Neonatal dural sinus thrombosis.

Dural sinus thrombosis in the newborn period has been infrequently documented and its clinical presentation remains obscure. Seventeen patients, all of whom were born at term with dural sinus thrombosis diagnosed in the neonatal period, were retrospectively identified and reviewed. Diagnosis was determined by unenhanced computed tomography which demonstrated a dense sagittal sinus with concomitant small ventricles. Two patients had ancillary studies (i.e., cerebral angiography and nuclear flow scan) which confirmed the diagnosis. Only 4 patients had evidence of perinatal asphyxia. Three patients were identified as having associated conditions known to predispose them to dural sinus thrombosis. None of the patients tested had an identifiable hypercoagulable state. Neonatal seizures were the initial presentation in 15 patients. Seizure onset predominantly occurred during the first week of life. Subsequent examinations were available in all 17 patients and ranged up to 6 years. Only 3 patients had seizures beyond the neonatal period. In 11 of 12 infants with no history of perinatal asphyxia, neurodevelopmental outcomes were normal. Two of 4 infants with perinatal asphyxia had neurologic sequelae. Dural sinus thrombosis represents an important and under-recognized cause of neonatal seizures in term infants. In the absence of perinatal asphyxia, normal neuro-developmental outcome is likely and the risk of seizure recurrence is low.

Management and follow-up of arterial thrombosis in the neonatal period.

The management and follow-up of 12 patients with major aortic thrombus formation occurring in the neonatal period between 1982 and 1987 are reported. Umbilical arterial catheters were inserted in 8 of the 12 patients before thrombus formation. Two patients had congenital thrombi. Hypertension, oliguria, hematuria, and elevated blood creatinine concentration were found at the time of diagnosis of the thrombus; nine of the patients had a patent ductus arteriosus. Supportive care was instituted in seven patients who were hemodynamically stable. Five of the patients had congestive heart failure, shock, or both, and were treated with surgical thrombectomy. Thrombolytic therapy was not used in either group. The five surgically treated patients and six of seven medically treated patients survived. Ultrasound examination suggested resolution of the thrombus in all survivors in 6 to 30 days. Sequelae from thrombus formation were present in all patients at the time of discharge and included hypertension in 9 of the 11 survivors and decreased renal function in six of them. Follow-up at 1 to 3 years revealed normal blood pressure, good growth, and good renal function in 10 of the survivors.

Antenatal and perinatal conditions correlated to handicap among 4-year-old children.

The purpose of this study was to investigate the impact of maternal prepregnancy and pregnancy-related risk factors, complicated delivery, and perinatal morbidity on subsequent handicaps in children. We surveyed a birth cohort of 4102 mothers and 4138 children in Frederiksborg County, Denmark. Maternal risk factors were defined according to guidelines published by the Danish National Board of Health, and perinatal morbidity and handicaps according to World Health Organization guidelines. The incidence of handicaps: (cerebral palsy, mental retardation [mild and severe], epilepsy, severe defects of vision and hearing); was 44 of 4038 children (twins and neonatal deaths were excluded). A combination of three or more maternal risk factors was found to be a predictor of risk for children with later handicaps; the incidence of handicaps was 11 times higher than in mothers with no risk factors. Eleven percent of all mothers had three or more risk factors and they had 43% of the handicapped children. Multiparity increased the risk in all risk categories. Of complications at delivery, intrapartum asphyxia, as evident from Apgar scores of less than 7 at 1 minute and less than 10 at 10 minutes in particular, was a strong predictor of a later handicap. Premature rupture of membranes for more than 24 hours was also significantly associated with later handicaps. Perinatal morbidity was correlated with a later handicap. The perinatal complication most strongly associated with later handicaps was low birthweight. Forty-eight percent of the affected children had a birthweight of less than 2500 gm and were small for gestational age. We conclude that the incidence of handicaps could possibly be reduced if the causes of the following maternal risk factors were identified and, if possible, eliminated: previous delivery of a child with a birthweight less than 2500 gm, previous delivery of a stillborn child, repeated abortions, severe infection during pregnancy, intrauterine growth retardation, and preterm delivery. Improved intrapartum diagnosis and prevention of asphyxia and treatment of children born with low Apgar scores would reduce the incidence of handicaps, as would intervention to prevent premature rupture of the membranes of more than 24 hour's duration.

