Consensus Guidelines for the Use of Fluorescence Imaging in Hepatobiliary Surgery.

OBJECTIVE: To establish consensus recommendations for the use of fluorescence imaging with indocyanine green (ICG) in hepatobiliary surgery. BACKGROUND: ICG fluorescence imaging has gained popularity in hepatobiliary surgery in recent years. However, there is varied evidence on the use, dosage, and timing of administration of ICG in clinical practice. To standardize the use of this imaging modality in hepatobiliary surgery, a panel of pioneering experts from the Asia-Pacific region sought to establish a set of consensus recommendations by consolidating the available evidence and clinical experiences. METHODS: A total of 13 surgeons experienced in hepatobiliary surgery and/or minimally invasive surgery formed an expert consensus panel in Shanghai, China in October 2018. By the modified Delphi method, they presented the relevant evidence, discussed clinical experiences, and derived consensus statements on the use of ICG in hepatobiliary surgery. Each statement was discussed and modified until a unanimous consensus was achieved. RESULTS: A total of 7 recommendations for the clinical applications of ICG in hepatobiliary surgery were formulated. CONCLUSIONS: The Shanghai consensus recommendations offer practical tips and techniques to augment the safety and technical feasibility of ICG fluorescence-guided hepatobiliary surgery, including laparoscopic cholecystectomy, liver segmentectomy, and liver transplantation.

Rare biliary cystic tumors: a case series of biliary cystadenomas and cystadenocarcinoma.

Cystic lesions of the liver are common and a major proportion is formed by parasitic cysts and simple cysts. Biliary cystic tumors (BCTs), namely biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCAC), are rare tumors which usually arise from the intrahepatic biliary tree. BCAs have malignant potential and are difficult to differentiate from BCAC pre-operatively on radiological imaging. Here we have presented 4 patients with BCTs and reviewed the literature pertaining to them.The data of four patients with BCA/BCAC diagnosed and treated at our institute were retrieved from our database and records were reviewed for age, sex, history, imaging, surgery, pathology and follow-up. Mean age of the patients was 53.5 years (range 30-71 years). Two male and two female patients presented with abdominal pain, of which one male patient had pancreatitis at diagnosis. Characteristic features were seen on pre-operative imaging (cystic lesions with internal septations) and biliary communication was identified in the patient with pancreatitis. Three patients were diagnosed with a BCA on final histology, while one patient had a BCAC. Following surgical resection, all the patients are asymptomatic and disease free with a mean follow-up of 24 months (range 10-40 months). In conclusion, BCTs should be suspected in the presence of a well-encapsulated, cystic hepatic lesion with internal septations. Although pre-operative distinction between BCA and BCAC is difficult, the lesion, whenever possible, should be completely resected as long-term outcomes are good, especially with BCA.

Choledochal cysts with chronic pancreatitis in adults: report of two cases with a review of the literature.

CONTEXT: Choledochal cysts, rarely present with chronic calcific pancreatitis. We report two patients with choledochal cysts who had concomitant chronic pancreatitis. CASE REPORT #1: A 27-year-old female with a history of recurrent abdominal pain, fever and jaundice presented with a type I choledochal cyst with calcifications in the uncinate process of the pancreas on CT scan. Her magnetic resonance cholangiopancreatogram (MRCP) revealed calcifications in the region of the uncinate process of the pancreas, the presence of a type I choledochal cyst with dilatation of the right and left hepatic ducts at their confluence suggesting an anomalous pancreaticobiliary ductal junction. She underwent choledochal cyst excision with a Roux-en-Y hepaticojejunostomy. CASE REPORT #2: A 35-year-old male with colicky abdominal pain of four months duration whose CT scan was suggestive of an atrophic pancreas with a 1 cm dilatation of the pancreatic duct and a calculus in the pancreatic duct near the ampulla. MRCP showed significant atrophy of the pancreas with an isointense filling defect seen in the pancreatic duct at its distal end near the ampulla. A diagnosis of chronic calcific pancreatitis with type I choledochal cyst was made. He underwent choledochal cyst excision with a cholecystectomy, hepaticojejunostomy (end-to-side) and side-to-side pancreaticojejunostomy. CONCLUSION: Chronic calcific pancreatitis is a rare occurrence in patients with choledochal cysts and only six cases have been reported in the literature. Our two patients with choledochal cysts associated with chronic pancreatitis were treated surgically.

