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weekly question 5/6/2022

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A one week-old, term infant develops tachypnea and hypoxemia with bilateral pleural effusions. Bilateral chest tubes are placed with drainage of approximately 30 ml/kg/day of fluid with 91% lymphocytes. The patient is treated with one week of bowel rest and total parenteral nutrition. This is followed by two weeks of octreotide infusion (up to 6 mcg/kg/hour). The chest tube output has not changed. The best next step is:

A Increase octreotide infusion to 12 mcg/kg/hour

B Instillation of bleomycin into the pleural spaces

C Thoracic duct ligation and bleomycin instillation into the pleural spaces

D Thoracic duct ligation and mechanical pleurodesis

E Thoracic duct ligation, pleurectomy and mechanical pleurodesis
 
correct answer
E Thoracic duct ligation, pleurectomy and mechanical pleurodesis

Severe congenital chlyothorax is a potentially lethal condition that requires careful management. In a term infant, a step-up approach of bowel rest and total parenteral nutrition (TPN) followed by octreotide infusion is reasonable. If there is no improvement after reaching the maximum dose of 6 mcg/kg/hour, then the patient should be brought to the operating room for thoracic duct ligation and pleurectomy. Other surgical options, such as chemical pleurodesis and/or thoracic duct ligation alone may be appropriate in patients with chylothorax due to thoracic duct injuries but are less effective in cases of congenital chylothorax. Furthermore, thoracic duct ligation, pleurectomy and pleurodesis is more effective than thoracic duct ligation and mechanical pleurodesis alone.

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Interesting, what do you mean by MCT diet?
An MCT (Medium-Chain Triglyceride) diet is a key treatment for chylothorax (lymph fluid in the chest), reducing lymphatic flow by providing fats absorbed directly into the bloodstream, bypassing the compromised lymphatic system, thereby promoting healing of the leak while ensuring adequate calories for recovery, often used before or with TPN (Total Parenteral Nutrition) or as an enteral nutrition (EN) strategy. This specialized, very low-fat diet replaces long-chain fats (LCTs) with MCTs, found in specialized formulas or skimmed milk, helping to decrease chyle production and allowing the thoracic duct to seal.
How it works
  • Bypasses Lymphatics: Unlike Long-Chain Triglycerides (LCTs) in normal fats, MCTs are absorbed directly into the portal vein, not the lymphatic system, reducing the load of chyle leaking into the chest.
  • Reduces Leakage: By lowering chyle production, it helps decrease the volume of fluid accumulating in the pleural space, encouraging healing.
  • Provides Energy: MCTs offer essential calories and energy, preventing malnutrition that can occur with traditional fasting or NPO (nothing by mouth) diets.
Diet specifics
  • Low LCT, High MCT: Focuses on foods rich in MCTs (like coconut oil, MCT oil supplements, or specific formulas) and severely restricts LCTs (found in butter, oils, nuts, fatty meats, whole milk).
  • Allowed Foods: Fruits, vegetables, white fish, skinless chicken breast, egg whites, low-fat dairy (skim milk), pasta, rice, and specialized formulas for infants.
  • Foods to Avoid: Nuts, seeds, avocado, fatty meats, whole dairy, butter, oils, and fried foods.
Implementation
  • First-line Treatment: Often used as a primary management step for chylothorax, particularly after surgery.
  • Formula Options: Can involve specialized low-fat, high-MCT infant formulas or, for older patients, a tailored low-LCT diet with MCT supplementation.
  • Success Rate: Highly effective, with studies showing success rates over 70-90% in resolving chylothorax, leading to the removal of drainage tubes.
 
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