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1.
Pediatr Transplant ; 28(1): e14537, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37550267

RESUMO

BACKGROUND: Endovascular management of portal vein thrombosis (PVT) is challenging. Transsplenic access (TSA) is growing as an access option to the portal system but with higher rates of bleeding complications. The aim of this article is to evaluate the efficacy and safety of transsplenic portal vein recanalization (PVR) using a metallic stent after pediatric liver transplantation. MATERIALS AND METHODS: This is a retrospective review of 15 patients with chronic PVT who underwent PVR via TSA between February 2016 and December 2020. Two children who had undergone catheterization of a mesenteric vein tributary by minilaparotomy were excluded from the patency analysis but included in the splenic access analysis. The technical and clinical success of PVR and complications related to the procedure via TSA were evaluated. RESULTS: Thirteen children with PVT were treated primarily using the TSA. The mean age was 4.1 years (range, 1.5-13.7 years), and the most common clinical presentation was hypersplenism (60%). Technically successful PVR was performed in 11/13 (84.6%) children, and clinical success was achieved in 9/11 (81.8%) children. No major complications were observed, and one child presented moderate pain in the TSA (from a total of 17 TSA). The median follow-up was 48.2 months. The median primary patency was 9.9 months. Primary patency in the first 4 years was 75%, and primary assisted patency was 100% in the follow-up period. CONCLUSIONS: Transsplenic PVR is a safe and effective method for the treatment of PVT after pediatric liver transplantation.


Assuntos
Hepatopatias , Transplante de Fígado , Trombose Venosa , Humanos , Criança , Pré-Escolar , Transplante de Fígado/efeitos adversos , Veia Porta/cirurgia , Resultado do Tratamento , Hepatopatias/complicações , Trombose Venosa/etiologia , Trombose Venosa/cirurgia , Estudos Retrospectivos
2.
Liver Transpl ; 29(8): 827-835, 2023 08 01.
Artigo em Inglês | MEDLINE | ID: mdl-36847137

RESUMO

Left lateral segment grafts have become a suitable option in pediatric liver transplantation (PLT). The correlation between hepatic vein (HV) reconstruction and outcome is relevant when assessing the safe use of these grafts. We retrospectively reviewed the medical records prospectively collected from a pediatric living donor liver transplantation database and conducted a comparative analysis of the different left lateral segment graft types according to HV reconstruction. Donor, recipient, and intraoperative variables were analyzed. Post-transplant outcomes included vascular complications such as hepatic vein outflow obstruction, early (≤30 d) and late (>30 d) PVT, hepatic artery thrombosis, and graft survival. From February 2017 to August 2021, 303 PLTs were performed. According to venous anatomy, the distribution of the left lateral segment was as follows: single HV (type I) in 174 (57.4%), close HVs, simple venoplasty for reconstruction (type II) in 97 (32.01%), anomalous hepatic vein (AHV) with a distance between the HVs orifices that allowed simple venoplasty (type IIIA) in 25 (8.26%) and AHV with a distance between the HVs orifices requiring homologous venous graft interposition (type IIIB) in 07 (2.31%) grafts. Type IIIB grafts came from male donors ( p =0.04) and had a higher mean donor height ( p =0.008), a higher mean graft weight, and a higher graft-to-recipient weight ratio, both p =0.002. The median follow-up time was 41.4 months. The overall cumulative graft survival was 96.3%, and comparative graft survival showed no difference (log-rank p =0.61). No hepatic vein outflow obstructions were observed in this cohort study. There was no statistically significant difference in the post-transplant outcomes between the graft types. The venous reconstruction of the AHV with homologous venous graft interposition had similar outcomes in the short and long term.


Assuntos
Transplante de Fígado , Humanos , Masculino , Criança , Transplante de Fígado/efeitos adversos , Estudos de Coortes , Estudos Retrospectivos , Doadores Vivos , Veias Hepáticas/cirurgia , Veias Hepáticas/anatomia & histologia
3.
J Pediatr Surg ; 57(12): 955-961, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35697543

