Case Report of an exceptional spontaneous abdominal heterotopic pregnancy with superfetation: Diagnosis and treatment: Heterotopic pregnancy with superfetation (8+1 WG & 5+4 WG).

Heterotopic pregnancy is an extremely rare condition in which an intrauterine and an extrauterine pregnancy co-exist. In spontaneous conceptions, heterotopic pregnancy occurs in only 1/30 000 pregnancies. The treatment of heterotopic pregnancy must be as minimally invasive as possible to preserve the development of the intrauterine pregnancy. Superfetation, defined as the coexistence of 2 or more foetuses of different gestational ages, remains particularly exceptional and poorly explained (second ovulation? embryonic diapause?). Here, we present an extremely rare case of a spontaneous heterotopic evolutive pregnancy with superfetation, consisting of an embryo in the pouch of Douglas estimated at 8 + 1 weeks of gestation (WG) and a progressive intrauterine pregnancy estimated at 5 + 4 WG. We treated the extrauterine pregnancy with an intra-cardiac injection of potassium chloride echo-guided via the vaginal route, and the patient then underwent exploratory laparoscopy 9 days later and lavage and aspiration of the abdominal heterotopic pregnancy due to pain and biological inflammatory syndrome probably caused by pelvic mass syndrome and peritoneal irritation from the foetal necrosis. She has not yet given birth and is currently at 36 WG.

Management of women with abnormal uterine bleeding: Clinical practice guidelines of the French National College of Gynaecologists and Obstetricians (CNGOF).

OBJECTIVE: To provide French guidelines for the management of women with abnormal uterine bleeding (AUB). DESIGN: A consensus committee of 26 experts was formed. A formal conflict-of-interest policy was developed at the beginning of the process and enforced throughout. The entire guidelines process was conducted independently of any industry funding (i.e. pharmaceutical or medical device companies). The authors were advised to follow the rules of the Grading of Recommendations Assessment, Development and Evaluation (GRADE®) system to guide assessment of quality of evidence. The potential drawbacks of making strong recommendations in the presence of low-quality evidence were emphasized. METHODS: The last guidelines from the Collège National des Gynécologues et Obstétriciens Français on the management of women with AUB were published in 2008. The literature seems now sufficient for an update. The committee studied questions within 7 fields (diagnosis; adolescents; idiopathic AUB; endometrial hyperplasia and polyps; type 0-2 fibroids; type 3 or higher fibroids; and adenomyosis). Each question was formulated in a PICO (Patients, Intervention, Comparison, Outcome) format and evidence profiles were compiled. The GRADE® methodology was applied to the literature review and the formulation of recommendations. RESULTS: The experts' synthesis work and the application of the GRADE method resulted in 36 recommendations. Among the formalized recommendations, 19 are strong and 17 weak. No response was found in the literature for 14 questions. We chose to abstain from recommendations rather than providing advice based solely on expert clinical experience. CONCLUSIONS: The 36 recommendations make it possible to specify the diagnostic and therapeutic strategies for various clinical situations practitioners encounter, from the simplest to the most complex.

[Management of women with abnormal uterine bleeding: Clinical practice guidelines of the French National College of Gynecologists and Obstetricians (CNGOF)]. / Prise en charge des ménorragies : recommandations pour la pratique clinique du Collège national des gynécologues et obstétriciens français (CNGOF).

OBJECTIVE: To provide French guidelines for the management of women with abnormal uterine bleeding (AUB). DESIGN: A consensus committee of 26 experts was formed. A formal conflict-of-interest (COI) policy was developed at the beginning of the process and enforced throughout. The entire guidelines process was conducted independently of any industrial funding (i.e. pharmaceutical, or medical devices). The authors were advised to follow the rules of the Grading of Recommendations Assessment, Development and Evaluation (GRADE®) system to guide assessment of quality of evidence. The potential drawbacks of making strong recommendations in the presence of low-quality evidence were emphasized. METHODS: The last guidelines from the Collège national des gynécologues et obstétriciens français (CNGOF) on the management of women with AUB was published in 2008. The literature seems now sufficient for an update. The committee studied questions within 7 fields (diagnosis; adolescent; idiopathic AUB; endometrial hyperplasia and polyps; fibroids type 0 to 2; fibroids type 3 and more; adenomyosis). Each question was formulated in a PICO (Patients, Intervention, Comparison, Outcome) format and the evidence profiles were produced. The literature review and recommendations were made according to the GRADE® methodology. RESULTS: The experts' synthesis work and the application of the GRADE method resulted in 36 recommendations. Among the formalized recommendations, 19 present a strong agreement and 17 a weak agreement. Fourteen questions did not find any response in the literature. We preferred to abstain from recommending instead of providing expert advice. CONCLUSIONS: The 36 recommendations made it possible to specify the diagnostic and therapeutic strategies of various clinical situations managed by the practitioner, from the simplest to the most complex.

