Postoperative care in pediatric liver transplantation

In the last 25 years, liver transplantation in children has become an effective, definitive, and universally accepted treatment for terminal liver diseases. Long-term survival exceeds 80% and improves each year as the result of constant technical advancements and improvements in immediate postoperative intensive care and clinical control

Experiência em fístula artério-venosa para hemodiálise crônica em crianças: detalhes e refinamentos técnicos

PURPOSE: The aim of this paper is to report our experience in the creation of arteriovenous fistulas in children by using microsurgical vascular techniques, with emphasis on the details of the surgical technique. METHODS: The children underwent surgery from July 1997 to March 2004. Operating loupes (magnification: 3.5X) were used by the entire surgical team. After dissection and adequate mobilization, the vein was anastomosed to the artery in an end-to-lateral fashion by using 4 separate 7/0 or 8/0 prolene running sutures. RESULTS: Twenty nine children underwent 33 fistula creations - 21 radiocephalic, 6 brachiocephalic, 4 brachiobasilic, and 2 saphenofemoral. Primary patency was achieved in 17/21 (80.9%) of the radiocephalic fistulas, 5/6(83.3%) of the brachiocephalic, 3/4 (75.0%) of the brachiobasilic, and 2/2 (100%) of saphenofemoral. Two patients developed fistula occlusion and radial artery thrombosis, and in 2 others, radiocephalic fistulas became occluded in the first or second postoperative week. In the late postoperative period, the saphenofemoral fistulas were responsible for significant edema formation in the lower extremity; an aneurysm formed in a brachiocephalic fistula after a long period of utilization. As to the patency of the different sites of fistulas, the brachiocephalic and brachiobasilic fistulas had the same incidence of patency as the radiocephalic fistulas (P >;.05). CONCLUSIONS: This experience indicates that the arteriovenous fistulas are a satisfactory method for providing hemodialysis in children. The utilization of microsurgical techniques and some technical refinements herein described permit the attainment of high patency rates of the fistulas.OBJETIVO: Relatar a experiência dos autores na execução de fístulas artério-venosas em crianças, com a utilização dos recursos da microcirurgia vascular, com especial ênfase aos detalhes de técnica cirúrgica. MÉTODOS: Os pacientes foram operados entre julho de 1997 e março de 2004. Foram utilizadas lupas cirúrgicas (aumento de 3,5 vezes). Após a dissecção da veia e da artéria, a anastomose veno-arterial foi realizada de forma término-lateral, com quatro suturas contínuas de fio 7/0 ou 8/0. RESULTADOS: Vinte e nove crianças foram operadas para a realização de 33 fístulas - 21 rádio-cefálicas, 6 bráquio-cefálicas, 4 bráquio-basílicas e 2 safeno-femorais. Obteve-se permeabilidade em 17/21 (80,9%) fístulas rádio-cefálicas, 5/6 (83,3%) bráquio-cefálicas, 3/4 (75,0%) bráquio-basílicas e 2/2 (100%) safeno-femorais. 2 pacientes apresentaram obstrução da fístula e da artéria radial e 2 outras fístulas rádio-cefálicas obstruíram-se entre a primeira e segunda semana de pós-operatório. Tardiamente, verificou-se que as fístulas safeno-femorais causaram edema do membro inferior correspondente e houve formação de aneurisma em uma fístula bráquio-cefálica. Quanto à permeabilidade, verificou-se que as fístulas bráquio-cefálicas e bráquio-basílicas apresentaram a mesma incidência de permeabilidade em comparação com as fístulas rádio-cefálicas (p>;0,05). CONCLUSÃO: A presente casuística demonstra que as fístulas artério-venosas persistem como um bom acesso vascular para hemodiálise em crianças. A utilização de microcirurgia e alguns detalhes técnicos aqui descritos permitem a obtenção de bons índices de permeabilidade das fístulas

