Porta Hepatitis Research Articles

S1926 A Young Female With Acute Cholangitis Due to Type IVa Choledochal Cyst With an Undifferentiated Spindle and Giant Cell Carcinoma Hiding Inside

Introduction: This patient is being presented to demonstrate the rarity and severity of extrahepatic biliary cystic masses as the primary etiology of acute cholangitis in a young patient, after failing endoscopic stent placement. Case Description/Methods: A 29-year-old female with no medical history presented with 2 days of sharp, epigastric pain radiating to the back associated with fevers and non-bilious vomiting. Patient’s exam noted scleral icterus and abdominal exam showed tenderness to palpation in the right upper quadrant. Labs showed a WBC of 26,300, AST of 114, ALT of 289, ALP of 343 IU/L, and total bilirubin of 9.76 mg/dL. Computed tomography (CT) of the abdomen showed dilated proximal extrahepatic CBD with a large complex cystic mass in the porta hepatitis measuring 10 cm x 9 cm x 8 cm and this was contiguous with gallbladder and proximal common bile duct with mass effect on duodenum and pancreatic head concerning for Type I choledochal cyst. An ERCP showed a significantly dilated CBD with cystic dilation and balloon sweep with 9-10-12 mm balloon catheter was performed with passage of a small stone and pus. Two plastic stents 10 Fr x 7 cm and 10 Fr x 9 cm were placed with appropriate bile drainage. Fevers continued and total bilirubin increased to 14.2 mg/dL and ALP reached 535 IU/L, therefore patient was taken for laparoscopy which showed large choledochal cyst intimately involved with the head of the pancreas and duodenum, necessitating a Whipple procedure for distal margin. Proximal margin entered the left and the right hepatic ducts, consistent with a type IVa choledochal cyst. Anatomic pathology of the 7.0 cm x 6.0 cm x 3.0 cm mass in the cyst showed an undifferentiated spindle and giant cell carcinoma, confined to the bile duct histologically, with lymphovascular and perineural invasion present. There was no gallbladder involvement and 25 lymph nodes were negative for malignancy. The patient improved and was discharged to home after extended recovery (Figure). Discussion: Undifferentiated carcinoma of the extrahepatic biliary system is extremely rare and has been previously found on middle-age to elderly patients. Diagnosis of undifferentiated carcinoma of the extrahepatic biliary system is difficult because the exact diagnosis cannot be determined prior to surgical resection. Patients with undifferentiated carcinoma of the extrahepatic bile duct may have a relatively better prognosis due to the early appearance of jaundice, compared to those with gallbladder involvement.Figure 1.: MRCP of Type IV choledochal cyst

Porta hepatis in relation to portal vein among Indians.

The porta hepatis / hilum of liver is a transverse fissure located in the inferior surface, where the major vessels and ducts enter or leave the organ. The major structures traversing the porta hepatis are the portal vein, the hepatic artery and the hepatic duct. Porta hepatis is an area of surgical and radiological significance. The knowledge of variations in structures traversing the porta hepatitis will reduce the risk of surgeries involving this area. Study was conducted in the department of anatomy dissection lab after obtaining ethical clearance. 30 liver specimens were used for these studies which were removed from the cadaver during under graduate teaching. Anatomical knowledge of variations in relations of structures present in porta hepatis is of immense help to surgeons and radiologists when they engage patients for clinical procedures like liver transplant, cholecystectomy and diagnostic procedures. Hence this study was aimed to observe the relations of portal vein in porta hepatis.

Implications of peritoneal cancer index distribution on patients undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.

ObjectivesPeritoneal cancer index (PCI) score is a common prognostication tool in peritoneal metastases (PM). We hypothesize that the distribution of PCI score and involvement of specific regions affects survival and morbidity outcomes.MethodsData was collected from a prospective database of patients who underwent CRS and HIPEC for PM at the National Cancer Centre Singapore. We evaluate the relationship between PCI, PCI distribution, and survival and morbidity outcomes.ResultsOne hundred and fifty-two patients underwent CRS and HIPEC with a median PCI score of nine (range 0–31). Median overall survival (OS) and progression free survival (PFS) were 43 and 17 months, respectively. Region six (pelvis) was most commonly involved and had the highest frequency of heavy disease burden. Presence of PM in the lower abdomen, flanks, and small bowel were associated with poorer OS (p=0.01, 0.03, <0.001) and PFS (p=0.04, 0.02, <0.001). Involvement of porta hepatitis predicted poorer OS but not PFS (p=0.03). Involvement of the gastric antrum resulted in higher rates of postoperative complications.ConclusionsThe pattern of PCI distribution may be associated with varying survival and morbidity outcomes.

