E. Vuillard

Congenital diaphragmatic hernia : antenatal prognostic factors : Does cardiac ventricular disproportion in utero predict outcome and pulmonary hypoplasia?

Abstract Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80 % mortality. It is impossible to predict which fetus with CDH will survive or not. Objective: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. Design: Retrospective study. Setting: Paediatric intensive care unit of a university childrens hospital. Patients and methods: Antenatal parameters and presence of left ventricular hypoplasia in utero were compared retrospectively to outcome and to presence of PH in 32 consecutive newborn infants with antenatally diagnosed CDH. Antenatal parameters included: gestational age at diagnosis, herniated organs, associated malformations and presence of polyhydramnios. Size of the cardiac ventricles, the aorta (Ao) and the pulmonary artery (PA) were obtained by fetal echocardiography, from which we calculated a cardioventricular index (left ventricle/right ventricle, LV/RV) and a cardiovascular index (Ao/PA). Delivery was planned in order to provide ventilatory and hemodynamic management. In case of death, PH was assessed according to the following criteria: the lung weight/body weight index and the radial alveolar count. For statistical comparisons, patients were separated into two groups: the hypoplasia group (H) and the non-hypoplasia group (NH). Results: Thirty-two pregnancies were delivered. Twenty-six newborns died (81 %), 6 survived (19 %). When comparing non-survivors to survivors, predictors of poor outcome were: mean gestational age at diagnosis (23 vs 28 weeks, p = 0.002), intrathoracic stomach (20 vs 1 s, p = 0.01) and associated malformations (6 vs 0). Cardiac ventricular disproportion, expressed by the LV/RV ratio, appeared to correlate well with a poor outcome (0.63 in non-survivors vs 0.93 in survivors, p = 0.03) and with PH (0.63 in the H group vs 0.95 in the NH group, p = 0.03). Conclusions: Our study confirmed the factors for a poor prognosis associated with CDH previously described in the literature, but none with a consistent demonstration of accuracy. LV hypoplasia may be a more accurate predictor of outcome and of PH but it has to be assessed by prospective studies with larger samples. Further basic science and Doppler-flow studies may be helpful to understand the natural history and pathophysiology of LV hypoplasia in CDH.

Highly differentiated teratoma and fetus-in-fetu: A single pathology?

A case of sacrococcygeal teratoma is presented with characteristics of fetus-in-fetu. This pseudo-fetus presented a rudimentary single cavity heart, which beat at a different rate to that of the affected infant. X-ray examination showed no spinal column. This case confirms that fetus in fetu can be a remarkably complex, well-differentiated, highly organized teratoma.

Use of ultrasound to distinguish between fetal hyperthyroidism and hypothyroidism on discovery of a goiter.

To determine whether sonography can be used to distinguish hyperthyroidism from hypothyroidism in pregnancies with fetal goiter.

Management of fetal thyroid goitres: a report of 11 cases in a single perinatal unit.

Fetal thyroid goitres may reveal hormonal imbalance. This can jeopardize neurological development and fetal outcome even when early postnatal treatment is provided. We report a series of 11 goitres diagnosed antenatally in women with past or present thyroid disorders or discovered fortuitously on ultrasound scan. Fetuses presented with hyperthyroidism in three cases and hypothyroidism in eight. Hypothyroidism was iatrogenic in five cases, due to maternal anti‐thyroid drugs. Hyperthyroidism was induced by transplacental transfer of thyroid stimulating antibodies (TSHrab). Accurate diagnosis of fetal thyroid status was obtained by fetal blood sampling but this invasive method was deemed necessary only in four cases as maternal clinical and biological data and ultrasound signs provided sufficient information to infer the type of thyroid disorder in the remaining patients. Fetal therapy relied on reduction of maternal antithyroid medication and, in selected cases, intra‐amniotic injection of levothyroxin in hypothyroidism, and on administration of antithyroid drugs in hyperthyroidism. All newborns were healthy and none displayed consequences of severe thyroid imbalance. No caesarean section was performed for dystocia. Fetal thyroid goitres can be managed successfully with selected use of invasive diagnostic and therapeutic techniques. Copyright

Amniotic fluid inflammatory proteins and digestive compounds profile in fetuses with gastroschisis undergoing amnioexchange

Objective  In gastroschisis, an inflammatory process related to the presence of digestive compounds may be involved in intestinal damage. We measured the amniotic fluid concentrations of total protein, ferritin and amylase, lipase, γ‐glutamyl transferase and bile acids before each amnioexchange performed in women whose infants had gastroschisis. We estimated the correlation among total proteins, ferritin and digestive compounds and postnatal outcome.

