Herlyn-Werner-Wunderlinch: An unusual presentation in a patient with Prader-Willi syndrome.

Herlyn-Werner-Wunderlich syndrome is an uncommon urogenital anomaly defined by uterus didelphys, obstructed hemi-vagina and unilateral renal anomalies. The most common clinical presentation is dysmenorrhoea following menarche, but it can also present as pain and an abdominal mass. Prader-Willi syndrome is a rare neuroendocrine genetic syndrome. Hypothalamic dysfunction is common and pituitary hormone deficiencies including hypogonadism are prevalent. We report the case of a 33-year-old female with Prader-Willi syndrome who was referred to the Gynaecology clinic due to vaginal bleeding and abdominal pain. Abdominal ultrasound revealed a haematometra and haematocolpos and computed tomography showed a uterus malformation and a right uterine cavity occupation (hematometra) as well as right kidney agenesis. Vaginoscopy and hysteroscopy were performed under general anaesthesia, finding a right bulging vaginal septum and a normal left cervix and hemiuterus. Septotomy was performed with complete haematometrocolpos drainage. The association of the two syndromes remains unclear.

An Algorithm to Predict the Lack of Pregnancy after Intrauterine Insemination in Infertile Patients.

Increasing intrauterine insemination (IUI) success rates is essential to improve the quality of care for infertile couples. Additionally, straight referral of couples with less probability of achieving a pregnancy through IUI to more complex methods such as in vitro fertilization is important to reduce costs and the time to pregnancy. The aim of the present study is to prospectively evaluate the threshold values for different parameters related to success in intrauterine insemination in order to provide better reproductive counseling to infertile couples, moreover, to generate an algorithm based on male and female parameters to predict whether the couple is suitable for achieving pregnancy using IUI. For that, one hundred ninety-seven infertile couples undergoing 409 consecutive cycles of intrauterine insemination during a two-year period were included. The first year served as a definition of the parameters and thresholds related to pregnancy achievement, while the second year was used to validate the consistency of these parameters. Subsequently, those parameters that remained consistent throughout two years were included in a generalized estimating equation model (GEE) to determine their significance in predicting pregnancy achievement. Parameters significantly associated with the lack of pregnancy through IUI and included in the GEE were (p < 0.05): (i) male age > 41 years; (ii) ejaculate sperm count < 51.79 x 106 sperm; (iii) swim-up alkaline Comet > 59%; (iv) female body mass index > 45 kg/m2; (v) duration of infertility (>84 months), and (vi) basal LH levels > 27.28 mUI/mL. The application of these limits could provide a pregnancy prognosis to couples before undergoing intrauterine insemination, therefore avoiding it in couples with low chances of success. The retrospective application of these parameters to the same cohort of patients would have increased the pregnancy rate by up to 30%.

Decline of Sperm Quality over the Last Two Decades in the South of Europe: A Retrospective Study in Infertile Patients.

Semen quality has a direct relation to male fertility. Whether sperm variables in humans have decreased over the last years is still uncertain, with some studies showing a decline and others reporting no changes. In this regard, previous research has suggested that lifestyle and environmental conditions may contribute to this variability, calling for regional studies. The present work is a retrospective, unicentric study that includes semen samples analyzed between 1997 and 2017 at the Parc Taulí Hospital (Barcelona metropolitan area). First, a multivariate analysis including the age as a confounding factor showed a statistically significant decrease in semen volume, pH, progressive motility, morphology and total motile sperm over time. Contrarily, no significant variation in sperm count or concentration was observed. Mean reductions per year were -0.02 mL for volume, -0.57% for progressively motile sperm and -0.72% for sperm with normal morphology. Interestingly, the average annual temperature registered by the Spanish Meteorology Agency negatively correlated to sperm morphology and sperm count (Rs = -0.642; p = 0.002 and Rs = -0.435; p = 0.049, respectively). In conclusion, the present study based on infertile patients from the Barcelona area found a decline in sperm motility and morphology, without effects on sperm count. Changes in temperature appeared to be associated to this decline, but further studies are needed to address the mechanisms linked to the observed variations.

