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Massive enlargement of a paraumbilical vein in a patient with advanced portal hypertension
Images in Surgery This section features outstanding photographs of clinical materials selected for their educational value or message, or possibly their rarity. The images are accompanied by brief case reports (limit 2 typed pages, 4 references). Our readers are invited to sumit items for consideration.
Massive enlargement of a paraumbilical vein in a patient with advanced portal hypertension Peter T. W. Kim, MD, MSc, Tony T. Chandy, MD, and Anand Ghanekar, MD, PhD, Toronto, Ontario, Canada
From the University of Toronto Liver Transplant Program, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada
A 28-year-old man with a 10-year history of mild ulcerative colitis presented with progressive jaundice, pruritus, abdominal distension, and fatigue of several weeks’ duration. The physical examination revealed several manifestations of chronic liver disease, including spider angiomata, peripheral muscle wasting, ascites, prominent abdominal wall veins, hepatosplenomegaly, and jaundice. Laboratory investigations revealed the following: hemoglobin, 100 g/L; platelets, 92 3 109/L; international normalized ratio, 1.30; aspartate aminotransferase, 74 U/L; alanine aminotransferase, 42 U/L; alkaline phosphatase, 431 U/L; total bilirubin, 109 mmol/L; creatinine, 59 mmol/L; and albumin, 28 g/L. Serologic studies revealed no evidence of viral hepatitis, and investigations for disordered copper and iron metabolism and alpha-1-antitrypsin deficiency were negative.
Accepted for publication July 7, 2011. Reprint requests: Anand Ghanekar, MD, PhD, Division of General Surgery, University Health Network, Toronto General Hospital, NCSB 11C-1227, 585 University Avenue, Toronto, ON M5G 2N2, Canada. E-mail: [email protected]. Surgery 2012;152:931-3. 0039-6060/$ - see front matter Ó 2012 Mosby, Inc. All rights reserved. doi:10.1016/j.surg.2011.07.025
Doppler ultrasound and contrast-enhanced computed tomography revealed an enlarged cirrhotic liver with patent hepatic vasculature accompanied by reversal of flow within the portal vein, numerous intraabdominal varices, ascites, splenomegaly, and enlarged periportal lymph nodes. A large, tortuous varix was observed to originate from the left portal vein and travel towards the umbilicus (Fig 1). Magnetic resonance cholangiography revealed diffuse intrahepatic bile duct strictures. A diagnosis of hepatic cirrhosis secondary to primary sclerosing cholangitis was made, and this was confirmed by transjugular liver biopsy. The patient proceeded to undergo orthotopic liver transplantation. At the time of surgery, a standard bilateral subcostal incision was used to gain access to the peritoneal cavity. General laparotomy confirmed the radiographic findings, and the liver was noted to be firm and cholestatic. A massively enlarged vein was observed traversing the operative field as a serpentine structure with a caliber similar to that of the small intestine (Fig 2). The structure arose from the umbilical fissure adjacent to the falciform ligament and took a tortuous course along the abdominal wall towards the periumbilical tissue. Several additional large varices were noted around the umbilicus. Safe exposure of the right SURGERY 931
932 Kim, Chandy, and Ghanekar
Fig 1. Contrast-enhanced coronal computed tomographic image revealing a large paraumbilical vein (white arrow) arising from the left portal vein in the umbilical fissure of the liver and coursing towards the periumbilical region, just beneath the anterior abdominal wall.
upper quadrant required partial resection of the vein after achieving proximal and distal control of the vessel with large vascular clamps. The caliber, turgor, and amount of blood flow within the vein precluded safe application of a vascular stapler. Orthotopic liver transplantation subsequently proceeded without complication, and the patient was eventually discharged home with good graft function. DISCUSSION In fetal circulation, oxygenated blood is returned to the fetus from the placenta via the umbilical vein. The umbilical vein courses along the margin of the falciform ligament towards the liver, where it enters the umbilical fissure and empties into the left portal vein. Blood is then returned to the inferior vena cava via the liver parenchyma and hepatic veins, or by passing through the ductus venosus, which connects the left portal vein directly to the left hepatic vein. Within days of normal respiration after birth, the umbilical vein and ductus venosus collapse and are obliterated, becoming the ligamentum teres and ligamentum venosum, respectively.1
Surgery November 2012
Fig 2. Intraoperative photograph following entry into the peritoneal cavity through a bilateral subcostal incision. A massively enlarged paraumbilical vein (black arrow), similar in diameter to the small intestine (white arrow), is visible traversing the operative field deep to the abdominal wall between the umbilical fissure of the liver and the periumbilical region. The patient’s head is located to the left of the photograph. (Color version of figure is available online.)
