Abstract Fanconi Anemia (FA) is a rare hereditary syndrome which commonly causes infertility. We present a case of a spontaneous successful pregnancy in a patient with FA. During pregnancy, she had a progressive worsening of baseline thrombocytopenia without improvement with corticosteroid or platelet transfusions. Due to inability to maintain a safe platelet level, therapy with a thrombopoietin receptor agonist (Eltrombopag), previously used in patients with refractory immune thrombocytopenia, was started with good response. As this report shows, pregnancy with good perinatal outcome in patients with FA is possible with appropriate multidisciplinary follow-up.

Resumo Introdução A hemorragia uterina anormal é mais frequente na adolescência. Apesar de maioritariamente de etiologia não estrutural, pode dever-se a qualquer causa descrita. Caso clínico Adolescente de 12 anos, sem antecedentes pessoais relevantes, com menarca há 1 mês, observada no serviço de urgência por hemorragia menstrual grave com agravamento progressivo e repercussão hemodinâmica com necessidade de suporte transfusional. Foi colocada a hipótese de disfunção ovulatória fisiológica associada a eventual coagulopatia desconhecida previamente e foi instituído tratamento médico. Por ausência de resposta a tratamento médico, foi submetida a observação sob sedação e aspiração uterina que evidenciou tratar-se de um Linfoma de Burkitt. Discussão Apesar de as causas estruturais, e particularmente as neoplasias, do foro ginecológico ou não, serem uma causa rara de hemorragia uterina anormal nesta faixa etária, elas devem ser levadas em consideração potenciando assim um tratamento mais célere e adequado.

Abstract Introduction Abnormal uterine bleeding is more frequent in adolescence. Although, most commonly, it has a non-structural etiology, it may be due to any cause described. Clinical case A 12-year-old adolescent, with no relevant personal history, menarche 1 month before, was observed in the emergency department for severemenstrual bleeding with progressive worsening, and hemodynamic repercussion in need of transfusion support. Physiological ovulatory dysfunction associated with possible previously unknown coagulopathy was considered to be the most likely diagnosis and medical treatment was initiated. Without response, the patient was submitted to sedated observation and uterine aspiration, which ultimately led to the diagnosis of a Burkitt Lymphoma. Discussion Although structural causes, and particularly malignancy, whether gynecological or not, are a rare cause of abnormal uterine bleeding in this age group, they must be considered, thus enhancing the fastest and most appropriate treatment.

Abstract Interstitial ectopic pregnancy is rare, but can cause severe complications. The diagnosis is challenging and the optimal treatment varies according to each case. The authors present two case reports of early interstitial pregnancies diagnosed in asymptomatic or mildly symptomatic women. One was submitted to laparoscopic excision of the ectopic mass without complications. As for the other, she was treated with intramuscular methotrexate. In both cases, human chorionic gonadotropin decreased consistently until it became undetectable 4 weeks after treatment. An early and accurate diagnosis is fundamental to avoid major complications. Conservative treatment is possible and should be recommended in selected cases.

Abstract Uterine septum is the most common uterine anomaly and is more prevalent in women with infertility and a history of recurrent miscarriage. There is no consensus on what the indications for treatment are, nor the specific surgical approach to be used. It is reasonable to consider incision of the septum in a patient with infertility, history of spontaneous abortion or adverse obstetric outcomes, but no solid published evidence supports surgical treatment in asymptomatic women. The decision on therapy must be shared with the patient, explaining the potential risks and benefits. The surgical technique must be chosen regarding the surgeon’s experience.

Abstract Overview and aims: Surgery for Pelvic Organ Prolapse (POP) is increasing with the aging of population. When maintenance of vaginal length and coital function is not a surgical goal, obliterative surgery is effective for POP treatment, especially in women with comorbidities. Our primary study goal was to evaluate the objective and subjective cure rates of different obliterative surgeries. We also aimed to characterize this population of women and the complications of this type of surgery. Study design: We conducted an observational retrospective cohort study Population: 82 patients who underwent obliterative surgery as a treatment for primary or recurrent POP. Methods: Retrospective review of clinical charts, with descriptive and bivariate analysis. Results: All women were sexually inactive and their mean age was 77.3 years-old. Overall, they had excessive weight and 97.6% of them had a POP-Q stage of III or more. The majority of these women had two or more comorbidities at time of surgery, and cardiovascular disease was the most severe in 40% of them. Twenty eight percent of them had already underwent a surgery for POP. There were only two cases of minor complications after surgery, specifically lower urinary tract infection. Overall, objective cure rate was 89.7% and subjective cure rate was 98.5%, for a mean time of follow-up of 18.5 months. We found no differences between different obliterative procedures. De novo urge urinary incontinence developed in 22% of patients. Conclusions: Obliterative surgery is a good treatment option for severe POP, especially in women with anesthetic risk and no desire for future vaginal coitus. In our cohort we found excellent objective and subjective POP cure rates after obliterative surgeries, combined with low risks..

