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Adult-onset type II citrullinemia (CTLN2) is a rare disorder of the urea cycle resulting in hyperammonemia, with a poor prognosis. Here we report a 48-year-old Japanese man who showed abnormal nocturnal behavior. Laboratory data indicated raised plasma concentrations of ammonia and citrulline, and a definitive diagnosis of CTLN2 was made by DNA analysis. Hyperammonemia was not improved by oral intake of branched-chain amino acids (BCAA), whereas venous infusion of BCAA was effective. Western blotting revealed heterozygotic expression of citrin protein in a liver biopsy specimen from the patient's brother. However, as symptomatic CTLN2 is very unusual in a heterozygotic carrier, we considered the brother suitable as a living-donor liver transplantation (LDLT) donor. The recipient's entire liver was removed, and replaced with the left liver graft. The plasma ammonia level remained low without infusion of BCAA after liver transplantation. From this case we conclude that venous infusion, rather than oral administration, of BCAA is useful for conservative treatment of CTLN2. However, liver transplantation is the only effective therapeutic option for CTLN2, and should be performed before irreversible encephalopathy occurs. Use of a graft from heterozygotic donors is permissible treatment for CTLN2.

The aim of this study is to assess the postoperative outcome of the two reconstruction methods using both a free jejunal graft and a venous-reconstructed gastric tube or a gastric tube alone after total pharyngolaryngoesophagectomy.We reconstructed the pharyngoesophagus using both a free jejunal graft and a gastric tube in 5 cases (JG group), including 4 in whom the gastric vein was reconstructed, and using a gastric tube alone in 8 cases (G group). We compared the two groups in terms of postoperative complications, operation time, blood loss and length of postoperative hospital stay.In the JG group, jejunal graft necrosis occurred in a case, although 4 cases with gastric venous reconstruction had no postoperative problems. In the G group, although anastomotic trouble occurred in 3 cases, 7 patients were discharged from hospital with oral feeding. In the JG group, postoperative stay was shorter, but the operation time was longer, than those in the G group.Reconstruction using a free jejunal graft and a venous-reconstructed gastric tube is useful especially for patients with a long defect in the pharyngoesophagus. These 2 methods may be used according to the length of the pharyngoesophageal defect.

To evaluate the expression and role of c-kit protein in the neoplasia of pancreatic neoplasms and its relationship to prognosis in invasive ductal carcinoma (IDC) of the pancreas.The immunohistochemical expression of c-kit protein was studied in normal pancreatic tissues, intraductal papillary-mucinous neoplasms of the pancreas (IPMN), mucinous cystic neoplasm of the pancreas (MCN), and IDC of the pancreas. The immunoreactive score (IRS) of c-kit protein expression was examined in normal pancreatic ductal cells, neoplastic cells, and pancreatic endocrine cells.The IRS values of c-kit protein expression in various neoplastic cells were significantly higher than those in normal ductal cells. No significant difference was seen between IRS values of c-kit protein expression in various pancreatic neoplastic cells of IDC of the pancreas, IPMN and MCN. No significant difference was seen between IRS values of c-kit expression in endocrine cells among various pancreatic tissues. Furthermore, a survival analysis in patients with IDC of the pancreas showed an obvious trend toward decreased survival in patients with c-kit-positive cancer, and c-kit protein expression did not correlate with any clinicopathological factors in IDC of the pancreas.c-kit protein expression may play an important role in neoplasia of pancreatic neoplasms. In view of the result that c-kit protein expression was found in 8% of IDC of the pancreas, a clinical trial for STI-571 (Glivec) against pancreatic cancer may be warranted for selected pancreatic cancer patients with c-kit protein expression.

We experienced a case in which a drainage operation for injury of the pancreas preserved the distal pancreas, spleen and the residual stomach after distal gastrectomy for complete laceration of the stomach. A 23-year-old male was crushed by a large truck while driving a car without a seat belt. Abdominal computed tomography (CT) scan showed a low-density area in and around the body of the pancreas, which suggested contusion of the pancreatic parenchyma. An emergent operation was indicated due to free air and massive bleeding in the abdominal cavity and liver damage. At the operation, the antrum of the stomach was completely lacerated and distal gastrectomy was performed. Drains were set around the body of the pancreas. Distal pancreatectomy with splenectomy would have led to total gastrectomy because of lack of blood supply to the residual stomach after distal gastrectomy. We limited the drainage operation for pancreatic trauma. As a result, the pancreatic body and tail were preserved, which is thought to be a much better long-term result for this patient.

