[Spontaneous adrenal hematomas. Retrospective analysis of 20 cases from a tertiary center]. / Hématomes surrénaliens non traumatiques : série rétrospective de 20 cas.

INTRODUCTION: Spontaneous adrenal hemorrhages (AH) are a rare condition with no consensus about their management. METHODS: Patients were identified using the Medicalization of the Information System Program database, imaging software and a call for observations to internists, intensivists and obsetricians working at our institution. Adult patients whose medical records were complete and whose diagnosis was confirmed by medical imaging were included. RESULTS: From 2000 to 2007, 20 patients were identified, including 15 were women. The clinical onset of AH was non-specific. In five cases, AH occurred during pregnancy; four of them were unilateral and right sided. The etiology of the other fifteen (bilateral adrenal hemorrhage in 11) were as follows: antiphospholipid syndrome (n=8), heparin-induced thrombocytopenia (n=4), essential thrombocythemia (n=3), spontaneous AH due to oral anticoagulants (n=1), complication of a surgical act (n=3), and sepsis (n=3). In seven cases, two causes were concomitant. The diagnosis of AH was often confirmed by abdominal CT. An anticoagulant treatment was initiated in 16 cases. Ten of the eleven patients presenting with bilateral adrenal hematomas were treated using a long-term substitute opotherapy. One patient died because of a catastrophic antiphospholipid syndrome. CONCLUSION: The clinical onset of HS is heterogeneous and non-specific. The confirmatory diagnosis is often based on abdominal CT. The search for an underlying acquired thrombophilia is essential and we found in this study etiological data comparable to the main series in the literature. Adrenal insufficiency is most of the time definitive in cases of bilateral involvement.

Prenatal diagnosis of pericallosal curvilinear lipoma: specific imaging pattern and diagnostic pitfalls.

We report the first series of cases of pericallosal curvilinear lipoma (CL) diagnosed prenatally and highlight the limitations in identifying a specific prenatal imaging pattern using ultrasound and magnetic resonance imaging (MRI). In all five of our cases, on ultrasound, the main feature leading to referral was a short corpus callosum. This subtle callosal dysgenesis was associated with a band of hyperechogenicity surrounding the corpus callosum, mimicking the pericallosal sulcus, which increased in size during the third trimester in three of the four cases in which sonographic follow-up was performed. On T2-weighted MRI, this band showed typical hypointensity in all cases; in contrast, on T1-weighted imaging, in only one case was there hyperintensity, suggestive of fat, as seen typically in the postnatal period. For appropriate prenatal counseling regarding outcome, it is important to identify or rule out CL when mild corpus callosal dysgenesis is observed. One should be aware of subtle diagnostic findings, such as a thin band of echogenicity surrounding the corpus callosum that is seen as a band of hypointensity on T2-weighted fetal MRI, and which may increase in size during gestation. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

[Macroscopic description of placental vascular anastomoses after dye injection for the comprehension of monochorionic pregnancy complications]. / Injection des anastomoses vasculaires pour la compréhension des complications propres aux grossesses monochoriales.

OBJECTIVES: The aim of the study is to compare placental monochorionic angioarchitecture complicated with twin-oligohydramnios-polyhydramnios sequence (TOPS), twin anemia polycythemia sequence (TAPS), twin reversed arterial perfusion (TRAP) and selective intra uterine growth restriction (sIUGR) to normal uneventful monochorionic placenta. METHODS: Between December 2012 and December 2015, monochorionic placenta has been studied at the multiple pregnancy care center of the Femme-Mère-Enfant Hospital in Lyon. Umbilical chords were catheterized and dye injected for macroscopic analysis of angioarchitecture at the anatomopathology department. Placentas treated with laser foetoscopic surgery were excluded. RESULTS: A total of 126 placentas were injected in the post-partum period. In total, 95% (119/126) of the placentas presented arteriovenous anastomoses (AVA). Median number of AVA was 7. The prevalence of at least one velamentous cord insertion was higher in TOPS and selective intrauterine growth restrictions P<0.01 and P<0.01 respectively, compared to uneventful pregnancies. Arterio-arterial anastomoses (AAA) were present in 82.7% (77/93) of uneventful placentas versus 33.3% of TOPS (P<0.01) and 28.5% of TAPS (P<0.01). The prevalence of veno-venous anastomoses was significantly higher in TOPS (P<0.01). All TAPS placentas showed marginal arteriovenous anastomoses. In TRAP placenta, the acardiac twin had no specific vascular territory. CONCLUSION: The study confirms literature findings on prevalence of vascular anastomoses in monochorial placentas, suggesting the protective role of AAA in TOPS and TAPS. The role of VVA is yet hard to determinate. Macroscopic observations of monochorionic placentas are valuable and essential keys for understanding, managing and treating anastomotic syndromes.

