[Venous infarction of the neonate]. / L'infarctus veineux hémorragique du nouveau-né

PURPOSE: To determine the imaging features of hemorrhagic infarction in neonates. PATIENTS AND METHODS: Retrospective study (1998-2008) of 19 children (17 premature and 2 term deliveries) with early lobar hyperechogenicity on transfontanel US (TFUS). Group I: 11 born infants with clinical as well as TFUS and MRI follow-up. Group II: 8 premature infants deceased within a week from multisystem pathology, with neuropathological study available in 3 cases. RESULTS: Group I (n=11): periventricular hyperechogenicity in a frontal (7), frontoparietal (2), parietooccipital (1) and temporoparietal (1) distribution with developing cavitary change (n=6). MRI showed a cortex sparing intraparenchymal hematoma. Group II (n=8): periventricular hyperechogenicity in a frontal (4), frontoparietal or parietal (3) and occipital (1) with developing cavitary change (3). Neuropathological examination showed characteristic lesions of venous hemorrhagic infarction. Clinical outcome was generally favorable for the surviving infants with contralateral motor deficit (n=5) non-correlated to the extent of the initial lesions. CONCLUSION: Venous hemorrhagic infarction mainly affetcs premature infants and typically involves the periventricular frontal white matter. Prognosis is generally favorable. It is thus important to differentiate this entity from asymmetrical cystic periventricular leukomalacia with much different prognosis.

[Cerebral infarction in full-term newborns: MR imaging features]. / Accident vasculaire cérébral (AVC) du nouveau-né à terme: aspects en IRM.

PURPOSE: Perinatal stroke is the most frequent cause of congenital hemiplegia. The MR imaging features of cerebral infarction in full-term newborns will be reviewed and the underlying etiologies discussed. PATIENTS AND METHODS: Retrospective study (1999-2005) including 15 newborn infants without history of fetal distress with early seizures (before day 7) with ischemic or hemorrhagic infarct on MR. All MR examinations were reviewed by consensus (MBD, CHA) using a checklist and results were correlated with findings at clinical follow-up (mean follow-up of 1 year). RESULTS: MR showed ischemic strokes in 10 infants (5 left, 4 right, 1 bilateral) in the middle (n=9) or anterior (n=1) cerebral artery distribution or with extensive bilateral distribution. The cortex and subcortical regions were involved in all cases with ipsilateral basal ganglia/internal capsule extension in 4 cases. The imaging features were related to the timing of the MR examination: within 5 days (n=4): cortical effacement on T1W and T2W images, increased signal on DWI with reduced signal on ADC; between 7-14 days (n=6): T1W hyperintense and T2W hypointense cortex. Five infants had unifocal (3/5) or multifocal (2/5) hemorrhagic infacrtion. Of 13 patients followed-up from 3 months to 3 years (1 lost to follow-up, 1 deceased), 11 had no clinical deficits, 1 had hemiparesis, and 1 had asymmetrical muscle tone. The latter 2 infants had involvement of the posterior limb internal capsule, with basal ganglia and hemispheric involvement. In the literature, this association of lesions is considered predictive of hemiplegia and a high rate of sequelae is reported following neonatal stroke: 50-75% with motor deficit and/or seizure disorder. The 4 infacts with hemorrhagic infarction followed-up for 14 months to 5 years had a globally favorable neurological outcome. CONCLUSION: Cerebral infacrtion in full-term newborns without associated risk factor is variable and should be recognized. Early MR, before day 2, with diffusion-weighted sequences, allows diagnosis but follow-up MR after day 7 is necessary to better depict the extent of permanent lesions. The presence of hemorrhage is not a predictive factor of poor neurological outcome.

Antenatal thalamic lesions in the newborn: two anatomoclinical cases.

We report two cases of antenatal bilateral thalamic lesions constituted by neuronal loss, gliosis and mineralized glial or neuronal cells. No etiology could be found. Neuroradiological findings were poorly correlated with histological changes. These cases are compared with the few previously reported cases of the same condition. We strongly recommend extensive etiological investigation as recurrence occurred in one family.

Developmental aspects of type II lissencephaly. Comparative study of dysplastic lesions in fetal and post-natal brains.