We present a 60-year-old woman who underwent three times hepatectomy and lung resection for metastasis originating from a carcinoma of the papilla of Vater after pancreaticoduodenectomy with lymphadenectomy during 12 years. Pancreaticoduodenectomy was performed in 1980 and histological examination of original tumor revealed a stage IIA papillary adenocarcinoma (pT3, pN0, pM0). Repetitive hepatectomy underwent in 1986 (S7,8), 1988 (S2), 1991 (S4) and bilateral partial resection of lung (right S1, left S2.3) in 1990. She died from multiple skin, bone and lung metastases 12 years after pancreaticoduodenectomy. The current case is very rare, however, if patients with carcinoma of the papilla of Vater have localized liver metastases and no local recurrence, liver metastases should be resected to improve the chances for long-term survival.

This study investigated the results of hepatectomy for multiple liver metastases and repeated hepatectomy for recurrent hepatic metastases. A proposed treatment strategy for liver metastases is discussed.Fifty-seven consecutive cases of liver metastases were studied. The metastases originated from colon cancer (24 cases), rectal cancer (11 cases), gastric cancer (14 cases), or gastrointestinal stromal tumors (two cases). The other cases included one each of gastric carcinoid, carcinoma of the papilla of Vater, cystic duct cancer, esophageal cancer, choriocarcinoma and breast cancer.The overall 5-year survival rate for the 57 cases was 45.4%; there was no significant difference between patients with colon cancer (56.3%), rectal cancer (45.5%), or gastric cancer (41.6%). The cumulative 5-year survival rates for synchronous and metachronous metastases were 38.3% and 50.8%, respectively (difference not statistically significant; NS). The survival rates for single and multiple metastases were 56.0% and 31.3% (NS), and those for monolobar and bilobar metastases were 48.5% and 40.9% (NS), respectively. Concerning the operative procedure, the survival rates for partial resection and hemi-hepatectomy were 49.5% and 26.9%, respectively (NS). The survival rates for surgical margins4mm and5mm were 45.9% and 45.4%, respectively (NS), and those for single and repeat hepatectomy were 40.5% and 58.2% (NS). Preoperative portal embolization was performed in seven cases because of multiple metastases or a tumor located in a deeper site in the liver. There was no hospital death among the 57 cases.These results show that hepatectomy may offer longer survival, even in patients with multiple or bilobar metastases. Neither the operative procedure nor the size of the surgical margin had any influence on survival after hepatectomy. The prognosis was improved not only for metastases from colorectal cancer, but also for gastric cancer. An increased survival benefit was obtained by repeat hepatectomy for recurrent hepatic metastases. Preoperative portal embolization extended the indication for hepatectomy and provided postoperative safety.

If the superior right hepatic vein (SRHV) is resected, segment 6 will be drained by the inferior right hepatic vein (IRHV) and congestion will not occur. However, early postoperative liver function and long-term regeneration of segment 6 after IRHV-preserving hepatectomy remains unclear.Three patients undergoing IRHV-preserving hepatectomy were examined. One (case 1) had liver metastasis from sigmoid colon cancer and two (cases 2 and 3) had hepatocellular carcinoma. Resection of segments 7 and 8 along with the SRHV was performed in cases 1 and 2. Anterior sectionectomy and resection of segment 4a along with the SRHV and middle hepatic vein (MHV) was performed in case 3. The volume of each sector was calculated by summation of each CT area.The warm ischemic time of the three cases was 50-78 min, operative blood loss was 496-914 g, and operating time was 4-7 h. There was no significant change in diameter of the IRHV and MHV before and after surgery. Intraoperative color Doppler ultrasonography was a useful technique for evaluating intrahepatic venous communication. Part of segment 6 had previously been resected in case 1, and at outpatient follow-up this segment was found to have become atrophic. In cases 2 and 3, the remnant posterior sector became hypertrophic.IRHV-preserving hepatectomy can retain a greater proportion of liver parenchyma than right hepatectomy. Postoperative liver function was well maintained in our patients and there were no serious complications. Long-term follow-up showed hypertrophy of the remnant posterior sector except in one case. To minimize surgical damage, for example in patients with poor liver function or preoperative complications, IRHV-preserving hepatectomy should be considered.

