Impact of prenatal estimation of the risk of respiratory distress in neonates with congenital pulmonary malformations on the choice of delivery site.

BACKGROUND: The vast majority of prenatally diagnosed congenital pulmonary malformations (CPM) remain asymptomatic at birth. The maximal value of the CPM volume ratio (CVRmax) predicts the risk of neonatal respiratory distress (NRD), and should allow for better assessment of the level of expertise needed at the delivery site. AIM: This study evaluated the level of maternity units currently chosen for the delivery of CPMs, and determined the impact of the choice of delivery site based on the CVRmax, with a threshold of 0.4 cm2. METHODS: Data were extracted from the French prospective MALFPULM cohort, with inclusion between March 2015 and June 2018. RESULTS: The final study population consisted of 383 women. Deliveries in level 1 or 2 maternity units (n = 98, 25%) involved CPMs with lower CVRmax (p<0.001), causing fewer signs of prenatal compression (p = 0.025). Among the 62 children (16%) who presented with NRD, only seven (11%) were born in level 1 or 2 units (p = 0.0078). Choosing the maternity level according to the CVRmax would have increased the number of births in level 1 or 2 maternity hospitals by 70%. In these maternity units, the percentage of children with NRD would have increased from 8% in the actual distribution to 10% in the new strategy. CONCLUSION: Our results showed an overuse of level 3 maternity hospitals for the delivery of newborns with a prenatal diagnosis of CPM. The use of CVRmax should enable a reduction in the use of expertise centers without an adverse impact on newborns.

[Prenatal and clinicopathological study of 6 cases of Pallister-Killian syndrome and review]. / Syndrome de Pallister-Killian : confrontation des données prénatales et fœtopathologiques de six cas et revue de la littérature.

OBJECTIVE: In multidisciplinary prenatal diagnosis centers, the search for a tetrasomy 12p mosaic is requested following the discovery of a diaphragmatic hernia in the antenatal period. Thus, the series of Pallister Killian syndromes (PKS: OMIM 601803) probably overestimate the prevalence of diaphragmatic hernia in this syndrome to the detriment of other morphological abnormalities. METHODS: A multicenter retrospective study was conducted with search for assistance from members of the French society for Fetal Pathology. For each identified case, we collected all antenatal and postnatal data. Antenatal data were compared with data from the clinicopathological examination to assess the adequacy of sonographic signs of PKS. A review of the literature on antenatal morphological anomalies in case of PKS completed the study. RESULTS: Ten cases were referred to us: 7 had cytogenetic confirmation and 6 had ultrasound screening. In the prenatal as well as post mortem period, the most common sign is facial dysmorphism (5 cases/6). A malformation of limbs is reported in half of the cases (3 out of 6). Ultrasound examination detected craniofacial dysmorphism in 5 cases out of 6. We found 1 case of left diaphragmatic hernia. Our results are in agreement with the malformation spectrum described in the literature. CONCLUSION: Some malformation associations could evoke a SPK without classical diaphragmatic hernia.

[Performance of prenatal diagnosis and postnatal development of congenital lung malformations]. / Performance du diagnostic anténatal et évolution postnatale des malformations pulmonaires congénitales.

OBJECTIVES: For many diseases, the comparison of prenatal diagnosis with a histopathological reality is not always possible. Fetal lung pathology, with its high rate of surgery in postnatal, allows this assessment. This study proposes an approach to the reliability of prenatal diagnosis and analysis of the postnatal development of all children in care for congenital pulmonary malformation (CPM). METHODS: This is a retrospective study of all cases of CPM diagnosed in Poitiers University Hospital from 1995 to 2011. Cases diagnosed prenatally were identified and the diagnostic accuracy was studied by histology when cases had surgery. The postnatal development of prenatally diagnosed cases is described and compared to children who did not receive prenatal diagnosis. RESULTS: Among the 45 cases of CPM supported at the Poitiers University Hospital, 30 had received prenatal diagnosis of isolated CPM. The diagnostic concordance between antenatal ultrasound and the final diagnosis is κ=0.67 (CI95% [0.38 to 0.94]). The sensitivity of ultrasound was 90% (CI95% [55-99.7]) in our series for the diagnosis of CAMP (cystic adenomatoid malformation pulmonary). We found a sonographic disappearance of lesions in 4 children, 1 child in regression, stable lesions in 21 cases. Four children showed an increase in volume of the malformation, with signs of poor tolerance in 3 cases. After birth, children who received a prenatal diagnosis were no more symptomatic than those whose diagnosis was made postnatal: 21 (70%) versus 11 (73%; P=1) respectively. Similarly, they often received prophylactic surgery: 18 (60%) versus 2 (13%) respectively (P<0.01) and less often suffered post-surgery complication: 3 (10%) versus 10 (67%) respectively (P<0.01). The number of children monitored was not significantly different in the two groups. CONCLUSION: Prenatal diagnosis allows for the precise nature of the lesion in 90% of cases in 2013 and had no impact on symptomatology at birth. When prenatal diagnosis is possible, preventive surgery probably reduces the occurrence of emergency surgery.