RESUMO

BACKGROUND: infants who require liver transplantation represent a treatment challenge because chronic liver disease at this early age affects the child's growth and development during a critical phase. The aim is to compare demographics, operative data, and long-term outcomes according to recipient weight at the time of LDLT. METHODS: This retrospective study included primary LDLT analyzed in 2 groups: BW ≤ 7 kg (n = 322) and BW > 7 kg (n = 756). A historical comparison between periods was also investigated. RESULTS: BW ≤ 7 kg had significantly lower height/age and weight/age z-scores, with median PELD score of 19. Transfusion rates were higher in the BW ≤ 7 kg group (30.9 ml/kg versus 15.5 ml/kg, P < 0.001). Higher frequencies of PV complications were seen in the BW ≤ 7 kg cohort. HAT and retransplantation rates were similar. Those with BW ≤ 7 kg required longer ICU and hospital stays. Patient and graft survival were similar. Patient survival in BW≤ 7 kg was significantly better in the most recent period. CONCLUSION: Malnutrition and advanced liver disease were more frequent in BW ≤ 7 kg. Despite increased rates of PVT and longer hospital stay, patient and graft long-term survival were similar between groups.


Assuntos
Transplante de Fígado , Doadores Vivos , Humanos , Criança , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Sobrevivência de Enxerto
4.
Case Rep Surg ; 2022: 1015061, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35223125

RESUMO

METHODS: We present a series of three patients with large hepatocellular adenoma lesions showing a central location, for which the living donor liver transplantation strategy was used as a backup procedure. RESULTS: Hepatocellular adenoma was confirmed by biopsy in all patients. Surgical resection was indicated because of the patients' symptoms and lesion size and growth. All patients had a lesion that was central or in close contact with major vessels. The final decision to proceed with the resection was made intraoperatively. A live donor was prepared for all three patients. Two patients underwent portal vein embolization associated with extended hepatectomy, and a total hepatectomy plus liver transplantation with a living donor was performed in one patient. All patients had good postoperative outcomes. CONCLUSIONS: In the treatment of hepatocellular adenomas for which complex resections are necessary and resectability can only be confirmed intraoperatively, surgical safety can be improved through the use of a living donor backup. Center expertise with living donor liver transplantation is paramount for the success of this approach.

5.
Pediatr Transplant ; 26(3): e14203, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-34881487

RESUMO

BACKGROUND: Acquired diaphragmatic hernia (DH) following liver transplantation (LT) is usually considered a surgical emergency. Interplay of contributing elements determines its occurrence but, in children, LT with partial liver grafts seems to be the most important causative factor. METHODS: This retrospective study describes the clinical scenario and outcomes of 11 patients with acquired DH following LDLT. RESULTS: During the study period, 1109 primary pediatric LDLT were performed (0.8% DH). The median age and BW of the recipients with DH at transplantation were 17 months and 11.1 kg, respectively; 63.7% of the cases had a weight/age Z-score of less than -2 at transplantation. The median interval between transplantation and diagnosis of DH was 114 days (32-538 days). A total of 6 (54.5%) of the patients had bowel obstruction due to bowel migration into the hemithorax. Ten defects were right-sided. Three patients required enterectomy and enterorrhaphy. Two patients required a new bilioenteric anastomosis, and one of them had complete necrosis of the Roux-in-Y limb. The patient with left-side DH presented gastroesophageal perforation. CONCLUSION: Most defects necessitate primary closure as the first treatment, and recurrence is rare. The associated problems encountered, especially related to intestinal complications, can determine increased morbidity following DH repair. Early diagnosis and intervention are required for achieving better outcomes.


Assuntos
Hérnia Diafragmática , Transplante de Fígado , Criança , Hérnia Diafragmática/complicações , Hérnia Diafragmática/cirurgia , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
6.
Pediatr Transplant ; 25(8): e14112, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34396644

RESUMO

BACKGROUND: The impact of the COVID pandemic on liver transplant (LT) programs varied among countries. Few data are available about that impact in pediatric liver transplant (PLT) programs. This study aimed at comparing the data of our program in Brazil (2019 vs. 2020). METHODS: Retrospective cohort study. RESULTS: One hundred and seventy-four PLT were performed in the period (93% living donors). Patients were divided into two groups according to the LT date: pre-COVID-19 period (march/2019-February/2020) and COVID-19 period (March/2020-February 2021). In the pre-COVID-19 period, 97 LTs were performed, and 77 LTs were performed in the COVID-19 period. Patients in the COVID-19 period were younger (10.9 months vs. 16 months, p 0.009), had higher PELD scores (15 vs. 14, p 0.04), more ascites (66.2 vs. 51.5%, p 0.03), and more frequently hospitalized before LT (27.3 vs. 17.5%). However, there was no difference in post-LT complications, retransplantation nor survival rates. Six (6.2%) patients from pre-COVID-19 period were COVID positive at a median of 15.5 months (14-17.5), and 6 (7.8%) patients from COVID-19 period were COVID positive at a median of 3 months (20 days-6 months) from LT. There was neither mortality nor complications in those patients. Four (33%) were hospitalized, and one had prolonged intubation. Four (33%) were asymptomatic, 4 (33%) had upper airways symptoms, and the remaining had gastrointestinal symptoms. CONCLUSION: Overall, PLT was not affected during COVID-19 period. Even though patients from COVID-19 period were sicker, there was no significant impact in LT outcomes. All the recipients who tested positive for COVID had a favorable outcome.