Personalized thromboprophylaxis using a risk score for the management of pregnancies with high risk of thrombosis: a prospective clinical study.

Essentials Pregnancy is a risk factor for thrombosis. Management of thrombosis risk in pregnancy remains a challenge. Prophylaxis needs to be personalized. Our score may be a helpful tool for the management of pregnancies at high risk of thrombosis. SUMMARY: Background Patients with thrombophilia and/or a history of venous thromboembolism (VTE) are at risk of thrombosis during pregnancy. A risk score for pregnancies with an increased risk of VTE was previously described by our group (Lyon VTE score). Objectives The aim of this prospective study was to assess the efficacy and safety of our score-based prophylaxis strategy in 542 pregnancies managed between 2005 and 2015 in Lyon University Hospitals. Patients/Methods Of 445 patients included in the study, 36 had several pregnancies during the study period. Among these 445 patients, 279 had a personal history of VTE (62.7%), 299 patients (67.2%) had a thrombophilia marker, and 131 (29.4%) thrombophilic women had a personal history of VTE. During pregnancy, patients were assigned to one of three prophylaxis strategies according to the risk scoring system. Results In the antepartum period, low molecular weight heparin (LMWH) prophylaxis was prescribed to 64.5% of patients at high risk of VTE. Among them, 34.4% were treated in the third trimester only, and 30.1% were treated throughout pregnancy. During the postpartum period, all patients received LMWH for at least 6 weeks. Two antepartum-related VTEs (0.37%; one with a score of < 3 and the other with a score of > 6) and four postpartum-related VTEs (0.73%; three with scores of 3-5 and one with a score of > 6) occurred. No case of pulmonary embolism was observed during the study period. The rate of bleeding was 0.37%. No serious bleeding requiring transfusions or surgery occurred during the study period. Conclusion The use of a risk score may provide a rational decision process to implement safe and effective antepartum thromboprophylaxis in pregnant women at high risk of VTE.

[Lucy's cancer(s): A prehistorical origin?] / Le(s) cancer(s) de Lucy : une origine préhistorique ?

OBJECTIVES: The recent discovery of the earliest hominin cancer, a 1.7-million-year-old osteosarcoma from South Africa has raised the question of the origin of cancer and its determinants. We aimed to determine whether malignant and benign tumors exist in the past societies. METHODS: A review of literature using Medline database and Google about benign and malignant tumors in prehistory and antiquity. Only cases with morphological and paraclinical analysis were included. The following keywords were used: cancer; paleopathology; malignant neoplasia; benign tumor; leiomyoma; myoma; breast cancer; mummies; soft tissue tumor; Antiquity. RESULTS: Thirty-five articles were found in wich there were 34 malignant tumors, 10 benign tumors and 11 gynecological benign tumors. CONCLUSIONS: The fact that there were some malignant tumors, even few tumors and probably underdiagnosed, in the past may be evidence that cancer is not only a disease of the modern world. Cancer may be indeed a moving target: we have likely predisposing genes to cancer inherited from our ancestors. The malignant disease could therefore appear because of our modern lifestyle (carcinogens and risk factors related to the modern industrial society).

[Elaboration of a national biobank for the study of gestational trophoblastic diseases]. / Création d'une biobanque nationale pour l'étude des maladies trophoblastiques gestationnelles.

