Surgical reconstruction of major bile duct injuries: Long-term results and risk factors for restenosis.

BACKGROUND: A single-institution retrospective analysis was undertaken to assess long-term results of definitive surgical reconstruction for major bile duct injuries and risk factors for restenosis. METHODS: Patients treated between January 1995 and October 2020 were reviewed retrospectively. The primary outcome measure was patency. RESULTS: Of 417 patients referred to a tertiary center, 290 (69.5%) underwent surgical reconstruction; mostly in the form of a hepaticojejunostomy (n = 281, 96.8%). Major liver resection was undertaken in 18 patients (6.2%). There were 7 postoperative deaths (2.4%). Patency was achieved in 97.4% of primary repairs and 88.8% of re-repairs. Primary patency at three months (including postoperative deaths and stents removed afterwards) in primary repairs was significantly higher than secondary patency attained during the same period in re-repairs (89.3% vs 76.5%, p < 0.01). The actuarial primary patency was also significantly higher compared to the actuarial secondary patency 10 years after reconstruction (86.7% vs 70.4%, p = 0.001). Vascular disruption was the only independent predictor of loss of patency after reconstruction (OR 7.09, 95% CI 3.45-14.49, p < 0.001), showing interaction with injuries at or above the biliary bifurcation (OR 9.52, 95% CI 2.56-33.33, p < 0.001). CONCLUSIONS: Long-term outcome of surgical reconstruction for major bile duct injuries was superior in primary repairs compared to re-repairs. Concomitant vascular injury was independently associated with loss of patency requiring revision.

[NA Rare Bacterial Pathogen in a Patient with Perihilar Cholangiocarcinoma: Erwinia persicina; First Case From Turkey]. / Perihiler Kolanjiyokarsinomlu Hastada Nadir Bir Bakteriyel Patojen: Erwinia persicina; Türkiye'den Bildirilen Ilk Olgu.

Members of the Erwiniaceae family, which can be found saprophytic in humans, have been identified several times as an infectious agent after their first identification in 1920. Erwinia persicina was first identified as a plant pathogen by being isolated from cucumber, tomato and banana in 1990, and it was shown to cause disease in many plant species in the following years. E.persicina was diagnosed as a urinary tract infection agent in an 88-year-old female patient in 1998. Our case, a 30-year-old male patient, was hospitalized for perihilar cholangiocarcinoma while being examined with the complaint of abdominal pain. In preparation for the operation, external drainage from the left lobe biliary tract was performed. The same bacterial growth was detected in the three bile fluid cultures of the patient taken on different dates. The bacterium was identified as E.persicina by MALDI-TOF Microflex LT/SH Smart MS (Bruker Daltonics, Germany) and Erwinia rhapontici with VITEK MS (Biomerieux, France), Rahnella aquatilis with VITEK 2 automated system, Pantoea agglomerans with BD Phoenix™ M50 (BD Diagnostics, USA) automated system. E.persicina identification was also obtained by Sanger sequencing. Antibiotic susceptibility results were evaluated according to the non-species related breakpoints criteria of the European Committee on Antimicrobial Susceptibility Testing (EUCAST). While resistance was found to cefuroxime and cefazolin, the isolate was found to be sensitive to many other beta-lactam antibiotics, quinolones and aminoglycosides. E.persicina is a bacterium that is rarely isolated as an infectious agent in humans. The reason for this may be that it is a plant pathogen on the one hand, and mistakes made in its diagnosis on the other. Many identification systems do not have this bacterium in their library. In this case report, our aim was to emphasize that mistakes made in the diagnosis of E.persicina may play a role in the rare occurence of the agent.

Choledochal cysts: Management and long-term follow-up.

