ABSTRACT Littoral cell angioma is an extremely rare splenic vascular tumor originating from the cells lining the splenic red pulp sinuses. Approximately 150 cases of littoral cell angioma have been reported since 1991. Its clinical manifestation is usually asymptomatic and is mostly diagnosed as an incidental finding through abdominal imaging. Herein, we present a case of littoral cell angioma in a 41-year-old woman with no previous comorbidities, which initially presented as a nonspecific splenic lesion diagnosed on imaging in the emergency room. The patient was treated through laparoscopic intervention. sinuses 15 1991 Herein 41yearold yearold 41 year old comorbidities room intervention 1 199 4 19

ABSTRACT BACKGROUND: The prevalence of primary sclerosing cholangitis (PSC) in the general population has not yet been clearly established. The management of PSC should focus on delaying the progression of the disease and restraining its complications. The only curative therapy for the disease remains liver transplantation (LT). PSC is currently the fifth most common indication for LT and corresponds to 5% of all LT indications in adults. AIMS: Our objective is to evaluate the indications and outcomes of PSC patients undergoing LT in three liver transplantation centers in southern Brazil – Hospital Santa Isabel in Blumenau, Santa Catarina state, and Hospital das Clínicas and Hospital Nossa Senhora das Graças, in Curitiba, Parana state). METHODS: This is a longitudinal observational study of patients with PSC who underwent LT in three major Brazilian medical centers. Electronic medical records and study protocols of all patients subjected to LT from January 2011 to December 2021 were retrospectively reviewed. RESULTS: Of the 1,362 transplants performed in the three medical centers, 37 were due to PSC. Recurrence of PSC occurred in three patients (8.1%) in 3.0±2.4 years (range, 1–4 years). The 1-year and 5-year survival rates after the first LT were 83.8 and 80.6%, respectively. The 1-year and 5-year graft survival rates were, respectively, 83.8 and 74.8%. CONCLUSIONS: Our experience with LT in patients with PSC demonstrated good patient and graft survival results. Most deaths were due to common factors in patients undergoing LT. BACKGROUND (PSC established complications . (LT) 5 adults AIMS Blumenau state Graças Curitiba state. state) METHODS 201 202 reviewed RESULTS 1362 1 362 1,36 3 8.1% 81 8 (8.1% 3024 0 2 4 3.0±2. range, range (range 14 1– years. years) 1year year 5year 838 83 83. 806 80 6 80.6% respectively 748 74 74.8% CONCLUSIONS results (LT 20 136 36 1,3 8.1 (8.1 302 3.0±2 80.6 7 74.8 13 1, 8. (8. 30 3.0± 80. 74. (8 3.0 ( 3.

RESUMO RACIONAL: A prevalência de colangite esclerosante primária (CEP) na população em geral ainda não foi claramente estabelecida. O manejo da CEP deve se concentrar em retardar a progressão da doença e gerenciar suas complicações. A única terapia curativa para a doença continua sendo o transplante hepático (TH). A CEP é atualmente a 5ª indicação mais comum de TH e corresponde a 5% de todas as indicações de TH em adultos OBJETIVOS: O nosso objetivo é avaliar as indicações e os resultados do transplante de fígado em pacientes com CEP submetidos a TH em três centros de transplante de fígado no sul do Brasil (Hospital Santa Isabel, Blumenau, Santa Catarina; Hospital das Clínicas, Curitiba, Paraná; e Hospital Nossa Senhora das Graças, Curitiba, Paraná). MÉTODOS: Este é um estudo observacional longitudinal de pacientes com colangite esclerosante primária que foram submetidos a transplante hepático em três grandes centros médicos brasileiros. Os prontuários eletrônicos de todos os pacientes submetidos a TH de Janeiro de 2011 a Dezembro de 2021, foram avaliados retrospectivamente. RESULTADOS: De um total de 1.362 transplantes realizados nos três centros médicos, 37 eram devidos à CEP. A recorrência de CEP ocorreu em 3 pacientes (8,1%) em 3±2,4 anos (intervalo, 1–4 anos). A taxa de sobrevida de 1 ano e 5 anos após o 1° TH foi respectivamente de 83,8 e 80,6%. As taxas de sobrevida dos enxertos em 1 ano e 5 anos foram, respectivamente, 83,8 e 74,8%. CONCLUSÕES: A nossa experiência com TH em pacientes com CEP demonstrou bons resultados de sobrevida dos pacientes e dos enxertos. A maioria dos óbitos ocorreu devido a fatores comuns em pacientes submetidos a TH. RACIONAL (CEP estabelecida complicações . (TH) ª OBJETIVOS Isabel Blumenau Catarina Clínicas Curitiba Paraná Graças Paraná. Paraná) MÉTODOS brasileiros 201 2021 retrospectivamente RESULTADOS 1362 362 1.36 8,1% 81 8 (8,1% 324 2 4 3±2, intervalo, intervalo (intervalo 14 1– anos. anos) 838 83 83, 806 80 6 80,6% 748 74 74,8% CONCLUSÕES (TH 20 202 136 36 1.3 8,1 (8,1 32 3±2 80,6 7 74,8 13 1. 8, (8, 3± 80, 74, (8 (

