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Cavernous Hemangioma Presenting as Budd-Chiari Syndrome Dean Y Kim, MD, Milan V Pantelic, MD, Atsushi Yoshida, MD, John Jerius, MD, Marwan S Abouljoud, MD, FACS, Henry Ford Hospital, Detroit, MI A 45-year-old moderately obese woman with a history of a herniated disc was admitted to the hospital after right upper quadrant pain progressed for 1 month. Inpatient workup revealed a mass in the liver. MRI and tagged red blood cell nuclear study confirmed the presence of a central cavernous hemangioma, measuring approxi- mately 6 cm in diameter between the right and left he- patic veins. The right hepatic vein was distended, but not occluded. She was treated symptomatically and with close observation. Despite best medical management over 6 months, the patient’s abdominal pain persisted and began to affect her epigastrium, causing abdominal fullness, worsening nau- sea, and early satiety. She was referred to our clinic for eval- uation. A repeat MRI revealed an 8.0 6.5 5.5-cm lobular, multiloculated hemangioma interposed between the medial and lateral segments of the left lobe of the liver, with significant narrowing of the intrahepatic inferior vena cava (A). The middle hepatic vein was absent, and the right hepatic vein was large and displaced posteriorly, demon- strating large peripheral veins in the dome (B) and large peripheral channels communicating with the inferior as- pect of one of two right hepatic veins (C). Because of the development of intrahepatic collaterals, potential for com- plete occlusion of the hepatic venous system, her worsening abdominal symptoms, and lower extremity edema, the pa- tient was offered a surgical resection. At operation, her left lobe was markedly swollen and the hemangioma was closely apposed to the right hepatic vein. A left trisegmen- tectomy was performed, carefully dissecting the hemangi- oma from the right hepatic vein. Her intraoperative course was uncomplicated and her hospital course was unremark- able. Her abdominal pain and fullness resolved, as did her lower extremity edema within 6 months. She remains symptom free 21 months after the resection. Hepatic hemangiomas are common benign mesen- chymal tumors of the liver, usually diagnosed between the ages of 30 and 50 years. They are frequently solitary, but may present as multiple lesions in one or both lobes of the liver. 1 These lesions are more frequent in women (3:1), and are usually asymptomatic. Most hepatic hem- 470 © 2005 by the American College of Surgeons ISSN 1072-7515/05/$30.00 Published by Elsevier Inc. doi:10.1016/j.jamcollsurg.2004.07.037

Cavernous hemangioma presenting as Budd-Chiari syndrome

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avernous Hemangioma Presenting as Budd-Chiari Syndrome

ean Y Kim, MD, Milan V Pantelic, MD, Atsushi Yoshida, MD, John Jerius, MD,

arwan S Abouljoud, MD, FACS, Henry Ford Hospital, Detroit, MI

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45-year-old moderately obese woman with a history ofherniated disc was admitted to the hospital after rightpper quadrant pain progressed for 1 month. Inpatientorkup revealed a mass in the liver. MRI and tagged redlood cell nuclear study confirmed the presence of aentral cavernous hemangioma, measuring approxi-ately 6 cm in diameter between the right and left he-

atic veins. The right hepatic vein was distended, butot occluded. She was treated symptomatically and withlose observation.

Despite best medical management over 6 months, theatient’s abdominal pain persisted and began to affect herpigastrium, causing abdominal fullness, worsening nau-ea, and early satiety. She was referred to our clinic for eval-ation. A repeat MRI revealed an 8.0 � 6.5 � 5.5-cm

obular, multiloculated hemangioma interposed betweenhe medial and lateral segments of the left lobe of the liver,ith significant narrowing of the intrahepatic inferior vena

ava (A).The middle hepatic vein was absent, and the rightepatic vein was large and displaced posteriorly, demon-trating large peripheral veins in the dome (B) and largeeripheral channels communicating with the inferior as-ect of one of two right hepatic veins (C). Because of theevelopment of intrahepatic collaterals, potential for com-lete occlusion of the hepatic venous system, her worseningbdominal symptoms, and lower extremity edema, the pa-

ient was offered a surgical resection. At operation, her left (

4702005 by the American College of Surgeons

ublished by Elsevier Inc.