We report neuropathological studies of five cases of type II lissencephaly from three fetuses and two infants. This comparative study allowed us to determine the developmental course of the cerebral lesions. Two distinct developmental events seem to generate this type of brain malformation: firstly, an early disturbance in cortex formation, which results both from a disorder of radial migration and a pial barrier disruption; secondly, a late perturbation of cerebral surface organization, resulting in fusion of the cerebral surface. All these features can be related to a primitive meningeal pathology, and more generally, to a neurocristopathy. Accordingly to our observations, this brain malformation appears during both migrational and post-migrational stages and may be considered more like a polymicrogyria than a lissencephaly.

Cardiac rhabdomyoma and tuberous sclerosis in three fetuses: a neuropathological study.

In spite of the development of modern imaging, most lesions of tuberous sclerosis (TS) remain difficult to detect before birth. Particularly, brain involvement at a fetal stage of development is poorly documented. We report three cases of fetuses examined after pregnancy was interrupted because of the detection of cardiac rhabdomyoma. In two of the three cases there were brain lesions suggestive of TS, including cortical tubers, subependymal nodules and scattered bizarre giant cells in the white matter. These observations confirm that brain lesions of TS can be present before birth; they can show, at an early period of development, an aspect quite similar to lesions described at an adult stage. The most characteristic cell abnormality is the so-called balloon cell. The majority of these cells exhibit a strong immunoreactivity with glial antibodies (GFAP, vimentin, S100). Immunoreactivity with neuronal markers (synaptophysin) is present in a small percentage of balloon cells.

Microgyric and necrotic cortical lesions in twin fetuses: original cerebral damage consecutive to twinning?

Extensive cortical necrosis associated with malformative microgyric-like lesions and with necrotic lesions of the white matter was observed in two male 25 week fetuses. These cases differed from previously reported cases of brain damage in monozygotic twins: both fetuses were affected and the lesions occurred early in pregnancy, before the end of neuronal migration, thus resulting in a cortical malformation associated with destructive lesions.

Bilateral porencephalic defect in a newborn after injection of benzol during pregnancy.

Porencephaly is usually considered to be a prenatal brain lesion due to a circulatory failure. We report a case of bilateral porencephaly with heterotopia and absence of the septum pellucidum in a newborn. The mother had received several injections of benzol during pregnancy with an intent of inducing abortion. The possibility of a causal relationship between the administration of benzol and the occurrence of the defect is supported by the existence of previously reported cases of cerebral malformations following maternal exposure to organic solvents.

Kearns-sayre syndrome. Two clinico-pathological cases.

Two clinico-pathological cases of Kearns-Sayre syndrome are reported. In both cases the typical triad (progressive external ophthalmoplegia, heart block, retinitis pigmentosa) was present and spongiosis was the main pathological finding. In one case there was also a marked capillary proliferation, significance of which is discussed. A deletion of the mitochondrial DNA was found in the muscle, spinal cord and brain of this last case.

Familial occurrence of prenatal encephaloclastic damage: anatomoclinical report of 2 cases.

Two brothers had a congenital cerebral malformation suggestive of a destructive process of the parenchyma; the first was hydranencephalic with extensive heterotopias. The second had focal microgyria and subcortical heterotopias. The precise origin of this disorder, which occurred two times in the same sibship, was not found; there is no reported genetic disease capable to cause such destructive brain lesion. The possibility of a persistent infectious disorder causing repetitive disturbance of gestation is discussed; it has not been proved. A fortuitous association cannot be excluded.

[Bilateral thalamic hemorrhage following severe asphyxia in a 9-month-old infant]. / Hémorragie thalamique bilatérale au décours d'une asphyxie sévère chez un nourrisson de 9 mois.

We report a case of post-asphyxic bilateral thalamic hemorrhage in a 9 month-old infant, consecutive to a hemophilus hypopharyngitis. The neuropathologic study revealed that it was in fact a hemorrhagic necrosis associated to diffuse necrosis of the hemispheres. This case is compared to the other reported cases of thalamic hemorrhage and the physiopathologic mechanism is discussed.

Neurodevelopmental outcome in cystic periventricular leukomalacia (CPVL) (30 cases).

