Congenital Chylothorax

Abstract

The objective of this paper is a retrospective study of all infants treated for congenital chylothorax at the Royal Children's Hospital (RCH), Melbourne, Australia and King Fahad National Guard Hospital (KFNGH), Riyadh, Kingdom of Saudi Arabia. The charts of all infants with congenital chylothorax admitted to RCH over a period of 13 years, June 1982-August 1994, and admissions to KFNGH over a 7-year period, June 1992-August 1998 inclusive, were reviewed including management outcome and complications. There were 19 infants, 13 from RCH and 6 from KFNGH; 11 females and 8 males. Three infants were managed antenatally. Fifteen infants presented immediately after birth. Seven were born with hydrops fetalis, 6 infants had syndromes and 10 infants were born prematurely. Regular infant feeding formula and/or breast milk were used successfully in 12 infants, while in 7 infants medium chain triglycerides (MCT) rich formula was used. Sixteen infants were mechanically ventilated with 75% of them ventilated for < or = 28 days. Fifteen infants received total parenteral nutrition (TPN), and in 80% for < or = 32 days. Hydropic infants had longer duration of mechanical ventilation and hospital stay with mean (range) of 33.9 (3-120) and 115 (23-225) days, respectively, compared with 18 (1-62) and 34.3 (14-88) days for nonhydropic infants. Five infants underwent surgery with failure in four. Sepsis and bronchopulmonary dysplasia were the main complications. The survival rate was 100% regardless of the mode of therapy. The prognosis of Isolated congenital chylothorax in term, and preterm infants is good even in the presence of hydrops. Breast milk and/or regular infant feeding formula should be used initially before proceeding to MCT-rich formula, which may be necessary in some cases. Surgery should be considered if conservative management of congenital chylothorax fails after 4-5 weeks.

Bilateral spontaneous chylothorax in a newborn and response to octreotide therapy

“…8,9 These procedures have their own complication and drawbacks as found in different studies. 7,10 Currently a more conservative approach like octreotide infusion has seen some success in the treatment of chylothorax. Octreotide is a long acting analogue of somatostatin which is preferred in treating gastrointestinal bleeding, intractable secretory diarrhea, pancreatitis, metastatic carcinoid syndrome and vaso active peptide secreting tumor.…”

Congenital chylothorax: current perspectives and trends

“…1 The most serious consequences of fetal chylothorax are pulmonary hypoplasia, congestive heart failure, and hydrops. 1,2,5 CC can be an isolated manifestation or may be associated with genetic conditions such as trisomy 21 (which is associated with 4.9% of isolated pleural effusions), monosomy X, 1,2 Turner, 4,6,7 and Noonan syndromes 4,6,7 (Table 1). Congenital lymphangiectasia can occur as part of these conditions.…”

“…It is a rare occurrence, estimated to affect 1 in 10,000 births, with a mortality rate ranging between 20% and 60%. [1][2][3][4] If chylothorax is associated with hydrops fetalis, mortality can be as high as 98%. 1 The most serious consequences of fetal chylothorax are pulmonary hypoplasia, congestive heart failure, and hydrops.…”

“…35 Fetuses with CC are at high risk for chromosomal abnormalities, and rapid diagnosis of fetal karyotype 36 and any other associated malformations is essential to guide further management and prognosis. Therapeutic measures during prenatal period include maternal dietary modification, 37 repeated thoracocentesis, 1,5,38 thoraco-amniotic shunting, 39 and pleurodesis with OK-432. [40][41][42][43][44] Maternal dietary management with lowfat, high medium-chain triglyceride (MCT) diet might help delay the need for thoracentesis in cases of fetal chylothorax.…”

How do we counsel patients carrying a fetus with pleural effusions?

“…It is the most common cause of pleural effusion in the neonatal period (8% of all cases of pediatric chylothorax over a 12-year period) 6,7 . Today, congenital hydrothorax has a perinatal mortality rate ranging from 15 to 30% 8 .…”