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Laparoscopic appearance of Cruveilhier-Baumgarten syndrome, dilated Sappey's veins, and early caput medusae
At the Focal Point
Kyosuke Tanaka, MD, Hideki Toyoda, MD, Ichiro Imoto, MD, Department of Endoscopic Medicine; Yasuhiko Hamada, MD, Masatoshi Aoki, MD, Ryo Kosaka, MD, Tomohiro Noda, MD, Yoshiyuki Takei, MD, Department
of Gastroenterology and Hepatology, Mie University School of Medicine, Tsu, Japan doi:10.1016/j.gie.2007.08.029
Commentary Inflammatory fibroid polyps (IFPs) occur throughout the GI tract but usually are seen in the colon, where they attend a variety of inflammatory disorders such as inflammatory bowel disease, and infectious diseases such as schistosomiasis, amebiasis, and ischemic colitis, among others. IFPs are formed either as an inflammatory mass or as an island of relatively spared mucosa that projects above surrounding ulcerated, and later re-epithelialized areas. Two special forms of IFPs in the colon are cap polyps and polyps that form at the sites of ureteral implantation. In the esophagus, IFPs are the consequence of GERD, and in the stomach IFPs usually occur in the antrum or prepyloric region and may be a source of bleeding or outlet obstruction. In the small intestine, IFPs usually occur in the ileum. IFPs have unique morphologic and immunohistochemical features, but on biopsy alone, the differential diagnosis may include sarcoma and other malignancies. The absence of mutational change may help to exclude malignant lesions. Just as the IFP in this patient shows, inflammatory polyps are often longer than they are broad, and in the extreme, exhibit a filiform appearance. To suspect that an observed lesion is an IFP, look for a polypoid lesion that occurs in the presence of an inflammatory disease, is longer than it is broad, and has a surface that may exhibit superficial areas of ulceration and mucus. Histology, immunochemistry, and EUS can help in establishing the diagnosis. Lawrence J. Brandt, MD Associate Editor for Focal Points
Laparoscopic appearance of Cruveilhier-Baumgarten syndrome, dilated Sappey’s veins, and early caput medusae
A 50-year-old man with diabetes mellitus and hepatitis B–related liver cirrhosis underwent investigation for GI bleeding of obscure origin. He presented with esophageal variceal bleeding 3 years earlier, and the varices were successfully obliterated by endoscopic therapy. He remained asymptomatic until recently, when he was found to be anemic and have occult blood in the stool. Signs of chronic liver disease were not evident on physical examination. EGD and colonoscopy did not reveal any source of bleeding. The results of a technetium-labeled red blood cell scan
and mesenteric angiography were unremarkable. A CTscan of the abdomen showed thickening of the proximal small bowel, but push enteroscopy did not show any mucosal lesions. Diagnostic laparoscopy was performed, and the small bowel was found to be normal. Interestingly, features of the Cruveilhier-Baumgarten syndrome, including a cirrhotic liver and patent umbilical (A) and dilated Sappey’s paraumbilical (B) veins, were seen at laparoscopy. Time will tell whether these features precede the development of radiologic or clinical caput medusae.
346 GASTROINTESTINAL ENDOSCOPY Volume 67, No. 2 : 2008
www.giejournal.org
At the Focal Point
DISCLOSURE The author reports that there are no disclosures relevant to this publication.
Kent-Man Chu, MB, BS, MS, FRCS(Ed), FACS, Division of Upper Gastrointestinal Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China doi:10.1016/j.gie.2007.08.013
Commentary Portal hypertension often produces a myriad of collateral vessels, which, in turn, are accompanied by a wealth of eponyms. Cruveilhier-Baumgarten either refers to a disease state in which the umbilical vein remains patent after birth, or a syndrome in which the umbilical vein is recanalized as a collateral because of portal hypertension (from cirrhosis). The sign of CruveilhierBaumgarten refers to the venous hum heard over the umbilicus, or a caput medusae that arises from hepatofugal flow through the recanalized umbilical vein. It is a tribute to the classical education of the early anatomists that they saw the snakes that made up Medusa’s hair in the tortuous ramifications of the collaterals surrounding the umbilicus. Sappey described the paraumbilical veins of the accessory portal system, which drain the falciform ligaments and surrounding areas to join the peripheral portal branches in the left hepatic lobe, the internal mammary veins, the diaphragmatic veins, and branches of the inferior epigastric veins around the umbilicus. What is the importance of all this? Only recognition that detection of such veins, whether by careful physical examination or by sophisticated imaging studies, means portal hypertension and probably cirrhosis. Examine the caput medusae carefully without fear of being turned to stone as humans were when they gazed upon the face of the chthonic Gorgon, Medusa. Lawrence J. Brandt, MD Associate Editor for Focal Points
An unusual gall bladder
