P. Gaucherand

Transvaginal Sonohysterographic Evaluation of Intrauterine Adhesions

We assessed the role of preoperative sonohysterography in the diagnosis of intrauterine synechiae.

Prenatal cerebral ultrasound and MRI findings in glutaric aciduria Type 1: a de novo case.

A 21-year-old primigravida with an unremarkable medical history was admitted to our institution at 33 weeks’ gestation for severe fetal macrocephaly (biparietal diameter 115 mm) associated with increased pericerebral spaces. Ultrasound examination showed an abnormal operculum with a wide Sylvian fissure and multiple bilateral subependymal cysts. Fetal cerebral magnetic resonance imaging (MRI) also demonstrated bilateral enlarged Sylvian fissures (abnormal operculum) with otherwise normal gyration, bilateral subependymal cysts, diffuse hyperintensity in the periventricular white matter on T2weighted images and severe increase of the supratentorial subarachnoid spaces. The basal ganglia were normal. After prenatal counseling, the parents decided to continue the pregnancy without any additional biological investigation, even though the imaging findings were consistent with glutaric aciduria Type 1 (GA 1). A 3540-g male with severe neonatal macrocephaly (cranial perimeter 40 cm) was delivered at 38 weeks’ gestation by Cesarean section. Neurological examination of the newborn was normal. Biological evaluation demonstrated abnormal elevation of the urinary excretion of 3-hydroxyglutaric-acids, which confirmed the diagnosis of GA 1. Neonatal cerebral MRI confirmed the prenatal imaging findings (Figures 1 and 2). The neonate was treated with dietary control. GA 1 is an autosomal recessive disorder that results from a deficiency of glutaryl-CoA dehydrogenase, which leads to the accumulation of glutaric and 3-hydroxyglutaric acids and secondary carnitine deficiency1–3. The first symptoms usually appear during early childhood (6–18 months) and are characterized by acute neurological crises associated with metabolic acidosis1–3. The neurological prognosis is severe without treatment but can be effectively improved by appropriate dietary control before clinical manifestations and acute striatal necrosis. This should prompt early biological diagnosis2. Molecular diagnosis is now available, allowing prenatal diagnosis via chorionic villus sampling in the presence of a positive family history4,5. It is noteworthy that no personal or familial history of GA 1 was present in our case, underlining the diagnostic value of the association of severe macrocephaly with suggestive cerebral imaging findings. Macrocephaly is the pivotal clinical sign in GA 1 patients in the postnatal period, as it is the earliest and the most consistent sign according to the largest series published6,7. Development of macrocephaly usually occurs early in the neonatal period, typically within the first weeks after delivery, but Figure 1 Prenatal (34 weeks’ gestation) (a) and postnatal (b) T2-weighted fast-spin echo magnetic resonance axial images showing abnormal operculum with widening of both anterior and superior parts of the Sylvian fissure and diffusely enlarged cerebrospinal fluid spaces.

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. Copyright

How deviations from STAN guidelines contribute to operative delivery for suspected fetal distress

OBJECTIVE To evaluate how deviations from STAN guidelines contribute to operative delivery for suspected fetal distress in a high-risk population. STUDY DESIGN This retrospective cohort study was conducted in a tertiary referral center with about 3000 deliveries a year. During the study period, STAN usage rate was 15.2%. All consecutive patients monitored with STAN who had an operative delivery for suspected fetal distress were included in the index group. Patients who delivered spontaneously or had an operative delivery for any reason other than suspected fetal distress were included as controls. Case review was performed by three referent obstetricians for STAN technology blinded to neonatal outcomes. Main outcome was agreement between decision made and decision recommended by STAN clinical guidelines. Secondary outcomes were reasons explaining guideline deviation and ST event to birth interval in cases with a significant ST event. RESULTS Eighty-three patients were included in each group. Decision made was consistent with STAN clinical guidelines in 124 patients (74.7%): 50 patients (60.2%) in the index group and 74 patients (89.2%) in the control group (p<0.05). Among these patients, no fetal metabolic acidosis was reported. Decision made was not consistent with STAN labor management guidelines in 42 patients (24.3%): 33 patients (39.8%) in the index group and 9 (10.8%) in the control group (p<0.05). Including ST event to birth interval, interventions were outwith STAN clinical guidelines in 51.4% of patients with suspected fetal distress. CTG misclassification was involved in over 75% of cases. CONCLUSION STAN guideline deviations contribute to an increased operative delivery rate in patients with suspected fetal distress and normal neonatal outcomes. Guideline deviations are more frequent in patients with suspected fetal distress than in controls. CTG misclassification was the leading cause of guideline deviation. STAN guideline deviations may contribute to alter STAN specificity.

