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Obstruction of a major hepatic vein, or major portal vein, or biliary tree branch causes atrophy of the related hepatic region, and frequently, hypertrophy in the remaining liver—the atrophy-hypertrophy complex (AHC). Whether hydatid cysts can cause AHC is controversial. The records of 370 patients who underwent surgery for hepatic hydatid disease between August 1993 and July 2002 were evaluated retrospectively. Excluding six patients with previous interventions on the liver, AHC had been recorded in the operative notes of 16 patients (4.4%); for all patients, a cyst located in the right hemiliver had caused atrophy of the right hemiliver and compensatory hypertrophy of the left hemiliver. The computed tomography images of seven patients were suitable for volumetric analysis. The median (range) right and left hemiliver volumes were 334 (0–686) ml and 1084 (663–1339) ml, respectively. The median (range) cyst volume was 392 (70–1363) ml. AHC due to Echinococcus granulosus was confirmed by objective volumetric analysis. The presence of AHC should alert the surgeon to two implications. First, pericystectomy may be hazardous due to association with major vascular and biliary structures. Second, in patients with AHC, the hepatoduodenal ligament rotates around its axis; this should be considered to avoid vascular injury if a common bile duct exploration is to be performed. [Copyright &y& Elsevier]

Primary pancreatic lymphoma (PPL) is a rare tumor that usually presents with the clinical picture of advanced adenocarcinoma but has a much better prognosis. A 38-year-old man was referred after percutaneous transhepatic external biliary drainage for obstructive jaundice. Abdominal magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography had revealed a 5-cm pancreatic head mass that caused biliary tract dilation. Computed tomography angiography showed that the mass encased the celiac trunk as well as the common hepatic and splenic arteries. MRI also revealed a metastatic lesion at the third lumbar vertebra. Serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels were within normal range. The initial diagnosis was inoperable pancreatic adenocarcinoma; however, Tru-Cut pancreatic biopsy showed a large B cell lymphoma. After 6 sessions of chemotherapy and 21 sessions of radiotherapy, both the pancreatic mass and the vertebral metastasis had disappeared. However, he had persistent distal common bile duct stricture that could not be negotiated by either the endoscopic or percutaneous route. A Roux-en-Y hepaticojejunostomy was performed. The patient stayed alive without recurrence for 52 months after the initial diagnosis and 45 months after completion of oncologic treatment. In conclusion, a large pancreatic mass with grossly involved peripancreatic lymph nodes, without ascites, liver or splenic metastasis, should alert the clinician to the possibility of PPL. Cure is possible by chemoradiotherapy even in the presence of vertebral metastasis. Persistent stricture in the distal common bile duct may require a biliodigestive anastomosis.

Aim: To investigate the eligible management of the cystic neoplasms of the liver., Methods: The charts of 9 patients who underwent surgery for intrahepatic biliary cystic liver neoplasms between 2003 and 2008 were reviewed retrospectively. Informed consent was obtained from the patients and approval was obtained from the designated review board of the institution., Results: All patients were female with a median (range) age of 49 (27-60 years). The most frequent symptom was abdominal pain in 6 of the patients. Four patients had undergone previous laparotomy (with other diagnoses) which resulted in incomplete surgery or recurrences. Liver resection (n = 6) or enucleation (n = 3) was performed. The final diagnosis was intrahepatic biliary cystadenoma in 8 patients and cystadenocarcinoma in 1 patient. All symptoms resolved after surgery. There has been no recurrence during a median (range) 31 (7-72) mo of follow up., Conclusion: In spite of the improvement in imaging modalities and increasing recognition of biliary cystadenoma and cystadenocarcinoma, accurate preoperative diagnosis may be difficult. Complete surgical removal (liver resection or enucleation) of these lesions yields satisfying long-term results.

