Tetra-amelia is a very rare genetic disorder, characterized by the complete absence of all extremities. Patients often have to be hospitalized because of pulmonary infection and associated abnormalities. In this patient group, venous access is possible by the insertion of either a peripheral intracath at the scalp/neck or a central venous catheter (CVC) at the internal jugular vein. Following central venous catheterization, many complications such as vessel and nerve injury, pneumothorax, hemothorax, cardiac tamponade, pulmonary embolism, arrhythmia, sepsis, and infection at the insertion site have been reported in the English literature. Chylothorax, a rare complication following central venous catheterization, can be decreased by preferring insertion of the CVC to the right internal jugular vein and using a flexible CVC of an appropriate length and gauge. Herein, insertion of CVC in a case with tetra-amelia and diagnosis and treatment of CVC-related chylothorax were summarized.

Keywords: Amelia, Central venous catheterization, Chylothorax, Total Parenteral Nutrition