Leiomyosarcoma of the Vena Cava. A Case Report of Resection of the Infrarenal Cava with Auto-transplantation of Both Kidneys

Introduction: Leiomyosarcoma of the IVC is a rare but potentially fatal condition where a complete R0 resection is the only potential cure. Since the first case of a leiomyosarcoma described back in 1871 there have been approximately 470 cases reported in the literature with varying techniques used to manage the cava and so a consensus on best surgical management has never been reached[1].We present a rare case of a Leiomyosarcoma of section II of the inferior vena cava with auto transplantation of both kidneys. Methods: Intra-operatively there was a very large bilobed leiomyosarcoma originating from the IVC at the level of the renal veins and growing around the porta hepatitis and below the liver. the liver was fully mobilised to allow exposure of the cava and facilitate tumour resection. In order to preserve both kidneys they were resected and auto transplanted into the pelvis at the end of the procedure. The tumour was resected with approximately 10cm of IVC and replaced with a PTFE tube graft. Results: Postoperative course was stormy complicated by perinephric haematomas, chest sepsis and delayed graft function. She spent 11 days in ICU (9 on CRRT) and was discharged home on day 27 with a creatinine of 61 Pathology was confirmed as a grade 3 leiomyosarcoma arising from the wall of the inferior vena cava. It wasn't possible to assess excision margins due to the fragmented specimen however sampled blood vessels and small amount of adherent liver parenchyma were clear of infiltration. Conclusion: Tumours involving the inferior vena cava remain a rare pathology with radical resection as the only option for improving long term survival. Resection of the cava can be achieved with or without replacement depending on collateral venous drainage and the location of the renal veins in relation to the resected segment. This case demonstrates a new surgical option for achieving R0 resection margins and avoiding renal dysfunction with autologous bilateral renal transplants. Disclosure: Nothing to disclose

2485 An Unusual Case of a Primary Mass in the Porta Hepatis

INTRODUCTION: Burkitt's Lymphoma is an aggressive subtype of B-cell non- Hodgkin Lymphoma which can present in three forms: endemic, sporadic, and immunodeficiency- associated. It commonly presents in the pediatric population and occurs in less than 1% of adult non-Hodgkin lymphomas. It classically presents with abdominal symptoms with ascites and can commonly involve the distal ileum, stomach, cecum, and mesentery. We describe an unusual case of Burkitt's lymphoma presenting as a mass in the porta hepatis, associated with obstructive jaundice. CASE DESCRIPTION/METHODS: A 74 y/o male presented with a two-week history of jaundice and intermittent epigastric pain. Physical exam findings were scleral icterus, jaundiced skin, and soft nontender abdomen with normal bowel sounds. Labs were pertinent for alkaline phosphatase 1,264, AST 166, ALT 95, total bilirubin 18.8, and direct bilirubin of 15.1. Ultrasound and MRI/MRCP demonstrated a 10.3 × 6.8 × 7.2 mass infiltrating the porta hepatis, peripancreatic, and periduodenal tissues. There were no other masses or adenopathy. At ERCP there was a friable mass replacing the ampulla. Cholangiogram showed dilated intrahepatic ducts proximal to an obstruction at the level of the common bile duct and a metal expandable stent was placed. Biopsy of the ampullary mass revealed high grade, mature B-cell lymphoma. Endoscopic ultrasound revealed a heterogenous mass and fine needle aspiration yielded necrotic cells. Tumor cells were positive for CD20, PAX5, BCL6, CD10, CD20, and LEF1. Pathology was positive for Myc rearrangement and translocation t(8;14). Combination chemotherapy was then initiated. DISCUSSION: Primary tumors of the porta hepatitis are uncommon as it is more often the site of metastatic disease from breast, pancreas, gastric, and colon cancer. This case is particularly unusual in that the primary tumor is a Burkitt lymphoma, which did not arise from lymph nodes or from the pancreas and which presented with obstructive jaundice.