Unilateral pulmonary agenesis: a report of four cases, two diagnosed antenatally and literature review.

Pulmonary agenesis is a rare congenital malformation of lung development defined as complete absence of lung tissues, bronchi, and pulmonary vessels; it may be uni- or bilateral. The right-sided form carries the poorest prognosis due to severity of co-existent anomalies. Its diagnostic circumstances are variables: first reported cases were diagnosed at autopsy, but early postnatal as well as fortuitous discovery have been reported. In recent years, progress in obstetrical imaging has made antenatal diagnosis possible so that fetal ultrasound and MRI allow early diagnosis and refinement by permitting the elimination of differential diagnoses (diaphragmatic hernia, cystic adenomatoid malformation of the lung, giant lobar emphysema, and situs inversus). This anomaly is compatible with normal life provided co-existent malformations are thoroughly investigated and managed in a multidisciplinary setting. We report four cases of lung agenesis two of which were diagnosed antenatally at 23rd and 30th weeks of gestation respectively. Our aim is to describe the circumstances having led to diagnosis and report both follow-up and outcome of our patients.

Pre-pregnancy body mass index and weight gain during pregnancy: relations with gestational diabetes and hypertension, and birth outcomes.

To study the relationship between pre-pregnancy body mass index (BMI) and weight gain during pregnancy with pregnancy and birth outcomes, with a focus on gestational diabetes and hypertension and their role in the association with fetal growth. We studied 1,884 mothers and offspring from the Eden mother-child cohort. Weight before pregnancy (W1) and weight after delivery (W2) were collected and we calculated BMI and net gestational weight gain (netGWG = (W2 - W1)/(weeks of gestation)). Gestational diabetes, hypertension gestational age and birth weight were collected. We used multivariate linear or logistic models to study the association between BMI, netGWG and pregnancy and birth outcomes, adjusting for center, maternal age and height, parity and average number of cigarettes smoked per day during pregnancy. High BMI was more strongly related to the risk of giving birth to a large-for-gestational-age (LGA) baby than high netGWG (odds ratio OR [95% CI] of 3.23 [1.86-5.60] and 1.61 [0.91-2.85], respectively). However, after excluding mothers with gestational diabetes or hypertension the ORs for LGA, respectively weakened (OR 2.57 [1.29-5.13]) for obese women and strengthened for high netGWG (OR 2.08 [1.14-3.80]). Low in comparison to normal netGWG had an OR of 2.18 [1.20-3.99] for pre-term birth, which became stronger after accounting for blood pressure and glucose disorders (OR 2.70 [1.37-5.34]). Higher net gestational weight gain was significantly associated with an increased risk of LGA only after accounting for blood pressure and glucose disorders. High gestational weight gain should not be neglected in regard to risk of LGA in women without apparent risk factors.

Influence of fetal and parental factors on intrauterine growth measurements: results of the EDEN mother-child cohort.