RESUMO Objetivo Analisar a influência da ligadura da tributária da veia porta no estímulo regenerativo hepático por meio de critérios imuno-histoquímicos. Métodos Dez suínos, divididos em dois grupos de cinco animais, foram submetidos à hepatectomia em dois estágios, sendo que os grupos foram diferenciados pela ligadura ou não da tributária da veia porta, responsável pela vascularização dos lobos lateral e medial esquerdos do fígado do suíno. Cinco dias após o procedimento, os animais foram reabordados para retirada de amostras hepáticas para posterior análise de histológica e imunoistoquímica com o marcador Ki67. Resultados O grupo submetido à hepatectomia com ligadura vascular apresentou incremento de 4% aproximadamente de hepatócitos em processo de regeneração, bem como grande número de células de Kupffer e células inflamatórias, quando comparado ao controle. Conclusão Em virtude da análise positiva da replicação celular observada por meio do marcador Ki67, pode-se observar que a ligadura de uma tributária da veia porta promoveu um maior estímulo de regeneração hepática, efeito observado com menor intensidade no grupo submetido apenas à ressecção hepática.

ABSTRACT Objective To analyze the influence of portal vein ligation in hepatic regeneration by immunohistochemical criteria. Methods Ten pigs divided into two groups of five animals underwent hepatectomy in two stages, and the groups were differentiated by ligation or not of the left portal vein tributary, which is responsible for vascularization of the left lateral and medial lobes of the pig liver. Five days after the procedure, the animals underwent liver biopsies for further analysis of histological and immunohistochemical with marker Ki67. Results The group submitted to hepatectomy with vascular ligation showed an increase of approximately 4% of hepatocytes in regeneration status, as well as a greater presence of Kupffer and inflammatory cells as compared to control. Conclusion As a result of positive cell replication observed through the Ki67 marker, we can suspect that the ligation of a tributary of the portal vein associated with liver resection promoted a greater stimulus of liver regeneration when compared to liver resection alone.

Hiperintesidades simétricas e bilaterais dos gânglios da base são observadas em imagens de ressonância magnética encefálica (RM) ponderadas em T1 na maioria dos pacientes com insuficiência hepática crônica devidas ao acúmulo de manganês. Nós relatamos o caso de um homem, com 53 anos de idade, com um complexo parkinsonismo-demência rapidamente progressivo associado com o acúmulo de manganês no cérebro, secundariamente a insuficiência hepática. Uma RM encefálica foi realizada e foram observadas imagens hiperintensas/hipersinal nas imagens ponderadas em T1 no globo pálido e, também, na substância branca dos hemisférios cerebrais ponderadas em T2. Devido à falta de resposta ao tratamento clinico optamos pelo transplante hepático. O paciente faleceu no 7º dia de PO. Nossos achados corroboram o conceito dos efeitos tóxicos do manganês nos gânglios da base/globo pálido. O tratamento desta forma de parkinsonismo é controverso e o transplante hepático não deverá ser considerada uma opção terapêutica de primeira linha, porém como um tratamento alternativo considerando-se os riscos-benefícios dessa escolha.

