[Contraception: CNGOF Guidelines for Clinical Practice (Short Version)]. / Contraception : Recommandations pour la Pratique Clinique du CNGOF (texte court).

The French College of Obstetrics and Gynecology (CNGOF) releases its first global recommendations for clinical practice in contraception, to provide physicians with an updated synthesis of available data as a basis for their practice. The French Health Authority (HAS) methodology was used. Twelve practical issues were selected by the organizing committee and the task force members. The available literature was screened until December 2017, and allowed the release of evidence-based, graded recommendations. This synthesis is issued from 12 developed texts, previously reviewed by experts and physicians from public and private practices, with an experience in the contraceptive field. Male and female sterilization, as well as the use of hormonal treatments without contraceptive label were excluded from the field of this analysis. Specific practical recommendations on the management of contraception prescription, patient information including efficacy, risks, and benefits of the different contraception methods, follow up, intrauterine contraception, emergency contraception, local and natural methods, contraception in teenagers and after 40, contraception in vascular high-risk situations, and in case of cancer risk are provided. The short/mid-term future of contraception mostly relies on improving the use of currently available methods. This includes reinforced information for users and increased access to contraception for women, whatever the social and clinical context. That is the goal of these recommendations.

[Contraception for adolescent : CNGOF Contraception guidelines]. / Contraception de l'adolescente. RPC Contraception CNGOF.

OBJECTIVE: The goal is to establish dialogue and determine the needs and skill levels of adolescence. This concerns sexuality, the prevention of STIs, the informed choice of contraception to avoid an unplanned pregnancy. MéTHODES: A systematic review based on literature about contraception AND teenagers was performed using Pubmed, Cochrane, national and international recommendations. RESULTS: The surveillance of the teenager contraception must integrate more specifically: global health with a stability of weight and corpulence, a sufficient calcium intake, the prevention of the sexually transmitted infections (STIs) and the vaccination against HPV. The 1st consultations with adolescent girls are an essential moment for dialogue in order to develop sexuality education. Main themes are: prevention of STIs with the use of condoms, detection of situations of precariousness or sexual abuse, and finally adherence to treatment to avoid unplanned pregnancy. Use of condoms associated with regular contraception is essential to assure a barrier against sexually transmitted infections (STIs) (NP1). To preserve the patient confidentiality, the patient is received alone (Grade B). She must be reassured about respect of anonymity and availability of free treatment. Clinical examination collects weight, height, BMI and blood pressure (Grade C). It is important to give them the choice of contraceptive method and provide objective information on the different contraceptive methods (NP2). If there are any contraindications, when the first prescription is a pill, it must be a 1st or 2nd generation pill with levonorgestrel. For some experts, it would be important to prescribe a pill at 30µg EE for better efficacy in case of forgetfulness in very young patients and for the good maintenance of bone mineralization (NP4). Information on long-acting reversible contraceptives, or LARCs, is essential. These contraceptive methods have proved their efficacy and their place in the first intention. (NP1). CONCLUSION: Prescribing contraception to a teenage girl requires the adaptation of the best treatment to her needs to prevent an unwanted pregnancy. This requires good information on prevention of STIs and on different methods of contraception in a confidence climate.

[Long-term outcomes after hypospadias surgery: Sexual reported outcomes and quality of life in adulthood]. / Vie sexuelle des jeunes adultes opérés dans l'enfance d'un hypospadias postérieur.

OBJECTIVE: To evaluate outcomes and long-term sexual quality of life after hypospadias surgery. Seventeen-years-old patients operated for a posterior hypospadias in childhood were included in a transversal study. PATIENTS AND METHODS: Fifteen patients, among the forty children treated since 1997, accepted to participate. These young men (mean age at the first surgery was 27.9±20months) were clinically reviewed and responded to questionnaires (EUROQOL 5, IIEF15 and non-validated questionnaire). This study arises about 8.4±5years after the last visit in paediatric department. RESULTS: Mean study age was 21.2±4.7years. One third of patients thought that global quality of life was distorted. Although 33% of the patients had erectile dysfunction, 80% were satisfied with their sexual quality of life. The most important complains were relative to the penile appearance. Number of procedures was not predictive of patient's satisfaction about penile function and appearance. Thirty-three percents of the patients would have been satisfied to have psychological and medical support. They would be interested in having contact with patients who suffered from the same congenital abnormality. CONCLUSION: These patients had functional and esthetical disturbances. This visit leads to a specific visit in 20% cases. In this study, medical follow-up does not seem to be counselling and had to be adapted. Adequate follow-up transition between paediatric and adult departments especially during adolescence seems to be necessary.