OBJECTIVE: In small-for-gestational-age neonates, parental and fetal characteristics can be used to distinguish between constitutionally small size and growth restriction, which is associated with a higher risk of morbidity and mortality. The aim of this study was to quantify relationships of parental and fetal characteristics with fetal ultrasound measurements. METHODS: The EDEN mother-child cohort included 2002 pregnant women with singleton pregnancies attending one of two university hospitals. Data from two routine ultrasound examinations for fetal biometry were recorded, at 20-25 and 30-35 weeks of gestation. Biparietal diameter (BPD), head circumference (HC), femur length (FL), abdominal circumference (AC) and estimated fetal weight (EFW) were studied as a function of prepregnancy maternal body mass index (BMI), maternal height, paternal height, fetal sex and gestational age. RESULTS: Data were obtained at the first scan from 1833 women and at the second scan from 1752 women. Parental anthropometric characteristics were significantly associated with ultrasound measurements at both scans. Maternal BMI was more strongly associated with AC and EFW, whereas both maternal and paternal height were more strongly associated with FL. An association was also found between fetal sex and all ultrasound measurements other than FL. CONCLUSION: Maternal and paternal anthropometric characteristics are significantly associated with ultrasound measurements in mid to late pregnancy. These relationships provide support for the use of these characteristics in ultrasound fetal size reference charts.

[Severe precocious pre-eclampsia : how to manage the feto-maternal conflict of interest]. / La pré-eclampsie sévère précoce : comment gérer le conflit d'intérêt foeto-maternel.

BACKGROUND: The HELLP syndrome is usually treated by rapid termination of pregnancy as the most effective way of limiting the risk of disease aggravation and maternal complications. The drawback is the risk of fetal complications due to prematurity when pregnancy is terminated before 32 weeks gestation. A controlled study has suggested that corticosteroid therapy could be effective in early HELLP syndrome. PATIENTS AND METHODS: From January 1, 1996 to March 1, 1999, we treated patients presenting early HELLP syndrome defined as platelet counts below 150,000 and ALAT above 50 U/l prior to 32 weeks gestation with dexamethazone (Soludecadron((R))) via intravenous administration of 10 every 12 hours to the end of pregnancy or until platelet counts rose above 150,000. RESULTS: Among 14 patients with early HELLP syndrome, 10 were treated (including 5 primiparous women). Six patients among the 10 had platelet counts between 50,000 and 100,000. Mean term at the first injection of dexamethazone was 29 weeks 3 days. Platelet counts rose and transaminase levels fell in all patients. Pregnancy was prolonged 7 2 days. The only maternal complication was one case of disseminated intravascular coagulation. There were no neonatal deaths. DISCUSSION: Our results are similar to those reported in the literature but such a treatment scheme can only be instituted within a rigorously controlled monitoring system in a unit with neonatal and maternal intensive care facilities. This protocol remains safe for patients with HELLP syndrome whose platelet count remains at least above 50,000.

[Impact of genital prolapse on the upper urinary tract]. / Le retentissement des prolapsus génitaux sur le haut appareil urinaire.

UNLABELLED: The authors reviewed five cases of genital prolapse affecting the upper urinary tract and causing renal failure. CLINICAL CASES: Five patients aged 55 to 75 years presented with genital prolapse, which was known by the patient in 3 cases for a period of 2 to 30 year, but left untreated. All cases had induced bilateral dilatation of the uretero-pyelocaliceal cavities with severe obstructive renal failure in 3 cases, moderate renal failure in 2 cases and associated with hypertension in 2 cases. Repair of prolapse, preceded by upper urinary tract diversion by ureteric and bladder catheters in 3 cases or pessary + bladder catheter because of the patient's age in 2 cases, cured or improved renal failure in 4 patients; only one patient had to be treated by dialysis because of the severity of the residual renal failure severity. DISCUSSION: The frequency (4%) of aetiopathogenic mechanisms (ureteric compression, progressive stretching of the ureter and posterior tilting of the trigone) are analysed. The delayed onset of sudden deterioration and the varying degrees of severity of renal failure demand emergency treatment of stage III prolapse with primary diversion of the upper urinary tract, currently by double J stents, until correction or improvement of renal function, after which surgical repair of the prolapse can be performed according to the usual surgical rules. Intravenous urography is still indicated in this situation. Palliative treatment is only indicated in elderly patients or patients with a high operative risk. The best treatment remains prevention by detection and treatment of prolapse before the development of this fortunately rare complication. CONCLUSION: This short clinical series emphasizes that undiagnosed prolapse can still be complicated by repercussions on the upper urinary tract with a risk of renal failure.