Mucinous Cystadenoma Arising in a Uterine Isthmocele: A Case Report.

Isthmocele is a discontinuation of the myometrium at the uterine scar site in a patient with a previous cesarian section (CS). The cause of isthmocele appears to be multifactorial. Poor surgical technique, low incision location, uterine retroflection, obesity, smoking, inadequate healing of scars, and maternal age are possible related factors. Most patients with this condition are asymptomatic. However, women can present with postmenstrual bleeding, pelvic pain, subfertility, dysmenorrhea, infertility, and scar abscess. Brazil has one of the world's highest cesarean section rates. One of the consequences of the rising rate of CS is the isthmocele, an emerging female health problem. Here we report a case of mucinous cystadenoma arising in a uterine isthmocele, a complication, as far as we could investigate, not yet described in the literature.

Istmocele é a descontinuidade do miométrio no local da cicatriz uterina em paciente com cesariana anterior. A causa da istmocele parece ser multifatorial. Má técnica cirúrgica, baixa localização da incisão, retroflexão uterina, obesidade, tabagismo, cicatrização inadequada de cicatrizes e idade materna são possíveis fatores relacionados. A maioria dos pacientes com esta condição é assintomática. No entanto, as mulheres podem apresentar sangramento pós-menstrual, dor pélvica, subfertilidade, dismenorreia, infertilidade e abscesso cicatricial. O Brasil tem uma das maiores taxas de cesariana do mundo. Uma das consequências da taxa crescente de cesarianas é a istmocele, um problema emergente de saúde feminina. Aqui relatamos um caso de cistoadenoma mucinoso originado em uma istmocele uterina, uma complicação ainda não descrita, até onde pudemos investigar.

Ten years of experience with endometrial cancer treatment in a single Brazilian institution: Patient characteristics and outcomes.

Few reports have described the clinical and prognostic characteristics of endometrial cancer, which is increasing worldwide, in large patient series in Brazil. Our objective was to analyze the clinicopathological characteristics, prognostic factors, and outcomes of patients with endometrial cancer treated and followed at a tertiary Brazilian institution over a 10-year period.This retrospective study included 703 patients diagnosed with endometrial cancer who were treated at a public academic tertiary hospital between 2008 and 2018. The following parameters were analyzed: age at diagnosis, race, body mass index, serum CA125 level before treatment; histological type and grade, and surgical stage. Outcomes were reported relative to histological type, surgical staging, serum CA125, lymph-vascular space involvement (LVSI), and lymph-node metastasis. The median patient age at diagnosis was 63 (range, 27-93) years (6.4% were <50 years). Minimally invasive surgeries were performed in 523 patients (74.4%). Regarding histological grade, 468 patients (66.5%) had low-grade endometrioid histology and 449 patients (63.9%) had stage I tumors. Tumors exceeded 2.0 cm in 601 patients (85.5%). Lymphadenectomy was performed in 551 cases (78.4%). LVSI was present in 208 of the patients' tumors (29.5%). Ninety-three patients (13.2%) had recurrent tumors and 97 (13.7%) died from their malignant disease. The robust prognostic value of FIGO stage and lymph node status were confirmed. Other important survival predictors were histological grade and LVSI [overall survival: hazard ratio (HR) = 3.75, p < 0.001 and HR = 2.01, p = 0.001; recurrence: HR = 2.49, p = 0.004 and HR = 3.22, p = 0.001, respectively). Disease-free (p = 0.087) and overall survival (p = 0.368) did not differ significantly between patients with stage II and III disease. These results indicate that prognostic role of cervical involvement should be explored further. This study reports the characteristics and outcomes of endometrial cancer in a large population from a single institution, with systematic surgical staging, a predominance of minimally invasive procedures, and well-documented outcomes. Prognostic factors in the present study population were generally similar to those in other countries, though our patients' tumors were larger than in studies elsewhere due to later diagnosis. Our unexpected finding of similar prognoses of stage II and III patients raises questions about the prognostic value of cervical involvement and possible differences between carcinomas originating in the lower uterine segment versus those originating in the body and fundus. The present findings can be used to guide public policies aimed at improving the diagnosis and treatment of endometrial cancer in Brazil and other similar countries.

Vaginal cuff brachytherapy in the adjuvant setting for patients with high-risk early-stage cervical cancer.