An Indian family of multiple endocrine neoplasia type 1 (MEN1): molecular diagnosis, treatment and follow up.

BACKGROUND/OBJECTIVE: Multiple endocrine neoplasia type 1 (MEN1) is an autosomal, dominant syndrome, characterized mainly by the combination of tumors involving the parathyroid, pancreatic and pituitary glands. Genetic sequencing leading to early treatment of family members has not yet been reported in Indian patients. METHODS: We performed molecular analysis of the MEN1 gene to identify mutations in an Indian family with MEN1 syndrome. The proband was identified with multiple peptic ulcers because of multifocal recurrent gastrinomas, as well as parathyroid and pituitary adenomas. All the 10 exons of the MEN1 gene were amplified using the polymerase chain reaction (PCR). The MEN1 gene was then screened by direct DNA sequencing. RESULTS: The proband is asymptomatic 3 years after total pancreatectomy and removal of parathyroid adenomas. DNA sequencing revealed the presence of a heterozygous Y227X mutation in exon 4 of the MEN1 gene in the proband. Four of the seven mutant-carrying family members are at present asymptomatic. Following screening, one asymptomatic child has been identified with and treated for insulinoma and parathyroid adenoma. CONCLUSION: Detection of the MEN1 gene mutation enables selection of family members for screening and long-term follow up.

Hepatic tuberculosis in absence of disseminated abdominal tuberculosis.

Liver involvement in tuberculosis in absence of miliary tuberculosis is rare. This study was performed to analyse the spectrum and response to treatment of hepatic tuberculosis in the absence of miliary abdominal tuberculosis. Retrospective analysis of seven cases of hepatic tuberculosis without miliary abdominal tuberculosis who presented at the single tertiary referral center were analyzed. All patients presented with fever and hepatomegaly. Five of them had pain in upper abdomen and vomiting. HIV serology was positive in one patient. All patients had normocytic normochromic anaemia, raised erythrocyte sedimentation rate (Mean 65). Mild elevation of liver enzymes and low albumin (Mean 2.4 gm%) with reversal of albumin globulin ratio (Mean 0.6) were seen in all. Two had jaundice. Prothrombin time was normal in all and lactate dehydrogenase values were elevated in all (Mean 794 IU/L). On ultrasonography, 2 had multiple hypodense lesion, 1 had coarse echotexture of liver, 1 had hyperechoic pattern and 3 had just hepatomegaly. Complete resolution of liver lesions on treatment with 4-drug anti-tuberculosis drug chemotherapy was seen. In conclusion, liver tuberculosis has protean manifestations with nonspecific alteration of liver function tests and is best diagnosed on liver biopsy. Overall response to therapy is satisfactory.

A prospective randomized trial of N-acetyl cysteine administration during cold preservation of the donor liver for transplantation.

AIMS: N-acetyl cysteine (NAC), an anti oxidant and a glutathione precursor, is effective in ameliorating liver injury of Tylenol overdose. There is experimental evidence that it also reduces ischemia reperfusion (I/R) injury. This clinical study was undertaken to study the effect of NAC administered in the donor operation. METHODS: 22 patients were randomized to receive NAC (IV & Portal flush) or no NAC (Control Group) during donor operation. Peak AST levels and 1-hour post-reperfusion biopsies were used to assess I/R injury. Episodes of acute rejection were recorded together with immunosuppressive drug levels. RESULTS: There were 4 exclusions (re-exploration for post-operative hemorrhage x3, OLT for acute liver failure x1). The two groups (n = 9 each) were matched for recipient and donor ages and sex. Viral hepatitis accounted for cirrhosis in 3 patients in NAC Group and 6 patients in Control Group. Statistically, Cold and warm ischemia times were not significantly different as was the use of blood and blood products in both groups. Serum peak AST levels were similar and post- reperfusion biopsy showed moderate to severe reperfusion injury in 3 recipients in the NAC Group and 4 in the Control Group. Excluding ones associated with low Tacrolimus levels (n = 4), there were 6 episodes of acute rejection (2- mild, 4- moderate) in the NAC Group and 5 in the Control Group (3- mild,1- moderate, 1- severe). CONCLUSION: In this pilot study, NAC administered during donor operation did not show a protective effect on I/R injury or on acute cellular rejection.

