[Immunosuppressive therapy and fertility preservation: Indications and methods]. / Traitements immunosuppresseurs et préservation de la fertilité : indications et modalités pratiques.

Fertility preservation is routinely performed in cancerology but less systematically used in the field of immune diseases, even though the use of gonadotoxic treatments in young patients may be required and even though the disease itself can alter fertility. This review aimed to clarify the indications and methods of fertility preservation in this context. Cyclophosphamide is the only immunosuppressive drug requiring fertility preservation in women. In men, fertility preservation should be proposed before treatment with cyclophosphamide, methotrexate, mycophenolate mofetil or mTOR inhibitors. Other factors inherent to the disease or the patient may alter fertility. Thus, screening for infertility and fertility preservation have to be implemented as much as possible to increase the chances of successful procreation in patients with immune disease. For women, the choice between the different preservation methods depends on the patient's age, disease activity, the time available before the start of treatment, the possibility of future pregnancy and the woman's and even couple's wishes. Before puberty, the only accepted method is cryopreservation of ovarian tissue. After puberty, the first-line method is the cryopreservation of mature oocytes. If the treatment has to be started in an emergency, if ovarian hyperstimulation/oocyte retrieval is contraindicated or if the patient refuses this option, cryopreservation of ovarian tissue or GnRH agonists could be proposed. For men, the accepted method is sperm cryopreservation. For prepubertal boys, the cryopreservation of spermatogonia after testicular biopsy is still experimental.

[Interstitial, angular and cornual pregnancies: Diagnosis, treatment and subsequent fertility]. / Grossesse interstitielle, angulaire et cornuale : diagnostic, traitement et futur obstétrical.

OBJECTIVE: Interstitial, angular and corneal pregnancies are not very frequent and often mistaken, each with its own definition and prognosis. The objective of this work is to relate 10 years experience of ectopic pregnancies at the UH in Dijon, based on the latest data from the literature in term of diagnosis, management and subsequent fertility. METHOD: This is a retrospective study carried out at the UH of Dijon from 01/01/2005 to 01/01/2015. From the medical records of each patient who presented a corneal, interstitial or angular pregnancy, we identified the risk factors for ectopic pregnancy (EG), the diagnostic and therapeutic means used, and the subsequent obstetrical events. RESULTS: In 10 years, 532 EG were managed including 10 interstitials, one angular and nine cornual. The main risk factors were previous EG (50%), salpingectomy (55%), curettage (45%) and smoking (40%). The localization of the EG was done in 75% by the endo-vaginal sonography, in 25% in peroperative. Thirty-five percent were treated with methotrexate, 20% had surgery and 40% had both. Seventy-five percent of patients had at least one ulterior pregnancy. In the case of caesarean section, no dehiscence of the corneal scar was identified. CONCLUSION: This study shows the presence of medical antecedents which are risk factors of the tubular EG. A methotrexate protocol should be proposed first. Even after corneal surgery, vaginal delivery may remain possible.

Early screening for type 2 diabetes following gestational diabetes mellitus in France: hardly any impact of the 2010 guidelines.

AIMS: Women who had gestational diabetes mellitus (GDM) have a high risk of type 2 diabetes mellitus (T2DM) in the years following pregnancy. Most follow-up screening studies have been conducted in limited geographical areas leading to large variability in the results. The aim of our investigation was to measure how the publication of guidelines affected early screening for T2DM after a pregnancy with GDM during the period 2007-2013, in France. METHODS: We conducted a retrospective cohort study in a representative sample of 1/97th of the French population using data from the "National Health Insurance Inter-Regime Information System," which collects individual hospital and non-hospital data for healthcare consumption. RESULTS: The sample included 49,080 women who gave birth in 2007-2013. In the following 3 months, only 18.49% of women with GDM had an oral glucose tolerance test or a blood glucose test in 2007. This rate had not significantly increased in 2013 (p = 0.18). The proportion of women with GDM who had the recommended glycemic follow-up at 3 months (20.30 vs. 21.58%, p = 0.19) and 6 months (32.48 vs. 37.16%, p = 0.08) was not significantly different before the guidelines (2008-2009) and after the guidelines (2012-2013). At 12 months, the difference was significant (46.77 vs. 54.05%, p = 0.009). CONCLUSION: Postpartum screening has improved only slightly since the guidelines and remains largely insufficient, with less than 25% of women with GDM screened in the first 3 months. In the first year after delivery, less than 60% of women were screened for T2DM.

