Severe Neurodevelopmental Disability Research Articles

Outcomes of children of extremely low birthweight and gestational age in the 1990s

Advances in perinatal care have improved the chances for survival of extremely low birthweight (<800 grams) and gestational age (<26 weeks) infants. A review of the world literature reveals that among regional populations, survival at 23 weeks' gestation ranges from 2 to 35%, at 24 weeks' gestation 17 to 62% and at 25 weeks' gestation 35 to 72%. These wide variations may be accounted for by differences in population descriptors, in the criteria used for starting or withdrawing treatment, in the reported duration of survival and differences in care. Major neonatal morbidity increases with decreasing gestational age and birthweight. At 23 weeks' gestation, chronic lung disease occurs in 57 to 86% of survivors, at 24 weeks in 33 to 89% and at 25 weeks' gestation in 16 to 71% of survivors. The rates of severe cerebral ultrasound abnormality range from 10 to 83% at 23 weeks' gestation, 9 to 64% at 24 weeks and 7 to 22% at 25 weeks' gestation Of 77 survivors at 23 weeks' gestation, 26 (34%) have severe disability (defined as subnormal cognitive function, cerebral palsy, blindness and/or deafness). At 24 weeks' gestation, the rates of severe neurodevelopmental disability range from 22 to 45%, and at 25 weeks' gestation 12 to 35%. When compared with children born prior to the 1990s, the rates of neurodevelopmental disability have, in general, remained unchanged. We conclude that, with current methods of care, the limits of viability have been reached. The continuing toll of major neonatal morbidity and neurodevelopmental handicap are of serious concern.

Favourable neurological outcomes following delivery room cardiopulmonary resuscitation of infants < or = 750 g at birth.

To study short- and long-term outcomes of infants < or = 750 g birthweight who received cardiopulmonary resuscitation (CPR) in the delivery room. A retrospective analysis of all inborn live births < or = 750 g birthweight from 1990 to 1996. Cardiopulmonary resuscitation was defined as positive pressure ventilation via an endotracheal tube and chest compressions. Univeriate analysis were conducted comparing patients according to the use of CPR or positive pressure ventilation alone. Cardiopulmonary resuscitation was administered to 16 infants: four received chest compressions only and 12 also received adrenaline. Cardiopulmonary resuscitation recipients had significantly lower Apgar scores at both 1 and 5 min, and had delayed onset of spontaneous respiration (P < 0.01). Seven patients died, and eight of nine survivors were free of major neurodevelopmental abnormalities at follow up. All CPR recipients with a 5 min Apgar score of < or = 5 and delayed onset of spontaneous respiration beyond 5 min had poor outcomes. Contrary to the majority of published evidence, delivery room CPR in our extremely small infants was not associated with a high risk of severe neurodevelopmental disability.

Outcomes of children of extremely low birthweight and gestational age in the 1990's.

Advances in perinatal care have improved the chances for survival of extremely low birthweight (< 800 g) and gestational age (< 26 weeks) infants. A review of the world literature and our own experience reveals that at 23 weeks gestation survival ranges from 2% to 35%. At 24 weeks gestation the range is 17% to 58%, and at 25 weeks gestation 35% to 85%. Differences in population descriptors, in the initiation and withdrawal of treatment and the duration of survival considered may account for the wide variations in the reported ranges of survival. Major neonatal morbidity increases with decreasing gestational age and birthweight. The rates of severe cerebral ultrasound abnormality range at 23 weeks gestation from 10% to 83%, at 24 weeks from 17% to 64% and at 25 weeks gestation from 10% to 22%. At 23 weeks gestation, chronic lung disease occurs in 57% to 70% of survivors, at 24 weeks in 33% to 89%, and at 25 weeks gestation in 16% to 71% of survivors. When compared to children born prior to the 1990's, the rates of neurodevelopmental disability have, in general, remained unchanged. Of 30 survivors reported at 23 weeks gestation nine (30%) are severely disabled. At 24 weeks gestation the rates of severe neurodevelopmental disability (including subnormal cognitive function, cerebral palsy, blindness and deafness) range from 17% to 45%, and at 25 weeks gestation 12% to 35% are similarly affected. In Cleveland, Ohio, we compared the outcomes of 114 children with birthweight 500-749 g born 1990-1992 to 112 infants born 1993-1995. Twenty month survival was similar (43% vs 38%). The use of antenatal and postnatal steroids increased (10% vs 54% and 43% vs 84%, respectively, P< 0.001), however the rates of chronic lung disease increased from 41% to 63% (P = 0.06). There was a significant increase in the rate of subnormal cognitive function at 20 months corrected age (20% vs 48%, P < 0.02) and a trend to an increase in the rate of cerebral palsy (10% vs 16%) and neurodevelopmental impairment. We conclude that, with current methods of care, the limits of viability have been reached. The continuing toll of major neonatal morbidity and neurodevelopmental handicap are of serious concern.

