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Objectives. – If the pattern of neonatal lenticulostriate vasculopathies (NLSV) is well-known, the prognosis is unknown except in TORCH syndromes. This study was aimed to describe the short, mid and long-term outcome of NLSV of various origins.Population and methods. – Of 9138 neonates (1981–2000) which were submitted to an early brain ultrasound study, 70 presented with a pattern of minor (35), moderate (27) or severe (8) NLSV, a single finding in 42 cases and in association with minor peri-intraventricular haemorrhage and/or leukomalacias in 28. The maternal and neonatal charts were reviewed, and the survivors were followed according to our usual policy.Results. – Of nine deaths, eight cases included severe congenital defects (metabolic or malformations or acquired: transfused monochorial twins). Of 61 survivors, eight were lost to follow-up within the first year, 53 were followed for 21 months to 9 years and 7 months (median 4 years 5 months). Of 53 children, 35 (66%) were strictly normal, eight had minor deviations, four had moderate and six had major neurodevelopmental deficits. Of 34 survivors with isolated NLSV and known follow-up, 27 were normal (79%) versus 8/19 (42%) in associated NLSV.Conclusions. – Minor or moderate isolated NLSV generally have a good long-term prognosis. Associated forms of any severity depend mainly upon the severity of periventricular leukomalacias. Major forms of NLSV must be a warning sign of a possible underlying congenital anomaly which will rule the vital and functional prognosis. [Copyright &y& Elsevier]

Background: Neonatal branching echogenic streaks in the basal ganglia or 'lenticulostriate vasculopathy' (LSV) has no clear comprehensive aetiology., Objective: To provide some clinical evidence, possibly relevant to aetiology, by analysis of a large series., Materials and Methods: Seventy cases (9 deaths, 3 post mortem) identified between 1981 and 2000 out of 9,138 neonates with routine brain sonograms (578 in a protocol for neonates from HIV+ mothers). Review of maternal/neonatal charts for clinical data and serologic status. Retrospective analysis of sonograms for grading and time course of LSV and coexistent abnormalities., Results: LSV was unilateral in 31 cases (22 right), bilateral in 39. Grading separated 8 major, 27 moderate and 35 minor patterns. In 42 cases, LSV was isolated, and in 28 it was mixed with peri-intraventricular haemorrhage grades I or II and/or leucomalacia. LSV was detected in the first postnatal week in 56 instances and appeared later in 13. Disappearance occurred mostly within 2 months. Perinatal circumstances: 13 recipient monochorionic twins (polycythaemia from twin-twin transfusion syndromes), 20 HIV+ mothers (possible immunological disturbances; only 2 infected neonates), 10 congenital anomalies, 1 toxoplasmosis, 1 postnatal cytomegalovirus infection, 25 miscellaneous conditions. Numerous maternal and neonatal factors were non-contributory., Conclusions: These results do not support congenital toxoplasmosis, rubella, cytomegalovirus, and herpes (TORCH) infections as the main causative factor. Polycythaemia and various immunological disturbances may be involved, as well as infectious agents not appropriately screened by routine serodiagnoses.

Objective: The aim of this study was to describe the clinical data and sonographic evolution of subependymal cysts (SEC) and to compare alone SEC versus SEC combined with other brain abnormalities., Methods: The diagnosis of SEC relied on the discovery of one/several cysts at the outer angle of one/both lateral ventricles detected by routine brain ultrasound tests in the first days of life. During the 1981-2000 period, 66 neonates had evidence of SEC in Port-Royal Department of Neonatal Medicine. The cases were divided into two groups: G-I, alone SEC; G-II, SEC combined with other brain abnormalities. Hospital charts were reviewed for gestational and delivery data and ultrasound scans were analyzed according to a preset list of items (dates, measurements, other findings)., Results: Patients with alone SEC had a higher gestational and birthweight, better birth conditions, and less respiratory morbidity. High rates of associated congenital anomalies were observed in both groups (19% in G-I, 13% in G-II, NS). Rare pre/perinatal infections did not show any association with SEC. SEC were uni or bilateral, single or multiple (string), with no difference between G-I and G-II. Unilateral SEC predominated in the left side (26/28, P<0.01). Serial examinations were carried out in 49/66 (74%), showing an increase in size of SEC in 21/49 (45%) in the first month of life, while 12 SEC (24%) disappeared. The 11 deaths occurred in neonates with other severe brain injuries (five peri-intraventricular hemorrhages, four periventricular leukomalacias) except one (diaphragmatic hernia)., Conclusions: Alone SEC and SEC combined with other brain abnormalities follow the same morphologic pattern and evolution. The striking predominance in the left side may suggest another etiology. Both are in favor of a developmental deviation and not a viral fetopathy.