[Quality of life after deep pelvic endometriosis surgery: Evaluation of a French version of the EHP-30]. / Qualité de vie après chirurgie de l'endométriose pelvienne profonde : évaluation d'une version française de l'EHP-30.

OBJECTIVE: To evaluate the changes in the quality of life of patients after deep pelvic endometriosis surgery, with a French version of EHP-30 questionnaire, and the capacity of the EHP-30 to carry out this measurement. MATERIAL AND METHODS: Study prospective monocentric, conducted in the obstetrics and gynecology department of the CHU Dijon during the period of October 2012 from October 2013. A EHP-30 questionnaire was given to patients before surgery. The same questionnaire was sent to their homes, away from surgery (3-6 months) to inform about their postoperative quality of life. The difference in preoperative and postoperative scores was tested using the test Wilcoxon signed ranks. A difference was considered significant if the p-value was less than or equal to 0.05. Sensitivity to change was calculated by the method of effect size (ES). The size of the effect is defined as the difference in mean preoperative and postoperative scores divided by the standard deviation of preoperative scores. A size effet of 0.20 indicates less change scores, of 0.50 a moderate change and of 0.80 a material change. RESULTS: We included 22 patients in total in the prospective analysis. The majority of patients had gynecological symptoms of dysmenorrhea with 69.7%, 75.7% and 75.7% dyspareunia chronic pelvic pain. Nineteen patients (57.6%) had gastro-intestinal symptoms. Urinary symptoms were less frequent. The results of the EHP-30 showed a significant improvement for the items "pain" (P=0.01), "control and powerlessness" (P=0.02), "emotional well-being" (P<0,01) "social relations" (P<0.01), "sexual intercourse" (P=0.03) and "relationship with the medical world" (P=0.05). We observed a non-significant improvement for the items "self-image" (P=0.44), "work" (P=0.48) and "relationships with children" (P=0.50). The size of the effect (ES) was low to high for all dimensions of the questionnaire, ranging from 0.1 to 0.6 for the entire group. A significant sensitivity to change was found for the items "pain" (ES=0.60), "control and powerlessness" (ES=0.62), "social relations" (ES=0.57). A moderate sensitivity to change was found for the items "emotional well-being" (ES=0.29), "relationship with the medical world" (ES=0.26). A low sensitivity to change was found for the items "relationships with children" (ES=0.06), "self-image" (ES=0.16), "work" (ES=0.18), "sexual intercourse" (ES=0.20). A size that is important to moderate effect corresponded to a statistically significant improvement of the score EHP-30. CONCLUSION: This study showed that the EHP-30 is a sensitive tool to change the health status and an appropriate instrument for the assessment of treatment effects in patients with deep pelvic endometriosis.

[Pheochromocytoma: rare lethal challenging diagnosis that may be encountered by gynecologists and obstetricians]. / Le phéochromocytome : piège diagnostique rare et mortel parfois rencontré en gynécologie-obstétrique.

Pheochromocytomas are rare but potentially lethal tumors responsible for malignant hypertension. They may be encountered by gynecologists and obstetricians. The diagnosis is difficult because it can be mistaken for diseases more frequent like preeclampsia or other pelvic tumors. We report two cases highlighting clinical clues such as labile hypertension, headache, sweating, palpitations and failure to respond to conventional treatment should prompt physicians to screen patients for pheochromocytoma by measuring the 24-hour urinary catecholamines. The surgery must be performed after using an appropriate preoperative treatment, in order not to trigger lethal outcome. During pregnancy, C-section is recommended.

