Double intestinal duplication and incidental neuroendocrine tumor of appendix, a rare case of acute abdomen.

INTRODUCTION: Intestinal duplication is rarely reported in adulthood and often remains undiagnosed until onset of complications. We describe the case of a 39 year old woman who came to our observation for acute abdomen due to a combination of double intestinal duplication (colon and ileum) and an incidental neuroendocrine tumor of the appendix. MATERIALS AND METHODS: A 39 year old woman who was admitted at with upper abdominal pain. Multisliced spiral CT scan showed a cystic lesion suggestive of an inflammed Meckel's diverticulum.The patient was underwent an urgent explorative laparoscopy. The intraoperative findings revealed a cystic lesion of the anti-mesenteric side of transverse colon, apparently dissectable from the bowel and a second lesion with a strongly adherent and unresectable from the anti-mesenteric aspect of the small bowel. A combined appendectomy was also performed. The histological diagnosis was consistent with a typical intestinal duplication for both intestinal lesionsand an incidental 2mm carcinoid tumor was also found in the appendix. The postoperative course was uneventful and the patient was discharged on p.o. day 5. At the presenttime she is well and following a regular oncologic follow-up. DISCUSSION: The rarity of this case is due to the concomitant presence of an incidental, sincronous, appendiceal NET. The elective treatment is surgical resection. CONCLUSION: Intestinal duplication in the adulthood is extremely rare and may either have an acute presentation as acute abdomen or represents an incidental finding of mass. We suggest that, once the diagnosis is suspected patient must undergo surgery.

Management of invasive and advanced thyroid cancer.

The majority of thyroid cancers are slow growing and have an excellent prognosis after surgical and medical therapy. However, a subset of thyroid cancers do not follow an indolent course, and exhibit aggressive behaviour. Advanced thyroid malignancies can cause distressing and life-threatening symptoms by local invasion, growth of distant metastases. Extensive surgery may have curative or palliative effects, although morbidity should be considered before planning debulking operations. Novel therapies using molecular targets and redifferentiation agents promise to expand our ability to treat patients with advanced thyroid malignancies in the future.

Male hypogonadism in cirrhosis and after liver transplantation.

Liver is deeply involved in the metabolism of proteins, hormones, enzymes, cytokines, as well as in sex hormones catabolism. Gonadal function requires a normal liver function, and it is well known that clinical signs of hypogonadism are common in patients with liver cirrhosis. Few studies have focused on hypothalamic- pituitary-gonadal alterations in male cirrhotic patients or after orthotopic liver transplantation (OLT). The pathogenesis of hypogonadism in cirrhotic patients is complex and not well explained. It involves both a gonadal and a hypothalamic- pituitary dysfunction. After OLT the hypothalamic-pituitary-gonadal function partially improves, showing that the hepatic dysfunction before OLT is deeply involved in its pathogenesis. After OLT some alterations persist in some patients, both because of pre-existing gonadal alterations (toxic-metabolic damage) and immunosuppressive pharmacological side effects. Further studies will explain the relationship between hypogonadism and OLT outcome, and the role of androgen therapy in hypogonadism after OLT, in the early months and in the long term.

Hepatitis C virus-related cirrhosis as a significant mortality factor in intention-to-treat analysis in liver transplantation.

INTRODUCTION: Liver transplantation represents the gold standard for the treatment of chronic liver disease. The whole transplantation process was assessed using an intention-to-treat analysis and considering patients from the time of their inclusion on the list and throughout lengthy follow-up. MATERIALS AND METHODS: From January 1, 1999 to June 1, 2004, 373 adults joined the waiting list for liver transplantation at our institution. The main variables analyzed were: age, gender, etiology, Model for End-stage Liver Disease score, Child-Pugh class, United Network for Organ Sharing (UNOS) status. Global survival was evaluated using intention-to-treat analysis from the time of patient inclusion in the list to the end of their late follow-up. RESULTS: The median waiting time was 20 months (range 0.1 to 70.2). By univariate analysis, the variables significantly influencing survival when patients joined the waiting list were: encephalopathy; ascites, poor nutritional status, Child-Pugh class C, UNOS 2, hepatitis C virus (HCV) and bilirubin > 2 mg/dL. By multivariate analysis, only HCV-related cirrhosis emerged as having an independent prognostic value. By intention-to-treat analysis, the 5-year survival rate was 67% and 79% for HCV-positive and HCV-negative patients, respectively (P = .0003). CONCLUSIONS: HCV-related cirrhosis is an independent prognostic factor for survival according to an intention-to-treat analysis. Different inclusion criteria or treatments while on the waiting list and after transplantation need to be considered in the future for HCV-positive patients.

