Long term outcome of small versus appropriate size for gestational age co-twins/triplets.

BACKGROUND: Small for gestational age (SGA) extremely low birthweight (ELBW < 1000 g) survivors often remain small and/or have subnormal school performance. Some are twins/triplets with larger appropriate size for gestational age (AGA) co-twins/triplets. OBJECTIVE: To assess whether SGA ELBW twins/triplets remain different from their AGA co-twins/triplets. DESIGN, SETTING: During 1981-1999, 353 SGA ELBW neonates were admitted to our neonatal intensive care unit: 267 survived, 54/267 were twins/triplets, and 36/54 had AGA surviving co-twins/triplets. This longitudinal study describes the growth, neurodevelopmental outcome, and school performance of these 36 sets (3-17 years). The children were classified as normal, or having minor, moderate, or severe deficiencies. RESULTS: Values for birth weight (mean intrapair z score difference 2.26), length (2.74), and head circumference (2.62) were lower in SGA neonates than in AGA co-twins/triplets. SGA survivors remained smaller at 3-6 years of age: mean intrapair z score difference in weight, 1.37, height, 1.54, head circumference, 1.21. From 6 to 17 years, smaller differences persisted. Former SGA children had a tendency to have motor deficiencies (nine SGA v three AGA) and mental retardation (seven v four), same hearing loss (two v two), but significantly more visual abnormalities (15 v 11), behavioural disturbances (14 v five), and speech problems (14 v eight). Twenty four sets were in the same normal level class, often supported by familial/professional help. CONCLUSIONS: Although raised in the same environment, SGA ELBW survivors remained smaller and had more visual/behavioural/speech problems, but most maintained grade level parity with their AGA siblings, with appropriate help.

[Neonatal lenticulostriate vasculopathy. Mid and longterm outcome (70 cases)]. / Devenir des enfants ayant présenté une vasculopathie lenticulostriée néonatale (70 cas).

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.

Macrocrania / Macrocephaly

Na maioria dos casos, é possível confirmar a macrocrania e segui-la para caracterizá-la como não evolutiva ou evolutiva. A ecografia trasnfontanelar, a tomografia de crânio e/ou a ressonância magnética foram usadas para medir os espaços pericerebrais, os espaços subaracnóides, a cisura inter-hemisférica, os ventrículos laterais e a cisterna magna. De acordo com os achados, é possível distinguir quatro combinações de achados na criança com macrocrania: a) um grande cérebro preenche todo o espaço (megaloencefalia); b) o cérebro parece preencher todo o espaço, mas existem cavidades com líquido em seu interior (hidrocefalia); c) o cérebro de tamanho normal ou reduzido está separado do osso por uma espessura de líquido, mas os ventrículos laterais são normais (hidrocefalia externa); d) o conteúdo é misto. Os resultados permitem, em geral, estabelecer um conjunto de diagnósticos coerentes, mas um certo tempo de evolução é frequentemente necessário.

Mid- and long-term outcome of 166 premature infants weighing less than 1,000 g at birth, all small for gestational age.

This longitudinal study reported the mid- and long-term neurodevelopmental outcome of 166 premature infants born with an extremely low birth weight (<1,000 g), all small for gestational age (<10th percentile birth weight for gestational age). Nine girls were lost to follow-up before 3 years of age, and 6 children were excluded a posteriori because of specific conditions diagnosed in the 1st year of life (severe abnormalities in 5). Of the remaining 151 children, 2% had cerebral palsy, 15% had lesser motor disturbances (reduced to 2% after psychomotor training), 8% had early severe developmental delays, and added mild and moderate delays and increasing cognitive gaps amounted to 28% in the 14th year. Visual deficits increased with age up to 63% of the older children. Seven children had deafness and 13 had hearing losses after otitis media. Language delays were observed at some stage in 31% of cases, as well as behavioral disturbances in 42% (severe problems in 12%). At last evaluation, 34% of the children were normal (12% of the older ones), 51% had minor deficiencies, 18% had moderate and 3% had major disabilities. Children entered the first grade in the 7th year in only 84% of cases, which dropped to 63% at proper level in the second grade; 47% entered high school at the proper age, 7/15 obtained the 'baccalauréat' in the 19th/20th year. The school performance was heavily dependent on the socioeconomic and cultural level of the family.

Catch-up growth in 166 small-for- gestational age premature infants weighing less than 1,000 g at birth.