[Pelvic organ prolapse-surgical technique (POP-ST): a classification of techniques of mesh augmented repairs for pelvic organ prolapse by vaginal route]. / Proposition d'une classification des techniques chirurgicales de cure de prolapsus (pelvic organ prolapse-surgical technique [POP-ST]) avec mise en place d'une prothèse par voie transvaginale.

OBJECTIVE: The aim of this study was to propose a classification of surgical techniques for treatment of prolapse by vaginal route using prosthetic reinforcements and to relate the evaluation of surgeons involved in the care of surgical patients. METHODS: A literature review was conducted searching for all articles relating novel technique of surgical management of patients with use of prosthetic reinforcements vaginally. The classification was made from descriptions found and then assessed by questionnaires filled out by surgeons. RESULTS: The classification takes account of all the techniques available today and can integrate new. Among the surgeons, 56.5% (13/23) found that the POP-ST is adapted to reflect the reality and variety of techniques and 60.8% (14/23) will be ready for daily use. CONCLUSION: A classification covering all the techniques put them at risk of a final tool too complex for routine use. The simplification would make it more usable but limited the comprehensiveness and evolutionary. Only 23 surgeons returned the questionnaire. A larger sample would be desirable. The POP-ST is the first classification of this type. We believe that it would assess the new techniques to better understand the complications.

[Spontaneous acute intussusception in a pregnant woman]. / Invagination intestinale aiguë spontanée chez une femme enceinte.

Acute intussusception in adults is rare and particularly so in pregnant women (between 1 / 5000 and 1 / 68,000 deliveries). It is a life-threatening condition for both the mother and the fetus. In adults, such intussusceptions are mainly secondary to an intestinal disease and frequently a tumour. We describe here a case of spontaneous intussusception during pregnancy and surgical management of the condition. We also analyse the causes specific to pregnancy.

[Uterine artery embolization with resorbable material prior to myomectomy]. / Embolisation artérielle utérine par matériel résorbable avant myomectomie.

PURPOSE: Fibroids are a frequent cause of gynecology referral. Myomectomy is a conservative treatment alternative. The main risk from this procedure is hemorrhage. The main objective of this study was to demonstrate the efficacy of preoperative uterine artery embolization with resorbable agents to reduce blood losses and facilitate myomectomy. Secondary objectives were to evaluate morbidity and subsequent fertility. PATIENTS AND METHODS: Retrospective study of 21 patients with preoperative uterine artery embolization prior to myomectomy at the University Medical Center of Dijon over a 3 year period. RESULTS: Myomectomy after uterine artery embolization with resorbable agents was associated with only minimal blood loss. Mean preoperative and postoperative hemoglobin levels were comparable (p<0.0001). Uterine suturing was technically simpler. The number of resected fibroids (p=0.2824) and the presence of preoperative anemia (p=0.474) had no statistically significant impact on the duration of hospital stay. Uterine synechiae occurred in three patients after the procedure, and were easily treated. Two patients had normal subsequent pregnancies. CONCLUSION: Preoperative uterine artery embolization with resorbable agents was effective in reducing surgical blood losses. This technique reduces the number of hysterectomies and hemorrhagic complications (hematoma, infection, weaker scar tissue). It should be considered in patients wishing uterine preservation when the hemorrhagic risk is high. Its use in patients seeking subsequent pregnancy should be further assessed with larger series.

[What are the neonatal risks in low-risk pregnancies: implications for the organization of birth centers]. / Quel risque néonatal pour les grossesses à bas risque: implications pédiatriques pour l'organisation des maisons de naissance?