Thrombophilic conditions in non-cirrhotic portal vein thrombosis.

OBJECTIVE: To study the prevalence of thrombophilic conditions in patients with acute and chronic portal vein thrombosis (PVT) and to compare it with those in patients suffering from deep vein thrombosis (DVT) after lower limb arthroplasty and in healthy subjects. METHODS: Twenty-six patients with spontaneous PVT (20 chronic, 6 acute) with normal liver function and not receiving anticoagulants were evaluated for thrombophilic conditions. Levels of protein C, protein S and antithrombin were compared with those in 50 healthy controls. Factor V gene 'Leiden' mutation (FVL) and high homocysteine levels were looked for in patients with PVT and in 18 patients developing post-arthroplasty lower limb DVT despite anticoagulation. RESULTS: Of 26 patients with PVT, 19 had at least one thrombotic condition (acute PVT 5/6, chronic PVT 14/20) and 12 had more than one such condition; in comparison, of 18 patients with DVT, eight had one thrombophilic condition and one had two such conditions (p=0.03). Patients with PVT had significantly lower levels of protein C, protein S and antithrombin than healthy subjects and those with DVT. Six patients had Factor VIII levels above 150%; four had elevated homocysteine levels and three had detectable anti-cardiolipin antibodies. Three patients with PVT (acute 2, chronic 1) were heterozygous for FVL mutation. CONCLUSIONS: Underlying thrombophilic conditions are common in Indian patients with spontaneous PVT. In many patients, multiple thrombophilic conditions are present and these may play a role in the pathogenesis of PVT.

Percutaneous stenting of the portal vein prior to biliary bypass in a patient with chronic pancreatitis and portal biliopathy.

Chronic pancreatitis (CP) often leads to obstruction of one or all the components of the spleno-mesenterico-portal (SMP) axis. This is often due to the ongoing inflammatory process which may lead to thrombosis of the surrounding veins. However, the inflammation also leads to progressive fibrosis and occlusion of the veins without thrombosis. This fibrosis is also responsible for the duodenal obstruction and biliary strictures associated with CP. These patients, with simple occlusion or encasement of one or a combination of components of the SMP axis, can be treated with venous stenting. Here, we present the case of a 58-year-old gentleman with portal vein occlusion and portal biliopathy secondary to CP. This patient was successfully treated with percutaneous transhepatic portal vein stenting and a subsequent surgical biliary bypass. In conclusion, portal venous obstruction in CP may be just due to local fibrosis and is amenable to treatment with a percutaneously placed portal venous stent.

Co-existence of peri-ampullary carcinoma with peripancreatic tuberculous lymphadenopathy.

CONTEXT: Pancreatic tuberculosis and peripancreatic tuberculous lymphadenitis are rare, mimicking various pathologies of the pancreas. The coexistence of peri-ampullary malignancy with peripancreatic tuberculous lymphadenitis has not been reported. CASE REPORT: We present the case of a young woman who had been operated on with a preoperative diagnosis of peri-ampullary adenocarcinoma in whom a frozen section of regional lymph nodes revealed tuberculosis. The final pathology confirmed the co-existence of tuberculosis with malignancy. The patient made an uneventful recovery. CONCLUSION: In countries with high endemicity for tuberculosis, the co-existence of malignancy and tuberculosis should be considered and resection, which is the only chance for cure, should not be abandoned due to observations based solely on frozen sections.

Emergency sclerotherapy for control of acute oesophageal variceal bleeding using 3% aqueous phenol: a 15-year experience.

