Cruveilhier-Baumgarten syndrome: anatomical and pathologic imaging of periumbilical venous network

Transcription

1 Cruveilhier-Baumgarten syndrome: anatomical and pathologic imaging of periumbilical venous network Poster No.: C-0442 Congress: ECR 2014 Type: Educational Exhibit Authors: J. Isogai, H. Sakamoto ; Asahi/JP, Tsukuba/JP Keywords: Veins / Vena cava, CT-Angiography, MR-Angiography, Shunts, Varices DOI: /ecr2014/C Any information contained in this pdf file is automatically generated from digital material submitted to EPOS by third parties in the form of scientific presentations. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do not in any way constitute or imply ECR's endorsement, sponsorship or recommendation of the third party, information, product or service. ECR is not responsible for the content of these pages and does not make any representations regarding the content or accuracy of material in this file. As per copyright regulations, any unauthorised use of the material or parts thereof as well as commercial reproduction or multiple distribution by any traditional or electronically based reproduction/publication method ist strictly prohibited. You agree to defend, indemnify, and hold ECR harmless from and against any and all claims, damages, costs, and expenses, including attorneys' fees, arising from or related to your use of these pages. Please note: Links to movies, ppt slideshows and any other multimedia files are not available in the pdf version of presentations. Page 1 of 25

2 Learning objectives 1. To demonstrate normal variants of the periumbilical venous system in macroscopic anatomy based on 20 dissected cadavers. 2. To discuss and illustrate various imaging of Cruveilhier-Baumgarten syndrome observed in 20 patients on reformatted CT or MR images. Background Cruveilhier-Baumgarten syndrome is characterized by the presence of the portosystemic collateral pathway between the recanalized paraumbilical vein and superficial or deep epigastric veins forming a network of dilated periumbilical veins giving a 'caput medusae' in cirrhosis patients with portal hypertension. The development of large recanalized paraumbilical vein with hepatofugal flow has been found to decrease esophageal varicose rupture and predispose to hepatic encephalopathy. An intraoperative mortality has been reported due to accidental disruption of unexpected varices, even in periumbilical varices, the true extent and complexity may be underestimated without explicit information about its course and size. Therefore a thorough understanding of vascular anatomy is essential for proper diagnosis. Findings and procedure details 1) Anatomy of the deep epigastric vein as a drainage of paraumbilical veins (Fig. 1) Paraumbilical draining veins are located immediately parallel to the urachus extending from the umbilicus to suprapubic or pericystic veins below the rectus abdominis muscle. These veins occasionally mimic urachal remnant, but anatomically differ from it by the presence of tortuous or bifurcated structures. Page 2 of 25

3 Fig. 1: Sagittal (a) and coronal oblique (b) reformatted CT images of the urachus (open arrows) and deep epigastric veins (arrows). References: Radiology, Asahi General Hospital - Asahi/JP 2) Classification of draining deep epigastric veins of paraumbilical flow in normal variants (Fig. 2) The most common pattern of drainage of paraumbilical veins is that of through the inferior epigastric veins into the external iliac veins. Page 3 of 25

4 Fig. 2: The diagram of classification of the deep epigastric vein as drainage veins of paraumbilical flow References: Radiology, Asahi General Hospital - Asahi/JP 3) Gross anatomy: Cadaveric and CT/MR imaging correlation at normal variants 3a) A connection between the deep epigastric vein and the inferior epigastric vein (Fig. 3) A cadaveric macroscopic exam displays the most common draining pathway of paraumbilical vein via the inferior epigastric vein in a high correlation with CT imaging. Page 4 of 25

5 Fig. 3: Anatomical reference at normal variants of macroscopic anatomy (a) and a coronal oblique reformatted CT image (b). A gross anatomy of deep epigastric veins (arrows) shows a drainage pattern of paraumbilical veins (yellow arrow) from the umbilicus (arrow head) through inferior epigastric veins (open arrows). References: Radiology, Asahi General Hospital - Asahi/JP 3b) A connection between the deep epigastric vein and the pubic vein (Fig. 4) Page 5 of 25

6 Fig. 4: Anatomical reference at normal variants of macroscopic anatomy (a), a coronal oblique reformatted CT image (b) and coronal MRI (c). A gross anatomy of the deep epigastric vein (arrows) shows a drainage pattern of paraumbilical veins (yellow arrows) from the umbilicus (arrow heads) through pubic veins (open arrows). Note that two normal-sized paraumbilical veins course downward to the umbilicus References: Radiology, Asahi General Hospital - Asahi/JP 4) The imaging of Cruveilhier-Baumgarten syndrome in a high correlation with cadaveric gross anatomy 4a) Cruveilhier-Baumgarten syndrome via the inferior epigastric vein (Fig. 5) The most common pathway of Cruveilhier-Baumgarten syndrome is a portosystemic collateral via deep epigasric veins into the inferior epigastric vein in a high correlation with cadaveric gross anatomy. Page 6 of 25

