Ascites

Nowicki, Michaeł; P, Bishop

doi:10.1016/b978-1-4377-0774-8.10018-1

Cited by 1 publication

(2 citation statements)

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“…Congenital chylous ascites (CCA) is a rare diagnosis of the exudation of chyle into the abdomen that occurs prenatally or within the first 3 months of life 1,2. In children, neonatal or infant chyloperitoneum occurs most often as an iatrogenic disruption of the thoracic duct from surgery or mass effect 3-5.…”

Section: Introductionmentioning

confidence: 99%

“…In children, neonatal or infant chyloperitoneum occurs most often as an iatrogenic disruption of the thoracic duct from surgery or mass effect 3-5. CCA is rarer than iatrogenic causes, estimated at 4% of identifiable cases, and only 50% of CCA cases have identifiable organic causes 1,2,5. Ehlers-Danlos syndrome type VI (EDS-VI) is an autosomal recessive connective tissue disorder.…”

Section: Introductionmentioning

confidence: 99%

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Congenital Chylous Ascites and Ehlers-Danlos Syndrome Type VI

Ermarth

Pohl

Esty

et al. 2016

ACG Case Reports Journal

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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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