BACKGROUND: The purpose of the present study was to compare the efficacy of emergency endoscopic variceal sclerotherapy (EVS) using 3% aqueous phenol as an initial procedure to control acute oesophageal variceal bleed. METHODS: One hundred and ninety-five consecutive patients presenting with acute variceal bleed were included in the study. Protocol based endoscopic sclerotherapy and management of bleeding oesophageal varices was done. Immediate cessation of bleeding, re-bleeding within the first 72 h, success of first injection, final success of EVS, short-term mortality, influence of aetiology of portal hypertension and severity of liver disease on these results were studied. RESULTS: Immediate cessation of bleeding was obtained in 191 out of 195 patients (97.9%). Twenty-seven (13.8%) patients re-bled within 72 h. On re-injection final success of EVS was 87.2% (170/195 patients). There was no significant difference between final success rate in cirrhotic versus non-cirrhotic patients (103/118 (87.3%) vs. 67/77 (87%)). Success of first sclerotherapy session was significantly higher than that of second sclerotherapy session. Surgical rescue was required in 25 (12.8%) patients. Mortality was 3.6%. Failure of EVS and mortality was significantly higher in Child's C group (P = 0.04, Relative risk = 0.5, confidence interval 0.22-1.16). CONCLUSION: EVS remains an effective and cost effective modality of treatment to control acute variceal bleeding irrespective of aetiology of portal hypertension provided strict protocol based management is followed. With timely surgical rescue for the failures, the overall mortality can be reduced to less than 5%.

Splenic artery embolization for variceal hemorrhage following blocked distal splenorenal shunt.

A blocked distal splenorenal shunt presents with torrential bleeding from gastric varices. Reoperation in the presence of portal hypertension in an unstable patient is difficult. Two patients with a blocked distal splenorenal shunt were subjected to splenic artery embolization. This stopped variceal bleeding from gastric varices resulting from the congested spleen due to thrombosis of the splenic vein. The procedure was successful in stopping the acute bleed in both patients. There were no serious complications or recurrent variceal bleeding and the varices were collapsed at one year on follow-up endoscopy. Splenic artery embolization is a safe and effective minimally invasive treatment for patients with bleeding from a blocked distal splenorenal shunt.

Presentation and natural history of variceal bleeding in patients with portal hypertension due to extrahepatic portal venous obstruction.

BACKGROUND: The natural history of portal hypertension due to extrahepatic portal venous obstruction (EHPVO) in the presence of a non-cirrhotic liver is not well understood. AIM: To evaluate the presenting features and the natural history of EHPVO by assessing the bleed patterns prior to definitive management in these patients. METHODS: Two hundred and seven consecutive patients presenting with history of variceal bleeding due to EHPVO were studied prospectively. Clinical, hematological and biochemical features on presentation, and pattern and severity of bleeding prior to institution of endoscopic therapy or surgery were recorded. RESULTS: Ascites was observed in 16% and was invariably transient. Splenomegaly was present in 82%, with hypersplenism in 22%; however, hypersplenism was symptomatic in only 6% of patients. In 127 patients having more than one episode of bleeding, the frequency of bleeding episodes was 0.94/year and transfusion requirment was 1.47 units/year. No fixed pattern of frequency of variceal bleeding was identified. However, number of bleed episodes and transfusion units requirement correlated with each other. The maximum severity of bleeding was between the ages of 12 and 19 years. CONCLUSION: Hypersplenism causing symptoms is rare in EHPVO presenting with variceal bleed.

Cystic variant of embryonal sarcoma of liver.

Embryonal sarcoma of the liver is an unusual tumor. The cystic form is rare and can mimic hydatid disease. We present a case that was mistakenly treated as a hydatid cyst for 3 months. Surgery was successful in removing the mass.

Obstructing enterolith as presenting feature in Crohn's disease.

We report a 54-year-old woman with Crohn's disease presenting with intestinal obstruction due to an enterolith impacted at the site of a small bowel stricture. The diseased bowel was successfully resected by laparoscopy-assisted surgery.

Methylene blue dye injection for identification of occult gastrointestinal bleeding.

The exact site of small bowel bleeding is difficult to detect intraoperatively. We present a simple method of on-table identification of the site of the bleed. A 55-year-old lady presented with recurrent episodes of melena and drop in hemoglobin. Digital subtraction angiography (DSA) revealed angiodysplasia of a vessel supplying the proximal jejunum. A microcatheter was placed at this site in the DSA suite, just prior to laparotomy. Two cubic centimeters of methylene blue dye was injected into the microcatheter on-table which demarcated the 6 in. of involved jejunum which was then resected. Patient is currently doing well over a 6-month follow up with no further episodes of melena. In conclusion, preoperative DSA and selective catheterization of the affected vessel allow for on-table localization of the exact site of bleed. This simple method avoids more invasive techniques of detection and enables limited segmental resection of the affected bowel.