AIM: This study was designed to identify pregnant women at low risk for severe neonatal morbidity. This population should apply for delivery in birth centers. POPULATION AND METHODS: This study was retrospective and included all livebirths in Burgundy over a 4-year period. Fifteen obstetric criteria recorded in the regional perinatal database were used to select pregnant women at low risk for severe neonatal morbidity. Incidence of severe neonatal morbidity in the low and high risk groups was assessed from the following markers: postnatal death; severe neurological conditions (ischemic encephalopathy, seizures, meningitis, intraventricular hemorrhage stage 3-4 and cystic periventricular leukomalacia in preterm infants); tracheal intubation; hospitalization in neonatal intensive care unit. RESULTS: The incidence of severe neonatal morbidity was significantly different (P<0.0001) in the low risk group (0.34% [IC 95%: 0.29-0.40]; N=46345) as compared with the high risk group (5.6% [IC 95%: 5.3-5.9]; N=24961). The main neonatal diseases in the low risk group were: respiratory diseases (29.8%); congenital heart diseases (17.9%) and perinatal asphyxia (15.3%). CONCLUSION: Even though the low risk criteria were associated with a low incidence of severe neonatal morbidity, residual morbidity should be considered in organization of birth centers in France.

Diffuse xanthogranulomatous pyelonephritis with psoas abscess in a pregnant woman.

We report the first case, to our knowledge, of xanthogranulomatous pyelonephritis (XGP) with psoas abscess occurring during pregnancy. A 37-year-old woman in the third trimester of pregnancy presented with low back pain and a fever. From sonographic features, a multidisciplinary team decided to perform computed tomography of the abdomen with contrast agent injection, which strongly suggested diffuse XGP of the left kidney with a psoas abscess. Cesarean section at 32 weeks was followed by extended nephrectomy. Pathological examination of the operative specimen confirmed the diagnosis. Outcomes were favorable in the mother and baby. The diagnosis and treatment of XGP during pregnancy are discussed.

Clinical, molecular, and genotype-phenotype correlation studies from 25 cases of oral-facial-digital syndrome type 1: a French and Belgian collaborative study.

Oral-facial-digital syndrome type 1 (OFD1) is characterised by an X linked dominant mode of inheritance with lethality in males. Clinical features include facial dysmorphism with oral, tooth, and distal abnormalities, polycystic kidney disease, and central nervous system malformations. Large interfamilial and intrafamilial clinical variability has been widely reported, and 18 distinct mutations have been previously reported within OFD1. A French and Belgian collaborative study collected 25 cases from 16 families. OFD1 was analysed using direct sequencing and phenotype-genotype correlation was performed using chi2 test. X inactivation studies were performed on blood lymphocytes. In 11 families, 11 novel mutations, including nine frameshift, one nonsense, and one missense mutation were identified, which spanned nine different exons. A combination of our results with previously reported cases showed that the majority of mutations (65.5%) was located in exons 3, 8, 9, 13, and 16. There was phenotype-genotype correlation between (a) polycystic kidney disease and splice mutations; (b) mental retardation and mutations located in exons 3, 8, 9, 13, and 16; and (c) tooth abnormalities and mutations located in coiled coil domains. Comparing the phenotype of the families with a pathogenic mutation to families with absence of OFD1 mutation, polycystic kidneys and short stature were significantly more frequent in the group with no OFD1 mutation, whereas lingual hamartomas were significantly more frequent in the group with OFD1 mutation. Finally, an X inactivation study showed non-random X inactivation in a third of the samples. Differential X inactivation between mothers and daughters in two families with high intrafamilial variability was of particular interest. Slight phenotype-genotype correlations were established, and X inactivation study showed that skewed X inactivation could be partially involved in the pathogenesis of intrafamilial clinical variability.

[Management of cervical intra-epithelial neoplasm during pregnancy]. / Prise en charge d'une néoplasie intra-épithéliale du col de l'utérus en cours de grossesse.