Catch-up growth was studied in 166 children born with an extremely low birth weight (<1,000 g) and small-for-gestational age (SGA, <10th percentile birth weight for gestational age). Of these children 159 were followed up for between 4 and 18 years (median 9 years). Group A, SGA <10th percentile of Lubchenco curves only; group B, <10th percentile of Mamelle's curves but >5th percentile, and group C, <5th percentile of Mammelle's curves. Catch-up growth was considered to be achieved when height, weight, and head circumference (HC) reached -2 SD of French reference data and remained above this limit afterwards. Catch-up growth in height was achieved in 126/156 children or 81% (group A 88%; group B 84%; group C 74%), before 3 years of age in 100/127 (78%). Seven children below -2 SD received growth hormone (1 child who caught up was excluded from the results). Catch-up growth in weight was achieved in 126/159 children or 79% (group A 86%; group B 82%; group C 73%), before 3 years of age in 87/126 (69%). Catch-up growth in HC was achieved in 126/156 or 81% (group A 78%; group B 92%; group C 77%), before 3 years of age in 102/127 (80%). Overall catch-up growth was achieved for all three parameters in 65% of children, two of three parameters in 19%, and one of three parameters in 8%. Eleven children never caught up on any parameter. While weight is a lesser concern and HC is not liable to intervention, a greater number of short children might benefit from growth hormone therapy.

Intracytoplasmic sperm injection pregnancy with trisomy 20p and monosomy 22q in a newborn resulting from a balanced paternal translocation.

In infertile men who carry a balanced reciprocal translocation, intracytoplasmic sperm injection (ICSI) may induce a pregnancy with an abnormal karyotype. This report describes a previously unreported paternal reciprocal translocation leading to a chromosomally unbalanced ICSI pregnancy. The triplet pregnancy resulted in 1 normal girl, 1 physically normal boy with the same balanced paternal translocation, and a severely malformed boy with trisomy 20p and monosomy 22q who died in the neonatal period.

[Mid- and long-term outcome of 77 triplets and their families]. / Devenir à moyen et long-terme de 77 fratries de triplés et de leurs familles.

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.

A risk-benefit assessment of natural and synthetic exogenous surfactants in the management of neonatal respiratory distress syndrome.

Alveolar surfactant is central to pulmonary physiology. Quantitative and qualitative surfactant abnormalities appear to be the primary aetiological factors in neonatal respiratory distress syndrome (RDS) and exogenous replacement of surfactant is a rational treatment. Available exogenous surfactants have a natural (mammal-derived lung surfactants) or synthetic origin. Pharmacodynamic and clinical studies have demonstrated that exogenous surfactants immediately improve pulmonary distensibility and gas exchange; however, this is achieved more slowly and with more failures with synthetic surfactants. The ensuing advantageous haemodynamic effects are not so striking and they include an inconvenient increased left to right ductal shunt. Two strategies of administration have been used: prophylactic or rescue therapy to treat declared RDS. All methods of instillation require intubation. In addition to the early benefits (improved gas exchange and reduced ventilatory support) the incidence of classical complications of RDS, especially air leak events, is decreased except for the uncommon problem of pulmonary haemorrhage. The incidence of bronchopulmonary dysplasia is neither uniformly nor significantly reduced although the severity appears to be lessened. The overall incidence of peri-intraventricular haemorrhages is not diminished although separate trials have shown a decreased rate. The most striking beneficial effect of exogenous surfactants is the increased survival (of about 40%) of treated very low birthweight neonates. A small number of adverse effects has been described. The long term outcome of survivor neonates with RDS treated with surfactants versus control neonates with RDS not treated with surfactants is similar in terms of physical growth, at least as good in terms of respiratory status, with a similar or slightly better neurodevelopmental outcome. There is not clear benefit of exogenous surfactant therapy in extremely premature infants (< 26 weeks gestational age, birthweight < 750 g). The potential risks of contamination, inflammatory and immunogenic reaction and the inhalation of platelet activating factor remain a theoretical concern of surfactant therapy which has not been confirmed in clinical practice. The optimal timing of treatment favours prophylaxis over rescue treatment and early rescue treatment rather than delayed therapy. Meta-analyses suggest the clinical superiority of natural surfactant extracts over a synthetic one (colfosceril palmitate). The economic impact of surfactant therapy is favourable and the costs per quality-adjusted life year (QALY) for surviving surfactant treated infants are low. In conclusion, the mid and long term benefit/risk ratio clearly favours the use of exogenous surfactants to prevent or to treat RDS in neonates who have a gestational age of > 26 weeks or a birthweight of > 750 g, especially with the prophylactic strategy using natural surfactant extracts.

[Prognosis and evolution of bilateral grade III intraventricular - hemorhages (III-III-IVH)] / Prognóstico e evolução cronológica das hemorragias intra-ventriculares de grau III bilateral (HIV-III-III).