Resultados obstétricos y perinatales en gestaciones tras técnicas de reproducción asistida / Obstetrical and perinatal outcomes in pregnancies after assisted reproductive techniques

Material y Métodos: estudio observacional retrospectivo que compara 704 gestaciones conseguidas tras técnicas de reproducción asistida en el periodo 2008-2012 (grupo estudio), con 2.507 gestaciones espontaneas (grupo control). Resultados: mayor riesgo de gestaciones múltiples en el grupo estudio 24,3% contra 1% del grupo control p < 0,001 RR 25,5 (17,3-37,6). El parto fue a las 37,79 semanas en grupo estudio y 38,99 grupo control p < 0,001. Manteniéndose las diferencias en gestaciones únicas con una media de 38,5 en grupo estudio y 39 en grupo control p < 0,001. Niños prematuros en grupo estudio 23,2%, contra 6,2% grupo control p < 0,001 RR 4,5 (3,6-5,8). Reduciéndose a la mitad el riesgo relativo cuando solo comparamos gestaciones únicas. Cesarea en 52% gestaciones por técnicas de reproducción asistida, contra 18,6% en gestaciones espontaneas p < 0,001 RR 2,85 (2,56-3,22). Manteniéndose diferencias comparando gestaciones únicas 15,6% en grupo estudio y 18,3% grupo control. No encontramos diferencias en el sexo del recién nacido. La tasa de bajo peso al nacer también fue mayor en grupo estudio 23,8% contra 7,97% grupo control p < 0,001 RR 3,6 (2,8-4,5); manteniéndose diferencias en gestaciones únicas con 11% recién nacido de bajo peso contra 5,8% p < 0,001 RR 2 (1,46-2,8). Malformaciones congénitas mayor en grupo control 9,45% respecto grupo estudio 3,69%. Conclusiones: la gestación tras técnicas de reproducción asistida presenta 25 veces más riesgo de gestación múltiple; las madres son 5 años más añosas; 4,5 veces más riesgo de tener un parto pretérmino; 2 veces mayor si comparamos gestaciones únicas; 3 veces más de tener recién nacido de bajo peso, el doble si comparamos gestaciones únicas; cesárea en más del doble tanto gestaciones únicas como en total gestaciones (AU)

Material and Methods: Retrospective observational study comparing outcomes of 704 pregnancies after assisted reproductive techniques 2008-2012 (study group), with 2507 spontaneous pregnancies (control group). Results: There are difference in outcome between assisted reproductive techniques (study group) and naturally conceived pregnancies (control group). We found differences in increased risk of multiple pregnancies in the study group 24.3% to 1% of control group p < 0.001 RR 25.5 (17.3-37.6). Mean weeks in which the delivery occurred study group was 37.79 and 38.99 in the control group p < 0.001. Maintaining differences when only singleton pregnancies compared with an average of 38.5 in the study group and control group 39 weeks p < 0.001. In the study group are at higher risk of preterm birth with 23.2% versus 6.2% control group, p < 0.001 RR 4.5 (3.6-5.8). Reduced to half the relative risk when compared only single pregnancies. Cesarean was performed in 52% of pregnancies by assisted reproductive techniques, while it was 18.6% incidence of spontaneous pregnancies p < 0.001 RR 2.85 (2.56-3.22). He maintained even comparing differences singleton pregnancies in both groups with 15.6% in the study group and 18.3% control group. Without differences in sex of newborns. The rate low birth weight was also higher in the study group 23.8% vs control group 7.97% p < 0.001 RR 3.6 (2.8-4.5); maintaining differences in single pregnancies with 11% low birth weight against 5.8%, study group against control group, p < 0.001 RR 2 (1.46-2.8); Although increased incidence of congenital malformations in the control group 9.45% from 3.69% in the study group. Conclusions: These results suggest that assisted reproductive techniques is associated with increased risk of multiple pregnancy, delivery preterm, higher risk of low birth weight (< = 2500g), and more cesarean section. The increased risk of preterm, low birth weight, and cesarean persisted in singleton pregnancies (AU)

Ovarian torsion and spontaneous ovarian hyperstimulation syndrome in a twin pregnancy: A case report.

INTRODUCTION: Ovarian hyperstimulation syndrome (OHSS) is extremely rare in spontaneous pregnancies. Spontaneous OHSS can result from glycoprotein hormones stimulating follicle-stimulating hormone receptors (FSHR). PRESENTATION OF CASE: We report a twin pregnancy in which ovarian torsion and hemoperitoneum complicating OHSS were treated with left adnexectomy and aspiration. The only trigger for spontaneous OHSS in this case was high levels of chorionic gonadotropin hormone. DISCUSSION: Multiple pregnancy, gestational trophoblastic disease, primary hypothyroidism, thyroid-stimulating hormone/gonadotropin-secreting adenomas, and mutations of the FSHR gene may trigger spontaneous OHSS. CONCLUSION: Spontaneous OHSS should be included in the differential diagnosis of acute abdomen in pregnant women; if spontaneous OHSS is diagnosed, the etiology should be determined in order to focus the treatment and avoid future complications.

The Value of Hysterosalpingography following Medical Treatment with Methotrexate for Ectopic Pregnancy.