Prise en charge des ménorragies : recommandations pour la pratique clinique du Collège National des Gynécologues et Obstétriciens Français (CNGOF) [Pré-print] / Management of women with abnormal uterine bleeding: clinical practice guidelines of the French National College of Gynecologists and Obstetricians (CNGOF) [Pré-print]

Émettre des recommandations pour la prise en charge des femmes ayant des ménorragies. Un comité de 26 experts a été constitué. Une politique de déclaration et de suivi des liens d'intérêts a été appliquée et respectée durant tout le processus de réalisation du référentiel. De même, celui-ci n'a bénéficié d'aucun financement provenant d'une entreprise commercialisant un produit de santé (médicament ou dispositif médical). Le comité devait respecter et suivre la méthode GRADE® (Grading of Recommendations Assessment, Development and Evaluation) pour évaluer la qualité des données factuelles sur lesquelles étaient fondées les recommandations. Les dernières recommandations du Collège National des Gynécologues et Obstétriciens Français (CNGOF) sur la prise en charge des femmes ayant des ménorragies ont été publiées en 2008. Nous avons souhaité réactualiser ces recommandations selon la méthodologie GRADE® en identifiant 7 champs différents (diagnostic ; adolescente ; ménorragies idiopathiques ; hyperplasie et polype de l'endomètre ; myomes de type 0 à 2 ; myomes de type 3 et plus ; adénomyose). Chaque question a été formulée selon le format PICO (Patients, Intervention, Comparison, Outcome). L'analyse de la littérature et les recommandations ont été formulées selon la méthodologie GRADE. Le travail de synthèse des experts et l'application de la méthode GRADE ont abouti à 36 recommandations. Parmi les recommandations, 19 ont été établies avec un accord fort et 17 avec un accord faible. Il n'a pas été possible de statuer pour 14 questions pour lesquelles nous avons préféré nous abstenir plutôt que de fournir des avis d'experts.Les 36 recommandations ont permis de préciser les stratégies diagnostiques et thérapeutiques des différentes situations cliniques des plus simples au plus complexes rencontrées par le praticien.

To provide French guidelines for the management of women with abnormal uterine bleeding (AUB). A consensus committee of 26 experts was formed. A formal conflict-of-interest (COI) policy was developed at the beginning of the process and enforced throughout. The entire guidelines process was conducted independently of any industrial funding (i.e. pharmaceutical, or medical devices). The authors were advised to follow the rules of the Grading of Recommendations Assessment, Development and Evaluation (GRADE®) system to guide assessment of quality of evidence. The potential drawbacks of making strong recommendations in the presence of low-quality evidence were emphasized. The last guidelines from the Collège National des Gynécologues et Obstétriciens Français (CNGOF) on the management of women with AUB was published in 2008. The literature seems now sufficient for an update. The committee studied questions within 7 fields (diagnosis; adolescent; idiopathic AUB; endometrial hyperplasia and polyps; fibroids type 0 to 2; fibroids type 3 and more; adenomyosis). Each question was formulated in a PICO (Patients, Intervention, Comparison, Outcome) format and the evidence profiles were produced. The literature review and recommendations were made according to the GRADE® methodology. The experts' synthesis work and the application of the GRADE method resulted in 36 recommendations. Among the formalized recommendations, 19 present a strong agreement and 17 a weak agreement. Fourteen questions did not find any response in the literature. We preferred to abstain from recommending instead of providing expert advice. The 36 recommendations made it possible to specify the diagnostic and therapeutic strategies of various clinical situations managed by the practitioner, from the simplest to the most complex.

[Guidelines for Clinical Practice of the French College of Obstetricians and Gynecologists 2021: Prophylactic procedures associated with gynecologic surgery]. / Recommandations pour Pratique Clinique du Collège des Gynécologues Obstétriciens français 2021 : gestes prophylactiques associés en chirurgie gynécologique.