Management of presumed benign ovarian tumors: updated French guidelines.

Transvaginal pelvic ultrasound is the first-line imaging examination for presumed benign ovarian tumors (PBOT) in adult women (Grade A). Ultrasound is sufficient for characterizing a unilocular anechoic cyst smaller than 7cm (Grade A). Magnetic resonance imaging is the recommended second-line investigation for indeterminate masses or masses larger than 7cm (Grade B). Serum CA-125 assay is not recommended for first-line diagnosis in adult women (Grade C). In women with a unilocular anechoic cyst, hormone therapy is ineffective and not recommended (Grade A). Ultrasound-guided aspiration is not recommended (Grade B). Abstention is an option in adult women with a unilocular asymptomatic anechoic cyst smaller than 10cm and no history of cancer (Grade B). If symptoms develop, laparoscopy is the gold standard for surgical treatment of PBOT (Grade A). Conservative surgical treatment (cystectomy) should be preferred to oophorectomy in pre-menopausal women without a previous history of cancer (Grade C). In cases of suspected adnexal torsion, laparoscopic surgical exploration is recommended (Grade B). Conservative treatment or detorsion without oophorectomy is recommended for pre-menopausal women regardless of the estimated torsion duration and macroscopic appearance of the ovary (Grade B). During pregnancy, expectant management is recommended for unilocular asymptomatic anechoic cysts smaller than 6cm (Grade C).

Benefits of primary surgical resection for symptomatic urethral prolapse in children.

OBJECTIVE: Urethral prolapse (UP) is a complete eversion of the distal urethral mucosa through the external meatus. UP must be distinguished by examination from trauma, prolapsed ureterocele, tumors or sexual abuse. Its management remains controversial. The aim of the study was to promote the benefits of primary surgical management for UP. METHODS: A retrospective multicenter review of children who received surgery for UP between 1991 and 2011 was carried out. Non-complicated UP was primarily treated conservatively. A total of 19 patients were referred for complicated UP and underwent resection of the prolapsed urethral mucosa. RESULTS: The mean delay in diagnosis was 2.2 days (range 1-6) and the most common symptoms were vaginal spotting and bleeding. No predisposing factor was found, but most patients had a mean weight, height and BMI greater than the 50th percentile. All patients underwent surgery successfully. One patient experienced a complication, i.e., dysuria. There was no case of recurrence after a mean 28 months of follow-up. CONCLUSION: Early detection is based on bedside examination. The first-line treatment strategy for uncomplicated UP should be conservative management. Surgical resection is safe and effective for patients with significant symptoms.

[Presumed benign ovarian tumors of childhood and adolescent]. / Tumeurs ovariennes présumées bénignes de l'enfant et l'adolescente.

Ovarian tumors in childhood are rare, often organic with 10% of malignant cases. Functional pathology dominates in adolescence and its management is the same as the adult. The clinical symptoms of PBOT (presumed benign ovarian tumor) are non-specific. The main clinical signs are acute pain, associated with peritoneal irritation syndrome, which can suggest an ovarian torsion, a mass or the development of secondary sexual characters. Hyperestrogenemia suggests a McCune-Albright syndrome or a granulosa tumor. Hyperandrogenism evokes a malignant tumor. Pelvic ultrasound is the main examination. Pure liquid cysts are benign but could be organic if persisting beyond 6 months. MRI and tumor markers are needed for heterogeneous cyst diagnosis. The protected extraction of a cyst is recommended during the laparoscopic cystectomy. If case of doubt of malignancy, laparoscopy allows the peritoneal cavity exploration. In case of torsion, ovarian untwisting must be performed. After untwisting, the ovary must be preserved because macroscopic aspect is not predictive of the ovarian function recovery. No medical treatment is effective. After resection, US follow up is required for five years.

[Urogenital bleeding revealing urethral prolapse in a prepubertal girl]. / Hémorragie génitale de la petite fille: penser au prolapsus urétral.

Urethral prolapse is a complete eversion of the distal urethral mucosa through the external meatus. It occurs primarily in prepubertal, primarly Black girls. Its pathophysiology has not been clearly identified. We report a case of a 5-year-old girl who came to the Emergency Department with a 1-day history of genital pain and "vaginal bleeding". Early recognition makes differential diagnosis with sexual abuse and staging allows prompt management under general anesthesic like prolapse reduction or surgical excision.

Ovarian cysts in prepubertal girls.