After an ectopic pregnancy (EP) fertility decreases, mostly due to tubal factor. Hysterosalpingography (HSG) is the most cost-effective tool for tubal patency assessment. Objective. To evaluate the usefulness of a HSG after a medical treatment for an EP, in order to counsel women on the most appropriate way to conceive future pregnancies. Methods. Between 1998 and 2008, 144 patients were submitted to medical treatment for an EP and performed HSG 3 months after the event. Results. 72.2% of normal HSG, 18.8% with unilateral obstruction, 6.3% tubal patency with defect, and 2.8% bilateral obstruction. Conclusion. Routine HSG following medical treatment for an EP does not seem necessary, as it does not change the initial management in 97.2% of the cases, but might be considered in selected risk cases, permitting timely referral of patients to in vitro fertilization.

Manejo conservador de un hematoma subcapsular hepático en un síndrome de HELLP / Conservative management of a subcapsular liver hematoma in HELLP syndrome

La sospecha clínica, el diagnóstico rápido y un abordaje multidisciplinar son fundamentales para mejorar la morbimortalidad de las pacientes con hematoma subcapsular hepático. El tratamiento de esta entidad abarca desde la conducta expectante bajo vigilancia clínica y radiológica, la embolización de las arterias hepáticas o la cirugía en caso de inestabilidad hemodinámica, persistencia de sangrado o incremento del dolor. Presentamos el caso de un síndrome de HELLP posparto complicado con un hematoma subcapsular hepático y que se resolvió exitosamente con embolización de la arteria hepática derecha (AU)

To improve morbidity and mortality in patients with subcapsular liver hematoma, clinical suspicion, a rapid diagnosis and multidisciplinary treatment are fundamental. The treatment of this complication includes expectant management with clinical and radiological observation, embolization of the hepatic arteries, and surgical treatment if there is hemodynamic instability, persistent bleeding or increasing pain. We report a case of postpartum HELLP syndrome that was complicated by a subcapsular liver hematoma, successfully treated by embolization of the right hepatic artery (AU)

Donovanosis genital / Genital donovanosis

Presentamos un caso de donovanosis genital en una gestante cuyo diagnóstico fue dificultoso dada la baja incidencia de esta enfermedad en nuestro país. La donovanosis o granuloma inguinal es una infección de transmisión sexual, causa de úlcera genital crónica no dolorosa. Es una enfermedad endémica en países tropicales y subtropicales. Debido al aumento de la inmigración procedente de estas zonas y al turismo sexual a países endémicos, estamos asistiendo a un aumento de esta patología infecciosa poco prevalente hasta ahora en nuestro medio (AU)

We report a case of genital donovanosis in a pregnant woman, whose diagnosis was difficult because of the low incidence of this disease in our country. Donovanosis or granuloma inguinale is a sexually transmitted infection that causes chronic genital painless ulceration. It is an endemic disease in tropical and subtropical countries. Due to the increase of immigration coming from these countries and sexual tourism in endemic countries, we are witnessing an increase in this infectious disease not very prevalent in our country until now (AU)

Displasia mesenquimal placentaria / Placental mesenchymal dysplasia

La displasia mesenquimal placentaria (DMP) es una anomalía vascular que presenta una baja incidencia (0,02%) y se caracteriza por una placentomegalia con múltiples vesículas en grano de uva en el examen ecográfico. El diagnóstico diferencial principal es la mola hidatiforme parcial, pero la DMP coexiste con feto viable. Se asocia a crecimiento intauterino restringido (CIR), muerte fetal y, en el 30% de los casos, síndrome de Beckwith-Wiedemann. El diagnóstico de sospecha de la DMP es ecográfico, pero el diagnóstico definitivo lo dará el estudio anatomopatológico. Presentamos 2 casos de DMP, cuyo diagnóstico no se realizó hasta el análisis histológico, que cursaron con resultados perinatales diferentes, así como una revisión bibliográfica de la displasia mesenquimal placentaria (AU)

Placental mesenchymal dysplasia (PMD) is a vascular abnormality with a very low incidence (0.02%) that is characterized by placentomegaly with multiple grapelike vesicles on ultrasound examination. The main differential diagnosis of PMD is partial hydatidiform mole. However, in contrast to a partial mole, PMD can coexist with a viable fetus. PMD is associated with fetal growth restriction, fetal demise and Beckwith-Wiedemann syndrome in 30% of cases. The suspected diagnosis is based on ultrasound examination, but the final diagnosis of PMD is made by histological analysis. We present two cases of PMD which were diagnosed at necroscopy and which had distinct outcomes. A literature review of placental mesenchymal dysplasia is also provided (AU)