OBJECTIVE: To draw up recommendations on the use of prophylactic gynecologic procedures during surgery for other indications. DESIGN: A consensus panel of 19 experts was convened. A formal conflict of interest policy was established at the onset of the process and applied throughout. The entire study was performed independently without funding from pharmaceutical companies or medical device manufacturers. The panel applied the GRADE (Grading of Recommendations Assessment, Development and Evaluation) system to evaluate the quality of evidence on which the recommendations were based. The authors were advised against making strong recommendations in the presence of low-quality evidence. Some recommendations were ungraded. METHODS: The panel studied 22 key questions on seven prophylactic procedures: 1) salpingectomy, 2) fimbriectomy, 3) salpingo-oophorectomy, 4) ablation of peritoneal endometriosis, 5) adhesiolysis, 6) endometrial excision or ablation, and 7) cervical ablation. RESULTS: The literature search and application of the GRADE system resulted in 34 recommendations. Six were supported by high-quality evidence (GRADE 1+/-) and 28 by low-quality evidence (GRADE 2+/-). Recommendations on two questions were left ungraded due to a lack of evidence in the literature. CONCLUSIONS: A high level of consensus was achieved among the experts regarding the use of prophylactic gynecologic procedures. The ensuing recommendations should result in improved current practice.

Borderline ovarian tumors: Guidelines from the French national college of obstetricians and gynecologists (CNGOF).

It is recommended to classify Borderline Ovarian Tumors (BOTs) according to the WHO classification. Transvaginal and suprapubic ultrasonography are recommended for the analysis of an ovarian mass (Grade A). In case of an undetermined ovarian lesion on ultrasonography, it is recommended to perform a pelvic MRI (Grade A) with a score for malignancy (ADNEX MR/O-RADS) (Grade C) included in the report and to formulate a histological hypothesis (Grade C). Pelvic MRI is recommended to characterize a tumor suspected of being BOT (Grade C). It is recommended to evaluate serum levels of HE4 and CA125 and to use the ROMA score for the diagnosis of indeterminate ovarian mass on imaging (grade A). If there is a suspicion of a mucinous BOT on imaging, serum levels of CA 19-9 may be proposed (Grade C). For Early Stages (ES) of BOT, if surgery without risk of tumor rupture is possible, laparoscopy with protected extraction is recommended over laparotomy (Grade C). For treatment of a bilateral serous ES BOT with a strategy to preserve fertility and/or endocrine function, bilateral cystectomy is recommended where possible (Grade B). For mucinous BOTs with a treatment strategy of fertility and/or endocrine function preservation, unilateral salpingo-oophorectomy is recommended (grade C). For mucinous BOTs treated by initial cystectomy, unilateral salpingo-oophorectomy is recommended (grade C). For serous or mucinous ES BOTs, routine hysterectomy is not recommended (Grade C). For ES BOTs, lymphadenectomy is not recommended (Grade C). For ES BOTs, appendectomy is recommended only in case of a macroscopically pathological appendix (Grade C). Restaging surgery is recommended in cases of serous BOTs with micropapillary architecture and an incomplete abdominal cavity inspection during initial surgery (Grade C). Restaging surgery is recommended for mucinous BOTs after initial cystectomy or in cases where the appendix was not examined (Grade C). If restaging surgery is decided for ES BOTs, the following procedures should be performed: peritoneal washing (grade C), omentectomy (grade B), complete exploration of the abdominal cavity with peritoneal biopsies (grade C), visualization of the appendix and appendectomy in case of a pathological macroscopic appearance (grade C) as well as unilateral salpingo-oophorectomy in case of a mucinous BOT initially treated by cystectomy (grade C). In advanced stages (AS) of BOT, it is not recommended to perform a lymphadenectomy as a routine procedure (Grade C). For AS BOT in a patient with a desire to fall pregnant, conservative treatment involving preservation of the uterus and all or part of the ovary may be proposed (Grade C). Restaging surgery aimed at removing all lesions, not performed initially, is recommended for AS BOTs (Grade C). After treatment, follow-up for a duration greater than 5 years is recommended due to the median recurrence time of BOTs (Grade B). It is recommended that a systematic clinical examination be carried out during follow-up of a treated BOT (Grade B). If the determination of tumor markers is normal preoperatively, the routine dosage of tumor markers in BOT follow-up is not recommended (Grade C). In case of an initial elevation in serum CA 125 levels, it is recommended to monitor CA 125 during follow up (Grade B). In case of conservative treatment, it is recommended to use transvaginal and transabdominal ultrasound during follow up of a treated BOT (Grade B). In the event of a BOT recurrence in a woman of childbearing age, a second conservative treatment may be proposed (Grade C). A consultation with a physician specialized in Assisted Reproductive Technique (ART) should be offered in the case of BOTs in women of childbearing age (Grade C). When possible, a conservative surgical strategy is recommended to preserve fertility in women of childbearing age (Grade C). In the case of optimally treated BOT, there is no evidence to contraindicate the use of ART. The use of hormonal contraception after serous or mucinous BOT is not contraindicated (Grade C). After management of mucinous BOT, for women under 45 years, given the benefit of Hormonal Replacement Therapy (HRT) on cardiovascular and bone risks, and the lack of hormone sensitivity of mucinous BOTs, it is recommended to offer HRT (Grade C). Over 45 years of age, HRT can be prescribed in case of a climacteric syndrome after individual benefit to risk assessment (Grade C).