7 Fig. 5: Anatomical reference at mild (a) and moderate (b) case of CruveilhierBaumgarten syndrome. Coronal oblique reformatted CT images show dilated and tortuous paraumbilical veins (yellow arrows) and deep epigastric veins (arrows) draining from the umbilicus (arrow head) through inferior epigastric veins (open arrows). References: Radiology, Asahi General Hospital - Asahi/JP 4b) Cruveilhier-Baumgarten syndrome via the pubic vein (Fig. 6) Page 7 of 25

8 Fig. 6: Anatomical reference at mild (a) and moderate (b) case of CruveilhierBaumgarten syndrome. Coronal oblique (a) and axial CT (b) images show dilated deep epigastric veins (arrows) draining from the umbilicus (arrow heads) through pubic veins (open arrows). Note an umbilical hernia containing paraumbilical varices. References: Radiology, Asahi General Hospital - Asahi/JP 4c) Cruveilhier-Baumgarten syndrome via the vesical venous plexus (Fig. 7) Page 8 of 25

9 Fig. 7: Anatomical reference at Cruveilhier-Baumgarten syndrome. Coronal oblique reformatted CT image shows dilated paraumbilical veins (yellow arrow) and a periumbilical tuft draining from the umbilicus (arrow head) through vesical venous plexus (open arrows) References: Radiology, Asahi General Hospital - Asahi/JP 5) Ectopic umbilical portosystemic collateral related to omphalomesenteric duct anomalies 5a) A connection between the ileal branch of superior mesenteric vein and the inferior epigastric vein (Fig. 8) A rare portosystemic collateral via the umbilicus is a pathway connecting the superior mesenteric vein to the deep epigastric vein which courses through inferior epigastric veins into external iliac vein. We think it one of the spectrum of omphalomesenteric duct anomalies. Page 9 of 25

10 Fig. 8: Rare cases of portosystemic collateral via the umbilicus. Sagittal (a/c) and coronal oblique (b/d) reformatted CT images show that the ileal branch of superior mesenteric vein (yellow arrows) communicates via the umbilicus (arrow heads) with inferior epigastric veins (open arrows) through deep epigastric veins (arrows). References: Radiology, Asahi General Hospital - Asahi/JP 5b) A connection between the ileal branch of superior mesenteric vein and vesical venous plexus in the space of Retzius (Fig. 9) Page 10 of 25

11 Fig. 9: A rare case of portosystemic collateral via the umbilicus. Axial (a), sagittal (b) and coronal oblique (c) reformatted CT images show that the ileal branch of superior mesenteric vein (yellow arrows) communicates via the umbilicus (arrow heads) with vesical venous plexus (open arrows) in the space of Retzius through deep epigastric veins (arrows). References: Radiology, Asahi General Hospital - Asahi/JP 6) Cruveilhier-Baumgarten syndrome via the superficial epigastric vein (Fig. 10) Page 11 of 25

12 Fig. 10: Severe (a) and moderate (b) case of Cruveilhier-Baumgarten syndrome. Volume rendered non-contrast MR image (a) and coronal oblique reformatted CT images show giant recanalized paraumbilical varices (yellow arrows) extending into the systemic circulation through superficial epigastric veins (arrows). References: Radiology, Asahi General Hospital - Asahi/JP 7) Cruveilhier-Baumgarten syndrome via the internal mammary vein (Fig. 11) Less commonly, the paraumbilical flow cranially returns to the systemic circulation (the intercostal and azygos veins) via the substernal or internal mammary veins. Page 12 of 25

13 Fig. 11: A cephalic course of Cruveilhier-Baumgarten syndrome. Sagittal (a) and coronal oblique (b) reformatted CT images show that recanalized paraumbilical veins (yellow arrows) communicate via the substernal or internal mammary veins (open arrows) with intercostal and azygos veins. References: Radiology, Asahi General Hospital - Asahi/JP Images for this section: Page 13 of 25

14 Fig. 1: Sagittal (a) and coronal oblique (b) reformatted CT images of the urachus (open arrows) and deep epigastric veins (arrows). Page 14 of 25