PURPOSE: To evaluate local control and survival of high-risk patients with early-stage cervical cancer submitted or not to vaginal cuff brachytherapy in the postoperative setting. METHODS AND MATERIALS: In this retrospective cohort of patients treated from 2010 to 2017, patients were eligible if they had confirmed histological diagnosis of cervical cancer treated with surgery and adjuvant radiotherapy with or without chemotherapy. Vaginal cuff brachytherapy (VCB) was indicated according to the radiation oncologist discretion. RESULTS: Seventy-nine patients were selected, with a median age at diagnosis of 47.5 years (26-77). Brachytherapy was delivered to 59 patients (74.7%). There were no significant differences between the VCB and the no-VCB groups. A total of 13 (16.5%) patients presented one or more events, 5 (25%) and 8 (13.5%) events in the no-VCB and VCB group, respectively. Most recurrences were pelvic and/or vaginal: 7/20 (35%) in the no-VCB group and 9/59 (10.2%) in the VCB group. There were eight systemic relapses with eight deaths. With a median followup of 45 months, mean overall survival and disease-free survival were, respectively, 85.1 and 83.8 months. No variables were correlated with overall survival. The only factor positively correlated to disease-free survival was VCB, with a mean of 86.9 and 68.4 months for patients who did and did not receive brachytherapy, respectively (p = 0.043). Vaginal recurrence was lower in the brachytherapy group, but with no statistical significance (p = 0.065). CONCLUSION: VCB was associated with a reduced recurrence rate in the postoperative setting of high-risk patients with early-stage cervical cancer.

Mucinous Cystadenoma Arising in a Uterine Isthmocele: A Case Report / Cistadenoma mucinoso surgindo em istmocele uterina: relato de caso

Abstract Isthmocele is a discontinuation of the myometrium at the uterine scar site in a patient with a previous cesarian section (CS). The cause of isthmocele appears to be multifactorial. Poor surgical technique, low incision location, uterine retroflection, obesity, smoking, inadequate healing of scars, and maternal age are possible related factors. Most patients with this condition are asymptomatic. However, women can present with postmenstrual bleeding, pelvic pain, subfertility, dysmenorrhea, infertility, and scar abscess. Brazil has one of the world s highest cesarean section rates. One of the consequences of the rising rate of CS is the isthmocele, an emerging female health problem. Here we report a case of mucinous cystadenoma arising in a uterine isthmocele, a complication, as far as we could investigate, not yet described in the literature.

Resumo Istmocele é a descontinuidade do miométrio no local da cicatriz uterina em paciente com cesariana anterior. A causa da istmocele parece ser multifatorial. Má técnica cirúrgica, baixa localização da incisão, retroflexão uterina, obesidade, tabagismo, cicatrização inadequada de cicatrizes e idade materna são possíveis fatores relacionados. A maioria dos pacientes com esta condição é assintomática. No entanto, as mulheres podem apresentar sangramento pós-menstrual, dor pélvica, subfertilidade, dismenorreia, infertilidade e abscesso cicatricial. O Brasil tem uma das maiores taxas de cesariana do mundo. Uma das consequências da taxa crescente de cesarianas é a istmocele, um problema emergente de saúde feminina. Aqui relatamos um caso de cistoadenoma mucinoso originado em uma istmocele uterina, uma complicação ainda não descrita, até onde pudemos investigar.

Pregnancy outcomes in severe polyhydramnios: no increase in risk in patients needing amnioreduction for maternal pain or respiratory distress.

INTRODUCTION: Polyhydramnios is a common complication of fetal malformations and has been described to have high risk of pregnancy complications such as prematurity and placental abruption. In a subgroup of women severe polyhydramnios may lead to maternal dyspnea or untreatable pain, and amnioreduction is the procedure indicated to relieve those symptoms. There is a lack of information concerning the increase in the risk for the pregnancy when the procedure is indicated. Therefore, this study sought to evaluate the risk of complications in pregnancies with severe polyhydramnios that needed amnioreduction in relation to the basal risk for the pregnancy of having polyhydramnios without the need for the procedure. MATERIALS AND METHODS: Patients with singleton pregnancies and severe polyhydramnios followed in our fetal medicine center were evaluated retrospectively. Pregnancy complications (prematurity rate, fetal death, premature rupture of membranes, placental abruption and chorioamnionitis) were studied in the group of patients needing the procedure and their risk was compared to the risk of having a pregnancy with severe polyhydramnios but with no need for the procedure. RESULTS: One hundred and thirty-five patients were evaluated. Forty-four patients (32.6%) needed amnioreduction. There was no increase in the risk of having complications when the procedure was needed OR = 1.4 (CI 0.46-1.26). CONCLUSION: Amnioreduction performed to relieve maternal symptoms did not statistically increase the risk of pregnancy complications with severe polyhydramnios in single pregnancies.

Clinical and obstetrical management of pregnant women with autoimmune hepatitis complicated by moderate or severe thrombocytopenia.

