Acute appendicitis in a kidney-pancreas transplant recipient: report of a case.

The prompt diagnosis and management of acute surgical conditions in immunocompromised solid organ transplant recipients are of critical importance. These conditions may or may not be related or to the transplanted allograft(s). This is a case report of a 41-year-old woman who received a simultaneous pancreas-kidney transplant. Nine years after the transplant, she developed acute appendicitis with a periappendiceal abscess and a fecalith, and she was treated with percutaneous drainage of the abscess and eventual semielective appendectomy. This is the first known report of acute appendicitis in a pancreas allograft recipient in the English literature.

In situ split liver transplantation for adult and pediatric recipients: an answer to organ shortage.

BACKGROUND: We report our initial experience with in situ split liver transplantation (SLT) for adult and pediatric patients. PATIENTS AND METHODS: From June 2003 to August 2005, 177 liver transplantations in 165 patients, 133 adults (81%) and 32 children (19%), were performed at our institution. Over this period, 45 liver transplantations (25%) were performed with an in situ split liver technique in 44 patients: 17 (39%) were adults and 27 (61%) children. All of the adult split liver recipients were transplanted with an extended right graft (ERG; segments I + IV-VIII), while pediatric recipients received in 23 cases a left lateral segment (LLS; segments II-III) and in 4 cases an ERG from a pediatric donor. The 45 split liver grafts (21 ERGs and 24 LLSs) were generated from 35 donors. In 10 cases we used both grafts generated with an in situ split procedure to transplant our patients, while in 25 cases the procurement procedure was performed in collaboration with other transplant centers. RESULTS: After a median follow-up of 9 months (range, 1-27 months), the overall patient survival rate was 88% for adult patients and 82% for pediatric patients. Graft survivals were 88% and 79%, respectively. Two adult patients (12%) died from sepsis in the early postoperative period. Five children (18%) died after their transplantations. Only one pediatric recipient (2%) of primary SLT underwent retransplantation. Vascular complications were absent in adult recipients, whereas 4 arterial (14%) and 4 venous (14%) complications developed in the pediatric population. The incidence of biliary complications was 23% in adult and 18% in pediatric recipients. CONCLUSIONS: The use of in situ SLT for adult and pediatric populations allowed us to expand the cadaveric donor pool, significantly eliminating pediatric waiting list mortality without penalizing the adult population.

Corticosteroid-free immunosuppression in pediatric liver transplantation: safety and efficacy after a short-term follow-up.

BACKGROUND: We report our results with the use of corticosteroid-free immunosuppression after pediatric liver transplantation, evaluating the efficiency and safety of this protocol in the early posttransplantation period. PATIENTS AND METHODS: From July 2003 to October 2005, 34 liver transplantations were performed in 32 pediatric patients (19 boys, 13 girls) at our institution. Recipient median age was 5 years (range, 0.2-14 years), and median body weight was 10 kg (range, 4-49 kg). Twenty-seven patients received a graft from in situ split liver transplantation, 5 a whole graft. Twenty-nine children (90%) received an immunosuppressive therapy based on methylprednisolone IV bolus at reperfusion (10 mg/kg) plus tacrolimus given at an initial dose of 0.08 mg/kg/d and then adjusted to obtain whole blood trough levels of 10 to 15 ng/mL during the first 3 months and 5 to 10 ng/mL after the 3rd month; basiliximab was given on postoperative days 0 and 4. Biopsy-proven acute rejection episodes were treated by methylprednisone IV boluses. RESULTS: After a median follow-up of 9 months (range, 1-27 months), the overall patient survival rate was 84% and graft survival rate was 79%. Three children (9%) died after their transplantations. Three (9%) experienced episodes of biopsy-proven acute rejection, always treated with IV steroid boluses. Mean RAI score was 4. One patient experienced PTLD that resolved with temporary reduction of immunosuppression. Cytomegalovirus infection rate was 14%. Sepsis occurred in 2 cases (6%). CONCLUSIONS: Initial results with a steroid-free immunosuppressive protocol are encouraging, with low rates of acute rejection and infectious complications as in steroid-based protocols.

A safe immunosuppressive protocol in adult-to-adult living related liver transplantation.