Assuntos
COVID-19/epidemiologia , Transplante de Fígado/estatística & dados numéricos , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Hospitais com Alto Volume de Atendimentos , Humanos , Lactente , Masculino , Pandemias , Complicações Pós-Operatórias/epidemiologia , SARS-CoV-2
7.
Pediatr Transplant ; 25(5): e13968, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33590638

RESUMO

BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2  < 50 mm Hg and those with PaO2  ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2  ≥ 50 mm Hg, 11 patients, and G2-PaO2  < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.


Assuntos
Síndrome Hepatopulmonar/cirurgia , Hipóxia/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Hipóxia/diagnóstico , Lactente , Tempo de Internação/estatística & dados numéricos , Cirrose Hepática/fisiopatologia , Masculino , Gravidade do Paciente , Cuidados Pós-Operatórios/estatística & dados numéricos , Estudos Retrospectivos , Análise de Sobrevida , Resultado do Tratamento
8.
Liver Transpl ; 26(12): 1644-1651, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-32852894

RESUMO

Pediatric living donor liver transplantation (PLDLT) is a successful therapeutic option for children with chronic and acute liver disease. After early transplant results, many technical advancements were introduced in the field to reduce the rate of complications and improve survival. The aim of this study is to present the outcomes of 975 primary PLDLTs in 3 periods: initial practice (period 1, 29 patients, January 1995 to December 1999), second period (period 2, 331 patients, January 2000 to December 2009), and third period (period 3 [P3], 615 patients, January 2010 to September 2019). Among the technical refinements introduced in P3 are the use of hyperreduced left lateral segment grafts, abdominal wall prosthetic mesh closure, double hepatic artery anastomosis, and increased use of vascular grafts for portal vein reconstruction. The outcomes included significant reductions of hepatic artery thrombosis (HAT), early portal vein thrombosis (EPVT), and retransplantation, with better patient and graft survival in P3. Additional analyses showed that the factors independently associated with worse 90-day patient survival were HAT, EPVT, and increasing Pediatric End-Stage Liver Disease score. In conclusion, the introduction of technical refinements in P3, in addition to improvements in patient care, determined a reduction in EPVT, HAT, and retransplantation. Consequently, patient and graft survival rates increased in all time points studied.


Assuntos
Doença Hepática Terminal , Transplante de Fígado , Criança , Doença Hepática Terminal/cirurgia , Sobrevivência de Enxerto , Artéria Hepática/cirurgia , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Estudos Retrospectivos , Índice de Gravidade de Doença
9.
Curr Pharm Des ; 26(28): 3402-3405, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32534559

RESUMO

The last decades have witnessed a significant improvement in the field of pediatric liver transplantation (LT), resulting in longer patient and graft survival; adequate graft selection, surgical refinement, the use of live donors and optimal postoperative care are among the reasons why pediatric recipients are living longer. With this new condition, pediatric recipients are now more exposed to the deleterious effects of immunosuppression, including metabolic, infectious and neoplastic complications, nephrotoxicity and neurotoxicity. Due to all those particularities, the approach to avoid overimmunosuppression or underimmunosuppression may be more difficult in children than in adult recipients. Moreover, pediatric recipients are exposed to growth issues and specific problems during adolescence, like nonadherence to immunosuppressive therapy. This article highlights the current immunosuppressive strategies for pediatric liver transplant recipients.


