Treatment of a Giant Haemangioma of the Liver With Kasabach-Merritt Syndrome by Orthotopic Liver Transplant

We describe a case of giant cavernous haemangioma of the liver with disseminated intravascular coagulopathy (Kasabach-Merritt syndrome) which was cured by orthotopic liver transplant.A 47 year old man presented with bleeding and tender massive hepatomegaly after tooth extraction. Investigations showed disseminated intravascular coagulopathy and a giant hepatic haemangioma involving both lobes of the liver. Initial treatment failed to resolve the coagulopathy and liver resection was attempted. At laparotomy the turnout was unresectable and the only option for cure was to offer a liver transplantation. The orthotopic liver transplant was performed 20 days after initial laparotomy. Subsequently, all coagulation parameters returned to normal and the patient remains well after 12 months. Orthotopic liver transplant can be considered for giant hepatic haemangioma with Kasabach-Merritt syndrome when resection is necessary and a partial hepatectomy is not technically feasible.     

Anesthesia for a patient with a giant hepatoma associated with severe acute hepatic damage]

We gave anesthesia to a patient for extensive right lobe hepatectomy. Although the liver function test revealed acute exacerbation just before the operation, we carried out anesthesia, diagnosing it to be due to giant liver tumor. The anesthesia was maintained with nitrous oxide, oxygen and epidural anesthesia with 1.5% lidocaine or 0.25% bupivacaine. A biopump inserted between the inferior vena cava and the left basilic vein was used during the right lobe resection to maintain sufficient venous return to the right atrium during the right lobe resection. Ketone body ratio was checked frequently in order to know the remnant liver energy charge and glucose was loaded properly. The surgery and anesthesia were uneventful. The resected right lobe weighed 2380 g, with necrosis of moderate size at the posterior-inferior segment. The serum transaminase decreased markedly after operation. It is important to have accurate diagnosis before we anesthetize patients with acute hepatic damage.     

Traumatic common hepatic artery injury causing isolated right hepatic ischemia due to a left accessory artery. A case report

IntroductionHepatic arterial liver flow is renowned for its redundancy. Previous studies have demonstrated that the common hepatic artery is not essential for liver survival. We present a case of a 31year-old involved in a high-speed motor vehicle accident whose liver survived thanks to the presence of an accessory hepatic artery.Presentaton of the caseWe present the case of a 31year-old male who sustained a traumatic injury of the proper hepatic artery following a motor vehicle accident. The patient suffered temporary right liver lobe ischemia due to the presence of an accessory left hepatic artery. This resulted in the selective formation of ‘biliary lakes' distinctively within the territory of the right hepatic artery supply.Simultaneously the patient developed a pseudo-aneurysm of the proper hepatic artery which required radiology intervention. At the time of pseudo-aneurysm embolisation, a rich network of arterial collaterals had formed between the accessory left hepatic and the inferior phrenic artery. On follow up the biliary lakes to the right lobe had resolved, but a small area at the periphery of the right lobe had encountered atrophy.DiscussionThis case report is an ‘in vivo’ demonstration of liver resilience to arterial flow re-distribution and demonstrates the ability of the biliary epithelium to recover from and ischemic injury.ConclusionParenchymal liver survival is mostly independent from flow within the common hepatic artery. Acute and chronic liver parenchyma changes following interruption of hepatic artery flow can still occur.     

Extended right lobectomy for caroli's disease: Report of a case and review of hepatectomized cases in Japan

A 27-year-old man was admitted to Nagasaki University Hospital complaining of fever. Percutaneous trans-hepatic cholangiography revealed multiple cystic dilatations of the intrahepatic bile ducts of the right lobe, as well as three obvious cystic dilatations and several small dilatations of the intrahepatic bile ducts of the left lobe. An extended right lobectomy was performed. However, the patient later suffered from repeated cholangitis and required the administration of antibiotics as a result of remnant cystic dilatations and stricture of the extrahepatic bile duct following hepatectomy. A slight enlargement of the remnant cystic dilatations, but no new cystic dilatations, were also recognized in the regenerating liver. We reviewed nine cases of hepatectomy for Caroli's disease reported in the Japanese literature, as well as our own case. Among these, four of the seven patients subjected to hepatic resection involving unilateral lobe demonstrated a good recovery. On the other hand, it is difficult to successfully treat Caroli's disease involving both lobes of the liver by hepatic resection.     