OGTT was performed in 28 liver transplants maintained with tacrolimus to investigate carbohydrate metabolism and assess risk factors for development of PTDM. None had PTDM that was detected by OGTT. Early PTDM in four cases (14.3%) resolved in follow-up. Five new cases (17.9%) demonstrated DCM (DCM = IGT +/- hyperinsulinemia). Fasting measurements were normal in two hyperinsulinemic cases. With one (20%, p > 0.05) exception none of the children with DCM were overweight or had a family history of diabetes. All five (100%) children with DCM had been given high cumulative dosage of steroids 18 (78.3%)--without DCM (p > 0.05). The median age of children with DCM was greater [4.3 (12.7-18.0) vs. 7.0 (2.3-18.0) yr, p < 0.01] and duration of follow-up longer [5.3 (2.3-7.0) vs. 2.5 (0.7-7.3) yr, p < 0.05]. Four children (80%) with DCM were pubertal (p < 0.05). However, neither age nor duration of follow-up or pubertal stage had significant effect on DCM development. Early PTDM is a transient phenomenon and is not predictive for future development of diabetes. DCM is frequently observed in liver transplanted children. Albeit the children with DCM were given high cumulative dose of steroids, were older, mostly were pubertal, and had longer duration of follow-up, we cannot draw firm conclusions on effects of the risk factors on carbohydrate metabolism because of the small sample size and relatively short duration of follow-up. Unlike fasting measurements, OGTT can detect all children with DCM.

A patient who developed necrotizing pancreatitis after transcatheter arterial chemoembolization (TACE) for hepatocellular carcinoma (HCC) is presented. A 55- year-old man had been followed for chronic hepatitis B infection for 10 years at another institution. He presented with multiple masses in the right lobe of the liver and a metastasis in the left adrenal gland. He was referred after a percutaneous liver biopsy which revealed a moderately differentiated HCC. He was treated by TACE. At the third session of TACE, the right hepatic artery was found to be thrombosed; however, angiography also demonstrated collateral feeder vessels (arising from the pancreaticoduodenal artery) which were used for treatment. He developed necrotizing pancreatitis, possibly due to regurgitation of the chemotherapeutic agents to the pancreas. He recovered without complications with imipenem-cilastatin prophylaxis. Acute pancreatitis is a rare but severe complication of TACE. Selective catheterization of the tumor vessels is the established standard in TACE. A careful risk-benefit analysis is mandatory in patients with abnormal collateral vessels. Treatment of acute necrotizing pancreatitis (ANP) after TACE is the same as the accepted approach to ANP due to other causes.

We report our success with somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation. A 48-year-old woman with cirrhosis due to autoimmune hepatitis underwent living-donor liver transplant; her graft-to-body weight ratio of the right lobe was 0.91%. After arterial reperfusion, portal pressure and flow were 24 cm H20 and 2.22 L/min (ie, 360 mL/100g graft/min), respectively. Following splenic artery ligation, the portal pressure decreased to 16 cm H20 and portal flow to 1.74 L/min (ie, 282 mL/100g graft/min). On the second postoperative day, small-for-size syndrome was diagnosed based on the marked prolongation of prothrombin time (international normalized ratio, 4.4), hyperbilirubinemia (359.1 micromol/L), rapid escalation of transaminases (alanine aminotransferase 2488 U/L, aspartate aminotransferase 1075 U/L) and very high portal flow rate (> 90 cm/sec). Oral propranolol (40 mg/day b.i.d.) and somatostatin infusion (250-microgram bolus followed by perfusion at a rate of 250 microgram/h for 5 days) were started. Prothrombin time and transaminase levels began to decrease the following day, although the bilirubin level increased to 495.9 micromol/L before returning to normal. The patient was discharged in excellent health 5 weeks after surgery. Despite reduction of portal pressure by splenic artery ligation, small-for-size syndrome may develop in patients with persistent high portal flow. To the best of our knowledge, this is the first report of the successful treatment of small-for-size syndrome by somatostatin and propranolol in the clinical setting.

An 11-month-old female infant underwent living-donor liver transplantation for secondary biliary cirrhosis 8 months after Kasai operation. The portal vein was hypoplastic, and its diameter was only 4 mm at the level of the splenomesenteric confluence. End-to-end anastomosis of the recipient suprarenal vena cava to the graft portal vein (a left lateral section from the patient's mother) was performed. An end-to-side portocaval shunt with the recipient portal vein was constructed to mitigate portal hypertension. The early postoperative course was relatively uneventful. However, persistent hepatitis caused by infection with Cytomegalovirus and chronic rejection resulted in progressive hepatic dysfunction. Nine months after the initial operation, a living-donor retransplantation (a left lateral section from the patient's grandmother) was performed. One month after retransplantation, severe acute rejection that eventually required OKT3 treatment developed. The patient was in excellent health until 4 months after retransplantation, when another acute rejection episode (for which she was successfully treated) developed. Cavoportal hemitransposition should be included in the armamentarium of the transplant surgeon for the management of extensive portal system thrombosis and portal vein hypoplasia. An additional shunt may be useful in mitigating portal hypertension.