Longitudinal follow-up of a cohort of newborn infants treated with inhaled nitric oxide for persistent pulmonary hypertension

Objective: To describe the outcome of a group of term newborn infants treated with inhaled nitric oxide for severe persistent pulmonary hypertension. Study design: We performed a prospective longitudinal medical and neurodevelopmental follow-up of 51 infants treated as neonates for persistent pulmonary hypertension of the newborn with inhaled nitric oxide. The original number of treated infants was 87, of whom 25 died in the neonatal period; of 62 infants who survived, 51 were seen at 1 year of age and 33 completed a 2-year evaluation. Statistical analysis used population medians, means, and standard deviations for parameters assessed. Paired t tests and chi-square analysis were used to compare outcomes measured at 1 year with assessments at 2 years for the 32 infants seen at both 1- and 2-year visits. Results: At 1-year follow-up median growth percentiles were 20%, 72.5%, and 50% for weight, length, and occipitofrontal circumference, respectively. Thirteen of 51 infants (25.5%) were <5th percentile in weight. Nine of 51 infants (17.6%) had feeding problems (need for gastrostomy feeding or gastroesophageal reflux), and 14 (27.5%) had a clinical diagnosis of reactive airways disease. Infant development as measured by the Bayley Scales of Infant Development was 104 ± 16 for the mental development index and 97 ± 20 for the psychomotor index. Six of 51 infants (11.8%) were found to have severe neurologic handicaps, defined as a Bayley score on either the mental development or psychomotor index of <68, abnormal findings on neurologic examination, or both. Fewer children (6.1% vs 15.7%) required supplemental oxygen at 2 years compared with 1 year, and performance on the psychomotor index of the Bayley Scales improved significantly. Conclusions: One- and 2-year follow-up of a cohort of infants with persistent pulmonary hypertension of the newborn who were treated with inhaled nitric oxide had an 11.8% (1 year) and 12.1% (2-year) rate of severe neurodevelopmental disability. There are ongoing medical problems in these infants including reactive airways disease and slow growth that merit continued close longitudinal follow-up. (J Pediatr 1997;131:70-5)

Neonatal burst suppression: Its developmental significance

Burst suppression was recorded on electroencephalograms of 15 of 274 term infants (5.4%) in our hospital within a 5 year period. These 15 infants were examined, their perinatal histories reviewed, and detailed neuro-developmental testing performed to examine the prognostic significance of burst suppression. Fourteen children (93%) had poor outcomes. Four (26%) died in infancy. Ten survivors were followed for a mean of 30 months (range: 18–61 months). Nine have severe motor deficits, 6 require total custodial care, 5 have intractable epilepsy, and 3 are blind. Their mean Gesell Developmental Quotient was 28; a score of less than 68 suggests severe retardation. A single patient, the only 1 with a perinatal history complicated by an easily-treated bacterial meningitis, achieved normal growth and development. This study documents the dire prognosis of neonatal burst suppression. The appearance of burst suppression, though transient, portended death or severe neuro-developmental disability in 93% of our patients.

Infant Feeding: Formula Feeding

We sought to describe long-term outcomes of infants with severe surgical necrotizing enterocolitis (NEC).Data were collected on infants with surgical NEC (2009–2018). Severe surgical NEC was defined by extensive bowel loss with residual bowel length < 30 cm, and “NEC totalis” was identified per operative report. Post-operative management and long-term outcomes were assessed, including enteral autonomy, severe neurodevelopmental disability, and educational attainment.Of 268 infants with surgical NEC, 41 (15%) had severe surgical NEC, and 14/41 were identified as “NEC totalis”. Zero severe NEC vs. 8 (57%) “NEC totalis” patients were placed on comfort measures following initial surgery (p < 0.001). Twenty-five patients (93%) with severe NEC survived vs 3/6 with “NEC totalis” (p < 0.001). The 28 survivors (68%) were followed for a median (IQR) duration of 8(4,10) years. Nine (32%) with severe NEC were weaned from parenteral nutrition. Eight (29%) had ≥ 1 marker for severe neurodevelopmental disability, and 11/16(69%) (7–16 years) were attending school at last follow-up.Long-term survival is excellent following initial discharge, and achievement of enteral autonomy is feasible in patients with severe surgical NEC. The majority of patients who survive do not have severe neurodevelopmental disability and participate in school. Given current survivals and outcomes, focus on measured residual bowel length may be more appropriate than the subjective term “NEC-totalis.”Prognosis Study.Level II.