Four hundred thirty consecutive pediatric living donor liver transplants: variables associated with posttransplant patient and graft survival.

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (São Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight (≤10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight ≤ 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight (≤10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.

Schistosoma mansoni infection in the liver graft: The impact on donor and recipient outcomes after transplantation.

The increasing number of transplants performed each year has led to the identification of unusual diseases in liver grafts from asymptomatic donors that were unrecognized before liver transplantation. Here we report our experience with patients who received liver grafts infected with schistosomiasis. From September 1991 to August 2010, 482 pediatric liver transplants were performed at A. C. Camargo Hospital/Sírio-Libanês Hospital (São Paulo, Brazil). For the identification of Schistosoma mansoni infections, pathology slides for the recipients were reviewed; these included postreperfusion and follow-up liver biopsy samples. We were able to identify 6 cases of schistosomiasis transmitted through infected grafts (5 of these grafts were from living donors). All living donors were confirmed to have normal liver chemistries, negative fecal tests for parasitic diseases, and normal abdominal ultrasound findings. Liver biopsy was not performed before transplantation. In all cases, features of schistosomiasis were absent in the liver explants. The living donors were treated with praziquantel and were taught to avoid risk factors for reinfection. No specific treatment for schistosomiasis was given to the recipients. There were no perioperative deaths, but 2 recipients died after living donor liver transplantation (LDLT) because of Kaposi's sarcoma and non-Hodgkin's lymphoma. In conclusion, using liver grafts infected with S. mansoni eggs did not compromise the results of LDLT in this pediatric cohort. Because of the parasite's life cycle and the therapeutic target of praziquantel, only donors should be treated for the infection. Three years of follow-up showed an uneventful recovery for the living donors.

Portal vein obstruction after liver transplantation in children treated by simultaneous minilaparotomy and transhepatic approaches: initial experience.

Portal vein thrombosis is a complication that occurs anytime after liver transplantation and can compromise the patient and graft survival. We describe a combined technique for PV recanalization in cases of PV obstruction after liver transplantation. Four children (1%), of 367 subjected to liver transplantation from June 1991 to December 2008, underwent PV recanalization through a combined approach (transhepatic and minilaparotomy). All children received left lateral hepatic segments, developed Portal vein thrombosis (n=3) and stenosis (n=1), and presented with symptoms of portal hypertension after transplantation. PV recanalization was tried by transhepatic retrograde access, and a minilaparotomy was performed when percutaneous recanalization was unsuccessful. Three patients underwent a successful portal recanalization and stent placement with the combined technique. In one patient, the recanalization was unsuccessful because of an extensive portomesenteric thrombosis. The other three children had the portal flow reestablished and followed with Doppler US studies. They received oral anticoagulation for three consecutive months after the procedure and the clinical symptoms subsided. In case of PV obstruction, the combined approach is technically feasible with good clinical and hemodynamic results. It' is a minimally invasive procedure and can be tried to avoid or delay surgical treatment or retransplantation.

Modified pediatric end-stage liver disease scoring system and pediatric liver transplantation in Brazil.

The Pediatric End-Stage Liver Disease (PELD) scoring system is a formula developed to provide a continuous numerical assessment of the risk of death in order to allocate livers to children in need of transplantation. The PELD scoring system was introduced in Brazil in July 2006. An important change was made in the system: the final number for listing patients less than 12 years old for transplantation was the calculated PELD score multiplied by 3. The consequences of this allocation policy were analyzed in 2 ways in this research: nationally and in the state of São Paulo (SP State). In the analysis of the national data, a comparison of the pre-PELD era (July 2003 to July 2006) and the post-PELD era (August 2006 to April 2009) showed that the total number of pediatric transplants for children under 12 years of age decreased 7%. Regionally, in SP State, there was a 62% increase in the number of deceased donor liver transplantation procedures for the pediatric population after the introduction of the modified PELD system. There was also a 6.1-fold increase in split liver transplantation as well as a statistically significant decrease in the time on the waiting list (P < 0.001). In conclusion, changing the allocation policy in Brazil in order to benefit pediatric patients on the waiting list had different results according to analyses of national and regional data. A significant increase in deceased donor liver transplantation/split liver transplantation and a shorter time on the waiting list were observed in SP State. The modified PELD scoring system is simple and optimizes the utilization of deceased donor liver grafts in centers performing pediatric transplants.