Management of borderline ovarian tumours during pregnancy: Results of a French multi-centre study.

OBJECTIVE: To assess the diagnostic and prognostic characteristics of borderline ovarian tumours (BOTs) detected during pregnancy, and to establish an inventory of French practices. MATERIALS AND METHODS: A retrospective multi-centre case study of 14 patients treated for BOTs, diagnosed during pregnancy between 2005 and 2017, in five French pelvic cancerology expert centres, including data on clinical characteristics, histological tumour characteristics, surgical procedure, adjuvant treatments, follow-up and fertility. RESULTS: The mean age of patients was 29.3 [standard deviation (SD) 6.2] years. Most BOTs were diagnosed on ultrasonography in the first trimester (85.7 %), and most of these cases (78.5 %) also underwent magnetic resonance imaging to confirm the diagnosis (true positives 54.5 %). Most patients underwent surgery during pregnancy (57 %), with complete staging surgery in two cases (14.3 %). Laparoscopy was performed more frequently than other procedures (50 %), and unilateral adnexectomy was more common than cystectomy (57.5 %). Tumour size influenced the surgical approach significantly (mean size 7.5 cm for laparoscopy, 11.9 cm for laparoconversion, 14 cm for primary laparotomy; P = 0.08), but the type of resection did not. Most patients were initially diagnosed with International Federation of Gynecology and Obstetrics stage IA (92.8 %) tumours, but many were upstaged after complete restaging surgery (57.1 %). Most BOTs were serous (50 %), two cases had a micropapillary component (28.5 %), and one case had a micro-invasive implant. BOTs were bilateral in two cases (14.2 %). Mean follow-up was 31.4 (SD 14.8) months. Recurrent lesions occurred in two patients (14.2 %) and no deaths have been recorded to date among the study population. CONCLUSION: BOTs remain rare, but this study - despite its small sample size - supports the hypothesis that BOTs during pregnancy have potentially aggressive characteristics.

Augmented Reality Guided Laparoscopic Surgery of the Uterus.

A major research area in Computer Assisted Intervention (CAI) is to aid laparoscopic surgery teams with Augmented Reality (AR) guidance. This involves registering data from other modalities such as MR and fusing it with the laparoscopic video in real-time, to reveal the location of hidden critical structures. We present the first system for AR guided laparoscopic surgery of the uterus. This works with pre-operative MR or CT data and monocular laparoscopes, without requiring any additional interventional hardware such as optical trackers. We present novel and robust solutions to two main sub-problems: the initial registration, which is solved using a short exploratory video, and update registration, which is solved with real-time tracking-by-detection. These problems are challenging for the uterus because it is a weakly-textured, highly mobile organ that moves independently of surrounding structures. In the broader context, our system is the first that has successfully performed markerless real-time registration and AR of a mobile human organ with monocular laparoscopes in the OR.

[Guidelines for surgical management of gynaecological cancer during pandemic COVID-19 period - FRANCOGYN group for the CNGOF]. / Prise en charge chirurgicale des cancers gynécologiques en période de pandémie COVID-19 ­ Recommandations du Groupe FRANCOGYN pour le CNGOF.

INTRODUCTION: Recommendations for the management of patients with gynecological cancer during the COVID-19 pandemic period. MATERIAL AND METHOD: Recommendations based on the consensus conference model. RESULTS: In the case of a COVID-19 positive patient, surgical management should be postponed for at least 15 days. For cervical cancer, the place of surgery must be re-evaluated in relation to radiotherapy and Radio-Chemotherapy-Concomitant and the value of lymph node staging surgeries must be reviewed on a case-by-case basis. For advanced ovarian cancers, neo-adjuvant chemotherapy should be favored even if primary cytoreduction surgery could be envisaged. It is lawful not to offer hyperthermic intraperitoneal chemotherapy during a COVID-19 pandemic. In the case of patients who must undergo interval surgery, it is possible to continue the chemotherapy and to offer surgery after 6 cycles of chemotherapy. For early stage endometrial cancer, in case of low and intermediate preoperative ESMO risk, hysterectomy with bilateral annexectomy associated with a sentinel lymph node procedure should be favored. It is possible to consider postponing surgery for 1 to 2 months in low-risk endometrial cancers (FIGO Ia stage on MRI and grade 1-2 endometrioid cancer on endometrial biopsy). For high ESMO risk, it ispossible to favor the MSKCC algorithm (combining PET-CT and sentinel lymph node biopsy) in order to omit pelvic and lumbar-aortic lymphadenectomies. CONCLUSION: During COVID-19 pandemic, patients suffering from cancer should not lose life chance, while limiting the risks associated with the virus.