The EEG evolution and neurological prognosis of neonates with perinatal hypoxia [corrected].

One hundred and seventy-three full-term newborns with hypoxic encephalopathy were subjected to polygraphic recordings (EEG, EOG, ECG and respiration) of 2.5-3.5 hr duration in the first 3 weeks in the period from 1970 to 1986. Their conceptional age ranged from 38 w to 42 w and birth weight from 1,750 g to 4,860 g. Their clinical outcome was evaluated in relation to the degree of depression of background EEG classified at each postnatal age. Cases with normal background before the 7th day, minimal or less depression before the 4th day and mild or less depression on the first day developed normally. Cases with marked or maximal depression at any day, moderate or more depression after the 7th day and mild or more depression after the 12th day developed neurological handicaps. The prognosis of others were not certain. Prognostic value of the neonatal EEG depended on the day of recording. Serial EEG recording was useful to prognosticate the clinical outcome.

Morbidity for survivors of extracorporeal membrane oxygenation: neurodevelopmental outcome at 1 year of age.

Extracorporeal membrane oxygenation is an important technology in the treatment of high-risk infants whose long-term outcome is being followed prospectively at our institution. The extracorporeal membrane oxygenation procedure allows temporary cardiopulmonary support for critically ill full-term neonates who are refractory to maximum ventilatory and medical management as a consequence of severe persistent pulmonary hypertension. The technique necessitates both the permanent ligation of the right common carotid artery and jugular vein and systemic heparinization. The survivors constitute a unique group of high-risk infants, from the standpoint of the hypoxic-ischemic insults preceding extracorporeal membrane oxygenation and the risks associated with the procedure. Our results indicate that most of our survivors are developing normally at 1 year. Major morbidity, in terms of either significant developmental delay (Bayley mental and motor indices less than 70) or significant neuromotor abnormality, occurred in only 10% of these infants. Poor outcome was associated with major intracranial hemorrhage and chronic lung disease. Ligation of the right carotid artery and jugular vein was not associated with a consistent lateralizing lesion. Long-term follow-up through school age is essential.

Differential effects of intrauterine and postnatal brain growth failure in infants of very low birth weight.

We investigated the contribution of brain growth failure to outcome in infants of very low birth weight (VLBW; less than 1.5 kg) who were appropriate for gestational age (AGA; n = 379) or small for gestational age (SGA; n = 102). Growth was assessed at birth, term, and 8 and 20 months' corrected age and results of a Bayley Mental Developmental Index (MDI) and neurologic examination were evaluated at 20 months of age. Both groups had similar 20-month MDI scores (93 vs 90) and similar rates of neurologic impairment (14% vs 10%). More SGA infants (25%) than AGA infants (14%) had a subnormal head circumference at 8 months' corrected age, indicating failure to catch up in head growth. Subnormal head size at 8 months predicted the 20-month MDI score for both groups. However, multivariate analysis indicated that, whereas subnormal 8-month head size was directly associated with the MDI score in AGA children, in SGA children 8-month head size was not significantly related to the MDI score when neonatal illness, neurologic impairment, socioeconomic status, and race were controlled for. We conclude that in AGA VLBW infants, subnormal head size at 8 months results from major neonatal illness and has adverse later effects. In SGA infants, subnormal head size at 8 months has many causes, including growth retardation in utero, which may not have an adverse effect on outcome; if subnormal head size is superimposed with neonatal illness, a poorer outcome is likely.

A comparison of obstetrical records of autistic and nonautistic referrals for psychoeducational evaluations.

Heretofore most studies dealing with the association between perinatal complications and autism have used a normal comparison group. In this study obstetrical records of 59 autistic children were compared to those of 28 nonautistic children whose intelligence has a similar range and distribution as the autistic sample. Using an optimality score to reflect number of obstetrical complications, we found that the nonautistic controls experienced less optimal conditions than the autistic sample. Abnormal presentation at birth is the only factor that occurred more frequently for the autistic sample than control sample.

Cluster of perinatal events identifying infants at high risk for death or disability.