Previously unreported high-grade complications of adrenalectomy

Serious complications of adrenalectomy are rare but the incidence may be underestimated if they occur outside major referral centers. We report five cases of high-grade complications after adrenalectomy that have not been previously described. The records of five cases of adrenalectomy performed at outside hospitals were reviewed. Four cases were referred for management of complications and one for medical-legal review. The nature of the adrenal lesion, operative approach, complication(s), and subsequent clinical course and complication management were assessed. Both open adrenalectomy (OA) and laparoscopic adrenalectomy (LA) cases were included. Operative indications were pheochromocytoma (N = 3), aldosteronoma (N = 1), and a nonfunctioning 6-cm hypervascular mass (N = 1). Complications of adrenalectomy included: case 1--complete transection of the porta hepatitis during right LA resulting in hepatic failure requiring emergent liver transplantation; case 2--ligation of the hepatic artery during right OA resulting in recurrent cholangitis and bile duct sclerosis requiring liver transplantation; case 3--ligation of the left ureter during LA resulting in postoperative hydronephrosis and loss of renal function; case 4--loss of left kidney function after OA, likely secondary to renal artery ligation ultimately requiring laparoscopic nephrectomy; case 5--LA of a normal adrenal gland for a 6-cm hypervascular mass thought to be arising from the adrenal gland. Three-month postoperative imaging demonstrated a persistent mass and the patient underwent hand-assisted laparoscopic nephrectomy for a left upper pole renal cell carcinoma that was missed at the time of LA. Despite the generally low morbidity of adrenalectomy, serious and potentially life-threatening complications can occur. Surgeon inexperience may be a factor in the occurrence of some of these complications which have not been previously described.

Intraoperative radiation therapy in recurrent ovarian cancer

To evaluate disease outcomes and complications in patients with recurrent ovarian cancer treated with cytoreductive surgery and intraoperative radiation therapy (IORT). A retrospective study of 24 consecutive patients with ovarian carcinoma who underwent secondary cytoreduction and intraoperative radiation therapy at our institution between 1994 and 2002 was conducted. After optimal cytoreductive surgery, IORT was delivered with orthovoltage X-rays (200 kVp) using individually sized and beveled cone applications. Outcomes measures were local control of disease, progression-free interval, overall survival, and treatment-related complications. Of these 24 patients, 22 were available for follow-up analysis. Additional treatment at the time of and after IORT included whole abdominopelvic radiation, 9; pelvic or locoregional radiation, 5; chemotherapy, 6; and no adjuvant treatment, 2. IORT doses ranged from 9-14 Gy (median, 12 Gy). The anatomic sites treated were pelvis (sidewalls, vaginal cuff, presacral area, anterior pubis), para-aortic and paracaval lymph node beds, inguinal region, or porta hepatitis. At a median follow-up of 24 months, 5 patients remain free of disease, whereas 17 patients have recurred, of whom 4 are alive with disease and 13 died from disease. Five patients recurred within the radiation fields for a locoregional relapse rate of 32% and 12 patients recurred at distant sites with a median time to recurrence of 13.7 months. Five-year overall survival was 22% with a median survival of 26 months from time of IORT. Nine patients (41%) experienced Grade 3 toxicities from their treatments. In carefully selected patients with locally recurrent ovarian cancer, combined IORT and tumor reductive surgery is reasonably tolerated and may contribute to achieving local control and disease palliation.

Pathogenesis and outcome of biliary atresia: current concepts.

Neonatal cholestatic disorders are a group of hepatobiliary diseases occurring within the first 3 months of life. Bile flow is impaired, and patients have conjugated hyperbilirubinemia, acholic stools, and hepatomegaly. Overall, 1 in 2,500 live births is affected with a neonatal cholestatic disorder (1). The two most common causes of neonatal cholestasis are biliary atresia and idiopathic neonatal hepatitis, accounting for up to 50% to 70% of cases. Other causes include a variety of neonatal infections (viral, toxoplasmosis, syphilis, bacterial), metabolic and genetic diseases, progressive familial intrahepatic cholestatic disorders (PFIC), paucity of interlobular bile duct disorders (e.g., Alagille syndrome), choledochal cyst, ischemia–reperfusion injury, association with parenteral nutrition administration, and other conditions (Table 1). Despite clinical improvement after the portoenterostomy procedure, approximately 70% to 80% of children with biliary atresia will eventually require liver transplantation; thus, biliary atresia alone accounts for almost 50% of all liver transplants performed in children (1). It should be noted that $77 million is spent each year in the United States on liver transplantation for children and the ensuing hospitalizations (2). This sum of money covers 0.2% of total health care expenditures related to children, even though these children represent 0.0006% of the total pediatric population. Importantly, this disproportionate expenditure for liver transplantation in children could be cut in half if improved therapies for biliary atresia were developed that could abrogate or further delay the need for liver transplantation. Remarkably, little is known about the etiopathogenesis of biliary atresia; consequently, there has been slow progress in developing improved therapies or preventative strategies during the past decade. The purpose of this review is to summarize recent advances in the diagnosis and management of biliary atresia, examine the clinical outcome, describe the evolving theories of the etiology and pathogenesis of this disorder, and highlight gaps in our current knowledge.