It is a commonly held view that portal hypertension leads to ‘‘recanalization’’ of the obliterated umbilical vein as a portosystemic shunt, thereby decompressing the portal circulation.2 Detailed angiographic and histopathologic studies, however, suggest that the obliterated umbilical vein never reopens, and that the large venous structures traveling from the liver towards the umbilicus are in fact enlarged paraumbilical veins, of which 2 or 3 are normally present in or adjacent to the falciform ligament.3 The observation that ‘‘recanalization’’ of an obliterated vessel does not actually occur may explain why patency of the ductus venosus, another portosystemic shunt, is so rarely observed in adults with advanced portal hypertension, because additional small veins do not run adjacent to this structure. Patency of the ductus venosus in adults is felt to more likely represent a primary failure of the structure to obliterate, and may have a genetic basis.4 Regardless of the anatomic origins, early recognition and appropriate management of these fragile vessels is required during surgery on patients with advanced portal hypertension in order to
Surgery Volume 152, Number 5
avoid massive hemorrhage. On occasion, these vessels may be successfully harvested and used as autologous vascular conduits.5 REFERENCES 1. Gray H. Anatomy of the human body. 20th ed. Philadelphia: Lea and Febiger; 1918. 2. Young TH, Lee HS. Images in clinical medicine. Recanalized umbilical vein. N Engl J Med 2007;357:e17.
Kim, Chandy, and Ghanekar 933
3. Lafortune M, Constantin A, Breton G, Legare AG Lavoie P. The recanalized umbilical vein in portal hypertension: a myth. AJR Am J Roentgenol 1985;144: 549-53. 4. Merkle EM, Gilkeson RC. Remnants of fetal circulation: appearance on MDCT in adults. AJR Am J Roentgenol 2005; 185:541-9. 5. Facciuto ME, Rodriguez-Davalos MI, Singh MK, Rocca JP, Rochon C, Chen W, et al. Recanalized umbilical vein conduit for meso-Rex bypass in extrahepatic portal vein obstruction. Surgery 2009;145:406-10.
Massive enlargement of a paraumbilical vein in a patient with advanced portal hypertension Peter T. W. Kim, MD, MSc, Tony T. Chandy, MD, and Anand Ghanekar, MD, PhD, Toronto, Ontario, Canada
From the University of Toronto Liver Transplant Program, Toronto General Hospital, University Health Network, Toronto, Ontario, Canada
A 28-year-old man with a 10-year history of mild ulcerative colitis presented with progressive jaundice, pruritus, abdominal distension, and fatigue of several weeks’ duration. The physical examination revealed several manifestations of chronic liver disease, including spider angiomata, peripheral muscle wasting, ascites, prominent abdominal wall veins, hepatosplenomegaly, and jaundice. Laboratory investigations revealed the following: hemoglobin, 100 g/L; platelets, 92 3 109/L; international normalized ratio, 1.30; aspartate aminotransferase, 74 U/L; alanine aminotransferase, 42 U/L; alkaline phosphatase, 431 U/L; total bilirubin, 109 mmol/L; creatinine, 59 mmol/L; and albumin, 28 g/L. Serologic studies revealed no evidence of viral hepatitis, and investigations for disordered copper and iron metabolism and alpha-1-antitrypsin deficiency were negative.
