LIVER TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS: A MULTICENTRIC STUDY.

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.

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.

Outcomes of Emergency Groin Hernia Repair in the Elderly: A Systematic Review.

Introduction: The number of surgeries for groin hernia (GH) among the elderly follows the increase in life expectancy of the population. The greater number and severity of comorbidities in this group increases the surgical risk, promoting discussion regarding the indication of elective surgery and the benefits of watchful waiting approach (WWA). The aim of the present study was to evaluate the outcomes of emergency hernia surgery among the elderly population. Materials and methods: A systematic review was performed in Pubmed and Scielo databases for the past early 10 years, until July 2022. The subject was groin hernia in the emergency setting focusing the elderly population. The PRISMA statement was followed and the classification of elderly was based on the World Health Organization's definition. Results: A total of 1,037 results were returned and we ended with nine original articles with emphasis in groin hernia in the emergency among the elderly population. In these subjects, the complications rate ranged between 21.2% and 28.9% and the mortality rate ranged between 1.2% and 6%. Cardiopulmonary disease, high ASA and Charlson's scales were associated with greater risk of complications and death. Conclusion: Emergency GH surgery in the elderly population carries an increased risk of complications and mortality. GH surgery is safe or, at least, less harmful when done electively. The risk and benefits of WWA and upfront surgery needs to be assessed and exposed to the patients. Our review sugest that elective surgery should be the option over WWA in this patient population.

Gallbladder agenesis a rare and underdiagnosed congenital anomaly: a case report and literature review.

Gallbladder agenesis (GA) is a rare congenital anomaly with conflicting epidemiology described in the literature. When present, it is misinterpreted as cholelitiasis, a highly prevalent condition. Nevertheless, surgeons and radiologists must be aware of it since it can lead to unnecessary invasive procedures. Diagnosis of GA is challenging due to the anatomical structures that sometimes resemble a shrunken gallbladder. We report the case of a 55-year-old man with preoperative diagnosis of cholelitiasis and further intraoperative find of GA. Since cholecystectomy is one of the most common surgeries worldwide, it demonstrates how relevant this case is to emphasize the need to recognize this diagnosis and be aware of its management to avoid unnecessary surgery.

Gastric adenocarcinoma in the excluded stomach 18 years after bariatric surgery: a case report.

Gastric cancer (GC) ranks fourth in overall cancer mortality. Bariatric surgical procedures, especially the gastric bypass surgery (GBS), raise a concern about the risk of GC in the excluded stomach (ES). Diagnosis of GC in the ES is challenging due to anatomical changes and impossibility of endoscopic access to the ES. There are few reports of GC after GBS, and it occurs more in the gastric stump than in the ES. We report a case of a 54-year-old female with GC in the ES 18 years after GBS. The increasing number of GBS and the aggressiveness of the GC show how relevant this case is to emphasize the need to consider this diagnosis in patients who develop upper abdominal symptoms, anemia or weight loss.

Mesenteric venous thrombosis in a pregnant woman at first trimester gestation: a case report.

Mesenteric vein thrombosis (MVT) in a pregnant patient is a rare condition that seems to be associated with the pregnancy pró-thrombotic state. This can lead to severe circumstances such as intestinal hemorrhagic ischemia, sepsis, abortion and death. Abdominal assessment is challenging due to the anatomical and physiological changes during pregnancy. MVT clinical and complementary evaluation are nonspecific, making essential an image exam. We report a case of a 33-years-old woman at 11 weeks of gestation. She sought medical evaluation due to abdominal pain and had an appendicitis diagnosis, which was treated by laparoscopic surgery. One week later, she came back complaining of nonspecific abdominal pain. So an extensive evaluation was made, and the diagnosis of MVT and intestinal ischemia was concluded. She underwent laparotomy exploration and anticoagulation, having a good evolution and so was discharged on the sixth post-operative day.

Pneumatosis cystoides intestinalis with pneumoperitoneum in an 87-years-old male patient: a case report.

Pneumatosis cystoides intestinalis (PCI) is a rare condition, characterized by gas-filled cysts in the intestinal wall. The mesentery and intra-abdominal ligaments can be affected. PCI is classified as primary or secondary and associated with multiple predisposing factors. An asymptomatic 87-year-old man underwent an abdominal tomography for follow-up of bladder carcinoma. The examination revealed intestinal and mesenteric pneumatosis associated with pneumoperitoneum. At laparoscopy, intestinal and mesenteric pneumatosis without intestinal infarction was identified. He was discharged on the fifth postoperative day. PCI is a benign condition that can be confused with mesenteric ischemia. Treatment is conservative, with periodic clinical evaluations. Surgical procedure is unnecessary for its diagnosis or management.

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.

Biliary tract melanoma metastasis mimicking hilar cholangiocarcinoma: a case report.

Malignant melanoma is the 19th leading cause of cancer worldwide. It is an aggressive neoplastic disease in which pathophysiological understanding and management has been in constant evolution in recent decades. The primary site is the skin, uvea and mucous membranes and has the capacity to metastasize to any organ. There are few reports of primary or secondary involvement of the biliary tract. We present the case of a 73-year-old woman with a bile duct lesion suggestive of cholangiocarcinoma and a final diagnosis of a single melanoma metastasis. Surgical treatment was performed due to oligometastatic stage IV melanoma with possibility of R0 resection followed by immune checkpoint therapy.