BACKGROUND: Choledochal cysts are congenital anomalies that can occur at any level of the biliary tree. They carry long-term risk of biliary complications and cancer development. Complete excision of all involved bile ducts is recommended. METHODS: Patients treated between 1995 and 2019 were reviewed retrospectively. RESULTS: Sixty patients; 46 female and 14 male with a median age of 41 years (range 13-83) were included in the study. Mild abdominal pain was the most common presenting symptom (60%). Majority of the patients had Todani type I cysts (67%). Concomitant biliary malignancy was diagnosed in five patients (9%). Eight patients were followed-up conservatively (13%). Twenty-five patients were treated by excision of the extrahepatic bile ducts and Roux-en-Y hepaticojejunostomy, liver resection was added in seven, pancreatoduodenectomy was done in three and liver transplantation in one. There was no perioperative mortality. Postoperative complications developed in 17 patients (34%), two requiring surgical treatment. Four of the five patients with malignancies died at a median 42 months (range 6-95) following surgery. Median 62 months (range 8-280) follow-up was available in 45 surgically treated patients, 19 followed-up for more than 10 years. None of the patients developed malignancy during follow-up. Four patients (17%) were readmitted for anastomotic strictures requiring treatment. CONCLUSION: The majority of choledochal cysts are Todani type-I and early cyst excision is the mainstay of management, which may decrease the risk of malignant transformation.

Observed or Predicted Albendazole Hepatotoxicity as an Indication for a Resection Procedure in Hepatic Hydatid Disease - A Short Series of Cases.

OBJECTIVE: To highlight the role of albendazole hepatotoxicity in the choice between drainage versus a resection procedure in hepatic hydatidosis. Methods: The charts of four patients were reviewed retrospectively. In three patients, albendazole caused more than 10-fold increases in transaminase levels and was stopped. One patient had concomitant autoimmune hepatitis. Results: In the first case, two large hydatid cysts involving the right and the left hepatic veins were detected. First, left lateral sectionectomy and ligation of the right posterior portal vein branches were performed. Hypertrophy of the remnant liver allowed a safe right posterior sectionectomy two months later. In the second patient, a 9-cm cyst in segments 6 and 7 was treated with pericystectomy. The third patient had a 6-cm centrally located cyst. Pericystectomy, removal of small vesicles from the anterior section bile duct, common bile duct exploration with a T-tube placement were performed. In the patient with auto-immune hepatitis, pericystectomy was chosen for two objectives: 1) to eliminate a cavity prone to recurrence in an immunosuppressed patient 2) to avoid albendazole that may complicate the interpretation of liver function tests. The postoperative period and early follow up of all patients was uneventful. The second and the fourth patients have been followed for 56 and 17 months respectively and no recurrence has been detected. Conclusions: A resection procedure eliminates the cavity and the need for adjuvant albendazole treatment. This is a vital advantage for the small subset of patients with severe albendazole hepatotoxicity.

Superselective Transarterial Chemoembolization as an Alternative to Surgery in Symptomatic/Enlarging Liver Hemangiomas.

BACKGROUND: Transarterial embolization of liver hemangiomas has not been considered to be consistently effective. METHODS: The charts of 25 patients who underwent superselective transarterial chemoembolization with the bleomycin-lipiodol emulsion were evaluated retrospectively. RESULTS: Twenty-two patients had abdominal pain; asymptomatic/vaguely symptomatic enlargement was the treatment indication in three patients. A single session was conducted in 17 patients, two sessions in 7 and three sessions in one. After the first session, lesion volume decreased by median (range) 51% (10-92%) from median (range) 634 (226-8435) to 372(28-4710) cm3 (p < 0.01), after a median period of 4 months (range 2-8). A second session was performed in eight patients (median (range) initial volume 1276 (441-8435) cm3) with persistent complaints and/or large lesions receiving feeders from both right and left hepatic arteries (staged treatment). Median (range) lesion size decreased further from 806 (245-4710) to 464 (159-2150) cm3 (p < 0.01). Three patients experienced a postembolization syndrome that persisted after the first week. Seventeen of the 22 symptomatic patients (77%) reported resolution or marked amelioration of complaints. Regrowth after initial regression was not observed during median (range) 14 (8-39) months of follow-up (n:18). CONCLUSION: Transarterial chemoembolization with the bleomycin-lipiodol emulsion is a potential alternative to surgery for symptomatic/enlarging liver hemangiomas. Volume reduction is universal, and symptom control is satisfactory. Centrally located and very large (>1000 cm3) lesions may require two sessions.