Bilateral and symmetric globus-pallidus hyperintensities are observed on T1-weighted MRI in most of the patients with chronic liver failure, due to manganese accumulation. We report a 53-year-old man, with rapid onset parkinsonism-dementia complex associated with accumulation of manganese in the brain, secondary to liver failure. A brain MRI was performed and a high signal on T1-weighted images was seen on globus-pallidus, as well as on T2-weighted images on the hemispheric white-matter. He was referred to a liver-transplantation. The patient passed away on the seventh postoperative day. Our findings support the concept of the toxic effects of manganese on the globus-pallidus. The treatment of this form of parkinsonism is controversial and liver-transplantation should not be considered as first line treatment but as an alternative one.

Hepatocellular carcinoma is one of the most frequent entities worldwide and partial hepatectomy has been the preferred option for treatment. We report the case of a 28-year-old Jehovah's Witness who came to us complaining about an abdominal mass. Angio-MRI showed a large heterogeneous mass in the left hepatic lobe. Ex situ liver resection followed by right hepatic lobe autotransplantation was performed, using extra-corporeal venous-venous bypass. He had an uneventful postoperative outcome and was discharged on the 10th postoperative day. The histology revealed hepatocellular carcinoma.

Adenosquamous carcinoma of the liver is a rare type of hepatic tumor first described in 1975. It is characterized by both glandular and squamous compounds. We describe a case of adenosquamous carcinoma of the liver. It was discovered in a 42-yr-old female with a 2-month history of vomiting, weight loss (7 kg). A left hepatic lobectomy disclosed an 11 x 9 x 7,5 cm tumor located at I, II, III, IV segments. This tumor is rare entity and is a variant of cholangiocarcinoma whose etiology and prognostic are not well known.

O objetivo do presente estudo é avaliar os efeitos do transplante hepático bem sucedido nas alterações dos ciclos menstruais e da fertilidade observadas em mulheres com hepatopatia crônica. Doze mulheres com idade de 17 a 54 anos submetidas a transplante hepático foram avaliadas. As seguintes variáveis foram analisadas: idade, etiologia da hepatopatia, padrão da menstruação, período de amenorréia, antes e após o transplante, e ocorrência de gravidez após o transplante. A idade média das pacientes foi de 36 ± 12,6 anos. Pacientes com cirrose biliar primária não apresentavam amenorréia antes do transplante. Os demais pacientes apresentavam amenorréia por 3 meses a 11 anos antes do transplante. Recuperação rápida da menstruação ocorreu em todos pacientes após o transplante (3,1 ± 1,2 meses). Duas pacientes engravidaram um e três anos após o transplante. Conclui-se deste estudo que a maioria das mulheres que apresenta amenorréia secundária à doença hepática avançada, volta a ter ciclos menstruais normais aproximadamente três meses após o transplante hepático e podem engravidar.

The objective of the present study is to evaluate the effects of successful liver transplantation on menstrual cycles abnormalities and on reproductive function of women with chronic liver disease. Twelve women with age between 17 and 54 years who underwent liver transplantation were evaluated. The following variables were analyzed: age, etiology of chronic liver disease, pattern of menstrual function and period of amenorrhea before and after transplantation, and occurrence of pregnancy after transplantation. The mean age of patients was 36 ± 12.6 years. Patients with primary biliary cirrhosis did not have menstrual abnormalities before transplantation. The other patients presented amenorrhea for 3 months to 11 years before the transplantation. Rapid recovery of menstrual function was observed in all patients after the transplantation (3.1 ± 1.2 months). Two patients became pregnant one and three years after the transplantation. It is concluded from this study that most women who present amenorrhea secondary to chronic liver disease have normal menstrual cycles in approximately three months following liver transplantation and they may become pregnant.