Visualization of follicles is perfectly physiological during childhood, their diameter generally does not exceed 10 mm. Ovarian cyst in childhood is well defined for a fluid image >20 mm. Generally mild and asymptomatic, ovarian cysts are fluid formations usually discovered incidentally by ultrasound. Some are hormonally active and cause the development of sexual characteristics. The natural history of functional cysts is eventual regression, and persistence is suggestive of organic tumor. The onset of pain is a sign of complication, and an abrupt sharp pain with vomiting is suggestive of ovarian torsion, in which case surgical intervention is urgent. In all cases, the diagnosis is based on pelvic ultrasound. MRI and tumor marker assays are required to determine the nature of an organic cyst before proceeding to surgery. These cysts may appear functional from the fetal period onward and will require management from the first days of life. Certain endocrine disorders such as precocious puberty, hypothyroidism, and aromatase deficiency cause functional cysts in girls. Recurrent bleeding is due to hormonally active cysts and suggests McCune-Albright syndrome. Although the persistence of a cyst suggests a neoplasm, a fluid character indicates benignity. Imagery is a useful aid in the diagnosis of epithelial tumors (cystadenomas) or teratoma (dermoid cyst).

Ovarian masses in adolescent girls.

Ovarian masses are the most frequent gynecological pathology seen in adolescent girls. Functional or organic tumors of the ovary are usually benign and the incidence rises with age. Most cysts are functional and adnexal torsion is the main complication, but a malignant etiology must nevertheless always be eliminated. The clinical presentation is quite variable. Ultrasonography is the investigation of choice: the sonogram will reveal a strictly fluid, benign functional cyst, suggest an adnexal torsion, and provide evidence of a heterogeneous mass. Emergency surgery is indicated only in the case of suspected ovarian torsion, in order to perform detorsion. In all other cases, serum tumor marker measurements will orient the diagnosis and MRI is an essential complement to imaging of tumors with heterogeneous solid components. Surgery and histopathological examination then determine the stage and the benign or malignant nature of the mass. Ovarian tumors are classified by the World Health Organization based on the cell of origin into epithelial tumors, germ cell tumors and sex cord-stromal tumors. Surgery should always follow oncological standards and be as conservative as possible to preserve future fertility.

[Klinefelter's syndrome and Turner's syndrome. For a better management]. / Syndrome de Klinefelter et syndrome de Turner : pour une meilleure prise en charge.

Klinefelter's syndrome (KS) affects one in 600 men and Turner's syndrome (TS), one in 2500 women. These 2 diseases are the most sex chromosome disorders characterized by one extra X in the SK male (47XXY) and the loss of an X in the girls with ST (45 X). Their common characteristic is the gonadal dysgenesis, which is the main cause of male or female infertility. Called "the forgotten syndrome", KS is under-diagnosed because apart from the large size, there are no dysmorphic features, along with a great ignorance of cognitive and language disorders in children. There are often comorbidities that lead to diagnosis such as autoimmune diseases or metabolic syndrome. TS is often diagnosed by the short stature. Management of Turner's girls has profoundly changed with Growth hormone therapy. There is an international consensus for a better management of associated diseases such as ORL, cardiac, renal, hepatic, autoimmune and metabolic diseases. Prenatal diagnosis allows early detection and management of cognitive deficiencies and of associated diseases.

[Central precocious puberty due to hypothalamic hamartoma in Cameroon]. / Puberté précoce de cause rare au Cameroun: L'Hamartome hypothalamique.

Hypothalamic hamartoma is an uncommon cause of central precocious puberty and sometimes of gelastic epilepsy and delayed development. The purpose of this report is to describe a case of central precocious puberty in an 18-month-old girl who was referred to our department for further investigation of vaginal bleeding that had appeared during the 8th month of life. The patient's puberty was compatible with Tanner stage 3, her psychomotor development was normal, and epilepsy was not observed. Diagnosis was based on abnormal hormonal workup findings and discovery of a hypothalamic hamartoma on brain magnetic resonance imaging. Neurosurgery was not performed. Administration of long-acting gonadotropin releasing hormone analog to slow puberty led to clinical improvement. Hypothalamic hamartoma must always be suspected in case of central precocious puberty. Although magnetic resonance imaging is the most reliable modality, it is not always available nor affordable in low-income countries. Neurosurgery is not indicated in patients without epileptic seizure. Gonadotropin releasing hormone agonists are usually indicated to slow down puberty and improve the adult height. However, the cost is prohibitive in developing countries.

Undervirilization in XY newborns may hide a 5α-reductase deficiency: report of three new SRD5A2 gene mutations.

