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Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI
Anna K Ermarth1, John Pohl1, Brittany Esty2
1Departments 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.
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.
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