Hepatocellular carcinoma with a solitary adrenal metastasis and poor hepatic functional reserve: Report of a case

A 54-year-old man with a 15-year history of liver disease, was found by his family physician to have multiple tumors in the right lobe of the liver and a large right retroperitoneal tumor. He was referred and admitted to our institute where a preoperative diagnosis of liver cirrhosis complicated by hepatocellular carcinoma and probable right adrenal metastasis was made. Because his hepatic functional reserve was so poor, only resection of the right adrenal tumor with a splenectomy for hypersplenism and a cholecystectomy for the prevention of cholecystitis secondary to the scheduled transcatheter arterial embolization was performed. The patient was discharged in good clinical condition 5 weeks after surgery.     

A safe combined nephrectomy and right lobectomy using the liver hanging maneuver for huge renal cell carcinoma directly invading the right lobe of the liver: report of a case

We herein discuss a patient who underwent simultaneous combined right nephrectomy and right lobectomy of the liver. A 64-year-old male was diagnosed with a huge right renal cell carcinoma (RCC), 13 cm in diameter, which was invading directly into the right hepatic lobe. This type of RCC has been rarely reported, and an anterior approach using the liver hanging maneuver was extremely useful during hepatic parenchymal dissection. The liver parenchymal dissection was performed prior to mobilization of the liver, because the mobilization of the right lobe of the liver was impossible. During the hepatic parenchymal resection, the liver was suspended with the tape and transected, and thereafter, retroperitoneal dissection, nephrectomy and right lobectomy of the liver were completed. The patient was discharged from the hospital on the 12th postoperative day with an uneventful clinical course. The anterior approach using the liver hanging maneuver during hepatic parenchymal resection can be safe and feasible for huge RCC invading the right hepatic lobe.     

Pringle′s法联合肝静脉阻断技术在复杂肝切除术中的应用

目的: 探讨Pringle′s法联合肝静脉阻断技术在复杂肝切除术中的应用价值。方法: 对37例第二肝门区肿瘤施行Pringle′s法＋肝静脉阻断切肝术患者的临床资料进行回顾性分析。结果:37例患者中原发性肝癌27例，转移性肝癌2例，肝巨大血管瘤8例。肿瘤平均直径12.7cm（6～35cm）。肿瘤侵犯1根主肝静脉6例，侵犯2根主肝静脉20例，侵犯3根主肝静脉11例。行右三叶切除11例，右半肝切除5例，中肝叶切除9例，Ⅷ段切除4例，左三叶切除5例，尾状叶切除3例。平均第一肝门阻断时间29min（17～48min），平均肝静脉阻断时间21min（8～32min）。行肝静脉修补1例。平均术中出血量950mL（200～4 000mL）。全组术后发生并发症18例次，均经治疗后愈。无死亡病例。结论:Pringle′s法联合肝静脉阻断技术在复杂肝切除术中既能达到减少术中出血的目的，又能防止术中肝静脉破裂导致空气栓塞，还避免了下腔静脉阻断所引起全身血流动力学紊乱，是一种更安全、有效的血流阻断技术。     

Surgical treatment of giant hemangiomas of the liver

Spontaneous rupture of liver hemangiomas is exceptional, they rarely increase in size. Elective surgery of liver hemangiomas is safe and effective. In our teaching hospital during a 5-year period 9 patients underwent elective surgery for giant liver hemangiomas, one more patient required urgent operation for spontaneous rupture. Indications for elective surgery were: abdominal pain in 5 patients, enlargement in 1 patient, and 3 patients were worried about the risk of rupture or having a tumor left in situ. The average age of our four male and six female patients was 44.5 years (30-58). The median largest dimension of the lesions was 8.5 cm (5.5-14); six of them located in the right, four in the left lobe. Enucleation was performed in most patients (8, mostly of them in the right lobe); anatomical resections were performed only in two cases (left lobectomy). There was no postoperative mortality, the only complication was mild pneumonia in one patient. Elective surgery is indicated only in a small number of patients with hemangiomas, it should be limited to giant, symptomatic tumors or those with a documented tendency to increase in size. The type of resection depends on the site and the size of the lesion. Enucleation can be performed rapidly and safely in most patients and as such it is preferable to anatomical resection.     