AIM: To generate a national biobank made up of samples of the highest quality for the purpose of inciting basic research on gestational trophoblastic diseases (GTD). MATERIAL AND METHODS: Three priority axes of research were defined to optimize the nature, method of collection, and storage of the samples. These are: to enhance our understanding of GTD, develop new diagnostic tests, and identify new therapeutic targets. The protocol for patient inclusion and sample processing was determined after extensive literature review and collaboration with international experts in the field of GTD. RESULTS: For each patient with a GTD and for control patients (legally induced abortions), chorionic villi, decidua and tumor samples (fresh, immersed in RNA-protective solution and fixed in formaldehyde), blood (serum, plasma, RNA, and peripheral blood mononuclear cells), urine (supernatant), and cell cultures of villous cytotrophoblasts are prospectively collected. Associations are then made between the collected samples and numerous clinical and biological data, such as human chorionic gonadotropic plasma levels following curettage in the case of a hydatidiform mole. CONCLUSION: Such a collection of high quality samples and their associated data open up new perspectives for both national and international collaborative research projects.

[Fetal transcerebellar diameter measured in screening ultrasound: Feasibility and reproducibility]. / Mesure du diamètre transverse du cervelet en échographie de dépistage : faisabilité et fiabilité.

OBJECTIVE: The aim of the study was to evaluate feasibility and reproducibility of fetal transcerebellar diameter measurement during second and third trimester ultrasound mass screening by junior and senior physicians. MATERIALS AND METHODS: A monocentric prospective study was conducted at the tertiary care teaching hospital in Lyon, including patients undergoing their second or third trimester planned ultrasound exam. For each patient, a resident and a senior practitioner measured each fetal transcerebellar diameter, during a blinded experiment, according to the transcerebellar plane described by the International Society of Ultrasound in Obstetrics and Gynecology. Images have been scored on 4 criteria. The inter-observer variability for transcerebellar diameter and image quality was assessed using an intra-class correlation coefficient. Image quality has been analyzed according to pregnancy term and to fetal presentation. RESULTS: Sixty-six patients were included, 44 patients before and 22 patients after 30 weeks. Inter-observer variability of transcerebellar diameter measurement was 0.4%. Inter-observer variability of image quality was 13.5%. Image quality was not significantly different between seniors and residents (P=0.06). Gestational age and fetal presentation did not affect significantly image quality (P=0.42) and (P=0.64) respectively. CONCLUSION: Transcerebellar diameter measurement during mass screening is simple and reliable. Posterior fossa abnormalities can be explored through its measurement.

Prenatal diagnosis of cobblestone lissencephaly associated with Walker-Warburg syndrome based on a specific sonographic pattern.

We report a specific sonographic cerebral pattern of cobblestone lissencephaly (CL) that has not been described previously. This pattern was encountered in four index cases and allowed prenatal diagnosis of CL associated with Walker-Warburg syndrome. The pattern included both an outer echogenic band with reduced pericerebral space, corresponding to an infra- and supratentorial extracortical layer of neuroglial overmigration on pathological examination, and a 'Z'-shaped appearance of the brainstem. This pattern was found as early as 14 weeks' gestation in one of our cases.

[Neonatal outcome of fetal hyperechogenic bowel]. / Devenir néonatal des fœtus présentant un intestin hyperéchogène.

OBJECTIVE: Echogenic bowel (EB) represents 1 % of pregnancy and is a risk factor of fetal pathology (infection, cystic fibrosis, aneuploidy). The aim of our study was to determine the fetuses' outcomes with isolated EB. PATIENTS AND METHODS: This is a retrospective study of all patients who presented singleton gestations with a fetal isolated echogenic bowel between 2004 and 2011 in two prenatal diagnosis centers. Search of aneuploidy, infection and cystic fibrosis was systematically proposed as well as an ultrasound monitoring. RESULTS: On 109 fetus addressed for isolate echogenic bowel five had other signs associated and 74 had a real isolated echogenic bowel (without dilatation, calcification, intrauterine growth restriction). In 30 cases, the EB was not found. Eighty-five percent of the patients had in the first trimester a screening for trisomy 21. None fetus with isolated EB had trisomy, infection or cystic fibrosis. One fetus died in utero and one newborn died of a metabolic disease without digestive repercussions. DISCUSSION AND CONCLUSION: The risk of trisomy 21 and the risk to have a serious disease appear low for the fetus with EB. It does not seem necessary to propose a systematic amniocentesis in case of isolated echogenic bowel.