Overview and Aims: Gestational diabetes (GD) is associated with increased risk for pregnancy and delivery complications. Ethnicity is a recognized risk factor for GD. Considering ethnic diversity in Portugal, it is important that health providers are familiar with differences in expected outcomes. This study was designed to compare Asian, Black and Caucasian women with GD. Study Design: Observational, retrospective study. Population: Women with singleton pregnancies and GD followed in our hospital, from 2012 to 2015. Materials and Methods: 323 women were divided according to country of birth and skin color - Caucasian (G1, n=230), Black (G2, n=79) and Asian (G3, n=14). Demographic characteristics, risk factors for GD and previous hypertension were compared. Maternal/fetal outcomes were analyzed - maternal weight gain, metabolic control, need of pharmacologic therapy, hypertensive complications, ultrasound biometric measures, intrauterine demise, mode of delivery, newborn weight and comorbidities. X2 and Fisher tests were performed. Significance was set at p-value<0.05. Results: G1 included women mainly from Portugal, G2 from Guinea Bissau, Angola and Portugal and G3 from India. Groups differed in level of education (p=0.023) and Body Mass Index categories (p=0.034) - G2 had the lowest level of education and the highest rates of overweight/obesity. Statistical difference was found in metabolic control, resulting in glycated haemoglobin below 6% (p=0.022) and mode of delivery (p<0,001); G2 and G3 had worse metabolic control than G1, with higher C-section rates. Neonatal comorbidity differed between groups (p=0.021), with G2 having higher rates, mainly hyperbilirrubinemia requiring phototherapy. Need or type of pharmacologic therapy (insulin/metformin) showed no difference between groups. Conclusions: Metabolic control in Asian and Black women with GD was more difficult to achieve than in Caucasians, with afterwards worse maternal/fetal outcomes. Health providers should acknowledge differences between ethnicities and a multidisciplinary and individualized approach should be provided.

Mammary-like neoplasms have been described through the mammary-line, axilla, vulva and prostate, and are rare lesions of un uncertain histological origin. Phyllodes tumor occuring in the vulva is rare with only a few cases reported. We describe a case of a 41-year-old woman with a vulvar swelling that revealed to be a phyllodes tumor after complete excision.

Laparoscopic and vaginal routes are the preferable routes to consider when planning a hysterectomy. Laparoscopy brings advantages to vaginal route when there are concomitant adnexal masses, endometriosis, pelvic inflammatory disease or previous abdominal surgeries. However, the learning curve in laparoscopy is slower and the surgery itself is, consequently, very dependent on the surgeon's experience. In this article, the authors review the first 300 total laparoscopic hysterectomy (TLH) surgeries in a new Obstetric/Gynecology Department. A retrospective analysis to clinical files of patients submitted to TLH between January 2013 and February 2016. Data was divided in 2 groups: group A, corresponding to the first 150 HTL and group B, with the 150 subsequent HTL. The results showed 2 similar groups, with median ages of 51 years old, with 27 median corporeal mass index, with 41% of the patients having previous abdominal surgeries. The main surgical indication was anomalous uterine bleeding secondary to mioma/adenomyosis (53%), and 309 concomitant surgeries were performed during surgical time (the majority in the adnexal area, 84%). Globally the intra-operatory and post-operatory complications rate was 1% and 2.3%, respectively, and most occurred in group A. The conversion rate to laparotomy or vaginal assisted laparoscopic hysterectomy was low (2.3%), and was only verified in group A, with statistic significance (p-value - 0.024). The blood loss volume was also statiscally different between the 2 groups (p-value < 0.001), with less volume blood loss in group B. The average uterine weight was similar in the 2 groups, with 187 and 168 grams, and the average surgical time was 155 and 148 minutes, which included the concomitant surgeries. In conclusions, despite the small number of cases, our study seems to show a tendency towards decrease in complications rate, conversion rate and blood loss, probably due to increased experience of the surgeons

Overview and aims: Deep endometriosis is defined as the presence of infiltrating lesions more than 5mm deep to the peritoneum and it corresponds to 5-10% of all endometriotic lesions. Laparoscopy is considered the gold standard for the diagnosis and treatment of endometriosis and it is successful in controlling pain, decreasing recurrence rates and improving fertility. This study aims to evaluate the work of a Laparoscopic unit specialized in the treatment of endometriosis. Study design, Population and Methods: Retrospective study of all laparoscopies performed for deep endometriosis between January 2013 and December 2014. Clinical presentation, pre-operatory exams, intra and post-operatory surgical data and follow-up were accessed. Results: Sixteen surgeries were done (15 patients). The patient's mean age was 35 years (28- 43); ten women were nulliparous of which nine were infertile. Dysmenorrhea was the most frequent symptom (n=12), followed by dyspareunia (n=8). In six women a node in the rectovaginal septum was present in the physical exam. In six cases a colpectomy was done in order to accomplish total excision of the rectovaginal node. One discoid excision of the rectum was done. No conversions to laparotomy were needed. The mean operative time was 142±55min, with estimated blood loss < 250ml in 15 surgeries. There was a case of an intra-abdominal abscess. Mean follow-up duration was eight months, with 81% of the patients referring clinical improvement. Conclusions: Laparoscopic surgery in the treatment of deep endometriosis requires a high degree of surgical expertise and the approach by a multidisciplinary team. Only then, a low rate of complications, symptoms regression and a potential fertility improvement can be achieved.