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Laparoscopic Splenectomy with Pericardial Devascularization for Hypersplenism and Esophageal Variceal Hemorrhage Due to Portal Hypertension
04:00

Laparoscopic Splenectomy with Pericardial Devascularization for Hypersplenism and Esophageal Variceal Hemorrhage Due to Portal Hypertension

Published on: November 15, 2024

Concurrent splenic peliosis and vascular Ehlers-Danlos syndrome

Arianne C van Bon1, Jón O Kristinsson, J H J M van Krieken

  • 1Department of Gastroenterology and Hepatology, Radboud University Medical Center, Nijmegen, The Netherlands.

Annals of Vascular Surgery
|September 23, 2008
PubMed

This case report describes concurrent splenic peliosis and vascular Ehlers-Danlos syndrome (EDS) in a 59-year-old male patient. After splenic rupture due to peliosis, the complicated postoperative period hinted at the possibility of vascular EDS. This diagnosis was confirmed by genetic testing, which revealed a novel point mutation in the COL3A1 gene, c.2545G-->C, leading to a codon encoding for arginine instead of glycine (p.Gly849Arg). In addition, a histological diagnosis of splenic peliosis could be established.

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