1/7. Longitudinal observation of deterioration of Doppler parameters, computerized cardiotocogram and clinical course in a fetus with growth restriction.We report on a fetus with intrauterine growth restriction detected at 27 weeks' gestation, who was longitudinally followed up until delivery by cesarean section 33 days later (31 5 weeks) due to severe decelerations in CTG. Longitudinal Doppler assessment of the umbilical artery (UA), the middle cerebral artery (MCA) and the main branch of the right pulmonary artery (RPA), the ductus venosus (DV) and the left coronary artery was compared to clinical course and computerized CTG. At first presentation (day--33) increased resistance in both the UA and uterine arteries with bilateral notches was found. Absent enddiastolic flow (AED) in the UA was found at day--19 and reverse flow (RED) at day--11. The MCA showed a decreased pulsatility first at day--19 and again at day--11 together with RED in the UA. The RPA initially (day--33) showed increased PI which returned to normal values at day--19 but increased again at day--1, when the DV showed RED and the coronary arteries became visible. The DV was normal until day--11, then its PI began to increase together with occurence of RED in the UA, but reverse flow in the DV occurred only on the eve (day--1) of severe decelerations in CTG. Short-term variability in computerized CTG was stable at 6 to 7 ms, except for an intermediate drop to 4 ms at day--10. Maternal hypertension was found at day--19 and mild preeclampsia developed at day--12. A reduction of fetal movements was noticed at day--5. This report shows that at 29 weeks gestation despite detection of AED resp. RED in the UA a prolongation of pregnancy for 19 resp. 11 days is possible. In addition to abnormal CTG, late signs of fetal deterioration are reverse flow in the DV and visibility of the coronary arteries. The role of increased resistance in the main branches of the pulmonary arteries should be examined in the future.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |
2/7. Cotyledonary-like placenta associated with severe intrauterine growth restriction.There have been no previous reports of human placentas mimicking the cotyledonary placenta of ruminants. We report a case of cotyledonary-like placenta associated with severe intrauterine growth restriction. A woman pregnant for the first time was referred to our hospital at 27 weeks' gestation because of severe intrauterine growth restriction. Characteristics of the placenta in the patient were examined by ultrasonographical and histopathological techniques. ultrasonography revealed that a hypo-echoic area intermingled with small segmented tissues in the placenta. The hypo-echoic area changed in size and shape according to uterine contractions. At 31 weeks' gestation, a caesarean section was performed because of non-reassuring fetal status. A female baby weighing 814 g was delivered. The placenta, which weighed 260 g, contained several long stem villi running over a distance of 6 to 7 cm without branching into intermediate or terminal villi. Small villous tissues had developed only at the tips of these stem villi. The present case shows an extraordinarily abnormal development of the villous trees in the placenta, which may have caused fetal IUGR and non-reassuring fetal status.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |
3/7. Very-early-onset discordant growth in monochorionic twin pregnancy.BACKGROUND: The significance of growth restriction in the first trimester in karyotypically normal fetuses is uncertain. CASE: We report a case of diamniotic monochorionic twin pregnancy with marked growth discordance noted in the first trimester. No major congenital or karyotype abnormalities or ongoing evidence of twin-twin transfusion syndrome were found during gestation. birth weight discordance in the twins was 49.5%. Histologic examination revealed immature villi, with a shortage of terminal villi and an abundance of intermediate mature villi, as well as hypoxic areas with altered villi in the part of the placenta supplying the smaller twin. CONCLUSION: The causes underlying discordant growth in karyotypically normal twin pregnancies without evidence of twin-twin transfusion syndrome are not entirely clear. There may be other conditions responsible for discordant growth that occur in the first or early second trimester of pregnancy.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |
4/7. Contribution of fetal MR imaging in the prenatal diagnosis of zellweger syndrome.zellweger syndrome (ZS), or cerebrohepatorenal syndrome, was the first described peroxisomal biogenesis disorder. It represents the most severe phenotype, and some of its multiple congenital anomalies can manifest prenatally. Fetal hypokinesia, renal hyperechogenicity, and cerebral ventricular enlargement are the most common reported fetal features. Single and/or late detectable manifestations account for most of the difficulties of prenatal diagnosis, as well as the limitations of ultrasonography itself. prenatal diagnosis, however, can be achieved through (1) assays of concentrations of peroxisomal metabolites (very-long-chain fatty acids, bile acids, intermediates, plasmalogens), (2) activities of peroxisomal enzymes (dihydroacetone-phosphate acyltransferase), or (3) molecular screening techniques, if available. We report on the contribution of MR imaging to the diagnosis of ZS in 2 unrelated fetuses. MR imaging was performed in the third trimester because of cerebral ventricular enlargement diagnosed on routine sonography examinations. In both cases, MR imaging revealed ZS-characteristic abnormal cortical gyral patterns, impaired myelination, and cerebral periventricular pseudocysts. In addition, MR imaging revealed renal microcysts and hepatosplenomegaly in one case. The high level of resolution of MR imaging, which allows analysis of cerebral gyration and myelination, facilitates the prenatal diagnosis of complex polymalformative syndromes such as ZS.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |
5/7. Diagnosis of unsuspected fetal metabolic storage disease by routine placental examination.GM1 gangliosidosis (type 1) is a rare hereditary, autosomal recessive, lysosomal storage disease characterized by a marked deficiency of active acid beta-galactosidase resulting in accumulation of gangliosides and mucopolysaccharides in tissues. disease status of newborns from affected kindreds may be diagnosed by placental examination. Typical findings include a characteristic vacuolar distension of the cytoplasm of syncytiotrophoblast and stromal Hofbauer cells. We report a case of unsuspected fetal storage disorder initially diagnosed by routine placental examination of a normal-appearing infant born to a previously unaffected family. Progressive, third-trimester oligohydramnios and fetal growth retardation had been documented by ultrasonography. Placental findings included vacuolization of syncytiotrophoblast, intermediate trophoblast, and stromal Hofbauer cells. Subsequent enzyme analysis confirmed the placental findings of storage disorder and diagnosed GM1 gangliosidosis.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |
6/7. Chorioangiomas of intermediate size and intrauterine growth retardation.Very large chorioangiomas are a rare but well recognized cause of neonatal morbidity, while small ones are clinically insignificant. This study emphasizes that some chorioangiomas of intermediate size may be causally related to intrauterine growth retardation, and that they may be surprisingly difficult to detect in the unfixed placenta.- - - - - - - - - - ranking = 5keywords = intermediate (Clic here for more details about this article) |
7/7. Preliminary definition of a "critical region" of chromosome 13 in q32: report of 14 cases with 13q deletions and review of the literature.We report on 14 patients with partial deletions of chromosome 13q. These patients exhibit a wide spectrum of phenotypes. Deletions limited to proximal bands q13-q31 are associated with growth retardation but not with major malformations. We review the literature since 1975 and summarize 13q deletion cases which have a phenotype involving one or more major malformations and mental retardation. Analysis of the breakpoints of these cases, as well as those reported by us, supports the hypothesis that only deletions involving at least part of band q32 are associated with major malformations and digital abnormalities. patients with more distal deletions have severe mental retardation but do not have major malformations or growth retardation. A group of patients in whom the breakpoint is stated to be within q32 has had an intermediate phenotype. This suggests that it may be possible to define subregions within q32 whose deletion is associated with particular developmental defects.- - - - - - - - - - ranking = 1keywords = intermediate (Clic here for more details about this article) |