Primary chylopericardium: A case report and literature review

Abstract

Abstract. Primary chylopericardium (CP) is a rare clinical condition in which chylous fluid containing high concentrations of triglyceride accumulates in the pericardial cavity. The present study reports a case of CP that was successfully treated by reconstruction surgery of thoracic duct. To improve the ability to diagnosis and treat this rare disease, the current study also systematically extracted 104 reported cases of primary or idiopathic CP from the past 60 years (January 1950 to December of 2015), and reviewed the clinical manifestation, etiology, diagnosis and treatment of these cases. The age at diagnosis varied between 6 weeks and 79 years with a mean age of 27.95±16.50 years. Asymptomatic patients accounted for 39.42% of cases, while the most common initial symptoms were dyspnea (44.23%) and coughing (10.58%). Jugular venous distention and distant heart sound was identified in 23 (22.12%) and 34 cases (32.69%), respectively. Cardiomegaly in X-ray scans was detected in the majority of patients (93.27%). In addition, lymphoscintigraphy and lymphangiography were helpful in identifying the source of chyle. Regarding the etiology, idiopathic cases accounted for 35.56% of the included cases in the present study. The most important cause of primary CP was abnormal connection or accumulation of lymph fluid in the pericardium (37.50%). Conservative therapy included low-fat or medium-chain triglyceride diet, as well as total parenteral nutrition. The majority of patients (71.2%) required surgery for definitive therapy, and thoracic duct ligation was the most preferred surgical procedure, performed in 44.23% of cases. Follow-up was reported in 64 cases, and all patients survived during the mean follow-up period of 12 months. Therefore, it is suggested that surgical management is the most successful treatment method and is associated with a favorable prognosis.

Idiopathic Chylopericardium: A case Report and Breif Review of Literature

“…In Yu et al study, 42 (40.38%) cases underwent lymphangioscintigraphy and 24 of them had abnormal accumulation within the pericardial sac. Among 24 cases of their series who underwent lymphangiography, 18 had abnormal findings [6]. In our study, there was no abnormal communication between the thoracic duct and pericardium.…”

“…CP occurs in all ages more dominantly in children and young adults [1]. The mean age in C. Dib study was about 36.78 ± 14.71 years with no gender specificity [1,2] and 27.95 ± 16.50 years in Yu et al study with 5 cases younger than 3 years [6]. The disease can be diagnosed in asymptomatic patients referred for annual health examination showing a cardiomegaly or detected by dyspnea, chest pain or cough [1].…”

“…C. Dib et al reported 33 patients with CP in a large cohort of 10 years [2], Yater et al found 3 cases among 100 non-traumatic patients with chylothorax [5]. The latest one in Beijing, China which analyzed 65 years of 92 English and Chinese studies reported 104 patients with CP in the literature [6]. CP occurs in all ages more dominantly in children and young adults [1].…”

“…Idiopathic CP is an exclusion diagnosis when no etiology is found. This has different possible explanations proposed by Dunn and reviewed by Yu: a) Function default of lymphatic valves that are connected to the thoracic duct and pericardium lymphatic vessel; b) lymphangectasia caused by elevated pressure in the thoracic duct; c) abnormal communication between the lymphatic vessel and the pericardial sac resulting in chylous reflux; d) congenital malformation [6]. The diagnostic is usually confirmed by pericardiocentesis, followed by cytology, chemistry and culture.…”

“…Furthermore, embolism of the thoracic duct is used in certain situation, and it is an alternative type of surgery, particularly for patients who refuse to be subjected or re-subjected to more invasive approaches or those who cannot tolerate anesthesia [6].…”