Objective: To compare the family status and neurodevelopment of triplets conceived by spontaneous pregnancy (SP), ovarian stimulation (OS), in vitro fertilization (IVF), and gametal intra-fallopian transfer (GIFT)., Design: A follow-up study consisted of neurodevelopmental examination and/or of interviews of parents/pediatricians/teachers and social workers. Outcome of the children was categorized as defective (mild, moderate, severe) or normal., Setting: The Port-Royal neonatal unit and follow-up clinic., Subjects: From 1/01/1987 to 31/12/94, one to all three neonatal triplets were admitted to the Port-Royal neonatal unit. Pregnancy was SP in 12, OS in 24, IVF in 36, GIFT in 5. All survivors (36 SP, 68 OS, 98 IVF, 14 GIFT) were included and their outcome (at 3 to 10 years of age) was known in 75 families., Results: Prematurity < 32 weeks was highest in the SP pregnancies, with no birth > 37 weeks, and more small-for-dates babies. As of this writing, the neurodevelopmental status was normal or included only a mild deficit (mainly visual corrections) in 82% of SP survivors, 94% of OS survivors, 98% of IVF survivors, all 14 GIFT survivors. Moderate and major deficits were found in one or two siblings of 7 sets of triplets, 5 of which were born < 32 weeks (1 SP, 3 OS, 1 IVF). In all groups, family status was characterized by the need to move, financial problems, maternal exhaustion and parental breakdowns. Five OS mothers, 5 IVF mothers, and one GIFT mother each had one to three subsequent SP pregnancies. In 1997, all families included one to eight children. Because of the cumulative effect of losses, deficits, and other post-natal difficulties, only one of four families had no problems., Conclusion: The overall outcome of surviving triplets is good but they are both a prenatal and post-natal hardship for the parents, chiefly the mother. We advocate a more cautious use of OS, no triple implantation at the first IVF, and more social help and guidance for the families during the pregnancy and the first 3 years of life of the triplets.

Based on results of epidemiological studies, dorsal or lateral sleeping positions are now recommanded in the prevention of sudden infant death syndrome (SIDS). This raises an ethical question about the attitude towards the ventral positioning therapy for gastroesophageal reflux (GOR). The consensus conference considers that the ventral position should only be recommanded in GOR when the benefit appears to outweigh the risk of SIDS that it induces. The conference proposes that for infants with simple uncomplicated reflux, sleeping in the prone position should not be introduced in the first line treatment. Prone positioning should be restricted to complicated cases resistant to dietary and medical measures.

Objective: To assess the physical and neurodevelopmental status of children born after in vitro fertilization (IVF) as well as the family condition., Design: Follow-up study including physical and developmental examination in available families, and/or interviews of parents/pediatricians/teachers/social workers., Setting: The Port-Royal neonatal intensive care unit and follow-up clinic., Subjects: From 1.1.1987 to 31.12.1992, in 32 single, 71 twin, 28 triple, and one quadruple pregnancies resulting from IVF (total 132), one or more neonates were admitted to the Port-Royal NICU. The outcome is presented for the 99 families followed longer than two years (199 neonates, 166/199 born prematurely)., Main Outcome Measures: Neurological sequelae (minor, moderate, major), developmental quotients, school level, behavioral disturbances, family conditions., Results: From 21 singletons (2 deaths, 1 lost), 18 survivors were followed: 17 were normal above 2 years (10 above 3 years went to school), 1 had a severe cerebral palsy with low IQ. From 57 twin pregnancies (7 fetal and 9 neonatal deaths), the 98 survivors were followed: 78 were normal above 2 years (65 above 3 years went to school), 20 had some neuro-developmental deficits (of 14 above 3 years, 12 went to school); 25 families had had or still had problems (deaths and/or anomalies (9 cases) and/or sequelae); 3 mothers were single parents (2 divorces and one split). From 20 triple pregnancies (1 fetal and 3 neonatal deaths), 54 out of 56 survivors were normal above 2 years (33 above 3 years went to school); 3 siblings (1 deaf and 1 with cataract) were put in foster care after severe maternal beating; 2 mothers were single parents (one divorce and one split); 4 mothers had repeated breakdowns; one father tried suicide. From one quadruple pregnancy (one fetal and one neonatal deaths), the 2 survivors were normal above 3 years and went to school. Most sequelae involved very low birthweight babies, in association with a number of sleep and behavioural disturbances. From 98 mothers, 16 had 18 spontaneous pregnancies (11 full-term newborns, 5 miscarriages, 1 extra-uterine pregnancy, 1 abortion) and 3 mothers had another IVF pregnancy (a few others had IVF failures).