Assuntos
Transplante de Fígado , Adolescente , Adulto , Criança , Rejeição de Enxerto/prevenção & controle , Sobrevivência de Enxerto , Humanos , Terapia de Imunossupressão , Imunossupressores/efeitos adversos
10.
Transplantation ; 103(3): 536-543, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-29847508

RESUMO

BACKGROUND: Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The largest published series includes only 3 cases. One great challenge of this procedure is to ensure adequate vascular stumps for the LD, the MSUD patient, and the recipient of the domino graft. Here, we describe our experience in 11 cases of LD-DLT in MSUD, highlighting the technical aspects of LD-DLT. METHODS: From September 2012 to September 2017, 11 patients with MSUD underwent LD liver transplantation at our institution, and MSUD livers were used as domino grafts in 11 children. RESULTS: (1) MSUD patients: 10 patients received a left lateral segment. The donor's left hepatic vein (HV) was anastomosed to the confluence of the recipient's 3 HVs. No vascular grafts (VG) were required for portal vein (PV) anastomosis. Single arterial anastomosis was performed with microsurgery in 10 of 11 patients. (2) MSUD graft recipients: In 8 cases, HV reconstruction was performed between the graft's HV confluence and the recipient's HV confluence, and in 3 cases, a vena cava triangulation was necessary; 6 MSUD grafts required HV venoplasty. No VG were needed for HV reconstruction. VG were used for PV reconstruction in 3 cases due to sclerotic PV. In 2 cases, double arterial anastomoses were performed in the MSUD liver. All patients remain alive and well. CONCLUSIONS: Living donor liver transplantation followed by DLT for MSUD is a complex procedure and demands technical refinement. Special attention must be paid to vascular reconstruction.


Assuntos
Transplante de Fígado/métodos , Doadores Vivos , Doença da Urina de Xarope de Bordo/cirurgia , Obtenção de Tecidos e Órgãos/métodos , Anastomose Cirúrgica , Índice de Massa Corporal , Criança , Pré-Escolar , Feminino , Sobrevivência de Enxerto , Artéria Hepática/cirurgia , Veias Hepáticas/cirurgia , Humanos , Lactente , Fígado/irrigação sanguínea , Fígado/cirurgia , Masculino , Veia Porta/cirurgia , Doadores de Tecidos , Transplantados
11.
Medicine (Baltimore) ; 97(52): e13517, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30593126

RESUMO

RATIONALE: In the setting of metastatic or locally advanced adrenocortical carcinoma, a limited number of therapies are available and their efficacy is generally below modest. The backbone of treatment remains surgery, even for metastatic disease, whenever it is possible, and mitotane. Chemotherapy can be used with limited results. A small subset of patients with adrenocortical carcinoma may have high mutational burden and harbor mutations in mismatch-repair genes. PATIENT CONCERNS: We report a 40-year old and a 28-year-old female patients with metastatic adrenocortical carcinoma refractory to multiple treatments. DIAGNOSIS: Next-generation sequencing detected high mutational burden (>10 mutations/megabase) in both patients, one of them with MSH2 mutation. INTERVENTIONS: They were treated with pembrolizumab (100 to 200 mg every 3 weeks). OUTCOMES: The patient harboring a MSH2 mutation experienced a long-term complete response after pembrolizumab, while the patient with high mutational burden and absence of mismatch repair deficiency did not have any response. LESSONS: To the best of our knowledge, this is the first report in the literature of a durable complete response after pembrolizumab in a patient with metastatic adrenocortical carcinoma. Differences in therapy sequencing, possibly abscopal effect related to multiple previous radiotherapy exposition, predictive values of high mutational burden and mutations in mismatch-repair genes are discussed.


Assuntos
Neoplasias do Córtex Suprarrenal/tratamento farmacológico , Neoplasias do Córtex Suprarrenal/genética , Carcinoma Adrenocortical/tratamento farmacológico , Carcinoma Adrenocortical/genética , Anticorpos Monoclonais Humanizados/uso terapêutico , Antineoplásicos Imunológicos/uso terapêutico , Carcinoma Adrenocortical/secundário , Adulto , Reparo de Erro de Pareamento de DNA/genética , Feminino , Humanos , Proteína 2 Homóloga a MutS/genética , Mutação , Resultado do Tratamento
12.
Pediatr Transplant ; 22(1)2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29131462

RESUMO

LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD. CVD patients had more abnormal EKG (32.4% vs 14.5%, P < .001), abnormal chest X-ray (11.8% vs 1.4%, P < .001), and altered echocardiography (89.7% vs 15.4%, P < .001) findings compared with the No-CVD group pretransplant. Post-transplant, significant differences between groups were observed related to abnormal EKG (14.7% vs 7.0%, P = .03) and echocardiography (48.5% vs 3.2%, P < .01) findings. Pretransplant ASD spontaneously closed in 22 patients. At 1 and 5 years post-transplant, there was no difference in the survival rate between groups (P = .96). The prevalence of CVD in recipients of LT was high, and its presence was associated with significantly higher cardiac decompensation before and after LT. Minor and moderate cardiovascular disease did not impact the long-term survival.