O objetivo do presente estudo é apresentar as complicações que ocorreram em seiscentos pacientes consecutivos com doença do refluxo gastroesofágico submetidos à fundoplicatura laparoscópica. O procedimento de Nissen-Rosetti (fundoplicatura de 360°) foi realizado em 587 pacientes (97,8%) e o de Toupet (fundoplicatura de 270°) em 13 (2,2%). Oitenta e um pacientes também foram submetidos à colecistectomia no mesmo ato operatório, e um a diverticulectomia faringoesofágica cervical com miotomia cricofaringeana. Trinta e nove pacientes tinham operação prévia no abdome superior. O período de internação hospitalar variou de 12 horas a 23 dias, com média de 1,2 dias. A via de acesso foi convertida em laparotomia em dez pacientes (1,7%). A principal causa de conversão foi a presença de aderências. A complicação intra- operatória mais freqüente foi pneumotórax, que foi observado em oito pacientes. Todos os pneumotóraces ocorreram nos cem primeiros casos. Cinco pacientes apresentaram hemorragia significante, sendo que dois deles necessitaram laparotomia para controle do sangramento. Úlcera gástrica foi diagnosticada em sete pacientes. Um paciente etilista morreu de pancreatite aguda e outro de síndrome de disfunção de múltiplos órgãos e sistemas conseqüente à perfuração gástrica. Outras complicações importantes foram: dois abscessos intra-abdominais, uma perfuração esofágica, uma sepse secundária à perfuração gástrica, um choque hemorrágico e uma obstrução gástrica secundária à herniação da fundoplicatura. Concluímos que a taxa de complicações da fundoplicatura laparoscópica é baixa e diminui significativamente com a experiência do cirurgião.

The objective of the present study is to present the complications that occurred in 600 consecutive patients with gastroesophageal reflux disease who underwent laparoscopic fundoplication. A Nissen-Rosetti procedure (360° fundoplication) was performed in 587 patients (97.8%) and a Toupet procedure (270(0) fundoplication) in 13 (2.2%). Eighty- one patients were also subjected to a concurrent cholecystectomy and one patient a concurrent cervical pharyngoesophageal diverticulectomy with cricopharyngeal myotomy. Thirty-nine patients had a previous upper abdominal operation. The period of hospitalization varied from 12 hours to 23 days, with an average of 1.2 days. The operation was converted to an open procedure in 10 patients (1,7%). The main cause of conversion was the presence of adhesions. The most frequent intraoperative complication was pneumothorax, that was observed in eight patients. All pneumothoraces occurred in the first 100 cases. Five patients had significant operative bleeding; two of them required laparotomy for bleeding control. Gastric ulcer was diagnosed in seven patients. One alcoholic patient died of acute pancreatitis and other with gastric perforation of multiple organ failure syndrome. Other major complications were two intrabdominal abscesses, one esophageal perforation, one sepsis due to gastric perforation, one hemorrhagic shock, and one gastric obstruction due to fundoplication herniation. It is concluded that complications of laparoscopic fundoplication are low and decrease significantly with the surgeon's experience.

The objective of the present study is to report a sucessful pregnancy in a 28 year-old female who underwent an orthotopic liver transplantation two years ago due to type I auto immune he patitis. Patient was taking cyclosporin, prednisone and azathioprine for maintenance immunossupression and had good graft function during all the pregnancy. The liver function tests were normal during the pregnancy. Fetal growth was monitored by ultrasonographic examination and showed normal development. At the end of the third trimester, she was subjected to a cesarean bearing a healthy girl weighting 2,320 kg. She was discharged with normal liver functions tests and no complications.

The aim of this paper is to present a case of a 37-year-old female patient with a benign tumor of the Ampulla of Vater and a brief review of the literature. The patient presented with progressive obstructive jaundice and weigth loss due to the presence of two adenomas of the second portion of duodenum. Laboratory tests confirmed the presence of obstruction of the biliary tree. Ultrasound and CT scan of the abdomen revealed bile duct dilatation. ERCP showed a tumor at the site of the Ampulla of Vater. The biopsies revealed tubular adenoma. She was submitted to local resection of the tumors and sphincteroplasty, since the frozen biopsy at the time of surgery showed no malignancy. During the post-operative follow-up she presented recurrence of symptoms. An upper GI endoscopy revealed a tumor at the Ampulla of Vater. She was then submitted to Whipple procedure with an uneventful recovery.