Accumulation of chylous fluid in the pleural space.

Two main types

• congenital chylothorax [MIM 603 523]: 1/7,000 to 10,000 births, often associated to a hydrops fetalis

·        as a result of a lymphatic malformation in the lungs: lymphangomatosis or lymphangiectases

·        associated with a syndrome with lymphangiectases: Turner, Noonan, trisomy 21, Gorham-Stout,  X-linked myotubular myopathy, yellow nail syndrome

·        mutation of a9ß1 integrins

• acquired chylothorax due to:

·        cervicothoracic surgery with an injury of the thoracic duct

·        cardiac surgery

·        thrombosis of the superior vena cava (especially after the placement of a central venous catheter)

·        Fontan surgical procedure

·        injury of the left thoracic duct during the placement of a catheter into the subclavian vein

·        tumor of the mediastinum

·        mediastinal lymphadenopathy

·        open or closed chest trauma

Diagnosis: milky pleural fluid (photo A), which divides into 3 layers after settling: an upper creamy layer that contains chylomicrons, an intermediate milky layer and a lower layer rich in cells. However, this milky aspect may not be present if the patient has not eaten recently (photo B)

Composition of the chyle: electrolytic content similar to plasma, > 1000 cells per L, including 80% of lymphocytes, presence of chylomicrons and > 110 mg / 100 ml of triglycerides.

Treatment:

-        drainage (sometimes antenatal in congenital forms)

-        enteral feeding with medium-chain triglycerides (not transported by the chylomicrons)

-        parenteral nutrition and continuous infusion of octreotide (0.3 to 1 µg/kg/h) of somatostatin (3.5 to 10 µg/kg/h) or etilefrine

-        in case of failure: thoracic duct ligation, pleurodesis (iodine povidone, 10 % if skin solution or 4% if scrub solution), temporary pleuro-peritoneal shunt

Anesthetic implications:

respiratory distress, hypoalbuminemia, malnutrition, depression of immunity (lymphopenia), sometimes increased risk for venous thrombosis

References :

-        Wheeler AD, Tobias JD.
Tension chylothorax in two pediatric patients.
Pediatr Anesth 2007; 17:488-91.

-        Wu ET, Takeuchi M, Imanaka H et al.
Chylothorax as a complication of innominate vein thrombosis induced by a peripherally inserted central catheter.
Anaesthesia 2006; 61:584-6.

-        Downie L, Sasi A, Malhotra A.
Congenital chylothorax : associations and neonatal outcomes.
J Paediatr Child Health 2014 ; 50 : 234-8.

-        Hmami F, Oulmaati A, Bouchilkhi C et al.
Chylothorax congénital : réponse rapide et totale à la polyvidone iodée.
Arch Pédiatr 2014 ; 21 : 1002-5.

-        Tutor JD.
Chylothorax in infants and children.
Pediatrics 2014 ; 133 : 722-33

Updated: February 2017