Approximately 30% of women diagnosed with cervical cancer are in their childbearing years. Prenatal care provides an excellent opportunity for cervical cancer screening. The incidence of abnormal Pap smear has been reported in 5-8% of pregnant women. But we must know that Pap smears have cytologic modifications because of pregnancy. All abnormal smears have to be referred to colposcopic examination. The squamocolumnar junction is visualized in almost 100% of cases. The sensitivity of colposcopy is nearly 87% with complete concordance in 72.6%. Colposcopically directed biopsies have a good correlation with the final diagnosis with very minimal risks for both mother and fetus. The high rate of complications (hemorrhage, abortion, premature labor) and residual lesions in half of cases do not encourage conization during pregnancy. The final treatment is carried out after delivery. The only absolute indication for conization in pregnancy is to rule out microinvasive disease or make the diagnosis of invasive carcinoma when such a diagnosis will alter the timing of delivery but also when there is a no satisfactory colposcopy and a high-grade Pap smear. In these cases conization is performed for diagnostic and not therapeutic purpose. We must be aware of the high rate of loss of follow-up (6-33%).

Antenatal detection of a communicating duodenal duplication.

We report a case of a subhepatic cystic mass diagnosed in utero by antenatal ultrasonography (US) at 15 weeks' gestation which subsequently proved to be a communicating duodenal duplication. In this male foetus, the differential diagnosis was choledochal cyst, congenital biliary atresia, foregut duplication or omentum cyst. Neonatal US examination lead to a diagnosis of duodenal duplication, also confirmed by barium gastrointestinal series. He was operated on day 8 and recovered uneventfully. We discuss the accuracy of antenatal US in the diagnosis of such right upper quadrant cystic masses. Now that antenatal findings are becoming increasingly sensitive in the detection of foetal anomalies, and parents need to be informed about the suspected pathology and its prognosis, we tried to determine, in the light of this case and a review of the literature, how antenatal US findings can offer more accuracy in the diagnosis of duodenal duplication.

Diagnostic and therapeutic dilemma with large prenatally detected cystic adrenal masses.

OBJECTIVES: The prenatal finding of a large cystic adrenal mass raises the dilemma of the differential diagnosis between adrenal hemorrhage and cystic neuroblastoma. The possibility of a neuroblastoma usually leads to surgical excision of such tumors. Nevertheless, an adrenal hemorrhage has to be recognized, so that unnecessary surgery may be avoided. METHODS: Three cases of large prenatally detected adrenal masses managed nonoperatively are reported. Data studied were: age at the diagnosis, prenatal and postnatal ultrasonographic consistency, and tumor marker levels. Size and sonographic evolution were also studied. RESULTS: In all 3 cases, a cystic mass, measuring more than 40 mm in size, was detected during the 3rd trimester of pregnancy. The sonographic appearance evolved from a sonolucent tumor to a heterogeneous mass with hyperechoic areas. The tumor marker levels were normal. All infants had a documented decrease in mass size at birth and were managed nonoperatively. All these tumors were considered adrenal hemorrhages. CONCLUSIONS: Prenatal ultrasonography rarely permits to distinguish an adrenal hemorrhage from a cystic neuroblastoma. The differential diagnosis, even in large masses, is based on close postnatal follow-up with serial sonography. Surgery is not mandatory, unless the size does not decrease. However, without pathologic proof, it is not possible to differentiate an adrenal hemorrhage from a spontaneously resolved neuroblastoma.

Familial orofaciodigital syndrome type I revealed by ultrasound prenatal diagnosis of porencephaly.

Porencephaly is a rare central nervous system (CNS) abnormality that can be caused by an intraparenchymal destructive process or a developmental defect. Here we report on a prenatal ultrasound diagnosis of complex CNS abnormalities including agenesis of the corpus callosum, agenesis of the cerebellar vermis, bilateral hydrocephaly, and bilateral porencephaly in fetus at 33 weeks' gestation. The diagnosis of familial orofaciodigital syndrome type I (OFD I) was raised after fetal autopsy, clinical examination of the family, and the X-linked dominant inheritance pattern. This is the fourth report of porencephaly in association with OFD I. We discuss the difficulties in genetic counselling since OFD I shows variable expressivity of the phenotypic features. Furthermore, we emphasize the importance of a detailed ultrasound examination after a prenatal diagnosis of porencephaly.