肝巨大血管瘤外科治疗体会

目的探讨肝巨大血管瘤手术指征及方法。方法回顾性分析1999年10月-2004年10月手术切除的28例肝巨大血管瘤的临床资料。全组肿瘤直径5~24cm,平均12cm,其中邻近第二肝门5例,与下腔静脉关系密切者7例,均采用肝血管瘤剥离术联合术中应用微波刀切除肿瘤。结果28例中12例出血量在400m l以下,未输血,5例出血量600~800m l,1例出血量为8000 m l。全组安全切除,均痊愈。病理检查为海绵状血管瘤。结论对于直径小于8 cm的肝血管瘤,可定期观察,但位于第二肝门的肝血管瘤,均应早期切除,肿瘤大于8 cm或合并有临床症状者,应手术切除。采用血管瘤剥离术联合术中应用微波刀切除肿瘤,出血量少,安全有效,并发症少,提高了手术切除率。     

Should Ruptured Liver Haemangioma Be Treated by Surgery or by Conservative Means ? A Case Report

Spontaneous rupture of a liver haemangioma is a rare but life-threatening acute clinical situation following haemorrhage within the liver, the subcapsular space and the peritoneal cavity in cases of capsular rupture. Rupture of a liver haemangioma has been reported to occur spontaneously in the majority of cases. In the past, prompt surgical treatment was recommended but was associated with high morbidity and mortality. Currently, conservative management and, in cases of recurrent haemorrhage, delayed surgery may be proposed. We report a case of spontaneous rupture of hepatic haemangioma treated by arterial embolisation and conservative means. The literature is also reviewed.     

Regeneration of Human Liver After Hepatic Lobectomy Studied by Repeated Liver Scanning and Repeated Needle Biopsy

Regeneration of the residual lobe of the liver after hepatic lobectomy in humans was studied by repeated liver scanning in seven noncirrhotic and three cirrhotic patients. Each patient was studied for several months during the study which lasted from 1-12 years. Regeneration was apparent in noncirrhotic liver remnants following hepatic lobectomy. In the case of a long standing, space occupying lesions such as benign giant cysts, the liver remnant would complete its regeneration process rather early, usually within a few months of hepatic lobectomy. In hepatoma cases, however, regeneration of the residual lobe after hepatic resection usually took five or six months for completion. On the contrary, no definite increase in the size of the liver remnant was seen on repeated liver scanning in cirrhotic patients. Histologic study of the residual lobe was repeated on needle biopsy specimens in two noncirrhotic and four cirrhotic patients. Regenerative hyperplasia of liver cells with large hyperchromatic, or double nuclei never seen in the preresection liver appeared in the liver remnant five, 11, and 27 days after hepatic lobectomy in noncirrhotic patients. In cirrhotics, however, there were no histologic changes between the preresection liver and the postresection remnant studied three, five, 15, 40 days or even two years and 8 months after hepatic lobectomy.     

Complex vascular reconstructions in living donor liver transplantation

We describe here the indications for and our experience with complex vascular reconstructions in living donor liver transplantation. From December 1999 to June 2002, 59 patients underwent liver transplantation, 51 receiving the right lobe, and 8 the left lateral lobe, as a graft from a living donor. The indication for interpositional grafts on the arterial side (6/59, 10%) were stenoses of the celiac trunk and after resection of the hepatic artery for oncological reasons in adults. In children, arterial interpositional grafts were performed in situations of long distances between the donor and recipient artery, or in cases of inflow release from the aorta in patients with small hepatic arteries. On the portal-venous side, one interpositional graft was performed after an oncological resection. Once the portal vein was partially arterialized because of insufficient inflow. We used veins from the recipient, and native or cryopreserved arterial homografts for these grafts. All patients were treated during the first 6 months after transplantation with aspirine only. During the follow-up we did not observe vascular complications. If required, vascular interpositional grafts in the arterial and portal-venous position can be performed without adding postoperative complications.     

Rupture of the hepatic artery after left hemihepatectomy for hilar bile duct carcinoma

Hilar bile duct carcinoma has a poor prognosis, but this has been improved in recent years by an aggressive surgical approach. We treated a 73-year-old woman who had obstructive jaundice due to bile duct carcinoma at the hepatic hilum. The jaundice decreased after percutaneous transhepatic biliary drainage. The tumor was resected with the left and caudate lobe of the liver and a part of portal vein. The right hepatic artery was located behind the common hepatic duct, and was suspected to be invaded by the tumor. We dissected the tumor from the arterial wall without carrying out combined resection of the hepatic artery. On the 6th postoperative day, the hepatic artery ruptured and the patient suffered hypovolemic shock. Resection of the hepatic artery and reconstruction were done, but the patient died 2 days later. Histological examination of the resected artery showed that the tumor had been curatively removed by dissection and that no tumor remained at the arterial wall. The rupture of the right hepatic artery was thought to have been caused by damage to the wall during the dissection procedure.     