OBJECTIVE: To describe the evolution of bilateral III IVH so as to define optimal dates for therapeutic moves.METHODS: Longitudinal study of brain ultrasound scans from day 1 over the first three months of life. Our team calls "massive III" a hemorrhage with an intraventricular clot diameter >8mm on the coronal view at the level of Monro's foramina of the day of maximal hemorrhage. The 90 neonates were divided into four groups. Group I included 29 premature neonates who died in the first seven days after birth (birthweight (BW), 1,114 -/+253 g, gestational age (GA), 28 weeks 3 days); with 21 massive uni/bilateral grade III PIVH, and early filling of the whole ventricular system and cisterna magna. Group II included seven premature neonates who died in the second week after birth (similar in BW and GA, but more heterogeneous group). Two groups of infants survived for more than 14 days. Group III included 30 premature neonates (BW, 1,299 -/+260 g, GA, 29 weeks 1 day), with 20 classical grade III PIVH, who had post-hemorrhagic dilatation regressed spontaneously in 13 cases and regressed after acetazolamide treatment in 17 cases (five deaths due to three bronchopulmonary dysplasias and two periventricular leucomalacias). Group IV included 24 premature neonates (BW, 1,344 -/+289 g, GA, 29 weeks 5 days) with 15 massive grade III PIVH, who suffered posthemorrhagic hydrocephalus through obstruction of the Sylvius aqueduct (15) and/or of the cisterna magna (21), 12 blocks affecting both levels. Blocks were observed from day 8, and 35 in the quasi totality of cases (17 deaths).CONCLUSIONS: From 1,183 consecutive peri-intraventricular hemorrhages (PIVH) diagnosed by brain ultrasound studies (01/01/81-12/31/94), 90 were grade (III-III) PIVH, with a 36% overall survival. The massive volume of grade III PIVG plays a heavy role in early deaths of extremely low birthweight neonates and it heralds a blocked hydrocephalus in more vigorous infants who survive PIVH.

Mid- and long-term outcome of 89 premature infants weighing less than 1,000 g at birth, all appropriate for gestational age.

OBJECTIVE: Advances in obstetrical and neonatal care have achieved dramatic reductions in the mortality of extremely low birth weight infants, but concerns regarding the long-term neurodevelopmental prognosis have increased. The study was performed to assess the physical and developmental status of premature infants weighing < 1,000 g at birth who were appropriate for gestational age (AGA). METHODS: A follow-up study including school level, physical and developmental examination and/or interviews of parents, pediatricians, teachers and social workers was carried out from January 1, 1981 to December 31, 1991, on 176 subjects who had been AGA premature neonates weighing < 1,000 g and were admitted to the Port Royal Neonatal Intensive Care Unit. Eighty-nine survived (50 girls, 39 boys). Five children were lost to follow-up after 3 years of age. Neurological sequelae, developmental and/or intellectual quotients, school level and results, vision, hearing, language, weight, height, and behavior were evaluated. RESULTS: At the last evaluation, 37% of children were completely normal (24% had always been normal); 52% were normal in the 4th year of life, decreasing to 31% in the 8th year. Minor disabilities increased from 31% in the 4th year to 53% in the 8th year. Moderate disabilities ranged yearly between 8 and 14%. Five children had major handicaps diagnosed before 3 years of age (4 cerebral palsies following severe neonatal brain lesions and 1 severe retardation with renal dwarfism). No difference was found between children with or without bronchopulmonary dysplasia. Twelve children (14%) had hearing loss (3 deafness) and 27 (32%) had visual deficits (no blindness). Nursery school begins at 3 for French children: 86% of the followed survivors were at nursery school at 3 years of age, 74% of 6-year-olds were in the first grade, primary school, and 50% of 7-year-olds were in second grade. Half the children with high socioeconomic/cultural risks had some school difficulties. Similar results were collected from various cross-sectional studies in the recent literature. CONCLUSIONS: While 20% of the children had moderate to severe disabilities, an increasing number of minor deficiencies appeared with age. Socio-economic/cultural factors had a deleterious effect even in structurally normal children, but they had an even worse effect in children with minor deficiencies, owing largely to the frequent lack of proper management at home.

Porcine surfactant replacement therapy in newborns of 25-31 weeks' gestation: a randomized, multicentre trial of prophylaxis versus rescue with multiple low doses. The French Collaborative Multicentre Study Group.