Functional, biochemical and molecular biological evidence for a possible beta(3)-adrenoceptor in human near-term myometrium.

The possible existence of a beta(3)-adrenoceptor (beta(3)-AR) in human near-term myometrium was investigated by in vitro functional and biochemical studies and analysis of mRNA expression. SR 59119A and SR 59104A and CGP 12177 (two selective agonists and a partial agonist, respectively, of the beta(3)-AR), salbutamol and terbutaline (beta(2)-AR agonists) each produced a concentration-dependent relaxation of the myometrial spontaneous contractions. There were no differences in pD(2) values for the relaxing potencies of terbutaline, salbutamol, CGP 12177 and SR 59119A. The rank order for their relaxing efficacies was SR 59119A>SR 59104A>terbutaline approximately salbutamol approximately CGP 12177 (E(max)=52+/-7%, 42+/-12% and approximately 30% respectively). Propranolol, a beta(1)- and beta(2)-AR antagonist, and ICI 118551, a beta(2)-AR antagonist (both at 0.1 microM), did not affect the SR 59119A-induced relaxation whereas SR 59230A, a selective beta(3)-AR antagonist (1 microM), significantly reduced the maximal relaxing effect of SR 59119A. SR 59119A and salbutamol induced a significant increase in cyclic AMP levels that was antagonized by SR 59230A but not by propranolol for SR 59119A, and by propranolol but not by SR 59230A for salbutamol. The beta(3)-AR mRNA was positively expressed in myometrium preparations in a reverse transcription polymerase chain assay. The results presented provide the first evidence for the existence of the beta(3)-AR subtype in human near-term myometrium and suggest that the effects of SR 59119A might be mediated through an increase in cyclic AMP level.

Transabdominal amnioinfusion to avoid fetal demise and intestinal damage in fetuses with gastroschisis and severe oligohydramnios.

BACKGROUND/PURPOSE: Despite dramatic improvement in survival rate for neonates with gastroschisis, significant postoperative morbidity and a low mortality rate still occur. Furthermore, even in recent publications, some fetal death has been reported. Does this mean that antenatal diagnosis of gastroschisis is a missed opportunity? In fact, decreased amniotic fluid (AF) volume is observed in some fetuses with gastroschisis. However, oligohydramnios is associated with an increased risk of fetal suffering. When severe oligohydramnios is observed, intrapartum amnioinfusion, to restore AF volume, may help avoid fetal complications. METHODS: Two fetuses with gastroschisis and severe oligohydramnios were treated antenatally with amnioinfusion of saline solution. In one case, fetal heart beat decelerations were observed at 27 weeks' gestation among with the oligohydroamnios and serial transabdominal amnioinfusions were performed. In the second case, severe oligohydramnios was observed at 31, weeks and an amnioinfusion was performed. The 2 babies were delivered at 31 and 34 weeks, respectively. RESULTS: In both cases, exteriorized bowel was nearly normal at birth, and primary closure could be performed. Outcome was favorable, and they were discharged home on day 43 and day 54, respectively. CONCLUSIONS: Because fetuses with gastroschisis and oligohydramnios are part of a particular high-risk group, serial ultrasound examination and computerized fetal heart beat monitoring are necessary during the third trimester. In selected cases of gastroschisis associated with severe oligohydramnios, serial amnioinfusion may be required.

Severe fetal cytomegalic inclusion disease after documented maternal reactivation of cytomegalovirus infection during pregnancy.

Recurrent cytomegalovirus infection during pregnancy is considered less dangerous for the fetus than primary infection. We present a case of severe fetal cytomegalic inclusion disease after maternal reactivation of cytomegalovirus during the first trimester of pregnancy. The possibility of such fetal injury is an argument for prenatal diagnosis in seropositive pregnant women when ultrasonographic findings suggest cytomegalovirus infection.