During the 1981-90 decade, 31 unilateral grade III intraventricular hemorrhages (III IVH), with controlateral grade I or grade II, and 65 bilateral grade III IVH were detected by brain ultrasound studies in the neonatal intensive care unit of Port-Royal. Varied hemorrhages were present on day 1 in 66% of unilateral III IVH and 72% of bilateral III IVH, and in all cases by day 4. Bilateral grade III was reached only between days 4 and 8 in 32% of cases. Death rate was 35% in unilateral III IVH and 66% in bilateral III IVH. Median dates of observation were: days 2-3, clots in the third ventricle and cisterna magna; days 29-35, disappearance of clots in lateral ventricles; days 2-4, onset of ventricular dilatation; days 15-20, maximal dilatation; days 22-27, onset of regressive dilatation; day 54-3 months, maximal regression of dilatation. The total number of true hydrocephalus was 3/31 in unilateral III IVH and 12/65 in bilateral III IVH. The 20 survivors after unilateral III IVH had 17 regressive dilatations (10 spontaneous and 7 with acetazolamide), 1 hydrocephalus treated by ventriculo-peritoneal shunt (VPS), 2 regressions with late hydrocephalus (2 VPS). The 21 survivors after bilateral III IVH had 19 regressive dilatations (6 spontaneous and 13 with acetazolamide), and 2 hydrocephalus (2 VPS). A late limited dilatation reappeared between 3 and 9 months of age in infants with neonatal periventricular leukomalacias.

During the period 1983-1987, 53 neonates (30 boys, 23 girls, mean birth weight 1,438 +/- 317 g, mean gestational age 30 +/- 2 weeks) were found to have cystic periventricular leukomalacias (CPVL) detected by brain ultrasonography. The neuro-developmental follow-up was 3-7 years. Seventeen CPVL were isolated and CPVL were associated with minor peri-intraventricular hemorrhage (0-1 to II-II) in 36 cases. CPVL included 16 minor forms (11 normal children; 2 minor, 3 moderate sequelae), 29 moderate forms (15 normal children; 4 minor, 7 moderate, 3 major sequelae) and 8 major forms (7 major sequelae, one moderate mental retardation without motor deficit). According to reverse analysis, a normal evolution (26 cases) followed CPVL which were sometimes widely, but thinly spread over the ventricles (thickness 1/5-1/3 of the cerebral mantle); minor sequelae (6 cases) were associated with a 1/4-1/3 thickness, moderate sequelae (11 cases) with a 1/3-1/2 thickness, major sequelae (10 cases) with a 1/2-2/3 thickness of CPVL. The thickness of the lesions appeared to be more predictive of sequelae than the sagittal extent, in particular that of the posterior CPVL which played a major role in the severity of sequelae, and mainly in the presence of motor deficits.

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.