BACKGROUND: In this series of 32 adult-to-adult living related liver transplantations, we assessed the efficacy and safety of basiliximab in combination with a tacrolimus-based regimen. Basiliximab, a chimeric monoclonal antibody directed against the alpha chain of the interleukin-2 (IL-2) receptor (CD25), has been extensively evaluated as induction therapy for cadaveric liver transplant recipients. PATIENTS AND METHODS: Thirty-two adult-to-adult living related liver transplantations were performed in the last 3 years. All patients received two 20 mg doses of basiliximab (days 0 and 4 posttransplantation) followed by tacrolimus (0.15 mg/kg/d; 10-15 ng/mL target trough levels) and steroids (starting with 20 mg IV switched to PO as soon as the patient was able to eat and weaned within 1-2 months). The average follow-up was 395 days after transplantation. RESULTS: Of the patients, 93.75% remained rejection-free during follow-up with an actuarial rejection-free probability of 92.59% within 3 months. Two patients (6%) had one episode of biopsy-proven acute cellular rejection (ACR). Actuarial patient and graft survival rates at 3 years were 86.85% and 81.25%. One patient (3%) experienced one episode of sepsis. There was no evidence of cytomegalovirus infections or side effects related to the basiliximab. We found zero de novo malignancy but we observed two patients with metastatic spread of their primary malignancy during the follow-up. CONCLUSION: Basiliximab in association with tacrolimus and steroids is effective as prophylaxis of ACR among adult living related liver transplant recipients.

Acceptance of marginal liver donors increases the volume of liver transplant: early results of a single-center experience.

To expand the donor pool, clinicians are continually modifying criteria to accept organs, particularly those in the so-called expanded or marginal donor pool. The concept and definition of a marginal donors continues to evolve. The impact of their use is the result of a combination of donor and recipient factors. Most clinicians accept steatosis above 30%, donor age over 60 years, prolonged ischemia time, prolonged intensive care unit stay, hypernatremia, previous cardiac arrest, prolonged episodes of hypotension, large use of inotrope drugs, and elevated liver function tests as criteria for designation of a marginal organ. In June 2003, we started to use marginal donors each year tripling the number of transplants per year at our center.

Pediatric liver transplantation: preliminary results at Istituto Mediterraneo Trapianti e Terapie ad Alta Specializzazione.

Between July 2003 and November 2004 14 pediatric liver transplantations (LTx) have been performed in 12 children using cadaveric donors. The primary diseases were as follows biliary atresia in 9 cases, whereas the other 3 children were affected by cystic fibrosis, Langherans cells histiocytosis, and hepatoblastoma, respectively. Median patient waiting time was 103 days (range, 2-158); no patient died while on the waiting list. Patients who underwent transplantation included 7 boys and 5 girls, ranging in age from 6 months to 14 years (median age, 5 years). Recipient median weight was 16 kg (range, 6-38). Donor median age was 19 years (range, 3-47), whereas donor median weight was 74 kg (range, 15-90). All children who underwent primary LTx were United Network for Organ Sharing (UNOS) status 2B. Of the 12 transplanted patients, 9 received a left lateral segment (LLS) from an in situ split liver, whereas 3 received a whole graft. Two children developed an episode of acute cellular rejection on the seventh postoperative day, which was treated successfully with a course of intravenous steroids for 3 days. After a median follow-up of 245 days, 10 children are alive but 2 children died due to primary nonfunction (PNF) on the second postoperative day and septic shock on the fifth postoperative day after retransplantation for acute hepatic artery thrombosis, respectively. One child who underwent retransplantation for hepatic artery thrombosis on the 31st postoperative day after primary LTx is currently alive. Evaluation of our initial data suggests that the split liver technique has the potential to meet the needs of pediatric LTx allowing grafting early in the course of the original disease and reducing waiting time.

Basiliximab in a triple-drug regimen with tacrolimus and steroids in liver transplantation.

BACKGROUND: Basiliximab, a chimeric monoclonal antibody (mAb) directed against the alpha chain of the interleukin-2 (IL-2) receptor (CD25), has been extensively evaluated as induction therapy for kidney transplant recipients, more frequently in combination with a cyclosporine-based regimen. In this study, we assessed the efficacy and safety of basiliximab in combination with tacrolimus and steroids following liver transplantation. METHODS: One hundred fifty-two liver transplant recipients (141 cadaveric donors and 11 living donors [LRLT]) in the last 4 years were treated with 2 20-mg doses of basiliximab (days 0 and 4 posttransplantation) followed by tacrolimus (0.15 mg/kg/d; 10-15 ng/mL target trough levels) and steroids (500 mg intravenous [IV] bolus at the reperfusion followed by 20 mg orally daily and weaning off in 1 or 2 months). Follow-up ranged from 104 to 1630 days after transplantation (mean, 665 days; SD +/- 442.65; median, 509 days). RESULTS: Eighty-five percent of patients remained rejection-free during follow-up with an actuarial rejection-free probability of 78% within 3 months. Nineteen patients had 22 episodes of biopsy-proven acute cellular rejection (ACR). Actuarial patient and graft survival rates at 3 years were 86.7% and 75.8%, respectively. Twenty-seven patients (20.6%) experienced 1 episode of sepsis, requiring temporary reduction of immunosuppressive therapy. There was no evidence of CMV infections or side effects related to basiliximab. We observed 2 de novo malignancies, 1 recurrence from an ileal carcinoid tumor and 1 pulmonary recurrence of hepatocellular carcinoma (HCC) in 1 recipient of LRLT. CONCLUSIONS: Basiliximab in association with tacrolimus and steroids is effective prophylaxis of ACR in liver transplant recipients and does not increase the incidence of infections or adverse effects.