Prognostic impact of model for end-stage liver disease score in patients undergoing liver transplantation with suboptimal livers.

BACKGROUND/AIMS: The aim of this retrospective study is to analyze the prognostic impact of Model for End-Stage Liver Disease (MELD) score in patients undergoing liver transplantation (OLT) with suboptimal livers. METHODS: Between January 2002 and January 2006, 160 adult patients with liver cirrhosis received a whole liver for primary OLT at our institution including 81 with a suboptimal liver (SOL group) versus 79 with an optimal liver (group OL). The definition of suboptimal liver was: one major criterion (age >60 years, steatosis >20%) or at least two minor criteria: sodium >155 mEq/L, Intensive Care Unit stay >7 days, dopamine >10 microg/kg/min, abnormal liver tests, and relevant hemodynamic instability. RESULTS: Baseline recipients characteristics were comparable in the two study groups. The SOL group had a significantly greater number of early graft deaths (<30 days) than the OL group, while the 3-year Kaplan-Meier patient survivals were similar. Using logistic regression, MELD score was significantly related to patient death only in the SOL group (P = .01), and the receiver operator characteristics curve method identified 17 as the best MELD cutoff with the 3-year survival of 93% versus 85% for MELD < or =7 versus >17, respectively (P > 05). In comparison, it was 94% and 72% in the SOL group (P < .05). Similarly, MELD >17 was significantly associated with early graft death rates only in the SOL group. CONCLUSION: This study advised surgeons to not use suboptimal livers for patients with advanced MELD scores, thus supporting a donor-recipient matching policy.

Combined liver and kidney transplantation: analysis of Padova experience.

BACKGROUND/AIM: The main indications for combined liver and kidney transplantation (CLKT) are as follows: (1) cirrhosis with renal damage dependent or not upon liver disease, (2) renal failure with dialysis and concomitant liver end-stage disease, (3) congenital diseases, and (4) enzymatic liver deficiency with concomitant renal failure. The aim of this study was to evaluate our results with CLKT both in adult and pediatric patients. METHODS: From September 1995 to September 2006, 15 CLKT (2.8%) among 541 liver transplantations included 4 pediatric patients (27%). The main indications for CLKT were hepatitis C virus (HCV) and polycystic diseases in adult patients, and primary hyperoxaluria in pediatric patients. RESULTS: The double transplantation was performed from the same donor in all cases. All adult patients received whole liver grafts, whereas 3 split transplants and 1 whole liver graft were transplanted in the pediatric patients. Median liver and kidney cold ischemia times were 468 and 675 minutes, respectively. After a median follow-up of 36 months (range, 1-125), the overall survival rate was 80%. Five-year patient and graft survival rates were 100% for adult CLKT, whereas they were 50% for pediatric patients. We observed only 2 cases (18%) of delayed renal function, requiring temporary hemodialysis with progressive graft improvement. There was only 1 case of kidney retransplantation due to early graft nonfunction in a pediatric patient. CONCLUSION: Although CLKT is related to major surgical risks, results after transplantation are satisfactory with an evident immunological advantage.

Pediatric liver transplantation: the University of Padua experience.

OBJECTIVE: The objective of this study was to analyze experience on pediatric liver transplantation (LT) between June 1993 and September 2006, including split liver transplantation (SLT), living donor liver transplantation (LDLT), and auxiliary partial orthotopic liver transplantation (APOLT). Furthermore, hepatocyte transplantation (HT) had a role in one patient with metabolic disease. METHODS: From November 1990 to September 2006, 657 LTs were performed including 63 pediatric LTs (9.6%) in 57 patients (32 boys and 25 girls). Six were retransplantations (9.5%). Thirty-two patients (57%) were younger than 5 years. The types of graft included the following: 26 whole organs (41%), 32 in situ split organs (51%), 4 reduced-size organs (6%), and 1 graft from a living donor (2%). Two patients received an APOLT, 4 patients received a combined kidney-liver transplantation (CKLT), and 1 patient received HT. Of the 63 pediatric LTs, 16 were behaved to be highly urgent (25%). RESULTS: Overall 1-, 3-, 5-, and 10-year patient survival rates were 82%, 82%, 78%, and 78%, respectively. Overall 1-, 3-, 5-, and 10-year graft survival rates were 76%, 76%, 72%, and 72%, respectively. In patients younger than 1 year, the 5-year survival rate was 100%. Perioperative mortality was 8.8%. Vascular complications occurred in 4 patients (6.3%). Six children required retransplantation due to primary nonfunction (PNF) in 4 cases (7%) and vascular thrombosis in 2 cases (3.5%). CONCLUSIONS: Cholestatic liver disease and age younger than 1 year were the best prognostic factors for excellent survival.