Reduced encephalopathy in pigs with ischemia-induced acute hepatic failure treated with a bioartificial liver containing alginate-entrapped hepatocytes.

To analyze the effects of an extracorporeal bioartificial liver containing alginate bead-entrapped hepatocytes on pigs with ischemia-induced acute hepatic failure. Prospective animal study. University and INSERM laboratory. Fifteen Large White/Pietrin female pigs weighing 20-30 kg. Acute hepatic failure was induced by end-to-side portocaval shunt and ligature of the whole porta hepatitis. The bioartificial liver was in a thermostabilized column, containing a fluidized bed of alginate beads that embedded porcine hepatocytes, connected to a plasmapheresis system. Intracranial pressure; survival; ammonia, total bilirubin, aminotransferases, alkaline phosphatase, and lactate concentrations; and clotting factors were studied. The groups were pigs with acute hepatic failure (group 1, n = 4), pigs with acute hepatic failure treated with bioartificial liver containing empty beads (group 2, n = 4), or porcine hepatocytes (group 3, n = 5). In group 1, survival of pigs averaged 10.9 +/- 1.0 hrs; intracranial pressure reached 32.3 +/- 3.8 mm Hg and was associated with coma and cerebral edema. After connection to the bioartificial liver, the survival of acute hepatic failure pigs was 12.1 +/- 1.4 hrs in group 2 and 14.8 +/- 2.5 hrs in group 3. In group 3, intracranial pressure and bilirubin concentrations were reduced significantly compared with both group 1 and group 2. Neither signs of encephalopathy nor cerebral edema was observed in any animal of group 3. In all animals, plasma ammonium, aminotransferases, alkaline phosphatase, and lactate concentrations increased and clotting factors decreased with no significant differences between the three groups. Autopsy revealed a total necrosis of the liver, which was histologically confirmed. The ischemia-induced model of acute hepatic failure in pigs is reproducible and provides measurable clinical and biological features. A bioartificial liver containing alginate bead-entrapped hepatocytes improves the signs of encephalopathy in pigs with ischemia-induced acute hepatic failure, suggesting that the bioartificial liver can clear out toxic compounds that are released from necrotic livers.

Management of biliary atresia in the liver transplantation era: A 15-year, single-center experience

Background/Purpose: The aim of this study was to address the perioperative aspects of hepatoportoenterostomy (HPE) for biliary atresia (BA), through the study of a 15-year, single-center experience of the management of this disease. Methods: One hundred twenty-seven patients were divided into three groups, depending on the variant of HPE performed: group A (n = 53) underwent HPE with external diversion of the Roux-en-Y anastomosis; group B (n = 54) underwent HPE with a long (35 to 40cm) Roux-en-Y anastomosis, without diversion; and group C (n =20) underwent the same kind of HPE as group B, but with a modified, “super extensive” dissection of the porta hepatitis. Eleven children in group B had an intussusception type antireflux valve in the Roux-en-Y loop. The porta hepatitis of 105 children was histologically classified in type I to III and correlated with rate of postoperative bile flow and age at surgery. Liver transplantation was performed after HPE in 20 patients. Results: Overall, biliary drainage was achieved in 72.5% of the children after HPE and 26.8% of all patients became jaundice free. Porta hepatitis type III was associated with a significantly higher rate of biliary drainage post-HPE then types I and II. There was no difference in the rate of bile drainage, relative number of jaundice-free patients, and mean number of episodes of cholangitis per year among surgical groups A, B, C. In group A, 43.7% of the patients had complications related to the stoma. The actuarial survival of children who underwent HPE followed by liver transplantation was 85%. Conclusions: (1) There is no correlation between type of porta hepatis and age at surgery for BA; (2) type III porta hepatis is associated with higher rates of bile drainage post-HPE; (3) children older than 16 weeks with BA should still be considered for HPE; (4) in these older patients, factors other than the type of porta hepatis, possibly the degree of liver damage, play a role in the lower rate of bile drainage observed; (5) external diversion of the Roux-en-Y in HPE is not beneficial and is detrimental because of stoma-related complications; (6) an antireflux valve in the Roux-en-Y loop does not reduce the incidence of cholangitis post-HPE; (7) Surgical reexploration does not restore biliary drainage; and (8) sequential therapy with HPE followed by liver transplantation remains the treatment of choice for BA.