Assuntos
Doenças Cardiovasculares/complicações , Doença Hepática Terminal/cirurgia , Transplante de Fígado , Adolescente , Doenças Cardiovasculares/diagnóstico por imagem , Doenças Cardiovasculares/mortalidade , Criança , Pré-Escolar , Ecocardiografia , Doença Hepática Terminal/complicações , Doença Hepática Terminal/mortalidade , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Transplante de Fígado/mortalidade , Masculino , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do Tratamento
13.
Radiographics ; 37(6): 1612-1631, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29019744

RESUMO

Liver transplant is considered to be the last-resort treatment approach for pediatric patients with end-stage liver disease. Despite the remarkable advance in survival rates, liver transplant remains an intricate surgery with significant morbidity and mortality. Early diagnosis of complications is crucial for patient survival but is challenging given the lack of specificity in clinical presentation. Knowledge of the liver and vascular anatomy of the donor and the recipient or recipients before surgery is also important to avoid complications. In this framework, radiologists play a pivotal role on the multidisciplinary team in both pre- and postoperative scenarios by providing a road map to guide the surgery and by assisting in diagnosis of complications. The most common complications after liver transplant are (a) vascular, including the hepatic artery, portal vein, hepatic veins, and inferior vena cava; (b) biliary; (c) parenchymal; (d) perihepatic; and (e) neoplastic. The authors review surgical techniques, the role of each imaging modality, normal posttransplant imaging features, types of complications after liver transplant, and information required in the radiology report that is critical to patient care. They present an algorithm for an imaging approach for pediatric patients after liver transplant and describe key points that should be included in radiologic reports in the pre- and postoperative settings. Online supplemental material is available for this article. ©RSNA, 2017.


Assuntos
Diagnóstico por Imagem , Transplante de Fígado/métodos , Complicações Pós-Operatórias/diagnóstico por imagem , Criança , Humanos
14.
Pediatr Transplant ; 21(8)2017 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-28881059

RESUMO

CMV infection plays an important role in the postoperative course following solid organ transplantation. We present the case of an 11-year-old male patient who underwent LDLT due to severe hepatopulmonary syndrome and biliary cirrhosis. Four weeks after LDLT, he developed persistent GI bleeding and was subjected to repeated endoscopic treatment and radiological arterial embolization to stop the bleeding from duodenal ulcers. Diagnostic workup was negative for CMV disease. Because the bleeding persisted, surgical treatment was indicated, and a pancreas-preserving duodenectomy was performed. Immunohistochemical staining of the surgical specimen demonstrated diffuse endothelial infiltration by CMV. Despite ganciclovir treatment, the patient developed new erosions in the jejunal mucosa and melena; ganciclovir was discontinued, and foscarnet was started, resulting in clinical improvement and the cessation of bleeding. This case highlights the technical aspects of performing a complex upper GI resection in a patient recently subjected to LDLT, taking care to avoid injury to the previous liver graft anastomosis and restore GI continuity. Moreover, CMV tissue-invasive disease compartmentalized in the GI tract may be difficult to diagnose, as indicated by the negative results of antigenemia and PCR assays and endoscopic superficial mucosal biopsies.


Assuntos
Infecções por Citomegalovirus/cirurgia , Duodenopatias/cirurgia , Duodeno/cirurgia , Transplante de Fígado , Doadores Vivos , Complicações Pós-Operatórias/cirurgia , Criança , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/etiologia , Duodenopatias/diagnóstico , Duodenopatias/etiologia , Humanos , Transplante de Fígado/métodos , Masculino , Pâncreas , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/virologia
15.
Pediatr Transplant ; 21(7)2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28736976

RESUMO

Although rare, ALF caused by disseminated HSV infection is associated with high mortality in the neonatal population. This condition is often diagnosed relatively late due to the absence of specific signs. We present a case involving a neonate with ALF submitted to living donor liver transplantation without a prior diagnosis. The patient had no skin or mucosal lesions, and IgM serology was negative for HSV-1 and HSV-2. Immunohistochemical staining of the liver explant was positive for herpes virus infection, and the patient subsequently received antiviral drug treatment, with a good outcome. Due to organ shortages and the rarity of the aforementioned condition, LT has seldom been reported for the treatment of ALF caused by herpes virus infection; however, LT may be the only option for neonates with fulminant hepatitis. The use of living donors in an urgent scenario is well established in Eastern countries and safely applicable for pediatric patients with ALF.


