Unilateral megalencephaly is a rare malformation of the central nervous system characterized by an overgrowth of one cerebral hemisphere due to an anomaly of neuronal cell migration. It shows macroscopic and histological alterations of the central nervous tissue.We report on a case of this malformation detected prenatally with the support of 3D ultrasonography. Copyright (C) 1999 John Wiley & Sons, Ltd.

Die Agenesie des Corpus callosum (ACC) ist eine relativ häufige Fehlbildung der zerebralen Mittellinie, sie ist mit dem Leben vereinbar und wird durch die zunehmende Verfügbarkeit moderner bildgebender Verfahren wie zerebrale Ultrasonographie (US), Computertomographie (CT) und Magnetresonanztomographie (MRT) auch immer häufiger diagnostiziert. Aus diesem Grund erlangt die Frage nach der weiteren neurologischen und mentalen Entwicklung der von dieser Fehlbildung betroffenen Kinder eine immer größere Bedeutung.

887 neonates at risk, referred to our neonatal unit underwent serial cranial ultrasound examinations and neurological follow-up over a period of 2 years. Our study focused on the prognosis of hemorrhagic and hypoxic-ischemic intracranial lesions. 194 patients with hemorrhages (subependymal hemorrhages [SEH] I degree-IV degrees according to Papile, hemorrhages of the choroid plexus [CPH], primarily intraparenchymal hemorrhages [PIH]) and/or hypoxic-ischemic lesions (infarcts of the major intracranial arteries and lesions of the periventricular white matter) were neurologically followed-up 12 to 24 months postnatally. A group of 266 patients with normal ultrasound scans out of the same population was equally followed-up and served as a control group. At the age of 12 months a preliminary neurodevelopmental diagnosis was made and the patients were divided into 3 groups. Group N (normal) had a normal neuromotor outcome, group S (suspect) showed minor neurological abnormalities without evidence of cerebral palsy and/or a developmental quotient between 80 and 90. Group A (abnormal) included patients with any degree of cerebral palsy (CP) and/or a development quotient below 80. A normal neurological outcome was seen in 88.3% of patients without intracranial lesion and in a comparable proportion of patients with SEH I degree (88.9%), SEH II degrees (84.8%) and CPH (81.3%). Patients with SEH III degrees developed normally in 72.7%, whereas only 25% of patients with SEH IV degrees and PIH were neurological normal at 12 months of age. For detailed statistical evaluation only preterm neonates (birthweight below 2500 grms) with and without hemorrhagic lesions were compared. Concerning the neurological short-term outcome our analysis revealed no statistically significant difference between patients with SEH I degrees, II degrees, III degrees, CPH and the control group. SEH IV degrees and PIH showed a unfavourable outcome. Only 2/8 surviving patients had a normal development, but small numbers of patients made a statistical analysis impossible. Two children with infarcts of the middle cerebral artery developed spastic hemiplegia of the contralateral body side. One child with an infarct of the posterior cerebral artery developed normally until the age of 1 year, but could not be followed-up further. Patients with periventricular lesions showed a normal neuromotor development in 88.9% and 75% when they had solitary periventricular cysts or wedge-shaped periventricular lesions, whereas none of 9 children who suffered from extensive cystic periventricular leucomalacia was neurologically normal at the age of 1 year.(ABSTRACT TRUNCATED AT 400 WORDS)

Veränderungen der periventrikulären weißen Substanz sind sonographisch deutlich von den bei Frühgeborenen häufig vorkommenden Keimlagerblutungen abgrenzbar.

Nachdem seit Jahren die Schädelsonographie routinemäßig an neonatologischen Abteilungen eingesetzt wird, drängt sich die Frage nach Inzidenz und prognostischer Bedeutung der diagnostizierbaren Veränderungen auf. Bezüglich Inzidenz und Mortalität herrscht — besonders was intrakranielle Blutungen betrifft — in der Literatur der letzten Jahre weitgehend Übereinstimmung, während andererseits die Bedeutung für die neuromotorische und mentale Entwicklung noch in vielen Punkten umstritten ist.

Zerebrale Malformationen stellen ein wichtiges Kapitel innerhalb der intra-kraniellen Pathologie von Neugeborenen und Säuglingen dar. Durch den weitverbreiteten Einsatz der Sonographie besteht nun die Möglichkeit, neue Erkenntnisse über Epidemiologie und Prognose zu erzielen. Die Inzidenz zerebraler Fehlbildungen ist abhängig von der Zusammensetzung des untersuchten Patientenkollektivs.

The aim of this retrospective study was to investigate differences with respect to infant morbidity and mortality following cardiotocographically monitored versus non-monitored deliveries. The observation period was 2 years. In particular, mortality was significantly higher in the non-monitored group of neonates weighing over 2500 g than in the monitored group of the same birth weight. The incidence of brain oedema in the group of infants where no cardiotocography had been employed during delivery was also higher. The results of this study are discussed.

The pyloric region of 58 infants who presented with vomiting was examined by real-time ultrasound. Transverse diameter of the pylorus and single wall thickness were measured. In 18 patients sonographic diagnosis of hypertrophic pyloric stenosis (HPS) was made and subsequently confirmed by surgery. The mean (+/- one standard deviation) of the transverse diameter and of the pyloric wall thickness in patients with HPS was 15 (+/- 1) mm. and 5.5 (+/- 0.7) mm., respectively. Forty infants matched for age and sex distribution without gastrointestinal symptoms served as controls. The measurements for the transverse diameter were 9.6 (+/- 1.8) mm. and for the wall thickness 1.9 (+/- 0.5) mm. Diameter values of patients with HPS were significantly (p less than 0.001) higher than those of the control group and those of the remaining 40 patients without HPS. Real-time sonography proved a reliable tool in the diagnosis of HPS.

Bei der gerichtlichen Obduktion eines 17 Tage alten Kindes wurde als Ursache einer umfänglichen Blutung in die Bauchhöhle ein Fall von infantiler multinodulärer Hämangiomatose der Leber festgestellt. Die behandelnden Ärzte hatten aufgrund der sonographischen Untersuchung eine solche Erkrankung bereits vermutet, sie waren aber aufgrund ihrer Untersuchungsergebnisse nicht in der Lage, eine endgültige Diagnose zu stellen. Durch das Ergebnis der Obduktion konnte auch ein Fremdverschulden der Geburtshelfer ausgeschlossen werden.

The results of 293 examinations of the skull by sonography in 170 newborn are presented. In 73 patients the results could be compared with the findings on CT or at post mortem. In 48%, abnormal sonograms of the skull were obtained, due to congenital abnormalities, hydrocephalus or intracerebral bleeds. High accuracy and wide application make real time sonography the diagnostic procedure of first choice for the examination of the brain of the newborn. An attempt has been made to determine the optimum time for the examination and suitable intervals for follow-up.