[Borderline Ovarian Tumours: CNGOF Guidelines for Clinical Practice - Short Text]. / Tumeurs frontières de l'ovaire. Recommandations pour la pratique clinique du CNGOF ­ Texte court.

This work was carried out under the aegis of the CNGOF (Collège national des gynécologues et obstétriciens français) and proposes guidelines based on the evidence available in the literature. The objective was to define the diagnostic and surgical management strategy, the fertility preservation and surveillance strategy in Borderline Ovarian Tumor (BOT). No screening modality can be proposed in the general population. An expert pathological review is recommended in case of doubt concerning the borderline nature, the histological subtype, the invasive nature of the implant, for all micropapillary/cribriform serous BOT or in the presence of peritoneal implants, and for all mucinous or clear cell tumors (grade C). Macroscopic MRI analysis should be performed to differentiate the different subtypes of BOT: serous, seromucinous and mucinous (intestinal type) (grade C). If preoperative biomarkers are normal, follow up of biomarkers is not recommended (grade C). In cases of bilateral early serous BOT with a desire to preserve fertility and/or endocrine function, it is recommended to perform a bilateral cystectomy if possible (grade B). In case of early mucinous BOT, with a desire to preserve fertility and/or endocrine function, it is recommended to perform a unilateral adnexectomy (grade C). Secondary surgical staging is recommended in case of serous BOT with micropapillary appearance and uncomplete inspection of the abdominal cavity during initial surgery (grade C). For early-stage serous or mucinous BOT, it is not recommended to perform a systematic hysterectomy (grade C). Follow up after BOT must be pursued for more than 5 years (grade B). Conservative treatment involving at least the conservation of the uterus and a fragment of the ovary in a patient wishing to conceive may be proposed in advanced stages of BOT (grade C). A new surgical treatment that preserves fertility after a first non-invasive recurrence may be proposed in women of childbearing age (grade C). It is recommended to offer a specialized consultation for Reproductive Medicine when diagnosing BOT in a woman of childbearing age. Hormonal contraceptive use after serous or mucinous BOT is not contraindicated (grade C).

[Borderline Ovarian Tumours: CNGOF Guidelines for Clinical Practice - Therapeutic Management of Early Stages]. / Tumeurs frontières de l'ovaire. Recommandations pour la pratique clinique du CNGOF ­ Prise en charge thérapeutique des stades précoces.

