Abstract Mitochondrial Myopathy is a rare pathology caused by a defect in the mitochondrial DNA metabolism, leading to defects in the formation of adenosine triphosphate, in the Krebs citric acid cycle, fatty acid oxidation and oxidative phosphorylation. It is manifested by exercise intolerance, muscle fatigue on small efforts, muscle weakness, tachycardia, and difficulty breathing. There are few case reports on the operative management of adult patients suffering from mitochondrial myopathy. With this report, we intend to describe the anesthetic management of a patient with mitochondrial myopathy who underwent laparoscopic gastroplasty and outline some anesthetic considerations about this pathology.

RESUMO Racional: Trombose de artéria hepática é importante causa de falência de enxerto e complicações biliares. Fatores de risco para trombose estão relacionados aos aspectos técnicos da anastomose arterial e fatores não cirúrgicos. Objetivo: Avaliar os fatores de risco para o desenvolvimento de trombose de artéria hepática. Métodos: A amostra consta de 1050 casos de transplante hepático. Foi realizado estudo retrospectivo e transversal, e as variáveis foram avaliadas em doadores e receptores. Resultados: A análise univariada mostrou que as variáveis relacionadas a trombose de artéria hepática são: MELD e tempo de isquemia quente. Na análise multivariada, o MELD=14.5 e tempo de isquemia quente =35 min foram fatores de risco independentes para trombose de artéria hepática. No teste de prevalência para avaliação do tipo de anastomose como variável, foi observado que a sutura contínua tem maior risco de trombose quando comparada com aquela em pontos separados. Conclusão: Tempo de isquemia quente prolongado, MELD calculado e idade do recipiente foram fatores de risco independentes para trombose de artéria hepática após transplante de fígado em adultos. Pacientes submetidos à anastomose com sutura contínua apresentaram mais trombose quando comparados com a em pontos separados. Retransplante por trombose está associado com maior mortalidade.

ABSTRACT Background: Hepatic artery thrombosis is an important cause of graft loss and ischemic biliary complications. The risk factors have been related to technical aspects of arterial anastomosis and non-surgical ones. Aim: To evaluate the risk factors for the development of hepatic artery thrombosis. Methods: The sample consisted of 1050 cases of liver transplant. A retrospective and cross-sectional study was carried out, and the variables studied in both donor and recipient. Results: Univariate analysis indicated that the variables related to hepatic artery thrombosis are: MELD (p=0.04) and warm time ischemia (p=0.005). In the multivariate analysis MELD=14.5 and warm ischemia time =35 min were independent risk factors for hepatic artery thrombosis. In the prevalence ratio test for analysis of the anastomosis as a variable, it was observed that patients with continuous suture had an increase in thrombosis when compared to interrupted suture. Conclusions: Prolonged warm ischemia time, calculated MELD and recipient age were independent risk factors for hepatic artery thrombosis after liver transplantation in adults. Transplanted patients with continuous suture had an increase in thrombosis when compared to interrupted suture. Re-transplantation due to hepatic artery thrombosis was associated with higher recipient mortality.

Resumo A pentalogia de Cantrell é uma anomalia congênita associada a defeitos na formação da parede abdominal, do esterno, diafragma e pericárdio diafragmático, além do desenvolvimento de anomalias cardíacas. É uma doença rara, com incidência estimada em um caso para cada 65.000 nascimentos, mais comum no sexo masculino (60% dos casos). Apresenta prognóstico reservado com mortalidade em torno de 63% e sobrevida após procedimento cirúrgico de no máximo nove meses. São escassos os relatos de casos referentes à pentalogia de Cantrell, o que se justifica pela raridade dessa patologia. Com este relato, os autores objetivam descrever um caso cirúrgico, em paciente do sexo feminino, e tecer algumas considerações anestésicas sobre essa malformação congênita rara.

Abstract Pentalogy of Cantrell is a congenital anomaly associated with defects in the abdominal wall, sternum, diaphragm, and diaphragmatic pericardium formation, in addition to the development of cardiac abnormalities. It is a rare disease with an estimated incidence of one case for every 65,000 births, being more common in males (60% of cases). It has a reserved prognosis with mortality around 63%, and a maximum of 9 months survival after surgery. There are few case reports addressing the pentalogy of Cantrell, which is justified by the rarity of this pathology. In this report our objective was to describe a surgical case of a female patient and make some anesthetic considerations about this rare congenital malformation.