An abbreviated scoring system for PIVH was derived from Papile's one: completed by a 0 for normal pattern, abcd divisions of each cerebral hemisphere for localization of parenchymal haemorrhages, and scoring of each side separately. The scoring system was applied to a consecutive series of 323 PIVH (1981-1984). Unilateral haemorrhages (123) accounted for 38% of all PIVH; they were almost exclusively grade I and II, and more frequently left-sided: 90 left and 33 right H. Of 200 bilateral haemorrhages, 127 were of equal severity on both sides, but left H. were more severe than right ones in 46/73 asymmetrical bilateral H. The survival rate was 91% in overall grade I H., 84% in overall grade II H., with little difference between uni- and bilateral H., 43% in overall grade III H. (34% in bilateral grade III), 28% in overall grade IV H. (1/11 in bilateral grade IV H). The extent of parenchymal H. only bore a partial relationship to the severity of the associated SEH-IVH, only half of the parenchymal haemorrhages being associated with grade III IVH on the same side, and only half of the unilateral PH being associated with a more severe SEH-IVH on the same side. The various former scoring systems have been reviewed. The interest of assessing both cerebral hemispheres separately and of evaluating the extent and location of PH is stressed.

In 1981, 151 neonates with birth weight (B.W.) less than or equal to 1500 g were admitted to the Port-Royal Intensive Care Unit (ICU) (31% of admissions), 81% being born outside. Total mortality was 26% (53% less than or equal to 1000 g). For children who died, the median hospital stay was 9 days; in survivors, it was 69d (109d for BW less than or equal to 1000 g 71d for BW 1001-1250 g, 61d for BW 1251-1500 g), similar in hypotrophic and eutrophic infants. Average weight at direct discharge from Port-Royal ICU was 2627 +/- 565 g. The smaller the infants were at birth, the later they were discharged after assumed term. Complications (more frequent in smaller infants) played a major part in discharge delay. The cost of initial care included every step from birth to final discharge, all hospital charges and transports. Average cost per survivor was 200,356 F, but it reached 336,545 F, in patients with bronchopulmonary dysplasia and 354,605 F in those with necrotizing enterocolitis. Although the mostly good quality of survival justifies these expenses, the authors advocate increased prevention of very early prematurity and severe intra-uterine growth retardation, and development of maternal transport, as well as of prenatal call for a neonatal team in cases of unexpected birth out a of a perinatal center.

Fibrinogen, factors V, II and VII + X assays have been performed by micromethod on day 1, 2 and 3 in 275 newborn infants (full-term and premature) over a period of one year -- 20 infants had proved bacterial infection (from maternal contamination) with positive cultures. The control group included 20 normal newborns in the same range of gestational age. The increase of the fibrinogen level in the infected infants appeared to be significant on day 1 and 2 but not on day 3 (Day 1: 4,06 g/l +/- 0,74 g/l versus 2,06 g/l +/- 0,28 g/l in normal newborn. p less than 0.0001; on Day 2: 4,20 +/- 0,86 g/l versus 2,32 g/l +/- 0,09 g/l p less than 0.001; on Day 3: 3,68 g/l +/- 0,62 versus 3,36 +/- 0,66 g/l). The level of fibrinogen measured in the newborn period by the micromethod was in a accordance with former works showing that it is up th the adult value after the 3rd day of life. Since the micromethod on capillary blood requires only 140 microliters of blood (whereas 2 ml with macromethod) this fibrinogen determination appears to be a rapid and reliable method for the diagnosis of neonatal infection due to materno-fetal contamination.

The new radiological technics show the incidence of intraventricular hemorrhage (IVH), intraparenchymal hemorrhage (IPH), or of porencephaly (PL), in the neonatal period. Discrepancies between the initial extent of lesions and the outcome have been observed. We tried to appreciate the EEG value in infants having had such lesions during the first month of life. We studied serial EEGs and neuroradiological exams in 34 babies: with IVH (group I, 17 cases), with IPH and/or PL (group II, 17 cases). Their gestational age (GA) was between 27 and 34 weeks; they all had a neurological follow-up between one and five years. Infants with favourable outcome (76% in gr. I, 47% in gr. II) had normal or slightly abnormal EEGs, whatever the extent of lesions. Nine babies had very abnormal EEGs (numerous positive rolandic spikes (PRSs) and/or EEG background without physiological rhythms); one developed moderate sequelae, the other 8 major sequelae (2 with infantile spasms). In gr. II, babies with major sequelae had a higher GA than babies with good outcome. The PRSs were more often observed with periventricular lesions than with IVH only and in babies with higher GA; they lasted up to 12 weeks on serial EEGs in one case with porencephaly (major sequelae with infantile spasms); they appeared before scan abnormality in another case which developed later porencephaly (major sequelae). Thus, EEGs give valuable information on brain function and help to forecast outcome in premature babies.