Thirty infant survivors had a wide spectrum of neonatal cystic periventricular leukomalacia (CPVL) diagnosed by ultrasound scans: 11 minor forms, 12 moderate forms, 7 major forms (categorized by both sagittal extent and coronal thickness). Eight cases were pure CPVL and 22 were associated with minor to moderate peri-intraventricular haemorrhage (PIVH), i.e. from (0-I) to (II-II). Mean gestational age: 31 +/- 2 weeks; mean birthweight: 1,532 +/- 356 g. The length of neurodevelopmental (ND) follow-up ranged from 13 months to 3 years and eight months. Of 11 children with minor CPVL, 9 were normal and two had moderate sequelae. Of 12 moderate CPVL, one was lost to follow-up, one had no motor disturbance but did have severe mental retardation (partial 7 p-), two were normal, one had a minor sequela, three had moderate and four had severe sequelae. Of the 7 major CPVL, one had moderate and 6 had severe sequelae. Cases including posterior lesions or presenting solely with such lesions had a worse outcome. In that range of PIVH, the outcome was related to the severity of CPVL and not to PIVH grade. All major late dilatations were associated with severe ND sequelae. Of 29 infants with early EEG tracings, the 7 children with early severe EEG abnormalities all had severe ND sequelae; 17 persistent EEG abnormalities, even mild, were associated with 9 severe and five moderate ND sequelae, and three normal outcomes. Combining US scans and EEG gives valuable prognostic information in minor and major CPVL, but less in the moderate forms.

Inverted neurons in agyria. A Golgi study of a case with abnormal chromosome 17.

An anatomoclinical observation of agyria is reported. The karyotype revealed a partial deletion of the short arm of chromosome 17. The etiology of agyria is reviewed in the light of this chromosomal abnormality. In addition we describe the peculiar pattern of neurons in the cortex: Golgi stain demonstrated many inverted pyramidal cells in the superficial part of the cortical layer. The mechanism of this abnormality is discussed.

Congenital hydrocephalus and eye abnormalities with severe developmental brain defects: Warburg's syndrome.

Five patients are reported with Warburg's syndrome, characterized by: (1) congenital hydrocephalus, (2) severe neonatal neurological dysfunction, (3) abnormalities of the anterior and posterior chambers of the eyes, (4) absence of known cause, and (5) severe developmental abnormalities of cortical gyration and architectonics. Fourteen similar published cases are reviewed. The syndrome can be diagnosed during life on the conjunction of the first four features listed. Evidence is adduced that this syndrome is a genetically determined condition with an autosomal recessive mode of inheritance and with a 25% recurrence risk for offspring of the parents of an affected infant.

[Long-term fate of very-low-birth-weight children]. / Devenir d'enfants de très faible poids de naissance.

75 neonates with birth weights less than or equal to 1,250 g were admitted to a neonatal intensive care unit. 34 were followed up for about 4 years. 44% (15) of the surviving children are normal; 35% (12) present with minor sequellae compatible with normal familial, social and school life; 14.7% (5) presents with moderate sequellae; 6% (2) present with major sequellae. None presents with hydrocephalus, bedridden encephalopathy, deafness or blindness. Among these 34 children, 12 have growth retardation. All children with sequellae presented with important neurologic involvement during the neonatal period. No silent period was observed during follow-up. An early screening for the disorders is possible. The neonatal risks, often unappreciated or underestimated are, in most cases, foreseeable when taking into account the mother's history and course of pregnancy. Thanks to improvement of in utero screening techniques, proper cooperation between obstetricians and pediatricians should allow for selecting a better management for all very low birth weight prematures.

[Very low birth weight neonates. Results of management. Future prospects]. / Nouveau-nés de très faible poids de naissance. Résultats. Perspectives d'avenir.

70 neonates with birth weights (BW) less than 1 250 g were treated at the intensive care unit for neonates (USINN), hôpital Port-Royal, from April 1st 1976 to July 31st 1977. 16 of them had intra-uterine growth retardation. In 20, BW cas under 1 000 g. Assisted ventilation was necessary in 54 (77%). 43 children (61%) survived, those who were born in the same hospital did so more often (88%) than those who came from other hospitals (53%) (p less than 0.01). These results show the needs and risks of the management of very low birth weight neonates: preventing hypothermia, assisted ventilation of long duration and risk of persisting ductus arteriosus, prolonged parenteral nutrition, risk of intracranial hemorrhage more especially as respiratory distress is more severe. In survivors, the high rate of respiratory sequellae (around 30%) suggests that the duration and intensity of assisted ventilation should be reduced as much as possible.