Assuntos
Hepatite Viral Humana/cirurgia , Herpes Simples/cirurgia , Falência Hepática Aguda/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Feminino , Hepatite Viral Humana/complicações , Herpes Simples/complicações , Humanos , Recém-Nascido , Falência Hepática Aguda/virologia
16.
Transplantation ; 100(9): 1944-54, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27362317

RESUMO

BACKGROUND: Biliary complications (BCs) remain an important cause of morbidity after pediatric liver transplantation. Technical factors have already been implicated in the development of BCs. Previous reports have associated the use of partial grafts, particularly living donor grafts, with a higher incidence of BCs. Our aim is to study the factors associated with the development of BCs in a large cohort of pediatric liver transplant recipients. METHODS: Retrospective cohort study of 670 children (<18 years of age) who underwent a primary liver transplant between March 2000 and January 2015. Patients who did and did not develop BCs were compared with identify associated factors. Univariate and multivariate analyses were performed. RESULTS: A total of 115 patients (17.2%) developed BCs (83 strictures and 44 leaks). Of the study participants, 594 had living donor liver transplants. Multiple arterial anastomoses was a protective factor for BCs, and a ductoplasty was a risk factor. Living donor grafts and multiple biliary anastomoses were more frequently associated with leaks. Patients with BCs had a higher reoperation rate and longer hospital stays. There was no difference in patient or graft survival. CONCLUSIONS: Technical factors play a major role in the development of BCs, particularly leaks. Strictures are more frequently associated with an inadequate arterial supply to the bile duct, and multiple arterial anastomoses may protect children from this complication. The use of partial grafts was not an independent factor for BCs in high-volume centers that are experienced with this technique.


Assuntos
Fístula Anastomótica/etiologia , Colestase/etiologia , Transplante de Fígado/efeitos adversos , Fatores Etários , Fístula Anastomótica/diagnóstico , Fístula Anastomótica/cirurgia , Procedimentos Cirúrgicos do Sistema Biliar/efeitos adversos , Distribuição de Qui-Quadrado , Pré-Escolar , Colestase/diagnóstico , Colestase/cirurgia , Feminino , Humanos , Lactente , Estimativa de Kaplan-Meier , Tempo de Internação , Transplante de Fígado/métodos , Doadores Vivos , Masculino , Análise Multivariada , Razão de Chances , Modelos de Riscos Proporcionais , Reoperação , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/efeitos adversos
17.
Pediatr Transplant ; 20(7): 994-999, 2016 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-27412691

RESUMO

The association between LT and gastrectomy is not common. Only two studies reported the gastrectomy/LT association in children. Here, we report three children who underwent LT who required a concomitant or sequential gastrectomy for different reasons. Patient 1, a 16-yr-old boy, during the LT, underwent a partial gastrectomy due to extensive injury to the duodenum. He had a previous and unusual portoenterostomy performed in the duodenum. Bowel reconstruction was performed using an intestinal loop that was first used for the bilio-enteric anastomosis and then connected to the gastric stump. Patient 2, a 22-month-old female child, underwent a partial gastrectomy with a Roux-en-Y reconstruction during a retransplantation. She had a large perforated gastric ulcer blocked by the allograft liver. Patient 3, a 26-month-old male child, five yr after living donor LT, was submitted to a partial gastrectomy because of gastric outlet obstruction. The histopathology was compatible with eosinophilic gastritis. The association between LT and gastrectomy in the pediatric population is extremely rare. Appropriate knowledge of the previous transplantation technique is very important. Further studies are required to assess the outcomes of the different types of gastric reconstruction in pediatric recipients.