Virchow Troisierʼs Lymph Node in Hepatocellular Carcinoma

Virchow-Troisier's lymph node is rarely found in patients with hepatocellular carcinoma. When present, it may or may not accompany spread of the tumor to mediastinal lymph nodes. In a 20-year-old South African black man with hepatocellular carcinoma, a Virchow-Troisier's node was evident clinically and confirmed at necropsy. Mediastinal lymph nodes were not involved by tumor, and the malignant cells probably gained access to the thoracic duct by way of lymphatic spread to the hepatic lymph nodes in the porta hepatitis and the celiac nodes, both of which were seen at necropsy to be extensively invaded by tumor.

Abdominal Manifestations of Non-Hodgkin's Lymphomas

25 children (18 boys and 7 girls) were treated for non-Hodgkin's lymphomas (NHL) during 1982-1990. The age at diagnosis was 2-15 years. Five of the 25 children died. Of the 25 children 6 patients had the diagnosis of intraabdominal NHL. The presenting abdominal symptoms for these 6 patients were abdominal pain in 4 cases, nausea 3, abdominal distention 2, icterus 1 and diarrhoea 1. Four of the 6 patients with abdominal NHL underwent laparotomy but the diagnosis was unknown before surgery. The indication for explorative laparotomy was in all cases a tumor of unknown etiology combined with ascites in 2 cases and icterus in 1 case. At surgery there was a tumor localized around the porta hepatitis in 1 case and in the right ovarium in 1 case. Furthermore, spread infiltrative growth of tumor was found in 3 cases, with infiltration of the peritoneum in 2 of these cases. A specimen for peroperative histological examination was taken and gave the conclusive diagnosis of NHL in 4 of 6 cases. Staging laparotomy, palliative tumor resection and radical surgery are preferably avoided. For proper treatment an adequate biopsy is important. The management of children with NHL is a multidisciplinary approach.

Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study.

Ten cases of rhabdomyosarcoma of the biliary tree (RMS/BT) in children are reported in this review of the Intergroup Rhabdomyosarcoma Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable tumors on IRS I and II. Intermittent obstructive jaundice with or without abdominal distention, fever, and loss of appetite is the typical presentation. Attribution of these symptoms to hepatitis commonly delayed definitive treatment. Demonstration of a mass in the porta hepatitis by ultrasound led quickly to surgical consultation and an exploratory laparotomy. Intraoperative cholangiography was a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. Resection of the mass with only microscopic or minimal gross residual disease was possible at the initial surgery in six of the ten patients. Included in this group are all four of the surviving patients. The continuity of bile flow was usually maintained by variations of a Roux-en-Y jejunostomy. Histology was embryonal RMS with some botryoid elements. Consultation with a surgical pathologist during the course of the surgery was of invaluable assistance. Multi-drug chemotherapy and radiotherapy consistent with the IRS protocols were given postoperatively. A second-look surgery was useful in evaluating residual or recurrent disease. Four patients are surviving free of disease at 6 months, 3, 6 1/4, and 6 1/2 years from diagnosis, respectively. This review demonstrates that a multidisciplinary approach in RMS/BT can lead to long-term survival.

Electron microscopy of lymphatics of the porta hepatitis.

The ultrastructure of the lymphatics of the porta hepatitis at the junction with the liver parenchyma has been examined. The lymphatics are composed of endothelium composed of flattened cells containing occasional mitochondria, pinocytotic vesicles and nuclei. The cells are bound by maculae adherentes, zonulae occludentes and desmosome-like structures. There are occasional fibers between endothelium and underlying collagen. Occasional pores closed by diaphragms are present. There is very little basement membrane. There is no definite communication with the spaces of Mall and Disse, but the lymphatic lumens are separated from these areas only by the junctional processes, which may be permeable. Lymphatics are present only in collagenized portal areas and not in the liver parenchyma proper.