OBJECTIVE: To describe the management of prenatal care and delivery in patients bearing autoimmune hepatitis associated with moderate or severe thrombocytopenia. METHODS: This study was performed in a tertiary level university hospital. Thirteen pregnancies in ten patients diagnosed with autoimmune hepatitis, complicated by thrombocytopenia, were retrospectively analyzed. The inclusion criteria were as follows: clinical diagnosis of autoimmune hepatitis, moderate or severe thrombocytopenia (platelet count < 100 x 103/mm3), gestational age at birth over 22 weeks, and patient followed-up by a specialized team at the institution. The variables studied were: maternal age, parity, treatment regimen, platelet count, examinations for investigation of hepatic function, type of delivery, weight at birth, and gestational age at the time of delivery. RESULTS: The average maternal age was 24.5 years (SD = 5.3) and six (50%) occurred in nulliparous women. During pregnancy, monotherapy with prednisone was adopted in 11 cases (92%). According to the autoantibody profiles, seven pregnancies (58%) had the autoimmune hepatitis type I diagnosis, two pregnancies had type II (17%), and three pregnancies (25%) had cryptogenic chronic hepatitis (undetectable titers of autoantibodies). Portal hypertension was featured in 11 pregnancies (92%). The average gestational age at delivery was 36.9 weeks (SD = 1.5 weeks), with an average weight at birth of 2,446 g (SD = 655 g). Eight infants (67%) were small for gestational age. At the time of delivery, severe thrombocytopenia was featured in four cases (33%) and cesarean surgery was performed in seven cases (58%). Complications at delivery occurred in three cases (25%), one patient presented uterine atony, and two patients presented perineal bruising. There was no perinatal or maternal death. CONCLUSION: The complications of thrombocytopenic patients with autoimmune hepatitis are elevated; nevertheless, with appropriate attention and care, they can be resolved. The association between two severe pathologies appears to increase the risk of prematurity and fetal growth restriction, demanding specialized prenatal care, as well as surveillance of newborn well-being.

Wilson's disease: an analysis of 28 Brazilian children.

OBJECTIVES: Clinical-laboratory and evolutionary analysis of twenty-eight patients with Wilson's disease. METHODS: Twenty-eight children (twelve females and sixteen males) with Wilson's disease were evaluated retrospectively between 1987 and 2009, with a follow-up of 72 months (1 - 240 months). The clinical, laboratory, and histologic features at diagnosis were recorded at the end of the study. RESULTS: The median age at diagnosis was 11 years (2 - 18 years). Twelve patients were asymptomatic, seven had hepatitis symptoms, five had raised aminotransferase levels, three had hepatomegaly associated with neurological disorders, one had fulminant hepatitis with hemolytic anemia, and six patients presented with a Kayser-Fleischer ring. A histological analysis revealed that six children had chronic hepatitis, seven had cirrhosis, two had steatosis, one had portal fibrosis, and one had massive necrosis. The treatment consisted of D-penicillamine associated with pyridoxine for 26 patients. Adverse effects were observed in the other two patients: one presented with uncontrollable vomiting and the other demonstrated elastosis perforans serpiginosa. At the end of the study, all 26 treated patients were asymptomatic. Twenty-four of the patients were treated with D-penicillamine and pyridoxine, and two were treated with trientine and zinc sulfate. A liver transplant was performed in one patient with fulminant hepatitis, but the final patient died 48 hours after admission to the intensive care unit. CONCLUSIONS: Family screenings associated with early treatment are important in preventing Wilson's disease symptoms and potentially fatal disease progression. The study suggests that Wilson's disease must be ruled out in children older than two years presenting with abnormal levels of hepatic enzymes because of the heterogeneity of symptoms and the encouraging treatment results obtained so far.

Manejo clínico e obstétrico em gestantes portadoras de hepatite autoimune complicada pela plaquetopenia moderada ou grave / Clinical and obstetrical management of pregnant women with autoimmune hepatitis complicated by moderate or severe thrombocytopenia

OBJETIVO: O presente trabalho tem como objetivo descrever o manejo do pré-natal e do parto em pacientes portadoras de hepatite autoimune associada à plaquetopenia moderada ou grave. MÉTODOS: Este trabalho foi realizado em hospital universitário, de nível terciário. Foram analisadas, retrospectivamente, 13 gestações em dez pacientes com diagnóstico de hepatite autoimune complicadas pela plaquetopenia. Os critérios de inclusão foram: diagnóstico clínico de hepatite autoimune, plaquetopenia moderada ou grave (contagem de plaquetas < 100 x 103/mm3), idade gestacional ao nascimento acima de 22 semanas e pacientes acompanhadas por equipe especializada da instituição. As variáveis estudadas incluíram idade materna, paridade, os regimes de tratamento, contagem de plaquetas, exames para investigação da função hepática, tipo de parto, peso ao nascer e idade gestacional no momento do parto. RESULTADOS: A média da idade materna foi de 24,5 anos (DP = 5,3) e seis (50%) ocorreram em nulíparas. Durante a gravidez, a monoterapia com prednisona foi adotada em 11 (92%) casos. De acordo com o perfil de autoanticorpos, sete (58%) gestações possuíam diagnóstico de hepatite autoimune tipo I, duas (17%) do tipo II e três (25%) eram portadoras de hepatite crônica criptogênica (títulos de autoanticorpos indetectáveis). A hipertensão portal foi caracterizada em 11 (92%) gestações. A idade gestacional média no parto foi de 36,9 semanas (DP = 1,5 semana), com média de peso ao nascer de 2446g (DP = 655g), sendo oito (67%) pequenos para a idade gestacional. No momento do parto, a plaquetopenia grave foi caracterizada em quatro (33%) casos e a cesárea foi realizada em sete (58%). As complicações no parto ocorreram em três casos (25%), uma paciente apresentou atonia uterina e duas, hematoma perineal. Não houve morte materna ou perinatal. CONCLUSÃO: As complicações em pacientes plaquetopênicas com hepatite autoimune são elevadas, no entanto, com os cuidados e atenção necessários, podem ser contornáveis. A associação de duas patologias graves parece aumentar o risco de prematuridade e restrição do crescimento fetal, demandando atenção pré-natal especializada, bem como vigilância do bem-estar do concepto.