Conjoined twins: twenty years' experience at a reference center in Brazil

OBJECTIVE: This study reports on the experience of one hospital regarding the surgical aspects, anatomic investigation and outcomes of the management of 21 conjoined twin pairs over the past 20 years. METHODS: All cases of conjoined twins who were treated during this period were reviewed. A careful imaging evaluation was performed to detail the abdominal anatomy (particularly the liver), inferior vena cava, spleen and pancreas, either to identify the number of organs or to evaluate the degree of organ sharing. RESULTS: There were eight sets of ischiopagus twins, seven sets of thoracopagus twins, three sets of omphalopagus twins, two sets of thoraco-omphalo-ischiopagus twins and one set of craniopagus twins. Nine pairs of conjoined twins could not be separated due to the complexity of the organs (mainly the liver and heart) that were shared by both twins; these pairs included one set of ischiopagus twins, six sets of thoracopagus twins and one set of thoraco-omphalo-ischiopagus twins. Twelve sets were separated, including seven sets of ischiopagus twins, three sets of omphalopagus twins, one set of thoracopagus twins and one set of craniopagus conjoined twins. The abdominal wall was closed in the majority of patients with the use of mesh instead of the earlier method of using tissue expanders. The surgical survival rate was 66.7%, and one pair of twins who did not undergo separation is currently alive. CONCLUSION: A detailed anatomic study of the twins and surgical planning must precede separation. A well-prepared pediatric surgery team is sufficient to surgically manage conjoined twins

Prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation

OBJECTIVE: Chronic rejection remains a major cause of graft failure with indication for re-transplantation. The incidence of chronic rejection remains high in the pediatric population. Although several risk factors have been implicated in adults, the prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation are not known. Hence, the current study aimed to determine the factors involved in the progression or reversibility of pediatric chronic rejection by evaluating a series of chronic rejection cases following liver transplantation. METHODS: Chronic rejection cases were identified by performing liver biopsies on patients based on clinical suspicion. Treatment included maintaining high levels of tacrolimus and the introduction of mofetil mycophenolate. The children were divided into 2 groups: those with favorable outcomes and those with adverse outcomes. Multivariate analysis was performed to identify potential risk factors in these groups. RESULTS: Among 537 children subjected to liver transplantation, chronic rejection occurred in 29 patients (5.4%). In 10 patients (10/29, 34.5%), remission of chronic rejection was achieved with immunosuppression (favorable outcomes group). In the remaining 19 patients (19/29, 65.5%), rejection could not be controlled (adverse outcomes group) and resulted in re-transplantation (7 patients, 24.1%) or death (12 patients, 41.4%). Statistical analysis showed that the presence of ductopenia was associated with worse outcomes (risk ratio=2.08, p=0.01). CONCLUSION: The presence of ductopenia is associated with poor prognosis in pediatric patients with chronic graft rejection

New concepts and outcomes for children with hepatoblastoma based on the experience of a tertiary center over the last 21 years

OBJECTIVE: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years. PATIENTS AND METHODS: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system. The following data were analyzed: age at diagnosis, comorbidities, prematurity, treatment modalities, histopathological findings, surgical details and complications, treatment outcomes, chemotherapy schedules, side effects and complications. Treatment outcomes included the occurrence of local or distant recurrence, the duration of survival and the cause of death. The investigation methods were ultrasonography, CT scan, serum alpha-fetoprotein level measurement and needle biopsy. Chemotherapy was then planned, and the resectability of the tumor was reevaluated via another CT scan. RESULTS: The mean numbers of neoadjuvant cycles and postoperative cycles of chemotherapy were 6±2 and 1.5±1.7, respectively. All children except one were submitted for surgical resection, including 50 partial liver resections and 7 liver transplantations. Statistical comparisons demonstrated that long-term survival was associated with the absence of metastasis (p=0.04) and the type of surgery (resection resulted in a better outcome than transplantation) (p=0.009). No associations were found between vascular invasion, incomplete resection, histological subtype, multicentricity and survival. The overall 5-year survival rate of the operated cases was 87.7%. CONCLUSION: In conclusion, the experience of a Brazilian tertiary center in the management of hepatoblastoma in children demonstrates that long survival is associated with the absence of metastasis and the type of surgery. A multidisciplinary treatment involving chemotherapy, surgical resection and liver transplantation (including transplantations using tissue from living donors) led to good outcomes and survival indexes

Language assessment of children with severe liver disease in a public service in Brazil