Extracerebellar ectopic brain tissue in the posterior fossa

Intracranial extracerebral ectopic brain tissue (also called glioneuronal heterotopia) is encountered in rare developmental disorders. These lesions are mainly located at the base of the brain in the supratentorial region and are frequently associated with craniofacial anomalies. We report the prenatal sonographic and magnetic resonance imaging (MRI) findings of isolated extracerebellar ectopic brain tissue in the fetal posterior fossa, which was confirmed by postnatal MRI examination in a healthy infant. A 27-year-old woman at 28 weeks’ gestation was referred to our department with the diagnosis of a round mass in the fetal posterior fossa located in the cisterna magna, which had been identified on a routine ultrasound scan. Ultrasound examination (Antares, Siemens, Munich, Germany) confirmed the presence of a round echogenic non-vascularized mass of 12 mm in diameter located in the midline of the cisterna magna posterior to a normal vermis (Figure 1). The cerebellum was anatomically normal with a normal transverse cerebellar diameter for gestational age (32 mm). The size of the cisterna magna was also within normal ranges. No other structural cerebral or extracerebral abnormalities could be seen. Biometric parameters were normal for gestational age. Fetal cerebral MRI showed a round and apparently solid mass, posterior to the vermis, with a signal similar to that of normal brain tissue on T2-weighted imaging (Figure 2) and with no hemorrhagic changes on T1weighted imaging. Vermian and hemispheric cerebellar anatomy were normal. Although this lesion has not been described previously in this location, imaging findings were suggestive of ectopic/heterotopic cerebral tissue. On the basis of normal cerebellar anatomy with a complete vermis and otherwise normal cerebral and extracerebral anatomy and, following prenatal counseling with neuropediatricians, the couple decided to continue with the pregnancy. Follow-up at 33 weeks’ gestation found the MR signal, size and echogenicity of the lesion to be unchanged. Since the patient was lost to follow-up for

OP07.03: “Isolated” Dandy‐Walker malformation on prenatal imaging: Look for subtelomeric deletion and post‐natal hydrocephalus

Objective: To determine the outcome of fetuses with a prenatal ultrasound (US) diagnosis of isolated Dandy-Walker variant (DWV), megacisterna magna (MCM) or Blake’s pouch cyst (BPC). Methods: Registers from our scan database diagnosed as isolated posterior fossa (PF) anomaly between 2002 and 2008 were retrospectively collected at Vall d’Hebron Materno Infantil Hospital in Barcelona. Information concerning karyotype, other image studies, pathology findings, perinatal outcome and postnatal evaluation was obtained from medical records. Infant’s development and behaviour was evaluated with the validated tool parent’s evaluation of development status (PEDS). Results: This series included 24 cases over seven years, we sonographically identified 7 fetuses with isolated DWV, 14 with isolated MCM, and 3 with isolated BPC. Mean gestational age at diagnosis was 24 weeks for DWV, 29 weeks for MCM and 20 weeks for BPC. Karyotypes were performed in 4, 8 and 2 fetuses with DWV, MCM and BPC respectively and all were normal. Of DWV cases 4 (57.4%) requested termination of pregnancy (TOP), one was lost after diagnosis, one had an intrauterine fetal demise and one had a healthy neonate. Of MCM cases one opted for TOP and 13 continued, all healthy neonates were born uneventfully, but one has developmental delay at follow-up. All cases of BPC continued pregnancies and finished with healthy babies. PEDS was used to detect possible developmental or behavioural problems in 16 cases with isolated PF anomalies in fetal US. We found one infant (6.2%) in group A-treatment needed; two infants (12.5%) in group B-perform wider evaluation; three infants (18.8%) in group C-close observation, and ten infants (62.5%) in group E-normal. Conclusions: Isolated DWV prognosis is still unclear, even when karyotype is normal, and both, isolated MCM and BPC, are generally associated to normal karyotype and have a high possibility of leading to a normal neonate and good long term prognosis.