OBJECTIVE: To describe the management of prenatal care and delivery in patients bearing autoimmune hepatitis associated with moderate or severe thrombocytopenia. METHODS: This study was performed in a tertiary level university hospital. Thirteen pregnancies in ten patients diagnosed with autoimmune hepatitis, complicated by thrombocytopenia, were retrospectively analyzed. The inclusion criteria were as follows: clinical diagnosis of autoimmune hepatitis, moderate or severe thrombocytopenia (platelet count < 100 x 103/mm3), gestational age at birth over 22 weeks, and patient followed-up by a specialized team at the institution. The variables studied were: maternal age, parity, treatment regimen, platelet count, examinations for investigation of hepatic function, type of delivery, weight at birth, and gestational age at the time of delivery. RESULTS: The average maternal age was 24.5 years (SD = 5.3) and six (50%) occurred in nulliparous women. During pregnancy, monotherapy with prednisone was adopted in 11 cases (92%). According to the autoantibody profiles, seven pregnancies (58%) had the autoimmune hepatitis type I diagnosis, two pregnancies had type II (17%), and three pregnancies (25%) had cryptogenic chronic hepatitis (undetectable titers of autoantibodies). Portal hypertension was featured in 11 pregnancies (92%). The average gestational age at delivery was 36.9 weeks (SD = 1.5 weeks), with an average weight at birth of 2,446 g (SD = 655 g). Eight infants (67%) were small for gestational age. At the time of delivery, severe thrombocytopenia was featured in four cases (33%) and cesarean surgery was performed in seven cases (58%). Complications at delivery occurred in three cases (25%), one patient presented uterine atony, and two patients presented perineal bruising. There was no perinatal or maternal death. CONCLUSION: The complications of thrombocytopenic patients with autoimmune hepatitis are elevated; nevertheless, with appropriate attention and care, they can be resolved. The association between two severe pathologies appears to increase the risk of prematurity and fetal growth restriction, demanding specialized prenatal care, as well as surveillance of newborn well-being.

Wilson's disease: an analysis of 28 Brazilian children

OBJECTIVES: Clinical-laboratory and evolutionary analysis of twenty-eight patients with Wilson's disease. METHODS: Twenty-eight children (twelve females and sixteen males) with Wilson's disease were evaluated retrospectively between 1987 and 2009, with a follow-up of 72 months (1 - 240 months). The clinical, laboratory, and histologic features at diagnosis were recorded at the end of the study. RESULTS: The median age at diagnosis was 11 years (2 - 18 years). Twelve patients were asymptomatic, seven had hepatitis symptoms, five had raised aminotransferase levels, three had hepatomegaly associated with neurological disorders, one had fulminant hepatitis with hemolytic anemia, and six patients presented with a Kayser-Fleischer ring. A histological analysis revealed that six children had chronic hepatitis, seven had cirrhosis, two had steatosis, one had portal fibrosis, and one had massive necrosis. The treatment consisted of D-penicillamine associated with pyridoxine for 26 patients. Adverse effects were observed in the other two patients: one presented with uncontrollable vomiting and the other demonstrated elastosis perforans serpiginosa. At the end of the study, all 26 treated patients were asymptomatic. Twenty-four of the patients were treated with D-penicillamine and pyridoxine, and two were treated with trientine and zinc sulfate. A liver transplant was performed in one patient with fulminant hepatitis, but the final patient died 48 hours after admission to the intensive care unit. CONCLUSIONS: Family screenings associated with early treatment are important in preventing Wilson's disease symptoms and potentially fatal disease progression. The study suggests that Wilson's disease must be ruled out in children older than two years presenting with abnormal levels of hepatic enzymes because of the heterogeneity of symptoms and the encouraging treatment results obtained so far.