Major bile duct injury during cholecystectomy represents potentially severe complications with unpredictable long-term results. If these lesions are not treated adequately, they can lead to hepatic failure or secondary biliary cirrhosis therefore requiring liver transplantation. We report a patient who required liver transplantation 15 years after open cholecystectomy. A l0-year old girl underwent open cholecystectomy and duodenal repair for cholelithiasis and cholecystoduodenal fistula. She required two surgical interventions, hepaticojejunostomy which was performed in another center and portoenterostomy for biliary stricture at our institution seven years after the cholecystectomy. Eight years after the third operation, she required recurrent hospitalization for treatment of hepatic abscesses. The extremely short intervals between the three life threatening episodes and the rapid progression to severe sepsis were taken into consideration and liver transplantation was performed at the age of 25. She is leading a healthy life at 4 years post transplantation. Although iatrogenic biliary injury can usually be treated successfully by a combination of surgery, radiological and endoscopic techniques, patients with severe injuries develop irreversible liver disease. This case report and review of the literature suggest that liver transplantation is a treatment modality for a selected group of patients with end-stage liver disease secondary to bile duct injury.

Background: Autoimmune pancreatitis is an evolving entity., Methods: A patient who had spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis is presented., Results: A 58-year-old diabetic woman with jaundice was referred for pancreatic head carcinoma diagnosed by magnetic resonance imaging (MRI). At laparotomy, a pancreatic head mass (4 x 3 cm) that involved the transverse mesocolon and two other hard masses (1 cm) in the pancreatic body and tail were found. The gallbladder was palpated as a hard tumor mass. Frozen section examination of the gallbladder and pancreatic biopsies revealed cholecystitis and pancreatitis with lymphoplasmacytic infiltration. The common bile duct was brittle and unsuitable for anastomosis. Starting 1 month after the operation, drainage from the biliary catheter decreased gradually and stopped. There was no parenchymal lesion on MRI examination in the 2nd postoperative month. Cholangiography from the percutaneous catheter showed flow of contrast agent into the duodenum. Serum immunoglobulin G, G4 and E levels were increased., Conclusion: To the best of our knowledge, this is the first report of spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis.

Background: We presented our experience with definitive treatment of traumatic biliary injuries., Methods: Six male patients (mean age 13 years; range 2 to 32 years) who were referred to our unit for definitive treatment of traumatic biliary injuries were retrospectively evaluated. Data were analyzed in terms of demographic characteristics, mechanisms of injuries, associated injuries, previous treatments, symptoms on admission, treatment at our unit, and the results of treatment. Outcome was assessed using modified Schweiser and Blumgart criteria., Results: The injuries were due to blunt abdominal trauma in all the patients but one who had a gunshot wound. In three patients, biliary injuries were missed at the initial operation. On admission, three patients had external biliary fistulas, two had biliary strictures. One patient was sent following inadvertent ligation of the hepatoduodenal ligament during attempts to control hemorrhage. Roux-en-Y hepaticojejunostomy was performed in three patients. Percutaneous biloma drainage was performed in two patients, resulting in fistula closure in 13 and 40 days, respectively. One patient was treated by endoscopic retrograde cholangiopancreatography and papillotomy, which enabled fistula closure in three days. One patient was lost to follow-up. One patient died from hepatic failure 11 years after the trauma. At the end of a mean follow-up of 49 months (range 15 to 75 months), three patients were in excellent condition, while one patient experienced occasional attacks of cholangitis., Conclusion: In patients with undetected biliary injuries and in those with unsuccessful repair attempts, biliary reconstruction should be performed in experienced hepatopancreatobiliary surgery units.

