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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 408-412 
    ISSN: 1433-0350
    Keywords: Walker-Warburg syndrome ; Miller-Dieker syndrome ; Lissencephaly ; Ventriculomegaly ; Spina bifida ; Cephalocentesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two cases of prenatal diagnosis of lissencephaly are presented in the context of a series of 118 cases of prenatally diagnosed hydrocephalus. Within this series there was one case of Walker-Warburg syndrome and another of Miller-Dieker syndrome. It is stressed that the cases reported here of ventriculomegaly diagnosed in utero show a very different outcome from those in published studies of fetal hydrocephalus which only deal with patients in whom the diagnosis was determined after birth. In those postnatal series there is a considerable selection bias, and the fate of the fetuses reported here was much worse than in postnatal series. Of the 118 fetuses 6 had fetal infections, 6 had chromosomal abnormalities, 26 had associated spina bifida, 64 fetuses had associated other anomalies, and only 28 had isolated hydrocephalus. Although it is difficult to determine the prognosis individually after prenatal diagnosis of ventriculomegaly, the data presented here may be helpful in counseling parents prenatally. The counseling should be performed with the collaboration of obstetricians, pediatricians, surgeons, and geneticists.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-1285
    Keywords: Key words Pregnancy – transposition of the great arteries – Mustard procedure – complications ; Schlüsselwörter Schwangerschaft – Transposition der großen Arterien – Mustard-Operation – Komplikationen
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Background: In patients after atrial switch operation (Mustard or Senning procedure) for transposition of the great arteries (TGA), the anatomic right (anterior) ventricle works as the systemic ventricle. Although this is associated with an increased rate of congestive heart failure, the prognosis for long-term survival is good. Therefore a large proportion of these patients has reached their reproductive years and consult cardiologists and obstetricians because they plan a pregnany or are already pregnant. Because of the substantial hemodynamic changes and the increase in cardiac output during pregnancy, potential risk factors and complications have to be considered. Patients: Initiated by the presentation of a pregnant patient with TGA after surgery in our hospital, we analyzed the information referenced in MEDLINE. Including our patient, there were reports on 27 patients after the Mustard procedure with a total of 39 pregnancies. Fetal outcome: Three abortions occurred during the first trimenon, another one was electively induced because of maternal cardiac deterioration. One late abortion occurred in 23rd week of pregnancy. 35 babies (one twinpair) were born healthy without cardiovascular anomalies. Maternal outcome: No maternal deaths occurred. Eight women developed clinical signs of systemic (= right) heart failure including all three patients with former complex TGA. Four of these patients recovered after delivery, four showed persistent reduction of physical abilities or signs of heart failure. Further complications were supraventricular tachycardias (five patients) and a high incidence of hypertension and pre-eclampsia (22% of patients). Conclusion: Overall, the outcome for mothers after Mustard procedure for TGA and their children ist good, but there is need for intensive and spzialized follow-up.
    Notes: Zusammenfassung Hintergrund: Patienten nach atrialen Umkehroperationen (Mustard- oder Senning-Operation) zur Korrektur einer Transposition der großen Arterien (TGA) unterscheiden sich in einem wesentlichen Punkt von anderen Patienten mit kongenitalen Vitien: Der anatomisch rechte (vorne gelegene) Ventrikel fungiert bei ihnen als Systemventrikel. Da eine wachsende Zahl derart operierter Patienten das Erwachsenenalter erreicht, gewinnt die Frage, ob eine Schwangerschaft für die so operierten Patientinnen wegen der dabei auftretenden, ausgeprägten hämodynamischen Veränderungen eine Gefährdung darstellt, zunehmende Bedeutung. Patienten: Initiiert durch die Vorstellung einer schwangeren Patientin in unserer Klinik konnten durch Literatursuche in MEDLINE weitere 26 Kasuistiken über Schwangerschaften nach Mustard-Operation identifiziert werden. Bei den somit insgesamt 27 Patientinnen wurde über 39 Schwangerschaften berichtet. Fetale Komplikationen: Es kam zu drei Frühaborten im 1. Trimenon und einer Fehlgeburt in der 23. Schwangerschaftswoche. Weiterhin wurde ein elektiver Abort im 1. Trimenon durchgeführt. 35 Kinder (ein Zwillingspaar) wurden lebend geboren ohne kardiovaskuläre Fehlbildungen. Mütterliche Komplikationen: Von den Müttern verstarb keine. Acht Patientinnen entwickelten eine therapiebedürftige Herzinsuffizienz, wobei alle drei Patientinnen mit vormals komplexer TGA (zusätzliche andere kardiale Fehlbildungen) betroffen waren. Von diesen erholten sich vier Patientinnen innerhalb der Nachbeobachtungszeit, die anderen vier Patientinnen zeigten eine anhaltende Verschlechterung ihrer körperlichen Leistungsfähigkeit oder klinische Zeichen einer Herzinsuffizienz. Als weitere Komplikationen kam es bei fünf Patienten zu supraventrikulären Tachykardien und bei 22% der Patientinnen zu einer Hypertonie/Prä-Eklampsie. Schlußfolgerung: Zusammenfassend zeigt sich eine gute Prognose für Mütter nach Mustard-Operation und ihre Kinder, ein intensives und spezielles Monitoring vorausgesetzt.
    Type of Medium: Electronic Resource
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