OBJECTIVES: To provide guidelines for clinical practice from the French College of Obstetrics and Gynecology (CNGOF), based on the best evidence available, concerning early stage borderline ovarian tumors (BOT). METHODS: Bibliographical search in French and English languages by consultation of Pubmed, Cochrane, Embase, and international databases. RESULTS: Considering management of early stage BOT, if surgery is possible without a risk of tumor rupture, the laparoscopic approach is recommended compared to laparotomy (Grade C). In BOT, it is recommended to take all the measures to avoid tumor rupture, including the peroperative decision of laparoconversion (Grade C). In BOT, extraction of the surgical specimen using an endoscopic bag is recommended (Grade C). In case of early stage, uni or bilateral BOT, suspected in preoperative imaging in a postmenopausal patient, bilateral adnexectomy is recommended (Grade B). In cases of bilateral BOT and desire of fertility preservation, a bilateral cystectomy is recommended (Grade B). In case of mucinous BOT and desire of fertility preservation, it is recommended to perform a unilateral adnexectomy (Grade C). In case of endometrioid BOT and desire of fertility preservation, it is not possible to establish a recommendation of treatment choice between cystectomy and unilateral adnexectomy. In case of mucinous BOT at definitive histological analysis in a woman of childbearing age who had an initial cystectomy, surgical revision for unilateral adnexectomy is recommended (Grade C). In the case of serous BOT with definitive histological analysis in a woman of childbearing age who has had an initial cystectomy, it is not recommended to repeat surgery for adnexectomy in the absence of residual suspicious lesion during initial surgery and/or on postoperative imaging (referent ultrasound or pelvic MRI) (Grade C). An omentectomy is recommended for complete initial surgical staging when BOT is diagnosed on extemporaneous analysis or suspected on preoperative radiological elements (Grade B). There is no data in the literature to recommend the type of omentectomy to be performed. If restaging surgery is decided for a presumed early stage BOT, an omentectomy is recommended (Grade B). Multiple peritoneal biopsies are recommended for complete initial surgical staging when BOT is diagnosed on extemporaneous or suspected on preoperative radiological elements (Grade C). In case of restaging surgery for a presumed early stage BOT, exploration of the abdominal cavity should be complete and peritoneal biopsies should be performed on suspicious areas or systematically (Grade C). A primary peritoneal cytology is recommended in order to achieve complete initial surgical staging when BOT is suspected on preoperative radiological elements (Grade C). In case of restaging surgery for presumed early stage BOT, a first peritoneal cytology is recommended (Grade C). For early serous or mucinous BOT, it is not recommended to perform a systematic hysterectomy (Grade C). For early stage endometrioid BOT, and in the absence of a desire to maintain fertility, hysterectomy is recommended for initial surgery or if restaging surgery is indicated (Grade C). For endometrioid-type early stage BOT, if there is a desire for fertility preservation, the uterus may be retained subject to good evaluation of the endometrium by imaging and endometrial sampling (Grade C). In case of surgery (initial or restaging if indicated) for early stage BOT, it is recommended to evaluate the macroscopic appearance of the appendix (Grade B). In case of surgery (initial or restaging if indicated) for early stage BOT, appendectomy is recommended only in case of macroscopically pathological appearance of the appendix (Grade C). Pelvic and lumbar aortic lymphadenectomy is not recommended for initial surgery or restaging surgery for early stage BOT regardless of histologic type (Grade C). In case of BOT diagnosed on definitive histology, the indication of restaging surgery should be discussed in Multidisciplinary Collaborative Meeting. For presumed early stage BOT, it is recommended to use the laparoscopic approach to perform restaging surgery (Grade C). Restaging surgery is recommended for serous BOT with micropapillary appearance and unsatisfactory abdominal cavity inspection during initial surgery (Grade C). Restaging surgery is recommended in case of mucinous BOT if only a cystectomy has been performed or the appendix has not been visualized, then a unilateral adnexectomy will be performed (Grade C). If a restaging surgery is decided in the management of a presumed early stage BOT, the actions to be carried out are as follows: a peritoneal cytology (Grade C), an omentectomy (there is no data in the literature recommending the type of omentectomy to be performed) (Grade B), a complete exploration of the abdominal cavity with peritoneal biopsies on suspect areas or systematically (Grade C), visualization of the appendix± the appendectomy in case of pathological macroscopic appearance (Grade C), unilateral adnexectomy in case of mucinous TFO (Grade C).

[Atypical Sézary syndrome in a young subject]. / Syndrome de Sézary atypique chez un sujet jeune.

INTRODUCTION: Sézary syndrome accounts for 5% of cutaneous T-cell lymphomas, with mean age of onset of 60 years. Erythroderma associated with palmoplantar keratoderma and lymphadenopathy is the usual clinical presentation, but the disease has potentially confusing polymorphic clinical features. PATIENTS AND METHODS: We report the case of a 27-year-old patient with no notable disease history, presenting generalized non-pruritic dermatosis for 3 months, with erythema and papules, and follicular distribution, localized to the limbs, the trunk and the face. Palmoplantar keratoderma was associated with acral edema. The clinical presentation was initially evocative of pityriasis rubra pilaris. Laboratory tests showed hyperlymphocytosis with Sézary cells in the blood. A diagnosis of grade IVA Sézary syndrome was made based on the skin biopsy results and the PET scan. Screening for KIR3DL2 on T-cells in blood was positive. Extracorporeal photochemotherapy was initiated but cutaneous relapse occurred, leading to combined treatment with bexarotene, which proved ineffictive. Despite numerous chemotherapies (cyclophosphamide, doxorubicin, vincristine, etoposide and prednisone, then dexamethasone, oxaliplatin and cytarabine, associated with brentuximab, vedotin, and, ultimately, clofarabine and endoxan), the patient died after 9 months. DISCUSSION: Our case illustrates an atypical clinical presentation of cutaneous lymphoma in a young patient. With a fatal outcome in 9 months despite 5 different lines of treatment, our case highlights the aggressive nature of Sézary syndrome as well as the difficulties involved in treating this disease. CONCLUSION: A diagnosis of Sézary syndrome must be considered in the event of atypical dermatosis in patients of all ages. The presence of lymphomatous clonal cells and Sézary cells in the blood, immunophenotyping of lymphocytes in blood and marrow, and a second reading of the cutaneous biopsy results enabled us to make a diagnosis of Sezary syndrome.

[Diagnostic strategies for endometriosis: CNGOF-HAS Endometriosis Guidelines]. / Stratégies diagnostiques dans l'endométriose, RPC Endométriose CNGOF-HAS.

