Severe hypertriglyceridemia, acute pancreatitis and chyloptysis

Hypertriglyceridemia is an important cause of acute pancreatitis and a level of >1000 mg/dl has been strongly associated with acute pancreatitis. Severe hypertriglyceridemia can be associated with lipemia retinalis, lipemic serum (Creamish-yellowish colored serum). Treatment of severe hypertriglyceridemia induced pancreatitis includes IV fluids, IV Insulin, IV Heparin and plasmapheresis. Chyloptysis is mostly seen in cases of abnormal lymphatics (Lymphangiectasis, Behcet’s disease) or thoracic duct abnormalities/obstruction (LAM, Trauma). Acute pancreatitis is an inflammatory disease of the pancreas associated with varying degrees of autodigestion, edema, necrosis and hemorrhage of the pancreatic tissue. Hypertriglyceridemia is considered to be the third most common cause of acute pancreatitis and a serum level >1000 mg/dl has been strongly associated with acute pancreatitis.1 However, finding triglycerides (TG) in the bronchial secretions (chyloptysis) in association with hypertriglyceridemia associated pancreatitis is extremely rare and has never been reported. A 52-year-old African-American male with a past medical history of hypertension and hyperlipidemia was admitted to the hospital with epigastric pain and nausea for two days. He reported drinking 4–5 12-ounce cans of beer a week. His physical examination was notable for guarding and tenderness in the epigastric region. His initial diagnostic workup is summarized in Table 1. Initial laboratory work up Initial laboratory work up Computed tomography (CT) of the abdomen showed diffuse inflammatory stranding around the pancreas concerning for pancreatitis with no evidence of gallstones or biliary sludge. He denied any history of gallstones, pancreatitis, new medications, or recent abdominal procedures. The patient was diagnosed with hypertriglyceridemia-induced pancreatitis. He was treated with intravenous fluids, insulin and heparin drip and therapeutic plasmapheresis was initiated (Figure 1a). His course was complicated by acute hypoxic respiratory failure requiring mechanical ventilation. While being mechanically ventilated, he was noted to have yellowish-brown secretions from the endotracheal tube which looked similar to his dialysate fluid (Figure 1b) and tested positive for TG. To confirm, bronchoscopy with bronchoalveolar lavage (BAL) was done and the fluid analysis showed a triglyceride level of 98 mg/dl. Over the next few days; he received three sessions of therapeutic plasmapheresis and his TG levels dropped to <500 mg/dl and his yellowish-brown secretions diminished and eventually resolved. The patient was eventually extubated. During his 3-month follow-up, he reported no recurrence of chyloptysis and his lipid panel showed TG levels of 134 mg/dl. Post dialysate fluid. Chyloptysis, or the expectoration of triglyceride-rich sputum is mostly seen in cases of intrinsic or extrinsic thoracic duct obstruction (lymphangioleiomyomatosis, lymphangioma, malignancy or iatrogenic from trauma). It can also be seen in cases of abnormal lymphatics (lymphangiectasis) or incompetent lymphatic valves (lymphatic dysplasia, Behcet’s disease) and in cases with high venous pressure such as untreated heart failure.2 Chyle from the lymphatic system may enter the airways when the lymphatic circulation is impaired by inflammation, malignancy or abnormal proliferation of smooth muscle cells. This impairment results in retrograde flow of chyle into the peri-bronchial lymphatics. With this circulatory overload in peri-bronchial lymphatics, chyle and lymph fluid ruptures through bronchial mucosa and enters bronchial lumen producing chyloptysis.3 We believe that the cause of chyloptysis in our case was a combination of peri-bronchial lymphatics circulatory overload caused by extreme hypertriglyceridemia, systemic vascular inflammation caused by pancreatitis and increased intrathoracic pressure from mechanical ventilation. In normal subjects, cholesterol, TG and fatty acids are absent in BAL fluid.4 Chyloptysis in adults should prompt a workup for evidence of thoracic duct obstruction. In the present case, the patient did not have any history of trauma, radiation or any malignancy. A CT abdomen did not show any intra-abdominal or intrathoracic masses which compressed or infiltrated the thoracic duct. His isolated event of chyloptysis that resolved after multiple sessions of apheresis which supports our hypothesis. To conclude, chyloptysis is a rare clinical entity and according to one report published in 2012, there were only 11 reported cases of chyloptysis in literature.5 During our literature review, we found 12 additional reported cases of chyloptysis from 2012 to 2018. Although Hypertriglyceridemia is a known cause of acute pancreatitis, its association with chyloptysis in the absence of thoracic duct abnormality or lymphatics is rare and has never been reported in the literature. Conflict of interest. None declared.