An observational cohort study of patients with lymphatic anomalies complicated by effusions
Gaglia, Michelle Theresa
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This observational study was conducted to characterize those patients with lymphatic anomalies complicated by effusions, as well as factors that are associated with the occurrence of effusions and poor outcome. Furthermore, this research aimed to better describe the natural histories of patients presenting with effusions resulting from lymphatic disorders, including morbidity and mortality rates. Of a cohort of 230 registry patients, 145 registered patients suffering from lymphatic disorders who experienced at least one effusion during follow-up were eligible for inclusion in this study. Information was collected primarily from structured patient interviews, medical records, and interdisciplinary conference reviews and clinic visits at the Vascular Anomalies Center at Boston Children's Hospital. Among other characteristics, the age at presentation, the age at pulmonary presentation, concurrent symptoms and disease features were assessed. Of patients with effusions, 37% present with their first effusions within the first year of their lives. Of the deceased cohort of patients who presented with their first effusion in the first year of their lives, 66.67% also died within the first year of their lives. Overall mortality for those patients suffering from effusions was found to be 17% (25/145). Of the deceased cohort with a known cause of death, 53% died due to respiratory or pulmonary complications of effusions. The median number of years from effusion presentation until death was 2.04 years. These data collected implied effusions are a critical determinant of mortality in patients with disorders of the lymphatic system.