Long-term biological consequences of donor right hepatectomy including the middle hepatic vein in adult-to-adult live donor liver transplantation.

The right lobe liver graft has become the workhorse of adult-to-adult live donor liver transplantation. Donor right hepatectomy is feasible only because of the immense regenerative ability of the liver. The long-term biological consequences of this very major donor procedure on the donor however are unknown. Twenty-nine donors of this procedure in our centre, all of whom included the middle hepatic vein, were studied. On long-term follow-up at a median of 47.4 months, there was a discernible but statistically insignificant decrease in size of the regenerated left lobe compared to the original whole liver volume. There was paradoxically a trend of incompleteness of regeneration in relation to the original liver volume for those with a larger remnant left lobe. The volume ratio of the regenerated left lobe to the original left lobe before hepatectomy was inversely proportional to the left lobe proportion preoperatively. This strong but inverse linear correlation reflected the good regenerative ability of the remnant left lobe. None of the donors developed thrombocytopenia. Although demonstrable decrease in white cell count, increase in serum alanine aminotransferase, aspartate aminotransferase, and creatinine did occur, the changes remained within normal limits and were of yet uncertain clinical significance. In conclusion, donor right hepatectomy including the middle hepatic vein is biologically acceptable to the live donor.     

Giant mesenchymal hamartoma of the liver in an adult

We report a case of hepatic mesenchymal hamartoma in an adult; this condition is extremely rare, with only 15 cases having been reported in the English-language literature worldwide. The patient was a 36-year-old woman who was seen at her local hospital for upper abdominal distension. A giant multilocular cystic tumor, which had almost entirely replaced the normal parenchyma of the right lobe of the liver, was diagnosed. She was referred to our hospital, where, with a diagnosis of biliary cystadenoma, the tumor was successfully removed by right hemihepatectomy. After an uneventful postoperative course, the patient was discharged from our hospital. On histological examination, the tumor consisted of numerous cystic lesions without epithelial lining cells; hepatocytes, bile duct, and vascular components, without either lobular structure or atypia, were observed in the pseudocyst wall, leading to a diagnosis of hepatic mesenchymal hamartoma. There have been a few previously reported cases of multifocal hepatic mesenchymal hamartoma reappearing in the remaining liver after hepatectomy, although these cases are considered to be extremely rare. Therefore, periodic follow-up will be necessary for the patient.     

Anterior approach for major right hepatic resection for large hepatocellular carcinoma

OBJECTIVE: To report the surgical and long-term outcomes of major right hepatic resection for large hepatocellular carcinoma (HCC) using the anterior approach compared with the conventional approach. SUMMARY BACKGROUND DATA: Great difficulty can be encountered during major right hepatic resection for large HCC using the conventional approach. Forceful retraction during mobilization of the tumor might result in serious complications, including dissemination of cancer cells, iatrogenic tumor rupture, and excessive bleeding, leading to unfavorable surgical and long-term outcomes. METHODS: In patients who had large HCC at the right lobe of liver and underwent major hepatic resection, the technique of anterior approach was used. After hilar control of the inflow blood vessels and without prior mobilization of the right lobe of liver and the tumor, parenchymal transection was performed using an ultrasonic dissector from the anterior surface of the liver until the anterior surface of the inferior vena cava was exposed. All venous tributaries, including the right hepatic vein, were controlled before the right lobe of liver was mobilized. Surgical and long-term outcomes were analyzed retrospectively and compared with patients who underwent surgery using the conventional approach. RESULTS: From 1989 to 1997, the anterior approach was used for major right hepatic resection in 54 patients with HCC of 5 cm or more in diameter. When compared with the 106 patients with similar clinical parameters who underwent hepatic resection using the conventional approach during the same period, the patients in the anterior approach group had significantly less intraoperative blood loss and blood transfusion, a lower hospital death rate, a lower incidence of pulmonary metastases, and a better median disease-free survival and median overall cumulative survival. CONCLUSION: The anterior approach is the preferred technique for major right hepatic resection for large HCC because it resulted in improved surgical and survival outcomes compared with the conventional approach.     