Prognostic factors of prenatally diagnosed gastroschisis.

OBJECTIVE To evaluate the prognosis of prenatally diagnosed gastroschisis. STUDY DESIGN In a retrospective study, we analyzed the clinical and echographic data of gastroschisis. These data were correlated with fetal outcome including delivery, surgical procedure, follow-up in the neonatal intensive-case unit and in the gastropediatric unit. RESULT Twenty cases were analyzed. The overall survival rate was 85%. Classical criteria were analyzed (maximal bowel dilatation, thickening of bowel wall). Fetuses with both severe perivisceritis and meconium-stained amniotic fluid were born earlier than fetuses with mild perivisceritis and normal amniotic fluid (p < 0.01). CONCLUSION Our data suggest that an inflammatory response could follow bowel exposure to amniotic fluid. This response could lead to perivisceritis and premature birth. This hypothesis is currently under investigation.

Iodine deficiency in northern Paris area: impact on fetal thyroid mensuration.

Introduction Iodine is essential for normal fetal and neonatal development. We studied the prevalence and impact on fetal thyroid development of iodine deficiency in pregnant women in the northern part of the Paris conurbation. Materials and Methods 110 patients underwent several determinations of urinary iodine excretion (UIE) and of serum FT4, FT3, and TSH. Fetal thyroid gland size was assessed using ultrasonography. Results We found evidence of widespread iodine deficiency (mean UIE, 49.8 µg/L [standard deviation, 2.11]). Iodine deficiency did not correlate significantly with maternal thyroid parameters but showed a significant negative correlation with fetal thyroid gland size (rho = 0.25, P = 0.02). Conclusion Iodine deficiency during pregnancy is still a problem in our geographical area and affects the fetal thyroid gland. Clinical Trials.gov NCT00162539

Trigonocephaly and valproate: a case report and review of literature.

Morgane Valentin1, Guillaume Ducarme1*, Carine Yver1, Edith Vuillard2, Nadia Belarbi3, Dominique Renier4 and Dominique Luton1 1Department of Obstetrics and Gynecology. Hôpital Beaujon, AP-HP, Université Paris 7, Clichy, France 2Department of Prenatal Diagnosis. Hôpital Robert Debré, AP-HP, Université Paris 7, Paris, France 3Department of Pediatric Radiology. Hôpital Robert Debré, AP-HP, Université Paris 7, Paris, France 4Department of Pediatric Neurosurgery. Hôpital Necker-Enfants Malades, AP-HP, Université 5, Paris, France

Prenatal Diagnosis of Pial-Superficial Arteriovenous Malformation

In the newborn, cerebral pial arteriovenous malformation has been recognized as a cause of congestive heart failure. Prenatal diagnosis allows early medical treatment of cardiac failure and increases the chance of successful neuroradiological intervention. This paper highlights the importance of careful prenatal cerebral examination in cases of cardiac ventricle enlargement.

OC209: Amniotic fluid exchange in gastroschisis

history. When abnormalities were detected CDTVU was repeated after 4–6 weeks, if the abnormality persisted, tumor markers, CT and laparoscopy were done. The mean age of the patients was 47 ± 12 year ranging from 16 to 88 years. Results: From 22.013 CDTVU performed 35 were diagnosed as suspicious of malignancy. Twenty nine of these patients have been surgically treated, in two the repeated CDTVU was normal and 4 were lost from follow-up. Nineteen of the surgically treated women had malignant lesions hystologically confirmed, 15 of them were tumors in stage I, 3 in stage III and one was a metastatic melanoma. In 10 patients benign lesions were diagnosed (3 serous cysts, 2 mucinous cysts, 2 endometriotic cysts, 1 cystadenofibroma, 1 cystic teratoma and 1 hydrosalpinx with paraovarian cyst). Conclusion: Although consensus about the benefits of using CDTVU as a screening procedure are not conclusive, our results suggest that it can help in diagnose of ovarian cancer in early stages.