Successful results of early nucleos(t)ide analogue treatment for liver transplantation candidates with severe acute hepatitis B infection.

BACKGROUND: Early treatment of severe acute hepatitis B virus (HBV) infection with nucleos(t)ide analogues may prevent progression to acute liver failure (ALF). PATIENTS AND METHODS: The charts of 24 patients who were treated for severe acute HBV infection (either INR ≥ 1.5 or INR≥ 1.4 and total bilirubin ≥ 20 mg/dL at the referring institution or after admission) between April 2021 and May 2023 (inclusive) were evaluated retrospectively. Twelve patients were women; median [range] age: 48 [35-68]. Entecavir (0.5 mg/day) (n = 16) or tenofovir disoproxil fumarate (245 mg/day) (n =8) were used depending on availability. RESULTS: Two patients required liver transplant which was performed successfully in one (no suitable donor for the other). Deterioration to ALF was prevented in 22 of the 24 cases (92%); these patients could be discharged after median (range) 12 (5-24) days following initiation of the antiviral drug. There was no significant difference in efficacy between the two antiviral agents. The anti-HBsAg antibody became positive in 16 patients (73%); one other patient became HBsAg negative at 1 month after discharge but was lost to follow up. Five patients (23%) are still HBsAg positive but all except one have started treatment in the last 6 months. One of the recently treated 4 patients stopped taking the antiviral drug at his own will and one has become anti-HIV antibody positive during follow up. CONCLUSION: Early treatment of severe acute HBV infection with entecavir or tenofovir disoproxil fumarate prevents the need for liver transplant and consideration of living donors.

Entecavir-induced neutropenia in an adult living donor liver transplant recipient: Successful conversion to tenofovir alafenamide.

At 22 weeks post-transplantation for HBV-related cirrhosis, an adult woman developed neutropenia which was aggravated by COVID-19 (ANC 0.4 × 109/L). Covid resolution and all "conventional" modifications were ineffective. Success within 2 weeks was achieved by switching entecavir to tenofovir alafenamide. A step-by-step judicious approach to post-transplant neutropenia is vital.

Dramatic response to albendazole in transplantation candidates with unresectable hepatic alveolar hydatid disease.

Long-term albendazole treatment should be given to all patients with unresectable hepatic alveolar echinococcosis as dramatic regression is possible in 15%-20%. It may be prudent to prepare a living donor for possible salvage transplant in case of a severe complication. Preemptive transplantation in mildly symptomatic patients should be discouraged.

Spontaneous regression of liver hemangiomas: a single-institution analysis of 46 patients.

OBJECTIVE: The aim of this study was to determine the nature of spontaneous regression of liver hemangiomas. PATIENTS AND METHODS: The records of the liver hemangioma patients who attended the out-patient clinic between 1988 and 2018 were evaluated. The data of the 716 adult patients who were followed for at least 3 years with cross-sectional imaging were analyzed. RESULTS: Spontaneous regression was documented in 46 patients (6.4%). Twenty-eight patients had a single hemangioma (61%), eight (17%) had two hemangiomas; the other 10 patients had 3-6 hemangiomas. Of the 87 lesions in 46 patients, 69 actually regressed during the study. Twelve patients with more than one lesion exhibited discordant courses - one of the hemangiomas of a patient with multiple lesions regressed, whereas the other enlarged or remained stable. Eleven of the regressed hemangiomas exhibited enlargement first, followed by spontaneous regression. Fourteen (20%) of the regressed hemangiomas acquired atypical characteristics that would have suggested a malignancy had the original films been unavailable. CONCLUSION: Spontaneous regression of liver hemangiomas is an underrecognized phenomenon. Enlargement should not be a straightforward indication for intervention because it may be followed by regression. A regressed hemangioma should be considered in the differential diagnosis of liver lesions suspicious for malignancy.

