title: Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI.
abstract: 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.
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Department of Pediatric Gastroenterology, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Anna K
authors.initials: AK
authors.lastname: Ermarth
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Department of Pediatric Gastroenterology, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: John
authors.initials: J
authors.lastname: Pohl
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Brittany
authors.initials: B
authors.lastname: Esty
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Jessica K
authors.initials: JK
authors.lastname: Sempler
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Department of Pediatric Genetics, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: John C
authors.initials: JC
authors.lastname: Carey
authors.affiliation: Departments of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah; Department of Pediatric Gastroenterology, University of Utah School of Medicine, Salt Lake City, Utah.
authors.forename: Molly A
authors.initials: MA
authors.lastname: O'Gorman