Accepted for publication July 7, 2011. Reprint requests: Anand Ghanekar, MD, PhD, Division of General Surgery, University Health Network, Toronto General Hospital, NCSB 11C-1227, 585 University Avenue, Toronto, ON M5G 2N2, Canada. E-mail: [email protected]. Surgery 2012;152:931-3. 0039-6060/$ - see front matter Ó 2012 Mosby, Inc. All rights reserved. doi:10.1016/j.surg.2011.07.025
Doppler ultrasound and contrast-enhanced computed tomography revealed an enlarged cirrhotic liver with patent hepatic vasculature accompanied by reversal of flow within the portal vein, numerous intraabdominal varices, ascites, splenomegaly, and enlarged periportal lymph nodes. A large, tortuous varix was observed to originate from the left portal vein and travel towards the umbilicus (Fig 1). Magnetic resonance cholangiography revealed diffuse intrahepatic bile duct strictures. A diagnosis of hepatic cirrhosis secondary to primary sclerosing cholangitis was made, and this was confirmed by transjugular liver biopsy. The patient proceeded to undergo orthotopic liver transplantation. At the time of surgery, a standard bilateral subcostal incision was used to gain access to the peritoneal cavity. General laparotomy confirmed the radiographic findings, and the liver was noted to be firm and cholestatic. A massively enlarged vein was observed traversing the operative field as a serpentine structure with a caliber similar to that of the small intestine (Fig 2). The structure arose from the umbilical fissure adjacent to the falciform ligament and took a tortuous course along the abdominal wall towards the periumbilical tissue. Several additional large varices were noted around the umbilicus. Safe exposure of the right SURGERY 931
932 Kim, Chandy, and Ghanekar
Fig 1. Contrast-enhanced coronal computed tomographic image revealing a large paraumbilical vein (white arrow) arising from the left portal vein in the umbilical fissure of the liver and coursing towards the periumbilical region, just beneath the anterior abdominal wall.
upper quadrant required partial resection of the vein after achieving proximal and distal control of the vessel with large vascular clamps. The caliber, turgor, and amount of blood flow within the vein precluded safe application of a vascular stapler. Orthotopic liver transplantation subsequently proceeded without complication, and the patient was eventually discharged home with good graft function. DISCUSSION In fetal circulation, oxygenated blood is returned to the fetus from the placenta via the umbilical vein. The umbilical vein courses along the margin of the falciform ligament towards the liver, where it enters the umbilical fissure and empties into the left portal vein. Blood is then returned to the inferior vena cava via the liver parenchyma and hepatic veins, or by passing through the ductus venosus, which connects the left portal vein directly to the left hepatic vein. Within days of normal respiration after birth, the umbilical vein and ductus venosus collapse and are obliterated, becoming the ligamentum teres and ligamentum venosum, respectively.1
Surgery November 2012
Fig 2. Intraoperative photograph following entry into the peritoneal cavity through a bilateral subcostal incision. A massively enlarged paraumbilical vein (black arrow), similar in diameter to the small intestine (white arrow), is visible traversing the operative field deep to the abdominal wall between the umbilical fissure of the liver and the periumbilical region. The patient’s head is located to the left of the photograph. (Color version of figure is available online.)
It is a commonly held view that portal hypertension leads to ‘‘recanalization’’ of the obliterated umbilical vein as a portosystemic shunt, thereby decompressing the portal circulation.2 Detailed angiographic and histopathologic studies, however, suggest that the obliterated umbilical vein never reopens, and that the large venous structures traveling from the liver towards the umbilicus are in fact enlarged paraumbilical veins, of which 2 or 3 are normally present in or adjacent to the falciform ligament.3 The observation that ‘‘recanalization’’ of an obliterated vessel does not actually occur may explain why patency of the ductus venosus, another portosystemic shunt, is so rarely observed in adults with advanced portal hypertension, because additional small veins do not run adjacent to this structure. Patency of the ductus venosus in adults is felt to more likely represent a primary failure of the structure to obliterate, and may have a genetic basis.4 Regardless of the anatomic origins, early recognition and appropriate management of these fragile vessels is required during surgery on patients with advanced portal hypertension in order to
Surgery Volume 152, Number 5
avoid massive hemorrhage. On occasion, these vessels may be successfully harvested and used as autologous vascular conduits.5 REFERENCES 1. Gray H. Anatomy of the human body. 20th ed. Philadelphia: Lea and Febiger; 1918. 2. Young TH, Lee HS. Images in clinical medicine. Recanalized umbilical vein. N Engl J Med 2007;357:e17.
Kim, Chandy, and Ghanekar 933
3. Lafortune M, Constantin A, Breton G, Legare AG Lavoie P. The recanalized umbilical vein in portal hypertension: a myth. AJR Am J Roentgenol 1985;144: 549-53. 4. Merkle EM, Gilkeson RC. Remnants of fetal circulation: appearance on MDCT in adults. AJR Am J Roentgenol 2005; 185:541-9. 5. Facciuto ME, Rodriguez-Davalos MI, Singh MK, Rocca JP, Rochon C, Chen W, et al. Recanalized umbilical vein conduit for meso-Rex bypass in extrahepatic portal vein obstruction. Surgery 2009;145:406-10.