Malignant Tumors Misdiagnosed as Liver Hemangiomas.

Background and Aim: To derive lessons from the data of patients who were followed for various periods with the misdiagnosis of liver hemangioma and eventually found to have a malignancy. Material and Methods: The records of 23 patients treated between 2003 and 2018 were analyzed retrospectively. Results: Twelve patients were men and 11 were women; median (range) age was 55 (35-80). The principal diagnostic modality for the initial diagnosis was ultrasonography (n:8), magnetic resonance imaging (MRI) (n:13), and computed tomography (CT) (n:2). At our institution, MRI was performed in 16 patients; the diagnosis was made with the available MRI and CT studies in five and two patients, respectively. In other words, the ultrasonography interpretations were not confirmed on MRI; in others, the MRI or CT examinations were of low quality or they had not been interpreted properly. Fifteen patients underwent surgery; the other patients received chemotherapy (n:6) or chemoembolization (n:2). The misdiagnosis caused a median (range) 10 (0-96) months delay in treatment. The final diagnoses were hepatocellular carcinoma in 12 patients, cholangiocarcinoma in four patients, metastatic mesenchymal tumor, metastasis of colon cancer, metastatic neuroendocrine carcinoma, sarcomatoid hepatocellular carcinoma, angiosarcoma, thoracic wall tumor, and metastatic tumor of unknown primary in one patient each. Conclusions: High-quality MRI with proper interpretation and judicious follow up are vital for the accurate differential diagnosis of liver lesions.

Complementary Roles of Cadaveric and Living Donor Liver Transplantation in Acute Liver Failure.

BACKGROUND: Living donor liver transplantation may complement cadaveric transplantation in acute liver failure (ALF) patients. METHODS: Between 2008 and 2017, 89 patients were treated for ALF; 15 patients (17%) recovered with intensive care treatment; 31 (35%) died without transplant. The records of the remaining 43 patients (median (range) age: 14 (1-62)) who underwent transplantation were evaluated. RESULTS: The etiologic factors were toxic agents (10; mushrooms: 8; herbs: 2), hepatitis viruses (7; A: 1; B: 6), Wilson's disease (7), autoimmune hepatitis (4), and Budd-Chiari syndrome (2); 13 cases were idiopathic. Cadaveric organs (whole, split, reduced) were transplanted to 32 patients; 11 patients underwent living donor transplantation. One patient (2%) died of septic shock on the second postoperative day. Bacterial infection was the most common early (< 3 months) complication in the remaining patients (31/42; 74%), followed by delirium (5/42; 12%) and acute rejection requiring steroid pulse (5/42; 12%). Seven other patients died during median (range) follow-up of 94 (14-142) months: various infections (5), leukemia (1), and acute myocardial infarction (1). The 1-, 5-, and 10-year survival rates were 100%, 96%, and 92% in children and 94%, 82%, and 65% in adults respectively. CONCLUSIONS: Cadaveric organ sharing and transplantation from living donors when appropriate yield a high survival rate, despite high early morbidity, in ALF patients whose conditions deteriorate despite intensive care treatment. Efforts to eliminate preventable causes of acute liver failure will lead to more efficient use of health care resources.

Steroid-induced reconstitution of the biliary tree ravaged by IgG4-related disease.

The steroid-induced, rapid healing of the biliary tree ravaged by IgG4-related disease shows that the point of irreversibility remains to be defined.

Investigation of impaired carbohydrate metabolism in pediatric liver transplant recipients.

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.

Does HepPar-1 immunoexpression have a role in differential diagnosis of periampullary cancer?