obe was markedly swollen and the hemangioma waslosely apposed to the right hepatic vein. A left trisegmen-ectomy was performed, carefully dissecting the hemangi-ma from the right hepatic vein. Her intraoperative courseas uncomplicated and her hospital course was unremark-

ble. Her abdominal pain and fullness resolved, as did herower extremity edema within 6 months. She remainsymptom free 21 months after the resection.

Hepatic hemangiomas are common benign mesen-hymal tumors of the liver, usually diagnosed betweenhe ages of 30 and 50 years. They are frequently solitary,ut may present as multiple lesions in one or both lobesf the liver.1 These lesions are more frequent in women

3:1), and are usually asymptomatic. Most hepatic hem-

ISSN 1072-7515/05/$30.00doi:10.1016/j.jamcollsurg.2004.07.037

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471Vol. 200, No. 3, March 2005 Kim et al Images for Surgeons

ngiomas are less than 5 cm; those larger than 5 cm arenown as giant hemangiomas. Giant hemangiomas mayause symptoms, including abdominal pain and rightpper quadrant fullness. Other causes of abdominalain must be ruled out before attributing it to heman-ioma. One series reported 54% of patients with ab-ominal pain and hemangiomas had pain attributed tother causes.1 Other symptoms include nausea, an-rexia, and early satiety from the large hemangiomaompressing adjacent organs. Acute pain occurs if theres thrombosis or bleeding within the hemangioma and

ay last several weeks.The natural history of cavernous hemangiomas is not

ell understood. Although there are reports that theajority remain stable over time,2 others have described

ignficant growth, with symptoms requiring surgical re-ection.3 For the asymptomatic patient and for lesionsess than 5 cm, reassurance and observation have beenecommended. For lesions greater than 5 cm, close ob-ervation with radiologic followup is warranted. Forhose who are symptomatic with a giant hemangioma,ther causes of pain need to be ruled out because up to5% of patients have persistent symptoms after surgicalesection.1 If surgery is indicated, there are four options:iver resection, enucleation, hepatic artery ligation, andiver transplantation. Mortality from a liver resection isnusual at specialized hepatobiliary-liver transplant cen-

ers. It has been reported that transfusion requirements

re less for enucleation compared with resection. De-ending on the presentation, enucleation may be a bet-er option for the symptomatic patient.

Cavernous hemangioma causing a Budd-Chiari-likeyndrome has been described once in the literature.4

hat lesion underwent cystic degeneration, causingymptoms. Our patient had a giant hemangioma in aocation compressing the retrohepatic vena cava. Also,ecause no early filling arteriovenous malformation oristula were demonstrated, the intrahepatic collateralsppeared to be from chronic dilatation secondary toompression of retrohepatic vena cava and right hepaticenous origin and occlusion of the middle hepatic veiny the hemangioma (which only demonstrates slowilling).

EFERENCES

. Farges O, Daradkey S, Bismuth H. Cavernous hemangiomas ofthe liver: are there any indications for resection? Word J Surg1995;19:19–24.

. Gandolfi L, Leo P, Solmi L, et al. Natural history of hepatichaemangiomas: clinical and ultrasound study. Gut 1991;32:677–680.

. Yoshida J, Yamasaki S, Yamamoto J, et al. Growing cavernoushemangiomas of the liver: 11-fold increase in volume in a decade.J Gastroenterol Hepatol 1991;6:414–416.

. Hanazaki K, Koide N, Kajikawa S, et al. Cavernous hemangiomaof the liver with giant cyst formation: degeneration by apoptosis?

J Gastroenterol Hepatol 2001;16:352–355.