Liver transplantation for the management of hepatoblastoma.

INTRODUCTION: Hepatoblastoma (HEP) is the most frequent liver malignancy occurring in childhood. Surgical resection currently represents the gold standard for treatment. In patients with initially unresectable tumors, chemotherapy may induce remarkable reductions in size. In nonresponder patients, liver transplantation (OLTx) may offer a chance of cure. MATERIALS AND METHODS: From 1990 to 2003, a total of 400 OLTx (31 pediatric transplants) have been performed at Padua University. Seven patients (4 males and 3 females) underwent OLTx for hepatoblastoma. All patients presented with bilobar liver involvement and had received chemotherapy according to the SIOPEL-1. In all patients preoperative staging was negative for extrahepatic involvement. RESULTS: The mean age of the pts was 8.2 years (range 6.4 months to 34 years). Mean follow-up after OLTx was 41.4 months (median 36, range 3 to 108 months). Actuarial patient survival rates after OLTx for hepatoblastoma are 83.3%, 83.3%, and 56% at 1, 3, and 5 years, respectively. Five of seven subjects with HEP are alive after transplant at 3, 12, 36, 65, and 108 months. Two patients died owing to recurrent disease after 6 and 60 months, respectively, from transplantation. Another subject, primarily treated with surgical resection, shows HEP recurrence at 40 months after OLTx. The remaining 4 patients are alive and well at a mean follow-up of 28 months (median 24, range 3 to 65 months). CONCLUSIONS: Liver transplantation may represent a valid therapeutic option for patients with unresectable HEP, but it is contraindicated in cases of recurrence following previous resection surgery. Neo-adjuvant chemotherapy is of paramount importance to obtain good long-term results.

Adjuvant chemotherapy for transplanted hepatocellular carcinoma patients: impact on survival or HCV recurrence timing.

INTRODUCTION: Hepatocellular carcinoma (HCC) is one of the most common tumors worldwide. In the Western world the current epidemic of cirrhosis due to the hepatitis C virus (HCV) is increasing the number of new cases. Liver transplantation (OLTx) represents a radical treatment for HCC and the underlying cirrhosis. Whether adjuvant chemotherapy is indicated in the postoperative period to prevent recurrence is controversial. MATERIAL AND PATIENTS: Forty-eight HCC patients underwent liver transplantation during 11 years, including 21 who were chemo-treated (CT) patients. Thirty-one patients (65%) had post-necrotic virus-C cirrhosis (PNC-C). Twenty-one cases (44%) were p-TNM stages III-IV, and 15 cases (31%) incidental HCC detected in the explanted liver. Seven HCV patients (15%) received chemotherapy (before 1998). RESULTS: One-, 3-, and 5-year overall survival rates were 100%, 85%, 79% (CT group), and 89%, 71%, 71% (no CT group), respectively. The HCV recurrence-free survival rates at 3, 6, and 12 months were 29%, 14%, 0% for the CT group, versus 76%, 38%, 25% for the no CT group (P =.005). CONCLUSIONS: Discontinuation of HCV-HCC patients by chemotherapeutic adjuvant protocols after transplantation appears rational due to the early hepatitis C recurrence confirmed in our series. Moreover, few studies have demonstrated that CT prolongs survival of HCC transplanted patients. New pharmacological approaches are necessary to solve these questions.

Extreme marginal donor: severe hypothermia as a rare preservation condition for explantable organs--a case report.