www.giejournal.org
Volume 67, No. 2 : 2008 GASTROINTESTINAL ENDOSCOPY 347
Kyosuke Tanaka, MD, Hideki Toyoda, MD, Ichiro Imoto, MD, Department of Endoscopic Medicine; Yasuhiko Hamada, MD, Masatoshi Aoki, MD, Ryo Kosaka, MD, Tomohiro Noda, MD, Yoshiyuki Takei, MD, Department
of Gastroenterology and Hepatology, Mie University School of Medicine, Tsu, Japan doi:10.1016/j.gie.2007.08.029
Commentary Inflammatory fibroid polyps (IFPs) occur throughout the GI tract but usually are seen in the colon, where they attend a variety of inflammatory disorders such as inflammatory bowel disease, and infectious diseases such as schistosomiasis, amebiasis, and ischemic colitis, among others. IFPs are formed either as an inflammatory mass or as an island of relatively spared mucosa that projects above surrounding ulcerated, and later re-epithelialized areas. Two special forms of IFPs in the colon are cap polyps and polyps that form at the sites of ureteral implantation. In the esophagus, IFPs are the consequence of GERD, and in the stomach IFPs usually occur in the antrum or prepyloric region and may be a source of bleeding or outlet obstruction. In the small intestine, IFPs usually occur in the ileum. IFPs have unique morphologic and immunohistochemical features, but on biopsy alone, the differential diagnosis may include sarcoma and other malignancies. The absence of mutational change may help to exclude malignant lesions. Just as the IFP in this patient shows, inflammatory polyps are often longer than they are broad, and in the extreme, exhibit a filiform appearance. To suspect that an observed lesion is an IFP, look for a polypoid lesion that occurs in the presence of an inflammatory disease, is longer than it is broad, and has a surface that may exhibit superficial areas of ulceration and mucus. Histology, immunochemistry, and EUS can help in establishing the diagnosis. Lawrence J. Brandt, MD Associate Editor for Focal Points
Laparoscopic appearance of Cruveilhier-Baumgarten syndrome, dilated Sappey’s veins, and early caput medusae
A 50-year-old man with diabetes mellitus and hepatitis B–related liver cirrhosis underwent investigation for GI bleeding of obscure origin. He presented with esophageal variceal bleeding 3 years earlier, and the varices were successfully obliterated by endoscopic therapy. He remained asymptomatic until recently, when he was found to be anemic and have occult blood in the stool. Signs of chronic liver disease were not evident on physical examination. EGD and colonoscopy did not reveal any source of bleeding. The results of a technetium-labeled red blood cell scan
and mesenteric angiography were unremarkable. A CTscan of the abdomen showed thickening of the proximal small bowel, but push enteroscopy did not show any mucosal lesions. Diagnostic laparoscopy was performed, and the small bowel was found to be normal. Interestingly, features of the Cruveilhier-Baumgarten syndrome, including a cirrhotic liver and patent umbilical (A) and dilated Sappey’s paraumbilical (B) veins, were seen at laparoscopy. Time will tell whether these features precede the development of radiologic or clinical caput medusae.
346 GASTROINTESTINAL ENDOSCOPY Volume 67, No. 2 : 2008
www.giejournal.org
At the Focal Point
DISCLOSURE The author reports that there are no disclosures relevant to this publication.
Kent-Man Chu, MB, BS, MS, FRCS(Ed), FACS, Division of Upper Gastrointestinal Surgery, Department of Surgery, The University of Hong Kong, Queen Mary Hospital, Hong Kong, China doi:10.1016/j.gie.2007.08.013
Commentary Portal hypertension often produces a myriad of collateral vessels, which, in turn, are accompanied by a wealth of eponyms. Cruveilhier-Baumgarten either refers to a disease state in which the umbilical vein remains patent after birth, or a syndrome in which the umbilical vein is recanalized as a collateral because of portal hypertension (from cirrhosis). The sign of CruveilhierBaumgarten refers to the venous hum heard over the umbilicus, or a caput medusae that arises from hepatofugal flow through the recanalized umbilical vein. It is a tribute to the classical education of the early anatomists that they saw the snakes that made up Medusa’s hair in the tortuous ramifications of the collaterals surrounding the umbilicus. Sappey described the paraumbilical veins of the accessory portal system, which drain the falciform ligaments and surrounding areas to join the peripheral portal branches in the left hepatic lobe, the internal mammary veins, the diaphragmatic veins, and branches of the inferior epigastric veins around the umbilicus. What is the importance of all this? Only recognition that detection of such veins, whether by careful physical examination or by sophisticated imaging studies, means portal hypertension and probably cirrhosis. Examine the caput medusae carefully without fear of being turned to stone as humans were when they gazed upon the face of the chthonic Gorgon, Medusa. Lawrence J. Brandt, MD Associate Editor for Focal Points
An unusual gall bladder
www.giejournal.org
Volume 67, No. 2 : 2008 GASTROINTESTINAL ENDOSCOPY 347