Transjugular intrahepatic portosystemic shunt in adult liver recipient with delayed graft function.

BACKGROUND: Transjugular intrahepatic portosystemic shunt (TIPS) has become an effective treatment for the complications of portal hypertension. We assessed the feasibility and outcome of TIPS in liver transplant recipients who developed delayed graft function (DGF) with portal hypertension. METHODS: From June 2003 to June 2004, 80 cadaveric orthotopic liver transplantation (OLTx) have been performed at our institution. Five patients (6.25%) developed DGF with hyperbilirubinemia and ascites with severe portal hypertension and were treated with TIPS placement (in the 6-month time period from the transplantation). RESULTS: There were no complications related to the procedure. No episodes of encephalopathy were seen. Four patients had better control of the ascites. In one case, we observed complete recovery of the transplanted liver with normalization of the liver function test. Three patients underwent retransplantation (within 7 days from the TIPS), whereas 1 is still on the list 6 months after TIPS placement with recurrent episodes of ascites. CONCLUSIONS: In our preliminary series, TIPS reduced dramatically the portosystemic gradient and improved clinical conditions. The results were negatively affected by the fact that the transplanted liver did not recover its function.

Thoracoabdominal bypass graft with liver retransplantation for the treatment of a pseudoaneurysm of the supraceliac aorta after liver transplantation.

Pseudoaneurysm following liver transplantation is a rare but life-threatening complication. Treatment is directed towards control of fatal bleeding. Ligation with or without revascularization of the graft is the treatment of choice. When revascularization is not possible, or the liver does not tolerate the arterial blood deprivation, retransplantation is the only option. We report a case of a 14-month-old girl who developed a pseudoaneurysm at the anastomosis between the recipient supraceliac aorta and the donor graft. The pseudoaneurysm was excised and the aorta was ligated above and below it. An extra-anatomical thoracoabdominal arterial graft was used to provide arterial blood supply to the lower torso and also to arterialize a new orthotopic liver graft. This is the first reported case of the use of thoracoabdominal jump graft to vascularize a transplanted liver.

En bloc heterotopic auxiliary liver and bilateral renal transplant in a patient with homozygous protein C deficiency.

An 8-year-old girl with homozygous protein C deficiency who had undergone maintenance dialysis since birth because of renal veins with thrombosis was treated with an en bloc heterotopic auxiliary liver and bilateral renal transplantation. The reconstitution of protein C activity by auxiliary liver transplantation facilitated successful renal transplantation.

Intestinal transplantation for short gut syndrome attributable to necrotizing enterocolitis.

BACKGROUND: Necrotizing enterocolitis (NEC) is a life-threatening condition of the neonatal age, which frequently requires surgical intervention. After extensive bowel resection, a small proportion of these patients may develop chronic short gut syndrome (SGS) and require chronic total parenteral nutrition (TPN) use. Intestinal transplantation has been performed in these patients as a life-saving option. This study reviews our experience with intestinal transplantation for SGS attributable to NEC emphasizing the mode of presentation, natural history, timing, and outcome. METHODS: A retrospective chart review was performed for all pediatric patients who underwent small bowel transplantation for NEC at the University of Miami between August 1994 and March 1999. RESULTS: Eleven transplants were performed for 10 patients with NEC (8 male and 2 female; median age: 1.75 years [range: 10 months to 10. 1 years]). Procedures performed were isolated intestinal transplants (n = 2), combined liver-intestinal transplants (n = 6), and multivisceral transplants (n = 3). All patients were born prematurely with median birth weight of 1.640 kg (range: 810 g to 2. 730 kg). They developed NEC in the first few days of life and subsequently underwent an average of 5 surgeries per patient before transplant. Transplant was indicated for liver failure in 8 patients and recurrent central line sepsis in 2 others. At present, 6 patients are alive with an overall 1-year and 3-year actuarial survival of 60% and a median follow-up of 29 months (range: 9-46 months). Six children have been weaned off TPN after a median time of 71 days (range: 19-131) from transplantation. All survivors are at home with functional grafts. CONCLUSION: Intestinal transplantation provided a reasonable outcome in patients with NEC-associated SGS who had already developed life-threatening complications related to TPN. Intestinal transplantation replaced the diseased intestine and liver, enfranchised patients from TPN, and conferred improved quality of life. These patients should be actively considered for intestinal transplantation and referred to a transplant center as soon as possible.