In this chapter we have examined the possibilities of screening endometriosis, both in the general population as well as in the target population. We then proposed decision trees, for primary and secondary care. Currently, there is not enough data in the literature to develop or organize a screening test for endometriosis. Screening for endometriosis is not recommended in the general population (level A). There is also no evidence to support systematic screening in a population with genetic risk factors (endometriosis in a relative), or with other clinical risk factors (increased menstrual volume, short cycles, early menarche) (level A). However, it is possible to propose a decision tree for the management of chronic pelvic pain symptoms (dysmenorrhea, dyspareunia, non-menstrual pelvic pain). The search for symptoms suggestive of endometriosis (intense dysmenorrhea [visual analogue scale >7/10, frequent abstention, resistance to level 1 analgesics], infertility) should be systematic. The search for localizing symptoms of deep endometriosis (deep dyspareunia, cyclic defecation pain, cyclic urinary signs) enables to orient the patient to second line evaluation. We propose a decision tree for second and third line evaluations, according to the suspicion and/or the discovery of deep lesions with specific locations, or the suspicion of superficial lesions.

Psychometric properties of the French version of the Endometriosis Health Profile-30, a health-related quality of life instrument.

INTRODUCTION: The objectives were to evaluate the acceptability, reliability and validity of the cross-cultural adaptation of the Endometriosis Health Profile-30 instrument into French. METHODS: This cross-sectional study was conducted between July and October 2015. We created an online link (REDCap platform) with the questionnaires. An endometriosis patients association spread the link on its website and with social networks. The translation and cultural adaptation of the EHP-30 was performed according to guidelines. Psychometric evaluation included data completeness, score distributions, floor and ceiling effects, factor analysis, internal consistency, item-total correlations corrected for overlap, convergent validity and test-retest reliability. RESULTS: The study included 913 women with endometriosis. In our results, data completeness was excellent. No floor effects were found and one ceiling effect was observed for the 'Infertility' scale. The highest means scores were found for 'Control and powerlessness' and 'Infertility' scales. Factor analysis confirmed the structure of the original EHP-30 questionnaire. Internal consistency was good (Cronbach's α range=0.72-0.96). The correlations of similar scale scores between EHP-30 and SF-36 were all significant (Spearman correlation coefficients ranging from -0.52 to -0.75). Test-retest reliability was good (intraclass correlation coefficients range=0.51-0.98). CONCLUSION: The French version of the EHP-30 is an acceptable, reliable and valid instrument for measuring health-related quality of life in women with endometriosis.

[Iodinated contrast in pregnant women and neonatal thyroid function]. / Injection de produit de contraste iodé en cours de grossesse et fonction thyroïdienne néonatale.

OBJECTIVES: There is a theoretical risk for neonatal hypothyroidism after prenatal exposure to iodinated contrast media. Current recommendations are in favour of neonatal thyroid function assessment. Our aim was to check if recommendations were observed, and if neonatal evaluation demonstrated anomalies. METHODS: Over the period from 01/01/2010 to 01/08/2015, maternal and newborn records were retrospectively reviewed. All pregnant women who underwent a computed tomography and their newborns were included. We collected thyroid-stimulating hormone (TSH), thyroxine (T4) and tri-iodothyronine (T3) levels. RESULTS: A total of 101 maternal and newborn records were reviewed. Mean gestational age at CT scan was 29.3±7.2 weeks. The mean dose of total iodine administered was 82.6±19.1mL. Only 21 newborns had a biological analysis (20.8%). All newborns had normal TSH and T4 levels at birth. Only 7 newborns had a T3 level above the upper threshold value, but according to expert opinion none have been considered pathological. CONCLUSION: Our study revealed that recommendations for neonatal thyroid function assessment after prenatal exposure to iodinated contrast media were not observed. This exposure seemed unlikely to have an important effect on thyroid function at birth.

[Alveolar capillary dysplasia with misalignment of pulmonary veins: a fatal cause of refractory neonatal cyanosis]. / Dysplasie alvéolo-capillaire avec mésalignement des veines pulmonaires : une cause de cyanose néonatale réfractaire létale.

Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare neonatal pathology that combines refractory hypoxemia with severe pulmonary arterial hypertension, and leads to death every time. Histologic examination of lung tissue confirms the diagnosis and is characterized by a decreased number of pulmonary capillaries, immature lobular development, and abnormal proximity between pulmonary arteries and veins. This abnormal proximity is responsible for an arteriovenous shunt. We report five cases confirmed by postmortem histology, which occurred over 14 years in Lower Normandy (France). The cumulative incidence is therefore of 1.8 for 100,000 births. In these five cases, the first symptoms appeared during the first hour of life and death occurred before 24h in four of five cases. The patient with the longest survival had mild histological lesions and delayed emergence of the first symptoms. Genitourinary and gastrointestinal anomalies were associated with ACD/MPV in two cases, and bilateral pulmonary hypoplasia in three cases. Optimized invasive ventilation, pulmonary vasodilators, vasoactive drugs, and pulmonary surfactant did not improve survival. Extracorporeal membrane oxygenation (ECMO) was not used. We present a review of the literature on ACD/MPV, a clinical and histological entity little known to both clinicians and pathologists, whereas a premortem diagnosis is possible and genetic counseling in affected families can be suggested.