What about the Misgav-Ladach surgical technique in patients with previous cesarean sections?

OBJECTIVE The Misgav-Ladach technique is recommended worldwide to perform cesarean sections but there is no consensus about the appropriate technique to use in patients with previous cesarean sections. This study evaluated the feasibility of the Misgav-Ladach technique in patients with previous cesarean sections. STUDY DESIGN This prospective cohort study included all women undergoing cesarean section after 36 weeks of gestation over a 5-month period, with the Misgav-Ladach technique as first choice, whatever the previous number of cesarean sections. RESULTS Among the 204 patients included, the Misgav-Ladach technique was successful in 100%, 80%, and 65.6% of patients with no, one, and multiple previous cesarean sections, respectively. When successful, the Misgav-Ladach technique was associated with a shorter incision to birth interval in patients with no previous cesarean section compared with patients with one or multiple previous cesarean sections. Anterior rectus aponeurosis fibrosis and severe peritoneal adherences were the two main reasons explaining the Misgav-Ladach technique failure. CONCLUSION The Misgav-Ladach technique is possible in over three-fourths of patients with previous cesarean sections with a slight increase in incision to birth interval compared with patients without previous cesarean section. Further studies comparing the Misgav-Ladach and the Pfannenstiel techniques in women with previous cesarean should be done.

OC10.04: Distortion of the anterior part of the interhemispheric fissure. Significance and impact on prenatal diagnosis

Methods: We evaluated the performance of 17 children, aged 2–7 years diagnosed in utero as macrocephalic and compared them to 17 normocephalic children, utilizing a standardized neuropsychological battery, which included attribution to cognitive, emotional and behavioral domains. Results: All the fetuses in the study group had a head circumference between two and three standard deviation (SD). No significant differences were found between the groups on the cognitive, language and motor domains. The study group scored significantly lower than the control on three parameters reflecting executive functioning, behavior and social-emotional development. Children with familial macrocephaly showed significantly better executive functioning compared to children with non-familial macrocephaly. Multiple linear regression analysis found paternal head circumference as the only significant variable in positively predicting the cognitive functioning of the child. Conclusions: Our results indicate that the prenatal diagnosis of isolated macrocephaly is not a risk factor for abnormal long-term neuropsychological development. Paternal macrocephaly was found to be most important in positively predicting cognitive performance of the child.

Critical events in obstetrics: a confidential enquiry in four high‐level maternities of the AURORE perinatal network

In Europe, disparities have been found in the quality of care for obstetric emergencies [1]. Compared with other European countries, France is in an average position (9 deaths per 100 000 births) [2], but experiences higher mortality than in Norway (3.3 per 100 000) [3]. The national committee of experts on maternal mortality analysed these cases and estimated that 87% of these deaths could have been avoided [4]. The dysfunctions identified related more to non-implementation of management protocols and inadequacies in the organization of care than to failures in the implementation of medical techniques [5]. Severe maternal morbidity can be used to assess quality of care and defined by the admission of pregnant or puerperant women to intensive care units (ICUs) [4,6]. Current research to improve the quality of obstetric care focuses on the impact of organizational factors [7,8]. Confidential inquiry has been defined as complementary to scientifically valid quantitative methods [9]. It involves peer review of the medical processes leading to serious, inopportune events in minute detail in order to remove weaknesses in the hospital system. Data were collected by means of interviewsrelated professional practices and the organization of care processes [10]. The aim of this study was to identify organizational dysfunctions in obstetric management of pregnant and puerperal patients who experienced a critical severe event requiring transfer to an ICU. Materials and methods