肝门部Glisson蒂交替血流阻断循Glisson鞘行肝右前叶下段解剖性切除术

目的探讨肝门部Glisson蒂交替血流阻断、循Glisson鞘行肝右前叶下段解剖性切除在肝癌手术中的应用。方法 15例肝右前叶下段肝癌患者中,8例采用肝门部Glisson蒂交替血流阻断、循Glisson鞘行肝右前叶下段解剖性切除术(A组)。7例采用间歇性第一肝门阻断(B组)行肝肿瘤局部切除术。对两组术中出血、术后肝功能情况进行比较。结果两组均无死亡病例,A组术中出血量、术后第1、3、5、7天的血清谷丙转氨酶(alanine transaminase,ALT)明显低于B组,两组比较差异具有统计学意义(P0.05)。结论肝门部Glisson蒂交替血流阻断、循Glisson鞘行肝右前叶下段解剖性切除术是减少术中出血和术后肝功能损害的有效的肝切除方法。     

Caudate lobectomy: tumor location, topographic classification, and technique using right- and left-sided approaches to the liver

BACKGROUND: Resection of the caudate lobe (involving segments I [dorsal sector] and/or IX [right paracaval region]) often presents a technical challenge. It is difficult to perform because of its deep location and adjacency to the major hepatic vessels (ie, the left and middle hepatic veins). METHODS: A literature review was performed based on a Medline search to identify articles on caudate lobectomy published from 1990 to 2005. This article describes the right and left-sided approaches to the liver for caudate resection according to caudate lobe tumor location and topographic classification. RESULTS: The results of 377 lobectomies were analyzed in this review. The left-sided approach to the liver was used in 55 (14.58%), the right-sided approach in 24 (6.36%), and both approaches in 298 (79.04%) caudate lobectomies. Primary benign and malign liver tumors, as well as secondary liver tumors, were resected. CONCLUSIONS: Access to and resection of the caudate lobe should be determined on the basis of tumor location and hepatic function. The left or right approach to the caudate lobe can be recommended for local resection of tumor located at Spiegel's portion or process portion. Approaches to caudate lobectomy are therefore largely dependent on size and location of the lesion, type of associated resection, and presence of scarring from previous resection.     

Surgical treatment of hepatic haemangiomas: a 15-year experience

BACKGROUND: Hepatic haemangiomas are congenital vascular malformations. They are the most common benign tumours of the liver and are often asymptomatic. Spontaneous or traumatic rupture, intratumoral bleeding, consumption coagulopathy and rapid growth are the mandatory surgical indications. We present our experience over the last 15 years with the surgical management of 15 liver haemangiomas to clarify the safety and effectiveness of this treatment. METHODS: There were 15 patients with hepatic haemangiomas who were surgically treated from 1990 to 2004. Indications for the operation were spontaneous or traumatic rupture, consumption coagulopathy, rapid growth, abdominal pain and uncertain diagnosis. Four of these lesions were located on the left lobe, nine on the right lobe; one lesion was located on the left and the right lobes and one on segments VII and VIII. Methods for diagnosis included ultrasonography, computed tomography scan, magnetic resonance imaging and selective hepatic arteriography or combinations of more than one technique. RESULTS: The procedures included five right-extended lobectomies, five right lobectomies, one left-extended lobectomy, two left lobectomies and two segmental resections. There was no death. The postoperative morbidity was minimal and was mainly correlated to two subdiaphragmatic collections, one intra-abdominal collection and one wound infection. The postoperative hospital stay was 12.7 days (range, 10-19 days). During the follow-up period, there was no recurrence. CONCLUSION: The resection of the hepatic haemangioma is safe. The indications for resection, however, should be carefully analysed before embarking on such a major operation.     

Living donor liver transplantation in a patient with giant hepatic hemangioma complicated by Kasabach-Merritt syndrome: Report of a case

We herein present a case of unresectable giant hepatic hemangiomas with Kasabach-Merritt syndrome which was successfully treated by living donor liver transplantation using a left lobe graft. The patient was a 45-year-old woman who complained of abdominal distension. Two sessions of transarterial embolization were performed, but failed to reduce the size of the tumor. The hepatic tumors were thus judged untreatable and the only option for a cure was to offer living donor liver transplantation, because of the tumor size, its location, and the association with Kasabach-Merritt syndrome. A left lobe graft with the middle hepatic vein donated by her 47-year-old brother was transplanted under venovenous bypass. The postoperative course of the recipient was complicated by small-for-size graft syndrome, which developed after episodes of acute cellular rejection on postoperative day 8 and sepsis on day 31. The patient successfully recovered from the complications and was discharged on day 72, and she remains well at 10 months after transplantation. In conclusion, living donor liver transplantation was found to be an effective option for the treatment of a patient with unresectable giant hepatic hemangiomas complicated by Kasabach-Merritt syndrome.