Three dimensional echocardiographic assessment of multiple rhabdomyoma in newborn.

BACKGROUND: Cardiac rhabdomyomas represent the most common primary cardiac tumour in children and are strongly associated with tuberous sclerosis complex. RESULTS: We reported a newborn for whom three-dimensional echocardiogram, with multiplane mode, real-time imaging, full volume and i-slice view, allowed detailed visualisation of multiple highly echogenic and well-circumscribed cardiac rhabdomyoma. CONCLUSIONS: Three-dimensional imaging allowed a better definition of the tumour characteristics and provided a better delineation of the spatial relationship of the mass with a tomographic perspective. Three dimensional imaging may facilitate a possible operative planning and should be included in cardiac mass evaluation and follow-up.

[Medical and economic evaluation of neonatal hospital at home structure]. / Évaluation médico-économique d'une structure d'hospitalisation à domicile de néonatologie.

BACKGROUND: Hospital at home (HAH) shortens hospitalization time by providing at-home hospital-level care. The aim of this study was to describe and assess the cost of the neonatal HAH stay and compare it to the incomes produced by activity-based payments during the 1st year of a neonatal HAH program. METHOD: Medical and economic cost study from the hospital's point of view. For children admitted to the neonatal HAH unit between May 2010 and May 2011, sociodemographic characteristics were identified, consumed resources evaluated, and costs compared to the incomes produced by activity-based payments. RESULTS: Over 75% of children admitted to neonatal HAHs were former preterm infants and 67% of stays included nutritional support. The average length of stay was 16.5 days (SD, 11). The 85 stays produced 152,582 euros of income, the median income was 1531 euros. The median cost of the HAH stay was 1945 euros, resulting in a loss of 45,518 euros for the hospital, but the filling rate was not at its maximum during this period of scalability (77%). Personnel was the most costly item (73% of the total cost) followed by general management and structural costs (20%). CONCLUSION: Economic aspects must be considered to preserve the financial viability of a HAH unit, but the secondary human benefits must be highlighted. A 100% occupation rate would nearly balance the neonatal HAH budget. However, fees must be adjusted to ensure the sustainability and development of these structures.

Is ilioinguinal/iliohypogastric nerve block always totally safe in children?

We report a case of accidental puncture of the small bowel during an ilioinguinal/iliohypogastric nerve block procedure for hernia repair. The diagnosis was made a few days later during a laparoscopic exploration owing to the progressive onset of clinical and radiological intestinal obstruction. A large, obstructing subserosal haematoma was found without any apparent perforation of the mucosa, and the damaged loop was resected. Such a complication has already been reported once in the literature after use of a long bevel needle for the puncture. This case is the first reported using an atraumatic short bevel needle. We discuss the technical aspects of the procedure and underline the fact that regional anaesthesia in children is never totally risk free.

Tracheobronchial injury by blunt trauma in children: is emergency tracheobronchoscopy always necessary?

PURPOSE: To discuss the usefulness of CT scan in initial management of well tolerated tracheobronchial injuries and the place of tracheoscopy. METHODS: We report our experience of three cases treated for tracheobronchial rupture resulting from three different mechanisms and review the literature. Three boys, aged 4 to 10 years, were referred to our institution for tracheobronchial rupture. Two of them presented with subcutaneous emphysema after a minor trauma, the third was a polytrauma referred after a severe car crash and was already intubated. We emphasise the importance of an initial CT scan, as this allowed us twice to confirm the tracheal wound prior to tracheoscopy. We discuss the necessity of performing a tracheoscopy in the case of a well tolerated lesion, as this procedure clearly worsened the ventilatory state in one of our cases. Moreover, one of our cases illustrates the fact that even a minor trauma can lead to life-threatening respiratory distress. All the lesions observed in our study were linear and were managed by thoracic drainage; they were then closely monitored and required no further surgical procedure. CONCLUSION: Tracheobronchial rupture in children can result from minor cervical traumas and in such cases special attention must be paid to mild discomfort or subcutaneous emphysema on admission. Initial CT scan can be very helpful in visualising the level of the rupture and its consequences with respect to the pulmonary parenchyma. One can question the necessity for tracheoscopy in well tolerated lesion, as its results do not always improve the therapeutic outcome.