Within 4 years 10 months (1981-1985), the Port-Royal Neonatal Intensive Care Unit admitted 2,400 neonates, one third with a birthweight below 1,501 g; 4,631 cranial ultrasound studies were performed in 1,488 of those neonates, mostly less than 1,501 g, detecting 392 consecutive peri-intraventricular hemorrhages (PIVH), of which 130 were major forms (from unilateral grade III to bilateral grade IV PIVH). Overall survival rates were 91% in grade I, 85% in grade II, 42% in grade III, 26% in grade IV; survival rate was significantly lower in bilateral than in unilateral grade II and III PIVH. In major PIVH, deaths occurred early (58% in the first week after birth). Post-hemorrhagic dilatation was constant but mostly regressive; true active hydrocephalus appeared in 1 unilateral grade III PIVH and 8 bilateral grade III PIVH, with ventriculo-peritoneal shunt in the second month of life in 5 infants (2 died), and 4 deaths (surgery not feasible). The neurological and developmental outcome of 42 of 46 survivors (4 losts to follow-up) was evaluated beyond one year of age in 12 unilateral grade III PIVH (10 normal children, 1 minor sequela, 1 moderate sequela), 16 bilateral grade III PIVH (7 normal children, 3 minor sequelae, 1 moderate sequela, 5 major sequelae), 13 unilateral grade IV PIVH (8 normal children, 1 minor sequela, 3 moderate sequelae, 1 major sequela), 1 bilateral grade IV PIVH (major sequela). A persistent major dilatation after 6-9 months of age bore an ominous prognosis.(ABSTRACT TRUNCATED AT 250 WORDS)

In 30 surviving neonates, close prolonged ultrasonographic brain studies demonstrated cystic periventricular leucomalacias (CPVL) of varying degree (11 minor forms, 12 moderate forms, 7 severe forms). Clinical histories were reviewed for each case. There were 18 boys, 4 twins, 2 small-for-dates. Mean gestational age was 31 +/- 2 weeks, mean birthweight was 1532 +/- 356 g. No pregnancy was normal, but prenatal events were of a common occurrence in 26/30 cases (premature labor, toxaemia, twins...). Immediate perinatal events included cord difficulties in 5 cases (3 tight cords around the neck, 1 prolapse, 1 case where loose cord around the neck came down with the head), abruptio placentae (2), acute fetal distress (10, of which 8 were severe), Apgar scores 0-1 (9, of which 7 occurred after fetal distress and 2 were unexpected). In four cases, CPVL were of antenatal origin (already in the cystic stage on days 1-2). In 2 cases, CPVL occurred postnatally (infective shock on day 1 and day 46). Plausible mechanisms for anoxic-ischaemic lesions could only be found in 13 cases and remained unknown in the other 17. However, clinical histories suggested the following; cumulative minor events might become as damaging as single major events; "minor" fetal distresses should be scrutinized; pregnant women should be taught not to wait until late to arrive at hospital because this results in non-monitored delivery. No obvious relationship was found between the severity of known events and the degree of CPVL, but a number of pre- and perinatal periods were poorly monitored. The legal importance of early ultrasonographic studies was stressed.

This analysis presents 400 cases of children at risk for sudden infant death syndrome (SIDS) who were followed at the Port-Royal SIDS-clinic from 1981 to 1984. The 400 cases included: 107 children seen after a severe accident ("near-miss"), 20 twins from pairs stricken by 1 SIDS and 9 twins from near-miss pairs, 264 SIDS-siblings. The following risk factors were searched for: a) low socioeconomical level, b) marital difficulties, c) psychological or psychiatric disturbances in mother and/or father, d) pathological gynecologic and/or obstetric history (sterility, gestational and/or delivery accidents), e) unwanted pregnancy, f) perinatal morbidity. In deaths from SIDS or near-miss infants, 34% cumulated 2 factors, 26% 3 factors, 8% 4 factors; only 2% had no known factor. In addition, a few deaths or severe accidents occurred within a short delay after a travel or a change in daycare; the role of these coincidences should be studied; meanwhile, the authors emphasize the need for a special attention to the quality of the child's environment, as a basic factor for a harmonious development.