Distortion of the anterior part of the interhemispheric fissure: significance and implications for prenatal diagnosis.

In order to illustrate the significance of a new anatomical finding, distortion of the interhemispheric fissure (DIHF) associated with impacted medial borders of the frontal lobes, we report a retrospective observational study of 13 fetuses in which DIHF was identified on prenatal imaging. In 10 cases there were associated anatomical anomalies, including mainly midline anomalies (syntelencephaly (n=2), lobar holoprosencephaly (n=1), Aicardi syndrome (n=2)), but also schizencephaly (n=1), cortical dysplasia (n=1) and more complex cerebral malformations (n=3), including neural tube defect in two cases. Chromosomal anomaly was identified in two cases, including 6p deletion in a case without associated central nervous system anomalies and a complex mosaicism in one of the cases with syntelencephaly. In two cases, the finding was apparently isolated on both pre- and postnatal imaging, and the children were doing well at follow-up, aged 4 and 5 years. The presence of DIHF on prenatal imaging may help in the diagnosis of cerebral anomalies, especially those involving the midline. If DIHF is apparently isolated on prenatal ultrasound, magnetic resonance imaging is recommended for careful analysis of gyration and midline, especially optic and olfactory structures. Karyotyping is also recommended.

Prenatal unilateral cerebellar hypoplasia in a series of 26 cases: significance and implications for prenatal diagnosis.

OBJECTIVE: To define imaging patterns of unilateral cerebellar hypoplasia (UCH), discuss possible pathophysiological mechanisms and underline the etiology and prognosis associated with these lesions. METHODS: In this retrospective study we reviewed the charts of 26 fetuses diagnosed between 2003 and 2011 with UCH, defined by asymmetrical cerebellar hemispheres with or without decreased transverse cerebellar diameter. The review included analysis of the anatomy of the cerebellar hemispheres, including foliation, borders and parenchymal echogenicity, and of the severity of the hypoplasia. Data from clinical and biological work-up and follow-up were obtained. RESULTS: Our series could be divided into two groups according to whether imaging features changed progressively or remained constant during follow-up. In Group 1 (n = 8), the progression of imaging features, echogenic cerebellar changes and/or hyposignal in T2*-weighted MR images were highly suggestive of ischemic/hemorrhagic insult. In Group 2 (n = 18), imaging features remained constant during follow-up; UCH was associated with abnormal foliation in three proven cases of clastic lesions, a cystic lesion was noted in three cases of PHACE (posterior fossa anomalies, hemangioma, arterial anomalies, cardiac abnormalities/aortic coarctation, eye abnormalities) syndrome and, in the remaining cases, UCH remained unchanged, with no imaging pattern typical of hemorrhage. In 24 cases the infant was liveborn and follow-up was continued in 23, for a mean period of 3 years. Among these, neurological complications were identified in seven (in one of seven (at a mean of 46 months) in Group 1 and in six of 16 (at a mean of 35 months) in Group 2). The surface loss of cerebellar hemisphere was > 50% in 19/24 fetuses and the vermis was clearly normal in appearance in 19/24. Predisposing factors for fetal vascular insult were identified in eight cases: these included maternal alcohol addiction, diabetes mellitus, congenital cytomegalovirus infection and pathological placenta with thrombotic vasculopathy and infarctions. CONCLUSION: UCH is defined as a focal lesion of the cerebellum that may be secondary to hemorrhage and/or ischemic insult, suggesting a clastic origin, particularly when imaging follow-up reveals changes over time. UCH may also be a clue for the prenatal diagnosis of PHACE syndrome. The amount of surface loss of cerebellar hemisphere does not correlate with poor prognosis. UCH with normal vermis is often associated with normal outcome.

Fractures in children younger than 18 months.