Left lateral segmentectomy for pediatric live-donor liver transplantation: special attention to segment IV complications.

BACKGROUND: During left lateral segmentectomy for live-donor liver transplant, the vascular inflow to segment IV can be compromised. An area of ischemia can be seen intraoperatively and further segment IV resection may be needed to prevent necrosis and abscess formation. METHODS: From July 1995 to February 2007, 324 consecutive living donor liver transplantations were performed at Hospital A. C. Camargo and Hospital Sirio-Libanes, Sao Paulo, Brazil. Two hundred eleven left lateral segments were transplanted in this period. Data on 204 left lateral segments donors were available for this analysis. RESULTS: There were 108 female and 96 male donors. Median age was 29 years (range, 16-48 years). Median follow-up time was 2.2 years (range, 2 months-11.8 years). Median intensive care unit stay was 1 day (range, 1-3 days), and median hospital stay was 5 days (range, 4-47 days). Postoperative complications were encountered in 39 donors (19.1%). Partial segment IV resection on the course of the primary surgery due to parenchyma discoloration was required in 107 cases (52.5%). Ten patients (4.9%) developed segment IV necrosis or abscesses, although four of them had had segment IVB resection intraoperatively. Segment IV necrosis or abscess significantly increased hospital stay and the number of readmissions, from 5.5+/-3.5 days to 8.4+/-3.7 days (P=0.012) and from 6 of 194 (3%) to 5 of 10 (50%) (P=0.001), respectively. CONCLUSIONS: Middle hepatic segment abscess or necrosis was the most frequent complication after left lateral segmentectomy (4.9%). Objective intraoperative strategies need to be developed to evaluate middle hepatic segment ischemia to identify and treat patients at higher risk.

Living donor liver transplantation for children in Brazil weighing less than 10 kilograms.

Infants with end-stage liver disease represent a treatment challenge. Living donor liver transplantation (LDLT) is the only option for timely liver transplantation in many areas of the world, adding to the technical difficulties of the procedure. Factors that affect morbidity and mortality can now be determined, which opens a new era for improvement. We have accumulated an 11-year experience with LDLT for children weighing<10 kg. From October 1995 to October 2006, a total of 222 LDLT in patients<18 years of age were performed; 129 primary LDLT and 7 retransplants (4 LDLT and 3 deceased donor grafts) were performed in 129 infants weighing<10 kg. Forty-seven patients received grafts with graft-to-recipient weight ratio (GRWR) of >4%. Two patients received monosegmental grafts, and 2 patients underwent delayed abdominal wall closure. Portal vein thrombosis occurred in 5.4% of the patients, hepatic artery thrombosis in 3.1%, and both in 1.5%. Among several variables studied, only the bilirubin level at the time of transplantation was associated with increased risk of death (P=0.009). Grafts with GRWR>4% had no negative effect on patient survival. There were 7 retransplants, and 4 patients received a second parental LDLT. Patient survival rates at 1, 3, and 10 years after transplantation were 88.8%, 84.7%, and 82% for all children, and 87.5%, 84.9%, and 84.9% for infants weighing<10 kg. LDLT has results comparable to other modalities of liver transplantation in infants. Monosegment grafts were rarely required in this series, although they may be necessary in patients with lower body weight.

Cancer metacronico em colon abaixado: alternativa cirurgica / Metachronous tumors in the pull-throughed colon

Os autores apresentam uma tecnica empregada para o tratamento de cancer de colon metacronico em colon abaixado. E sugerida, como alternativa a amputaçao do reto, a ressecçao do segmento que aloja o tumor, mantendo-se a arcada vascular. Esse procedimento e viavel emcasos onde o tumor aparentemente nao envolve a arcada marginal e quando nao ha comprometimento evidente dos ganglios de drenagem intermediarios e principais

Reservatório vascular totalmente implantável para quimioterapia: experiência inicial / Totally implantable vascular reservoir for chemotherapy: initial experience

Os autores relatam sua experiência inicial com a colocaçäo de reservatórios vasculares totalmente implantáveis para quimioterapia. Foram avaliados 31 reservatórios colocados em 29 pacientes. Foram observados 19,3% de complicaçöes, sendo que em 12,9% dos casos houve a necessidade de se fazer a retirada do reservatório. Em nenhum caso retirou-se o reservatório por infecçäo ou obstruçäo tardia. Baseados nesses resultados, os autores concluem que o reservatório vascular totalmente implantável constitui-se em recurso extremamente valioso para pacientes necessitando de quimioterapia por períodos prolongados

Síndrome de Budd-Chiari associada a policitemia "vera": indicaçäo de transplante hepático ou derivaçäo venosa? / Budd-Chiari syndrome associated to polycythemia vera: indication for liver transplantation or venous shunts?