Inclusion of entire pancreas in the composite liver and intestinal graft in pediatric intestinal transplantation.

An entire pancreatico-duodenal complex was included in the liver and intestinal graft in eight children who received small-size grafts. This method showed several advantages compared to the traditional approach. They included reducing time for graft preparation by eliminating donor pancreas resection, no necessity of biliary reconstruction and leaving natural tissue support for blood vessels. The method was not associated with an increased risk of complications such as pancreatitis or rejection. It should be considered in pediatric liver and intestinal transplant recipients who require small-size grafts.

Multivisceral transplantation for megacystis microcolon intestinal hypoperistalsis syndrome.

BACKGROUND: Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare autosomal recessive disorder causing a functional neonatal bowel obstruction. Its etiopathogenesis is not fully understood. The prognosis is poor in the majority of cases; most patients die before the age of 6 months. In this report, we describe our experience with three patients with MMIHS in whom multivisceral transplantation was performed. METHODS: Three patients with MMIHS underwent multivisceral transplantation. All patients were females with a history of long-term total parenteral nutrition (TPN) with TPN-related cholestatic liver disease. RESULTS: Patient 1 died 17 months after transplantation because of aspiration after revision of her feeding gastrostomy. At the time of death, the graft was functioning and the patient was completely off TPN. Patient 2 is alive 17 months after transplant. She is a fully functional, active 2-year-old and has also recently begun oral feeding after intensive rehabilitation. Patient 3 died on day 44 of multisystem failure. CONCLUSIONS: This is the first report in the literature of multivisceral transplantation for MMIHS. Although one of the three patients died 44 days after surgery from multiorgan system failure, the other two patients had long-term survival after transplant and both grew well on enteral feeding alone. One patient died 17 months from a non-transplant-related complication, while the other is living at home off of TPN, with almost complete dietary rehabilitation 17 months after transplant. Our case reports suggest that multivisceral transplantation is a valuable therapeutic option for patients affected by MMIHS with TPN-induced liver failure.

Juvenile polyposis: case report and assessment of the neoplastic risk in 271 patients reported in the literature.

A case of juvenile polyposis is reported and 271 cases are collected from the literature. The risk for neoplasia is analyzed and the endoscopic or surgical therapy is discussed. The authors conclude that juvenile polyposis should be considered as a challenge to the surgeon with regard to familial adenomatous syndromes, and strongly recommend a close follow-up of patients with juvenile polyposis.

[Biliary lithiasis in childhood. A spectrum of diseases with different clinical significance during fetal life, childhood and adolescence]. / La litiasi biliare in età pediatrica. Uno spettro di patologie con diverso significato clinico nella vita fetale nel lattante, nell'infanzia e nell'adolescenza.

On the basis of a research on fetal cholelithiasis, a review on the various form of cholelithiasis in pediatric age has been carried out. These include, in addition to fetal cholelithiasis, lithiasis in the first year of life and lithiasis in infancy and adolescence. These various expressions of the same pathology differ for incidence, predisposing factors, clinical situation, therapy and follow-up. The research conducted on fetal cholelithiasis showed an incidence of 0.39%, higher than expected. There isn't any maternal, obstetrical or fetal predisposing factor. The diagnosis is purely instrumental and is not correlated with known clinical or humoral data. The most frequent evolution is spontaneous resolution of the biliary echogenic images in absence of clinical manifestations; the complications are rare and not well documented. Cholelithiasis in the first years of life is correlated with malformative, pharmacologic or iatrogenic predisposing factors. The clinical situations include symptomless cases with spontaneous resolution and cases with serious complications. Therapy is to be chosen in each case in accordance with clinical features. Pediatric cholelithiasis beyond the first year of life, especially in the later childhood and adolescence, can be similar to cholelithiasis of adults for epidemiology, pathogenesis, symptomatology and therapy.