Liver transplantation in patients with primary sclerosing cholangitis: a multicentric study / Transplante hepático em pacientes com colangite esclerosante primária: estudo multicêntrico

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.

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.

Management of pneumatosis cystoides intestinalis with pneumoperitoneum: 5-years systematic review

Introduction: Pneumatosis cystoides intestinalis (PCI) is a rare condition characterized by gas-filled cysts in the intestinal wall. Although rare, it may also involve other regions, such as the mesentery. PCI is classified as primary or secondary and is associated with multiple predisposing factors. It may be associated with either a benign condition or a potentially fatal condition, such as mesenteric ischemia. The objective of this study was to review the medical literature on the rare benign presentations of PCI, excluding cases associated with intestinal ischemia.Methods: We conducted a systematic literature review according to the PRISMA statement. We searched PubMed and LILACS databases for articles published between January 2015 and December 2020 using the following Medical Subject Headings: "pneumatosis cystoides intestinalis" and "pneumoperitoneum," "pneumatosis intestinalis," and "pneumoperitoneum" or "mesenteric pneumatosis."Results: We included 51 articles comprising 58 patients with PCI and pneumoperitoneum. Most patients were men, and mean patient age was 64.9 years. We identified an idiopathic etiology in 29.31% of cases, and the most common predisposing factor was immune dysfunction (29.31%). A total of 24.13% of patients were asymptomatic. The most commons symptoms were abdominal pain (43.10%), nausea and vomiting (41.37%), and abdominal distention (37.93%). Diagnostic surgery was conducted in 26 patients (44.82%). Only 1 patient underwent surgical treatment.Conclusions: PCI is a clinical condition that may have a benign etiology and not require surgery. Treatment of the benign etiology is conservative. Thus, life-threatening conditions should be excluded in all cases.

Retroperitoneal fetus in fetu in a 65-year-old man

Fetus in fetu (FIF) is a rare congenital anomaly in which a malformed fetus is incorporated within the body of its twin. It was first described in the late 18th century and has an incidence of 1:500,000 live births. In most cases, the diagnosis is made in infants or young adults. To date, the oldest patient reported in the literature was 47 years old. We describe the case of a 65-year-old patient with FIF, now the oldest reported in the literature. Our patient meets all the diagnostic criteria for FIF, including the presence of a limb in advanced formation inside the lesion. The treatment was surgical excision. FIF should be considered in the differential diagnosis of abdominal masses, typically recognized in infancy. Symptoms arise from mass effects. Surgical resection should be performed due to the potential for malignant transformation.