Assuntos
Gastrectomia/métodos , Falência Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Transplante de Fígado/métodos , Adolescente , Anastomose em-Y de Roux/métodos , Anastomose Cirúrgica , Pré-Escolar , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Masculino , Reoperação , Estudos Retrospectivos
18.
Pediatr Transplant ; 20(6): 813-8, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27329427

RESUMO

Re-LT is the only recourse for patients with liver graft failure. However, survival rates after re-LT are lower than those of primary transplants. Few reports are available regarding re-LT with LDs in children. The objective of this study was to describe our cohort of patients retransplanted with LD and emphasize the technical aspects of a re-LT with LD. This is a retrospective report of a series of 18 children (<18 yr old) submitted to 20 re-LT from January 1997 to December 2013 at Hospital Sirio-Libanes and Hospital AC Camargo Cancer Center. The one- and five-yr survival for patients retransplanted with LD was 70.6% and 58.6%. Little technical modifications from a regular LD primary transplant were needed in patients retransplanted with LD. Seven (38.8%) patients presented vascular complications following re-LT and three presented biliary complications (16.6%). In conclusion, a re-LT with LD is an acceptable alternative for children who experience liver graft failure and it does not compromise the donor pool. Further experience with re-LT with LD may support this therapy.


Assuntos
Transplante de Fígado , Doadores Vivos , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Transplante de Fígado/métodos , Transplante de Fígado/mortalidade , Masculino , Avaliação de Resultados em Cuidados de Saúde , Reoperação , Estudos Retrospectivos
19.
Pediatr Transplant ; 20(5): 717-722, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27174167

RESUMO

Many publications discuss the various strategies for vascular reconstruction (VR) in pediatric LDLT. Having knowledge of alternative techniques is helpful in planning transplants. This article presents three case reports that illustrate some of the alternative techniques for HV, PV, and HA reconstruction in pediatric LDLT. It also reviews the available alternative strategies reported for VR in pediatric LDLT. In the first case, a 13-month-old girl presented a PRETEXT III HB with invasion of the retrohepatic vena cava. An LLS graft HV was anastomosed to a DD iliac vein graft and subsequently implanted in a "standard" fashion in the recipient. In the second case, a 44-month-old boy presented with multifocal HB and portomesenteric thrombosis and the portal inflow was done through a renoportal anastomosis. In the third case, a 22-month-old child with a failed Kasai procedure had extensive HA thrombosis. The HA reconstruction was performed with an interposition of the recipient's IMV graft. The use of alternative techniques for VR in pediatric LDLT is paramount to the success of such a complex procedure. Imaging studies can help transplant surgeons outline surgical strategies and define the best technique to be used in each case.

20.
Transplantation ; 100(5): 1066-72, 2016 05.
Artigo em Inglês | MEDLINE | ID: mdl-27014791

RESUMO

BACKGROUND: Hepatic artery thrombosis (HAT) increases morbidity and mortality after liver transplantation (LT). The identification of risk factors for HAT may aid transplant teams in the development of strategies aimed at reducing HAT. This article describes the risk factors for HAT and outcomes after LT. METHODS: This report describes a retrospective study (1995 to 2015) of primary pediatric living donor LT (LDLT). Pretransplant and technical variables were included in the study. Binary logistic regression was used for data analysis. RESULTS: This study included 656 primary LDLT. The median age, body weight, and pediatric end-stage liver disease score at the time of transplant were 13 months, 8.4 kg and 15, respectively. Twenty-one (3.2%) patients developed HAT. Intraoperative HAT (odds ratio, 62.63; 95% confidence interval, 12.64-310.19; P < 0.001) and the use of liver grafts with a graft-to-recipient weight ratio less than 1.1% (odds ratio, 24.46; 95% confidence interval, 4.55-131.56; P < 0.001) retained statistical significance in the multivariate model. Patient and graft survivals were significantly worse in cases with HAT. The overtime trend analysis revealed a decrease in the incidence of HAT (P = 0.008) and an increase in the use of 2-arterial anastomosis (P < 0.001). CONCLUSIONS: A graft-to-recipient weight ratio of 1.1% or less and intraoperative HAT were independently associated with HAT. Trend analysis further revealed a significant reduction in the incidence of HAT over time, as well as the increased use of 2 hepatic arteries for anastomosis during graft implantation. The double artery anastomosis may represent an extra protection to pediatric recipients undergoing LDLT.


Assuntos
Artéria Hepática/cirurgia , Transplante de Fígado/métodos , Doadores Vivos , Trombose/patologia , Adolescente , Adulto , Anastomose Cirúrgica , Anticoagulantes/química , Peso Corporal , Criança , Pré-Escolar , Seleção do Doador , Feminino , Sobrevivência de Enxerto , Humanos , Lactente , Estimativa de Kaplan-Meier , Fígado/cirurgia , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Razão de Chances , Análise de Regressão , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos , Adulto Jovem
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