universal consensus on management of CC is unavailable, and data on the safety of medication use for CC in neonates are sparse.

A case of congenital chylothorax

“…The mortality rate of CC ranges from 20-60% 1 . Iatrogenic trauma, malignancies and some granulomatous infections cause secondary chylothorax in infants and children 1,2 .…”

“…The mortality rate of CC ranges from 20-60% 1 . Iatrogenic trauma, malignancies and some granulomatous infections cause secondary chylothorax in infants and children 1,2 . Antenatally detected CC can act as a space occupying lesion interrupting normal lung development 3 .…”

“…Chylothorax is the commonest cause of pleural effusion encountered during the perinatal period 1 . Congenital chylothorax (CC) is rare with a prevalence of 1:10,000 1 .…”

“…Chylothorax is the commonest cause of pleural effusion encountered during the perinatal period 1 . Congenital chylothorax (CC) is rare with a prevalence of 1:10,000 1 . CC could be due to congenital lymphatic malformation and may be associated with syndromes 2 .…”

Octreotide Infusion for the Treatment of Congenital Chylothorax

“…The wide variance in reported mortality rates can be attributed to maturational age of the newborn, severity of the pleural effusion, and other associated factors [ 2 ]. Despite this, no guidelines have been unanimously established in the treatment and management of congenital chylothorax [ 3 ].…”