Fractures in children younger than 18 months occur before the usual walking age. The prognosis is favourable across fracture types and circumstances of occurrence. The cause is obvious in obstetrical injuries, whose risk factors have been well documented. Diaphyseal fractures are easy to recognise, whereas challenges may arise with the diagnosis of physeal injuries. Fractures occurring after the neonate is discharged home may be due to accidental falls related to clumsiness on the part of the carers. Other possibilities, however, are child abuse and abnormal bone fragility. Thus, the aetiological diagnosis has major medical, social, and legal implications. Identifying the aetiology is often extremely difficult and benefits from the involvement of a multidisciplinary team. The literature review presented herein is designed to assist orthopaedic surgeons in the diagnosis and management of children with fractures before 18 months of age, in compliance with French legislation, which has undergone major changes over the last quarter century.

[Ogilvie's syndrome after cesarean section: A case report]. / Syndrome d'Ogilvie après une césarienne : à propos d'un cas.

Ogilvie's syndrome is a rare postsurgical complication. The high mortality rate (normally 15 to 31% and up to 45% after caecal perforation) explains the seriousness of this clinical situation. The early diagnosis is made by plain abdominal X-ray. Conservative treatment is usually effective and surgery should be reserved for complicated cases or refractory to conservative treatment. We report a case of Ogilvie's syndrome after cesarean section, which has been treated by conservative methods.

Campomelic dysplasia: echographic suspicion in the first trimester of pregnancy and final diagnosis of two cases.

OBJECTIVE: Campomelic dysplasia (CD) is a rare skeletal dysplasia characterized by marked femoral and tibial angulations, hypoplasic scapulae, normal upper limbs and sex reversal in 3/4 of 46,XY fetuses. Most cases are lethal in the neonatal period. Heterozygous mutations in the SOX9 gene are responsible for CD. The diagnosis is not usually made until the mid-second trimester or later. METHODS: We describe 2 cases of CD suspected by ultrasonography in the first trimester. RESULTS: The 2 cases presented with hygroma colli along with anomalies in the lower but not the upper limbs. Terminations of pregnancy were obtained at 14+3 and 20+6 gestational weeks. Fetopathological examinations confirmed sonographic findings. CONCLUSION: When first trimester hygroma colli is accompanied by specific findings of the lower limbs, the diagnosis of CD can be investigated through SOX9 mutation analysis.

Abnormal Sylvian fissure on prenatal cerebral imaging: significance and correlation with neuropathological and postnatal data.

OBJECTIVE: To illustrate and determine the significance of abnormal Sylvian fissure development (or abnormal operculization) in cases in which prenatal cerebral imaging is suggestive of underlying cortical dysplasia. METHODS: This was a retrospective study of 15 fetuses at 24-34 weeks in which abnormal operculization was identified on prenatal cerebral imaging and for which follow-up data were available. The imaging findings were correlated to macro- and microscopic neuropathological data (n = 11) or to postnatal clinical and imaging findings (n = 4). RESULTS: On microscopic examination of fetuses from 11 terminated pregnancies, abnormal operculization was associated with cortical dysplasia in four cases and the cortex was normal in seven. Abnormal operculization was associated with cortical dysplasia in only one of the four liveborn infants. Cases of abnormal Sylvian fissure development with normal cortical architecture were classified, according to associated anomalies of the central nervous system, into one of five groups: those with neural tube defects, microcephaly or frontal hypoplasia, glutaric aciduria, other cerebral abnormalities, and extracerebral anomalies. CONCLUSION: Abnormal operculization on prenatal imaging does not systematically reflect underlying cortical dysplasia. It may be related to extracortical factors such as abnormal cerebral volume or other developmental anomalies of the central nervous system. An understanding of the significance of abnormal Sylvian fissure development could be useful in integrating its analysis into a more general one of the whole central nervous system.

[Closure or non closure of the peritoneum at cesarean section in 2008?]. / Suture péritonéale ou non au cours de la césarienne en 2008?

