%0 Journal Article
%T Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
%A Anna K. Ermarth
%A Brittany Esty
%A Jessica K. Sempler
%A John C. Carey
%A John Pohl
%A Molly A. O¡¯Gorman
%J Archive of "ACG Case Reports Journal".
%D 2016
%R 10.14309/crj.2016.159
%X We report the first observation of a patient with contgenital chylous ascites (CCA) and Ehlers-Danlos syndrome type VI due to primary lymphatic defect with additional vascular anomaly. CCA is a rare condition, and there is limited understanding of its pathophysiology and treatment options. We also review the patient¡¯s treatment course mitigated with octreotide and total parenteral nutritional support, as there are no current established guidelines for CCA. Early recognition of possible association with Ehlers-Danlos syndrome is important for quick intervention and successful management of pediatric patients
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5226192/