É apresentado um caso de síndrome de Budd-Chiari associado a policitemia vera, em que foi indicado o transplante ortóptico do fígado. Após revisao da literatura acerca da etiologia e terapêutica dessa associaçao, discutem-se os fatores que devem influenciar a decisao a ser tomada entre um transplante de fígado ou uma derivaçao venosa.

Tratamento do hiperesplenismo por embolizaçäo esplênica em uma paciente com câncer de ovário para possibilitar a administraçäo de quimioterapia antineoplásica / Treatment of hyperplenism in patient with ovarian cancer to allow chemotherapy

O hiperesplenismo é uma síndrome caracterizada por esplenomegalia que, por sua vez, produz um quadro de anemia, leucopenia, trombocitpenia ou qualquer combinaçäo destas citpenias no contexto de uma medula óssea normal ou hiperplásica. Estas citopenias, por definiçäo, se corrigem com a esplenectomia. o quadro de leucopenia, anemia e/ou trombocitopenia decorrentes do hiperesplenismo é especialmente preocupante em pacientes oncológicos. Nestes pacientes, o hiperesplenismo muitas vezes se constitui em um fator limitante à administraçäo de doses terapêuticas de drogas quimioterápicas mielotóxicas. Apresentamos aqui um caso de uma paciente de 61 anos com esplenomegalia à doença de Caroli e portadora de cistoadenocarcinoma seroso papilífero invasivo estádio III (FIGO), cuja quimioterapia foi complicada por pancitopenia prolongada. O mielograma revelou uma medula óssea hipercelular sem evidência de alteraçöes mielodisplásicas ou megaloblásticas compatível com a presença de hiperesplenismo. Com a condiçäo clínica precária desta paciente precluia a esplenectomia, procedeu-se à embolizaçäo parcial esplênica (EPE) que resultou em melhora importante e prolongada dos seus níveis hematimétricos. Pudemos, assim, administrar quimioterapia antineoplásica para esta paciente sem a ocorrência de mielosupressäo importante. Concluímos, portanto, que a embolizaçäo esplênica é uma alternativa para casos de pacientes com câncer e necessidade de quimioterapia e com pancitopenia intensificada pela presença de hiperesplenismo.

Tratamiento de várices rectales a través de ligadura elástica endoscópica / Treatment of rectal varices using endoscopic elastic ligation

Las várices rectales sangrantes son raras y cuando aparecen representan un riesgo muy alto para estos pacientes. Relatamos el caso de un paciente de 50 años con cirrosis, que presentó sangrado por várices rectales y fue tratado con ligadura elástica endoscópica con excelente resultado

Transplante hepático com doador vivo familiar / Liver transplantation from living related donors

Objetivos: Apresentar a experiência com os primeiros 12 transplantes hepáticos com doadores vivos realizados no Hospital Sírio-Libanês de Säo Paulo. Métodos: Foram utilizados como doadores 6 pais e 6 mäes com idade entre 30 e 48 anos. Todos os doadores form submetidos ao termo de consentimento esclarecido, à avaliaçäo clínica, laboratorial e radiológica, e à coleta prévia de sangue para autotransfusäo. A idade dos receptores variou de 7 meses a 10 anos e, o peso, de 6,3 a 34 kg. Seis receptores foram considerados de alto risco devido a complicaçöes da doença hepática avançada e foram submetidos ao transplante em caráter de urgência. Resultados: A mortalidade dos doadores foi nula, e o tempo médio de internaçäo foi de 10 dias. Foram observadas complicaçöes técnicas em 4 receptores, enquanto que, em 7, ocorreram um ou mais episódios de infecçäo bacteriana, viral ou fúngica. Um ou mais episódios de rejeiçäo, comprovados por biópsia, foram diagnosticados em 7 pacientes. A sobrevida dos receptores foi de 67 por cento, sendo 83 por cento nos casos eletivos e 50 por cento nos casos urgentes. O seguimento pós-operatório variou de 8 a 25 meses. Dos 8 sobreviventes, 7 apresentam excelente qualidade de vida e funçäo hepática normal...