Background and Aims: To evaluate treatment results in iatrogenic biliary injuries with concomitant vascular injuries., Patients/methods: Between January 1998 and May 2002 (inclusive), angiography was performed in 45 of the 105 patients treated for iatrogenic biliary tract injury. The charts of these 45 patients and 5 other patients in whom vascular injury was diagnosed at operation were evaluated retrospectively. Twenty-nine patients had concomitant vascular injury, the biliovascular injury group (BVI), and the remaining 21 patients had isolated biliary tract injury (IBTI)., Results: The most frequent initial operation was a cholecystectomy. The frequency of high-level (Bismuth III or IV) strictures was 90% in the BVI group and 62% in the IBTI group ( P<0.05). Perioperative mortality was 7% in the BVI group and 5% in the IBTI group ( P>0.05). The morbidity in the BVI group was significantly higher ( P<0.05). Two patients in each group were lost to follow up. During a median (range) follow up of 31 months (5-51 months), a successful functional outcome was achieved in 96% of the BVI group and 100% of the IBTI group with a multimodal approach ( P>0.05)., Conclusions: The frequency of high-level biliary injury and morbidity were significantly higher in the BVI group. However, concomitant vascular injury had no significant effect on mortality and medium-term outcome of biliary reconstruction. Thus, routine preoperative angiography is not recommended.

Background: Radical resection is the only potentially curative treatment for hepatic alveolar echinococcosis (AE). Although Turkey is an endemic region, population screening is not performed and early diagnosis is rare. Consequently, surgeons are compelled to explore possibilities such as near-total resection and biliodigestive anastomosis for palliation of jaundice., Methods: Surgery was performed in 32 patients with hepatic AE with the following indications: (1) resection; (2) palliation of jaundice; (3) definite assessment of operability; (4) failure in the management of cavity infection by percutaneous methods. Curative resection (R0 = complete resection of all parasitic mass [n = 9], and R1 = a resection in which a small remnant was left on a vital structure [n = 8]) were performed in 17 patients, intrahepatic cholangiojejunostomy in 7, laparotomy-external drainage in 7, and debulking in 1., Results: Perioperative mortality rates were 2/17, 0/7, 2/7 and 1/1, respectively. Twelve patients in the curative resection group are alive without recurrence/progression of the small remnant during a median follow-up of 59 (range 27-116) months. One patient developed an inoperable recurrence that was treated with albendazole. One patient was lost to follow-up. Long-term albendazole treatment was effective in all R1 patients except a patient who had slow asymptomatic progression. Successful palliation of jaundice was achieved in 5 of the 7 intrahepatic cholangiojejunostomy patients., Conclusions: The results of R1 resection in alveolar hydatid disease are similar to those of R0 resection; a small remnant is successfully controlled by albendazole. In patients with jaundice due to hilar invasion, biliary diversion from segment 3 or 5 is effective for palliation of the jaundice and facilitates albendazole treatment., (Copyright 2003 S. Karger AG, Basel)

A 54-year-old woman was referred with the diagnosis of hepatic angiosarcoma, made by percutaneous biopsy under ultrasonographic guidance. Ultrasonography (US) had revealed a 48 x 42 x 35 mm mass in the right lobe. Standard biochemical tests and whole blood count had yielded normal results. At our institution, magnetic resonance imaging demonstrated a hypervascular mass in the right lobe. Alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 levels were normal. Serological tests were negative for hepatitis B and C viruses. There was no evidence of metastasis. A right hepatectomy was performed. Histopathological examination confirmed the diagnosis of angiosarcoma. However, there was a suspicion of microscopically positive margins. Relaparotomy and resection of a 1-cm-thick slice of hepatic parenchyma was performed. Histopathological examination revealed necrotic tumor cells at the previous margin. The new surgical margin was tumor free. Due to the expected poor prognosis, prophylactic chemoembolization of the remnant liver (lipiodol + adriamycin + mitomycin) was performed at 3 and 7 months postoperatively. She has been alive without recurrence for 5 years and 4 months. Hepatic angiosarcoma has two distinct presentations: multiple tumors and a solitary tumor. The reported poor results largely stem from the predominance of the multiple tumors and consequent unresectability. Long-term survival is possible in solitary resectable hepatic angiosarcomas.