AIMS: Histological subtyping of periampullary carcinomas is considered as a criterion for prognosis and therapeutic implications of these tumours. We assessed the immunoexpression rates of HepPar-1, CDX2 and MUC2 antibodies in different subtypes of periampullary adenocarcinomas (PAC), intestinal and pancreatobiliary, in order to assess their impact on differential diagnosis of this group of cancers. The expression of antibodies was also measured in ductal adenocarcinoma of the pancreatic head (DAPH). METHODS: Sixty-five patients with PAC and DAPH who underwent pancreatic Whipple resection constituted the study cohort. Of these, 46 (71%) had PAC, and 19 (29%) had DAPH. Among PACs, 20 (44%) were intestinal and 26 (56%) were pancreatobiliary type. RESULTS: HepPar-1 immunoreactivity was detected in 18 (39%) of all PACs. The rate of HepPar-1 expression was significantly higher in intestinal type PAC (75%) than it was in pancreatobiliary type (12%). The sensitivity, specificity, and accuracy of HepPar-1 immunoexpression for diagnosing intestinal type PAC were 75% , 89%, and 83%, respectively. Similarly, the rates of both CDX2 and MUC2 expressions were significantly higher in intestinal type PAC (80%) than they were in pancreatobiliary type (8%). The sensitivity, specificity, and accuracy of both CDX2 and MUC2 immunoexpressions for intestinal type PAC were 80%, 92%, and 87%, respectively. CONCLUSION: HepPar-1 antibody was found to be a highly sensitive and specific marker for distinguishing intestinal type from pancreatobiliary type among PACs. In addition to CDX2 and MUC2 antibodies, HepPar-1 immunoexpression seems to have a potential role in differential diagnosis of PACs.

Somatostatin and propranolol to treat small-for-size syndrome that occurred despite splenic artery ligation.

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.

Transplantation for Large Liver Tumors in the Setting of Abernethy Malformation.

In this study, we report our experiences on the role of transplantation in 2 patients with large liver tumors in the setting of Abernethy malformation. Patient 1 was a 17-year-old boy who was referred for hepatic masses and recurrent hepatic encephalopathy episodes. Computed tomography and magnetic resonance imaging showed 2 large tumors (4 and 8 cm) in the liver. The portal vein drained directly into the vena cava. Core biopsy of the larger mass revealed fibrosis and regenerative hyperplasia. There were hyperintense signals in the T1-weighted images in the globus pallidus. The Stanford-Binet intelligence scale showed moderate mental retardation (IQ 39); however, the patient showed good ability for caring for himself. His cognitive defect was ascribed partially to chronic encephalopathy. The patient received a right hepatic lobe from his older brother. The congenital portacaval shunt was disconnected to provide inflow to the graft. Pathologic examination of the explanted liver revealed no evidence of malignancy. His IQ improved to 75 at 29 months posttransplant. The hyperintensity of the globus pallidus on magnetic resonance imaging disappeared. The patient has maintained a normal life during 9 years of follow-up. Patient 2 was a 17-year-old girl who was referred for multiple hepatic masses; she had no symptoms at admission. Magnetic resonance imaging showed type 1 Abernethy malformation and multiple hepatic masses (largest was 10 cm), which appeared to be hyperplastic lesions. Because malignancy could not be definitely excluded, she received a right lobe without the middle hepatic vein from her uncle. Pathologic examination of the explanted liver showed localized nodular hyperplasia; there was no evidence of malignancy. She has maintained normal life activities during 3 years of follow-up. Liver transplant is a curative treatment option for patients with large liver tumors, replacing the hepatic parenchyma in the setting of Abernethy malformation.

Hepatic atrophy-hypertrophy complex due to Echinococcus granulosus.

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.

A rare intraabdominal tumor: giant hepatic artery aneurysm.

A 55-year-old man was investigated for right upper abdominal quadrant pain. He had no history of abdominal trauma or surgery. Imaging studies showed a common hepatic artery aneurysm involving the gastroduodenal artery. Following aneurysmectomy, examination with a hand Doppler apparatus yielded clear arterial signals from the liver surface. Therefore, vascular reconstruction was not performed. He had an uneventful postoperative course.

Successful living-donor liver transplantation and retransplantation with cavoportal hemitransposition: a case report.

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.