15 Fig. 2: The diagram of classification of the deep epigastric vein as drainage veins of paraumbilical flow Page 15 of 25

16 Fig. 3: Anatomical reference at normal variants of macroscopic anatomy (a) and a coronal oblique reformatted CT image (b). A gross anatomy of deep epigastric veins (arrows) shows a drainage pattern of paraumbilical veins (yellow arrow) from the umbilicus (arrow head) through inferior epigastric veins (open arrows). Page 16 of 25

17 Fig. 4: Anatomical reference at normal variants of macroscopic anatomy (a), a coronal oblique reformatted CT image (b) and coronal MRI (c). A gross anatomy of the deep epigastric vein (arrows) shows a drainage pattern of paraumbilical veins (yellow arrows) from the umbilicus (arrow heads) through pubic veins (open arrows). Note that two normal-sized paraumbilical veins course downward to the umbilicus Page 17 of 25

18 Fig. 5: Anatomical reference at mild (a) and moderate (b) case of CruveilhierBaumgarten syndrome. Coronal oblique reformatted CT images show dilated and tortuous paraumbilical veins (yellow arrows) and deep epigastric veins (arrows) draining from the umbilicus (arrow head) through inferior epigastric veins (open arrows). Page 18 of 25

19 Fig. 6: Anatomical reference at mild (a) and moderate (b) case of Cruveilhier-Baumgarten syndrome. Coronal oblique (a) and axial CT (b) images show dilated deep epigastric veins (arrows) draining from the umbilicus (arrow heads) through pubic veins (open arrows). Note an umbilical hernia containing paraumbilical varices. Page 19 of 25

20 Fig. 7: Anatomical reference at Cruveilhier-Baumgarten syndrome. Coronal oblique reformatted CT image shows dilated paraumbilical veins (yellow arrow) and a periumbilical tuft draining from the umbilicus (arrow head) through vesical venous plexus (open arrows) Page 20 of 25

21 Fig. 8: Rare cases of portosystemic collateral via the umbilicus. Sagittal (a/c) and coronal oblique (b/d) reformatted CT images show that the ileal branch of superior mesenteric vein (yellow arrows) communicates via the umbilicus (arrow heads) with inferior epigastric veins (open arrows) through deep epigastric veins (arrows). Page 21 of 25

22 Fig. 9: A rare case of portosystemic collateral via the umbilicus. Axial (a), sagittal (b) and coronal oblique (c) reformatted CT images show that the ileal branch of superior mesenteric vein (yellow arrows) communicates via the umbilicus (arrow heads) with vesical venous plexus (open arrows) in the space of Retzius through deep epigastric veins (arrows). Page 22 of 25

23 Fig. 10: Severe (a) and moderate (b) case of Cruveilhier-Baumgarten syndrome. Volume rendered non-contrast MR image (a) and coronal oblique reformatted CT images show giant recanalized paraumbilical varices (yellow arrows) extending into the systemic circulation through superficial epigastric veins (arrows). Page 23 of 25

24 Fig. 11: A cephalic course of Cruveilhier-Baumgarten syndrome. Sagittal (a) and coronal oblique (b) reformatted CT images show that recanalized paraumbilical veins (yellow arrows) communicate via the substernal or internal mammary veins (open arrows) with intercostal and azygos veins. Page 24 of 25

25 Conclusion The majority of paraumbilical flow returns to the systemic circulation via one of the two inferior epigastric veins in a high correlation with the cadaveric anatomy. Radiologists must be aware of potentially expansile periumbilical venous system especially for interventional procedures to severe portosystemic shunts. Personal information 1 2 J. Isogai, H. Sakamoto 1 Dept. of Radiology, Asahi General Hospital, Asahi, Japan 2 Dept. of Health Science, Tsukuba collage of technology, Tsukuba, Japan References 1. Veins of the abdomen and pelvis : hepatic portal system. In:Williams PL, et al,eds. th Gray's anatomy. 38. New York, NY: 1999; Nadia JK, Erik GE (1998) Imaging of the Umbilicus and Periumbilical Region. RadioGraphics; 18: Moubarak E, Bouvier A (2012) Portosystemic collateral vessels in liver cirrhosis: a three-dimensional MDCT pictorial review. Abdom. Imaging; 37(5): Dahnert W. Radiology Review Manual, 6th Edition, 2007 p Kiyosue H, Ibukuro K, (2013) CT Anatomy of Drainage Routes of Gastric Varices: A Pictorial Review. RadioGraphics. 33 (1): Page 25 of 25