Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI | Zendy

Anna Ermarth | Zendy; John F. Pohl | Zendy; Brittany Esty | Zendy; Jessica Sempler | Zendy; John C. Carey | Zendy; Molly O’Gorman | Zendy

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Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI

Author(s) -

Anna Ermarth,

John F. Pohl,

Brittany Esty,

Jessica Sempler,

John C. Carey,

Molly O’Gorman

Publication year - 2016

Publication title -

acg case reports journal

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.112

H-Index - 4

ISSN - 2326-3253

DOI - 10.14309/crj.2016.159

Subject(s) - medicine , ehlers–danlos syndrome , chylous ascites , octreotide , ascites , pediatric surgeon , lymphatic system , intervention (counseling) , pathophysiology , intensive care medicine , surgery , pathology , pediatric surgery , somatostatin , psychiatry

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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