The observation of ambiguous genitalia in the newborn signals a medical, surgical and psychological emergency. The most crucial decision will be the choice of sex assignment. Rapid and precise diagnosis is thus essential. In XY newborns with normal/high plasma testosterone (T), partial androgen insensitivity syndrome (PAIS) is usually the first diagnosis evoked, which implies an androgen receptor (AR) defect. The diagnosis of steroid-5-alpha-reductase deficiency is rarely considered by the paediatrician. We report three new SRD5A2 gene mutations in four newborns from France, Morocco and Turkey. The newborns presented with ambiguous genitalia and normal plasma T values and the initial diagnosis\PAIS. In all four cases, normal sequences of the complete AR gene excluded this diagnosis and raised the hypothesis of 5α-reductase deficiency. The entire coding region (5 exons) of the SRD5A2 gene was assessed by PCR and direct sequencing analysis. For patient 1, we identified a new homozygous 2bp deletion in exon 1 (c.122_123delAG). Patient 2 had a known homozygous mutation, p.G115D, in exon 2. New compound heterozygous mutations in exon 4 (p.A215V) and exon 5 (p.X255Q) were found in patient 3. Patient 4 presented a new substitution in exon 1 (p.S14R) associated with a known polymorphism (p.V89L). Our data confirm our previous experience and clearly demonstrate that a 5-α reductase defect should be considered in all XY newborns with ambiguous genitalia and normal plasma T secretion, whatever their geographic area or ethnic group; moreover, this defect was not linked to specific phenotype. Early molecular diagnosis is indispensable for the crucial decision of the newborn's sex of rearing.

[Juvenile granulosa-cell tumor: clinical and molecular expression]. / Tumeurs juvéniles de la granulosa: expression clinique et moléculaire.

Ovarian sex cord-stromal tumors are rare tumors that originate from the nongerminal cells of ovary. Two decades ago, the identification of juvenile granulosa-cell tumors (GCT), as a specific entity inside this group, allowed a better treatment of these tumors in children. However, little data have been reported on the natural course of the disease and reliable prognostic factors have not been yet defined. We here review the clinical and genetics aspects of granulosa tumors, based on a series of 40 children. This national collaborative study involved the French Society of Children Cancer and eight clinical departments of pediatric endocrinology. We found that early diagnosis of a tumor, revealed by clinical signs of hyperoestrogeny, is an important prognostic factor. The pathophysiology of these tumors is still debatable and several cellular- and molecular-abnormal signals could be implicated in their development. The role of growth factors and oncogenes through the signaling pathway of MAP kinase is still discussed. According to our data, FSH signaling-transduction pathway, such as a constitutionally activated Galphas, could also be implicated in the induction of granulosa cell proliferation and seems to modulate the invasiveness of the tumor. Last, we have described a low-expression pattern or an extinction of an ovarian-determination gene, FOXL2, which is related to a worse prognosis of this tumor.

[Construction of a neo-vagina by sigmoidocolpoplasty]. / Reconstruction vaginale par sigmoïdoplastie pour aplasie utérovaginale: Technique chirurgicale et avantages.

Construction of a neo-vagina by sigmoidocolpoplasty has been effective in the treatment of vaginal aplasia, a condition most commonly seen with the Mayer Rokitansky Syndrome. This article describes the surgical technique and principal complications, and reviews the literature to compare this technique with other methods of repair, particularly the Davydov technique.

[Turner's syndrome and procreation. Ovarian function and Turner's syndrome]. / Syndrome de Turner et procréation. Fonction ovarienne et syndrome de Turner.

Ovarian failure is a typical feature in Turner's syndrome. The majority of follicles disappears prematurely after a normal determination of the ovary. This results from an accelerated loss of oocytes from the ovaries after the 18th week of fetal life or over a few postnatal years, usually before the onset of puberty. The cause and mechanism of this loss are unknown. X chromosomal anomaly due to deletions or haploinsufficiency of genes can explain the various degrees of ovarian failure. Spontaneous puberty occurs in 20-30% of Turner syndrome patients and their fertility rates vary from 5 to 10%. This indicates the possible presence and maturation of follicles in their ovaries in adolescence. In ovarian failure, the hormone replacement therapy (HRT) is necessary to achieve the development of normal female sexual characteristics, the self image or social functioning and to prevent osteoporosis. Pregnancy is now possible with oocyte donation. A careful cardiovascular follow-up is necessary. Cryoconservation represents one way for preserving the future fertility, but the optimal age of ovarian biopsy has to be studied.