UNLABELLED: The progressive increase in patients with end stage liver disease has lengthend the waiting- list for liver transplantation. Unfortunately this has not been followed by a suitable increase in the number of donors. The expanding "donor pool" has required use of "marginal" donors (ICU stay > 10 days, sepsi; steatosis > 30-40%, hypernatremia > 155 mmol/L, inotropic drugs). We report the case of a skier who remained for more than 1 hour in cardio-respiratory arrest under the snow; the 49-year-old women was extracted from the snow after 1 hour and 12 minutes and found to be asystolic, fixed pupils and deep hypothermia (27.2 degrees C). After cardiopulmonary resuscitation, partial cardio-respiratory activity was re-established. In the ICU severe hypothermia (26.7 degrees C) was treated with extracorporeal circulation until a re-establishment of satisfactory cardio-circulatory conditions was obtained. Unfortunately cerebral anoxic cerebral death was established and multiorgan procurement performed 3 days later. After liver transplantation into a 59 year-old patient with PNC-C was performed. The course was uneventful and the patient was discharged on the 19th postoperative day. CONCLUSIONS: Organ procurement from donors involved in accidental traumatic events with cardio-respiratory arrest and hypothermia, is similar to the non-heart-beating donor (NHBD) condition. Correct cardiopulmonary resuscitation and the use of extracorporeal circulation for gradual restoration of body temperature are necessary for optimal organ perfusion. In the present case the anoxic insult induced by the cessation of the cardio-respiratory function, was probably mitigated (if not even annulled) by the hypothermia.

[Blood coagulation changes in patients with post-splenectomy persistent thrombocytosis]. / Le alterazioni emocoagulative nei pazienti con trombocitosi persistente post-splenectomia.

To clarify the possible role of persistent thrombocytosis after splenectomy as being a predisposing factor causing thromboembolism. Blood coagulation profiles were studied in 35 patients (20 M and 15 F, mean age 42 +/- 17.5) suffering from thrombocytosis (> 500,000/dl) who underwent splenectomy for non-malignant and non-traumatic diseases. Seventy healthy subjects acted as a control group. Tests were performed 6 months after the operation and for both groups (patients and controls) blood samples were collected for: platelets, fibrinogen, PT, APTT, AT III, plasminogen, F1 + 2, t-PA and DNA analysis for F V, F II and MTHFR. After one year all subjects were controlled for thrombocytosis, genomic abnormalities and venous thrombosis. All the analyses were performed according to the Statistical Package for Social Science. The significance of the differences in means was evaluated by non-parametric tests, differences with a P value < 0.05 being considered significant. Increased plasma levels of fibrinogen, D-dimer, F1 + 2 and PAI-1 were found in the patients compared with the control group. TPA was significantly lower in the patients than in the controls. At the one year follow-up, two patients with genetic polymorphism had suffered deep venous thrombosis. Our findings indicate that splenectomy contributes to abnormal platelet aggregation and endothelial cell activation with hypercoagulability.

Sutureless skin closure in varicose vein surgery: preliminary results.

OBJECTIVE: To describe the preliminary results of a special method of wound closure in varicose vein surgery using the tissue adhesive butyl-2-cyanoacrylate. METHODS: Eighteen consecutive young women (mean age 23 years) underwent partial stripping of the greater saphenous vein for varicose veins of the lower limbs by an external phleboextractor. Their wounds were closed without sutures by means of the adhesive butyl-2-cyanoacrylate. The cutaneous edges were drawn together by linear traction between forceps and the adhesive was applied and allowed to set. Less than 0.5 ml of adhesive was required to complete the entire procedure. Wounds were evaluated at 7 days for infection, dehiscence, and tissue reactions. At 6 months all wounds were rated for cosmesis using a validated visual analog scale, that is, a 100 mm line with "worst scar" at the right end of the line and "best scar" at the left end. All patients were interviewed about their acceptance of tissue adhesive skin closure. RESULTS: The mean time required to close the epidermis with the adhesive was 117 seconds. All patients were followed up for 6 months. At 7 days no adverse outcomes had occurred. Results of wound evaluation at 6 months by the visual analog scale showed scores of 22.2 +/- 13.8 mm (optimal). The percentage of optimal scores was 94.4%, and only one patient (5.6%) had a suboptimal score. Inquiry into the patient's opinions suggested that this procedure was very acceptable. CONCLUSION: Preliminary results with sutureless skin closure in varicose vein surgery have been very encouraging. This fast and cosmetic method of wound repair can replace the need for skin sutures in varicose vein surgery.