The aim of the study was to determine if prophylaxis with multiple low doses of porcine surfactant would increase survival, without bronchopulmonary dysplasia, compared with rescue therapy, for respiratory distress syndrome in newborns of 25-31 weeks' gestation. Compared with rescue therapy (n = 122), prophylaxis (n = 134) decreased the need for oxygenation and ventilatory support within 3-72 h. It did not, however, increase survival without bronchopulmonary dysplasia (60% versus 46%) (odds ratio (OR) = 1.53, 95% confidence interval (CI) = 0.90-2.61). Furthermore, prophylaxis decreased the incidence of severe peri-intraventricular haemorrhage (3% versus 16%) (OR = 0.28, 95% CI = 0.09-0.84) and retinopathy of prematurity (2% versus 11%) (OR = 0.18, CI = 0.04-0.78). We conclude that prophylaxis did not increase survival without bronchopulmonary dysplasia. The decreased incidence of severe peri-intraventricular haemorrhage and retinopathy of prematurity after prophylaxis requires further study.

[In vitro fertilization neonates transferred to neonatal medicine. Mid-and long-term outcome in 99 families]. / Nouveau-nés de FIV transférés en Médecine Néonatale: devenir de 99 familles à moyen et long-terme.

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).

[Chronology of ultrasonographic course of neonatal intraventricular hemorrhage stage III]. / Chronologie de l'évolution échographique des hémorragies intraventriculaires néonatales de stade III.

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.

[Mid-term and long-term outcome in newborn infants with periventricular leukomalacia (53 cases)]. / Devenir à moyen et long terme des nouveau-nés atteints de leucomalacies périventriculaires (53 cas).

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.

[Treatment of severe hyaline membrane disease with a single-dose of natural exogenous surfactant of porcine origin. A randomized trial: immediate effects and outcome at 28 days of life]. / Traitement de formes sévères de la maladie des membranes hyalines par une dose unique d'un surfactant exogène naturel d'origine porcine. Un essai randomisé: effets immédiats et devenir à 28 jours de vie.

The results obtained with porcine surfactant (Curosurf) administration for the treatment of hyaline membrane disease (HMD) are reported. Thirty premature infants weighing 700 to 2,000 g with severe HMD (mechanical ventilation and oxygen requirement (FiO2) greater than 60% were randomly allocated at 2 to 15 hours postnatal age. Eight of the 30 patients included in this group participated in a multicenter european trial. The fifteen infants with mean gestational age (GA) of 29.5 weeks included in the treatment group (T), were treated at 8.6 hours of life with a single dose of 200 mg/kg Curosurf given intratracheally while 15 infants of mean GA 30 weeks formed the control group (C). Infants in the T group showed an immediate, dramatic and sustained improvement of oxygenation as reflected by increased PaO2/FiO2 and arterial to alveolar PO2 ratios within 1 hour. This significant improvement in favor of T group (p less than 0.005) persisted for 2 days when control infants began to recover. This improvement in oxygenation allowed a significant decrease of FiO2 (p less than 0.005) and mean airway pressure (p less than 0.01) in the T group within 1 hour and up till the second day. Despite this early improvement obtained with Curosurf the survival rate at 28 days of life and the incidence of associated HMD complications were not significantly modified. However the tendency was towards decreased respiratory morbidity. The discussion will consider the value of multiple doses.

Neonatal diagnosis of respiratory distress syndrome.

After a brief historical recall, this review states the needs for an accurate diagnosis of the neonatal respiratory distress syndrome (RDS). The clinical features consist of disturbances of respiratory rate, grunting, intercostal retractions, and cyanosis, but early mechanical ventilation tends to suppress most of them. Laboratory findings include hypoxemia, hypercapnia, and mixed acidosis. Positive radiological diagnosis remains an important criterion but early ventilation with positive end-expiratory pressure has made grading obsolete. The biochemical diagnosis addresses the basic lung surfactant deficiency, by determination of the lecithin/sphingomyelin ratio and phosphatidylglycerol ("modified lung profile") in lung effluents at birth. If clinical and radiological diagnosis remains adequate for daily practice and epidemiological studies, biochemical diagnosis should be mandatory for therapeutic trials. However, the problem of atypical RDS in very low birth weight infants has not been totally solved. RDS has now been known for more than 80 years; yet its diagnosis is still a matter of controversy.

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.

[Neonatal cystic leukomalacia. Perinatal case histories of 30 survivors]. / Leucomalacies kystiques néonatales. Anamnèse périnatale chez 30 survivants.

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.

[Short, middle and long-term outcome of major peri-intraventricular hemorrhages]. / Devenir des hémorragies péri-intraventriculaires majeures à court, moyen et long terme.

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)