Non closure of the peritoneum at cesarean is still debatable, despite the national and international guidelines. This review aims at exposing risks and benefits of non closure of the peritoneum, focusing on the peritoneum adhesions. Many studies demonstrated no benefits at peritoneum closure in the duration of surgery, the immediate postoperative period and the short-term complications. Data about pelvic adhesion risk are more inconsistent. Different criteria were considered in the studies: adhesions incidence and density during subsequent cesareans or pelvic surgeries, duration of surgery and the delay between incision and birth during the subsequent cesarean and fertility known to be impaired by thick-pelvic adhesions. Most of the studies are exhibiting serious bias, leading to weak conclusions. However, two randomised controlled trials compared pelvic adhesion in the subsequent c-section, in step with closure or non closure of the parietal and visceral peritoneum at first caesarean. The results showed that non closure of the peritoneum does not increase or even reduce the adhesions risk. These results are consistent with results from three studies reporting no modification of patient fertility. As a conclusion, current data are supporting the national and international medical society recommendations about the benefits of the non closure of the peritoneum at caesarean section.

[Laparoscopic adjustable gastric banding and pregnancy]. / Anneau gastrique et grossesse.

UNLABELLED: Laparoscopic adjustable gastric banding and pregnancy. OBJECTIVES: We intend to understand the interactions between pregnancy and laparoscopic adjustable gastric banding in order to ensure better care for patients. MATERIALS AND METHOD: We undertake an observation retrospective study, from January 2004 to December 2005, at Edouard Herriot hospital, of births from women with laparoscopic adjustable gastric banding with a study of gastric banding interaction with pregnancy, neonatal outcomes and postpartum; we read through literature. RESULTS: We studied 35 pregnancies from women with a gastric banding out of 5773 pregnancies (0.6%); we noted lower obstetrical complications with loss of weight that we attributed to the gastric banding comparing with the pregnancies before the gastric banding: three hypertensive diseases and one gestational diabetes had been avoided; we observed a complication due to the gastric banding: occlusive syndrome, which implied gastric banding calibration during immediate postpartum. CONCLUSION: Our record folders are in accordance with literature and confirm improvement in obstetrical prognostic. Waiting for stabilization of weight is recommended before starting a pregnancy after gastric banding surgery; the adjustment of the gastric banding during pregnancy must be undertaken individually according to symptoms (vomiting, gain of weight...).

Effects of residential distance to hospitals with neonatal surgery care on prenatal management and outcome of pregnancies with severe fetal malformations.

OBJECTIVES: To examine the effect of maternal origin and distance between maternal residence and the nearest maternity ward with a neonatal surgical center in the same hospital, on prenatal diagnosis, elective termination of pregnancy, delivery in an adequate place and neonatal mortality for pregnancies with severe malformations requiring neonatal surgery, and to examine the effect of the place of delivery on neonatal mortality. METHODS: This was a retrospective study, through the France Central-East malformation registry, of 706 fetuses with omphalocele (n = 123), gastroschisis (n = 99), diaphragmatic hernia (n = 222), or spina bifida (n = 262), but without chromosomal anomalies. Maternal origin was classified as Western European and non-Western European. Adequate place for delivery was defined as birth in a Level-III maternity ward with a neonatal surgical center in the same hospital. RESULTS: The prenatal diagnosis rate was 67.7% in 1990-1995 and 80.2% in 1996-2001 (odds ratio (OR), 2.07 (95% CI, 1.24-3.45)). On multivariate analysis, the rate was significantly lower for women living 11-50 km (adjusted OR, 0.49 (95% CI, 0.25-0.94)), or > 50 km (adjusted OR, 0.39 (0.20-0.74)) from the closest adequate place of delivery, compared with those living < 11 km from it, but there was no difference for maternal origin. Non-Western European women had fewer elective terminations of pregnancy (adjusted OR, 0.34 (95% CI, 0.14-0.81)) and fewer deliveries in an adequate place (adjusted OR, 0.40 (95% CI, 0.18-0.89)). Neonatal mortality was lower in the case of delivery in an adequate place (adjusted OR, 0.22 (95% CI, 0.07-0.72)) and was not associated with maternal origin and distance from nearest maternity ward with a neonatal surgical center. CONCLUSION: Rate of prenatal diagnosis decreases with increasing distance between parental residence and referral center. Non-Western European women are diagnosed prenatally as often as are Western Europeans, but terminate their pregnancy less often, perhaps for cultural reasons. Non-Western European women with malformed fetuses deliver in adequate centers less often, probably because of the way the perinatal care system is run.