The therapies for treating tumors of the papilla of Vater remain controversial because accurate preoperative diagnosis is difficult. Treatments include endoscopic resection, pancreaticoduodenectomy and total papillectomy. We report the case of a 69-year-old man who underwent total papillectomy for a borderline malignant tumor of the papilla of Vater. In our institution, the decision to perform a total papillectomy for borderline malignant adenoma is based on whether it is intestinal type or pancreaticobiliary type. Carcinoma of the papilla of Vater is classified into two types: an intestinal type and a pancreaticobiliary type and the prognosis of the intestinal type is much better than that of the pancreaticobiliary type. We suggest that total papillectomy can be performed for an intestinal, borderline malignant tumor.

Recently, the significance of preserving the spleen has received a lot of attention. Since our first trial and success of spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein for chronic pancreatitis, this procedure has been more frequently performed and reported. In this study, we introduce the technique and indications for the procedure for intraductal papillary mucinous tumor of the pancreas.Nine patients underwent spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein. We performed this procedure in two patients with intraductal papillary mucinous tumor. The splenic vein is identified behind the pancreas and within the thin connective tissue membrane. The connective tissue membrane is cut longitudinally above the splenic vein. An important technique is to remove the splenic vein from the pancreas from the body of the pancreas toward the spleen. There are many branches from the splenic vein on both sides, and these branches should be carefully ligated and cut. The pancreas is removed from the splenic artery from the spleen toward the head of the pancreas. This procedure is much easier than removing the pancreas from the vein.The postoperative course was uneventful in all nine cases, but one, in which pancreatic fistula continued for more than several weeks. The mean and standard deviation of the operative blood loss, the duration of the operation and the postoperative hospital stay in seven cases, excluding two cases, in which either Puestow's procedure or simultaneous subtotal esophagectomy was performed, were 413+/-385 mL, 298+/-55 min, and 39+/-15 days, respectively. Severe complications were not found in any of the nine cases. The two patients with intraductal papillary mucinous tumor have been followed as outpatients without any recurrence.Spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein is easy and safe, and should be performed for some of the patients with intraductal papillary mucinous tumor of the pancreas.

Preservation of the spleen at distal pancreatectomy has recently attracted considerable attention. Since our first trial and success with spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein for tumors of the pancreas and chronic pancreatitis, this procedure has been performed more frequently. Three patients with intraductal papillary-mucinous tumor underwent spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein. In this procedure, the splenic vein is identified behind the pancreas and the connective tissue membrane is cut longitudinally above the splenic vein. An important point is to remove the splenic vein from the pancreas from the body of the pancreas toward the spleen. In one patient with intraductal papillary-mucinous tumor in the body of the pancreas who had undergone distal gastrectomy for duodenal ulcer 32 years previously, residual proximal gastrectomy could be avoided with this procedure. In this case, the histological diagnosis was a pseudocyst, and epithelial dysplasia was found in other pancreatic ductuli. In another case, epithelia were borderline between hyperplasia and adenoma. In both of these cases, the histological diagnosis was different from the preoperative diagnosis. Even with advances in imaging techniques, diagnosis of a cystic lesion of the pancreas is still very difficult. Ordinary distal pancreatectomy with splenectomy would have been oversurgery in these two cases, which could be avoided using our procedure. Severe complications were not found in any of the three cases and the postoperative course was uneventful. The patients have been followed as outpatients without any recurrence. Spleen-preserving distal pancreatectomy with conservation of the splenic artery and vein is easy and safe, and should be performed for some patients with intraductal papillary mucinous tumor of the pancreas.

