Idiopathic congenital chylothorax treated with octreotide

Abstract

Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects.

Low Dose of Octreotide Can be Helpful in the Management of Congenital Chylothorax

“…After thoracentesis the baby had all criteria for diagnosis of CC. (Triglyceride content ≥ 1.13 mmol/l with a cell count ≥ 1000 cells/µL (80% or more lymphocytes)) ( 2 ) All options of therapeutic approaches for management of CC usually begin with supportive and conservative methods. Ligation of thoracic duct, pleural absorption, and pleurodesis as the surgery methods can be useful when all medical and conservative methods fail to reduce the pleural effusion and treatment of CC ( 4 ), conservative methods include long hospitalization, thoracentesis and insertion of chest tube, total parenteral nutrition, and introduction of a MCT diet ( 1 , 4 ).…”

“…Intravenous infusion is necessary for somatostatin while octreotide can be used subcutaneously or intravenously ( 1 ). Table 1 shows the studies that have previously used octreotide for the treatment of CC ( 2 - 4 , 7 - 16 ). Based on this table , octreotide has been successfully used for the treatment of CC in some studies; however, it should be taken into account that this treatment method cannot be applied in all cases of CC.…”

“…A rare condition in newborns called congenital chylothorax (CC) occurs when lymphatic fluid accumulates within the pleural cavity ( 1 ). Diagnosis of CC is confirmed when analysis of pleural fluid shows a triglyceride content equal to or more than 1.13 mmol/l with a cell count equal to or more than 1000 cells/µL (80% or more lymphocytes) ( 2 ). In the post natal period, supportive methods like ventilator care, immediate drainage of effusion, and total parenteral nutrition (TPN) must be considered as first line treatment.…”

“…In the post natal period, supportive methods like ventilator care, immediate drainage of effusion, and total parenteral nutrition (TPN) must be considered as first line treatment. Recently, using different doses of octreotide with different treatment responses was reported in some case reports ( 2 , 3 ). Surgical methods can be useful as a final option when using those managements could not help ( 4 ).…”

Rare case of congenital chylothorax and challenges in its management

“…Other cases have found octreotide to be helpful. In one case where total parenteral nutrition had failed, 16 days of octreotide treatment is reported to have resulted in full resolution of the chylothorax 13. One additional case reported a patient to have received octreotide for 151 days, resulting in resolution of the chylothorax 14.…”