OBJECTIVE: The aim of this research was to compare language development (expressive and receptive skills) in children awaiting liver transplantation with that of children who have already undergone the surgical procedure. METHODS: An observational, descriptive, cross-sectional study was conducted with 76 children divided into groups, as follows: 31 children who were candidates for liver transplantation (Group 1; G1), 45 children who had already undergone liver transplantation (Group 2; G2), and a control group (CG) of 60 healthy, normally developing children. Health status information was gathered, and the Test of Early Language Development (TELD)-3 was used to assess language skills. Family household monthly income data were also gathered using a specific questionnaire. RESULTS: G1 had poorer language performance compared with G2 and the CG. G2 had lower language performance when compared with the CG. However, when considering the TELD-3 standard scores, G2 had scores within normal limits. The regression analysis indicated age as a risk factor for language deficits in Group 1 and family income as a risk factor for language deficits in G2. CONCLUSIONS: The results suggested that children with chronic liver disease have delays in language development. Transplanted children have linguistic performance within normal limits, but their scores tended to be lower than the CG

Modelos experimentais de hepatectomia e regeneração hepática em ratos recém-nascidos e recém-desmamados

OBJECTIVES: Liver regeneration is a complex process that has not been completely elucidated. The model most frequently used to study this phenomenon is 70% hepatectomy in adult rats; however, no papers have examined this effect in developing animals. The aims of the present study were: 1) to standardize two models of partial hepatectomy and liver regeneration in newborn suckling and weaning rats, and 2) to study the evolution of remnant liver weight and histological changes of hepatic parenchyma on the days that follow partial hepatectomy. METHODS: Fifty newborn and forty-four weaning rats underwent 70% hepatectomy. After a midline incision, compression on both sides of the upper abdomen was performed to exteriorize the right medial, left medial and left lateral hepatic lobes, which were tied inferiorly and resected en bloc. The animals were sacrificed on days 0 (just after hepatectomy), 1, 2, 3, 4 and 7 after the operation. Body and liver weight were determined, and hepatic parenchyma was submitted to histological analysis. RESULTS: Mortality rates of the newborn and weaning groups were 30% and 0%, respectively. There was a significant decrease in liver mass soon after partial hepatectomy, which completely recovered on the seventh day in both groups. Newborn rat regenerating liver showed marked steatosis on the second day. In the weaning rat liver, mitotic figures were observed earlier, and their amount was greater than in the newborn. CONCLUSIONS: Suckling and weaning rat models of partial hepatectomy are feasible and can be used for studies of liver regeneration. Although similar, the process of hepatic regeneration in developing animals is different from adults.OBJETIVOS: A regeneração hepática é um processo complexo não completamente elucidado. O modelo mais utilizado para o estudo desse fenômeno é a hepatectomia a 70% em ratos adultos. Não há trabalhos utilizando modelos em animais em crescimento. Desta forma, os objetivos deste estudo foram: 1. padronizar dois modelos de hepatectomia parcial e regeneração hepática utilizando ratos recém-nascidos e recém-desmamados; 2. estudar a evolução do peso do fígado remanescente e as alterações histológicas do parênquima hepático nos dias subseqüentes à hepatectomia parcial. MÉTODOS: Cinqüenta ratos recém-nascidos e quarenta e quatro ratos recém-desmamados foram submetidos à hepatectomia a 70%. Após laparotomia mediana, foi realizada compressão bilateral no abdome superior do animal, levando à exteriorização dos lobos hepáticos direito medial, esquerdo medial e esquerdo lateral, que foram ligados na base e ressecados em bloco. Os animais foram sacrificados logo após a hepatectomia e no 1º,2º,3º,4º, e 7º dias após a cirurgia. O peso corpóreo e do fígado foram determinados, e o parênquima hepático submetido à análise histológica. RESULTADOS: Os índices de mortalidade dos animais recém-nascidos e recém-desmamados foram 30% e 0% respectivamente. Em ambos os grupos, houve uma diminuição significativa na massa hepática logo após a hepatectomia, com recuperação completa no sétimo dia de pós-operatório. O parênquima hepático dos animais recém-nascidos apresentou acentuada esteatose no segundo dia. O fígado do animal recém-desmamado exibiu figuras mitóticas mais precoces e mais numerosas que o do recém-nascido. CONCLUSÕES: Os modelos de hepatectomia parcial em ratos recém-nascidos e recém-desmamados são factíveis e podem ser usados para estudos da regeneração hepática. Embora semelhante, o processo de regeneração hepática em animais em crescimento não é igual ao do animal adulto