The inferior vena cava ligament is a fibrous membrane located around the inferior vena cava. Few reports exist on the ligament's location, attachment to the liver, or the inferior vena cava.We obtained 16 specimens of human liver and inferior vena cava from cadavers. The inferior vena cava ligament was photographed and then dissected for histological examination. Relationships among the ligament, inferior vena cava, and liver were examined microscopically. The numbers and diameters of veins, arteries, and lymph vessels at least 1 mm in diameter were recorded.The cranial margin of the inferior vena cava ligament was ended in a blind loop. The cranial portion above the mid-portion of the Spiegel lobe was thicker than the caudal portion. The ligament was attached to the right and left hepatic veins. The mean length of the right side of the inferior vena cava ligament was 37.0 mm and the mean width 15.6 mm. The inferior vena cava ligament had a mean thickness of 0.8 mm (thin end) and 2.5 mm (thick end). Although the inferior vena cava ligament was usually tightly continuous with the liver capsule, microscopically the attachment between the ligament and the inferior vena cava was loose. The mean number and diameter of veins in the inferior vena cava ligament was 1.0 and 1.4 mm, respectively. The mean number and diameter of arteries was 0.2 and 2.4 mm, respectively. The mean number and diameter of lymphatic vessels was 2.8 and 1.7 mm, respectively.After dissection of the inferior vena cava ligament, major hepatic veins can be dissected extrahepatically. Because the ligament is wider caudally, the forceps should be inserted caudocranially during separation. Since both the number and diameters of lymphatic vessels in the ligament are large, the ligament should be ligated and cut.

Hilar bile duct cancer progresses slowly but easily invades the nearby portal vein or hepatic artery. Thus, in some cases, curative resection is impossible, so we need to determine the best non-surgical treatments for this tumor.We classified 98 patients with hilar bile duct cancer into 3 categories: a non-surgical group (34 cases), an exploratory laparotomy group (9 cases), and a surgical resection group (55 cases). Survival rates were examined in the light of clinical factors.In the non-surgical group, extensive vessel invasion was the most common reason for unresectability (13 cases), with broad biliary extension the second most common (11 cases). In the exploratory laparotomy group the most common reason for unresectability was severe vessel invasion (6 cases). Cumulative 1- and 2-year survival rates for patients with unresectable tumors without distant metastasis were 26.9% and 7.2%, respectively. One- and 2-year survival rates for patients with unresectable tumors and with total bilirubin of less than 2 mg/dL on discharge were 36.8% and 9.8%, respectively. The 1-year survival rate with placement of an expandable metallic stent was as high as 55.6%; without the stent it was 7.1% (P = 0.005). Radiation therapy gave a better prognosis than did no radiation (P = 0.01).Portal and arterial invasion were the principal reasons for unresectability. Use of an expandable metallic stent or radiation therapy, and a total bilirubin level of less than 2 mg/dL on discharge, were factors that enhanced survival in unresectable cases, but distant metastasis, dissemination, and poor general condition or liver function were negative factors for survival.

Although the progression of hilar bile duct cancer is slow, without adequate treatment the prognosis becomes poor. Margin-positive or negative resection has often been reported to correlate with the prognosis. Moreover, the value of combined vessel resection for hilar bile duct cancer is still obscure.Fifty-five out of 98 cases of hilar bile duct cancer were treated with surgery. The patients were classified as a microscopic margin-positive resection group with 12 cases and a microscopic margin-negative resection group with 43 cases. The agreement between preoperative diagnosis and pathological findings of resected specimens was examined, as were cumulative survival rates according to clinical and pathological factors.In diagnosis of portal invasion with computed tomography, the rate of coincidence, overestimate and underestimate were 69.8%, 13.2% and 17.0%, respectively. Corresponding rates in diagnosing arterial invasion were 58.5%, 15.1% and 26.4%, respectively, and for lymph node metastasis 54.7%, 9.4% and 35.8%, respectively. The 5-year survival rates for margin-positive and negative resection groups were 17.9% and 26.5%, respectively (NS). Patients surviving for more than 1 year in the margin-positive resection group tended to exhibit exposed cancer cells only at the bile duct, rarely showing lymph node metastases. Combined arterial resection had a poor prognosis and high, usually lethal, complication rates.Accurate preoperative diagnosis rates of vessel invasion and lymph node metastasis were 60-70% and 56%, respectively. The long-term survival was expected even in margin-positive resection cases without lymph node metastasis. Combined hepatic arterial resection showed no clinical advantage.