To determine the prognostic import of neonatal seizures according to the presence or absence of certain other postnatal characteristics, we studied a population of 39,000 infants with birth weight greater than 2500 gm. Children with clinically recognized neonatal seizures and 5-minute Apgar scores less than or equal to 5 and who had at least one of five signs compatible with neonatal encephalopathy had a risk for first-year death of 33%. Survivors of this cluster of events (low Apgar score-abnormal signs-seizures) had a risk for motor disability of 55%. In contrast, survivors of neonatal seizures who did not have poor Apgar scores or other abnormal signs had a risk for motor disability of only 0.13%. Thus, among infants with neonatal seizures the risk for cerebral palsy was 420 times greater if there had been a low 5-minute Apgar score and other neonatal signs. Low Apgar score-abnormal signs-seizures constituted a cluster of events that served to identify, within the first days of life, a tiny subgroup of term newborn infants in whom risk for chronic motor disability was 55%, and for death or disability was 70%.

Decreased incidence of neurologic disability among neonates at high risk born between 1975 and 1984 in Alberta.

We report the outcome at 2 or 3.5 years of 1463 neonates at high risk born between 1975 and 1984 and cared for in a regional perinatal program in Alberta. Although the number of surviving infants of very low birth weight (1250 g or less) increased over the study period, the incidence rate of neurologic impairment fell significantly, from 19% to 13% (p less than 0.01), so that there was no significant increase in the absolute number of disabled children. This finding remained valid when two other groups of infants at high risk (those weighing more than 1250 g at birth and having a positive neurologic history and those born at term with asphyxial encephalopathy) were included in the analysis, so that over the decade there was a significant decrease in the incidence of disability among the total group of neonates (p less than 0.01) and no increase in the absolute number of disabled children (23 in 1975 and 19 in 1984). We conclude that neonatal intensive care has contributed to improved survival of neonates at high risk without increasing the burden of major neurologic disability.

Cervical cerclage for the temporary treatment of patients with placenta previa.

Cervical cerclage as a temporizing measure for the treatment of patients with placenta previa was evaluated in 25 patients admitted to the hospital for vaginal bleeding between 24-30 weeks' gestation and sonographic evidence of a placenta previa. The patients were randomly assigned to either cerclage (13) or conventional management (12). Cerclage patients had significantly better perinatal outcome, as indicated by more advanced gestational age at the time of delivery (34.9 +/- 3.0 versus 31.6 +/- 2.9 weeks; P = .02), larger birth weight (2709 +/- 511 versus 1812 +/- 506 g; P = .002), and fewer neonatal complications (two of 13 versus ten of 12 infants; P = .001). Maternal bleeding was more frequent and severe for patients in the control group. The total hospital cost was less for cerclage patients than for those receiving conventional expectant management ($9898 +/- 3943 versus $27,271 +/- 9901; P = .02). These results support the use of cervical cerclage for the treatment of patients with symptomatic placenta previa early in gestation.

Etiological factors associated with the development of periventricular leukomalacia.

Prenatal, intrapartum and postnatal factors are compared between 15 preterm infants, known to have periventricular leukomalacia (PVL) on ultrasound and 15 infants of similar birthweight and gestation who ultrasonographically showed no evidence of cystic lesions, and who are known to be neurologically normal at follow up. Prenatally, the incidence of antepartum haemorrhage was significantly higher in the PVL group. Intrapartum factors were similar between the two groups but postnatally, the PVL group had significantly lower PaCO2 readings during the first 72 h of life. It is postulated that a severe maternal bleed in late pregnancy and neonatal hypocarbia could significantly decrease cerebral perfusion and cause areas of ischaemia and infarction resulting in periventricular leukomalacia.

A chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome. A specific entity analysed in 30 patients.

We have studied the clinical presentation and course of a chronic inflammatory disease occurring in childhood and observed in 30 patients. The first symptoms were generally present at birth, except in a few patients where they were first noticed in early infancy. All the patients had the association of three main symptoms: neurological, cutaneous and articular. The skin rash was the first symptom observed in all the patients and looked like a chronic non pruritic urticaria varying during the day. The articular manifestations involved knees, ankles and feet, elbows, wrists and hands unaffecting the other joints. They could be mild giving arthritis during flare-ups or severe with major radiological modifications affecting the epiphysis, metaphysis and growth cartilage. The neurological manifestations were characterized by a chronic meningitis and symptoms indicating meningeal irritation: headaches, seizures, spasticity of legs. Most patients had a cerebral atrophy and a low IQ. Sensory organ involvement occurred progressively during the follow-up: ocular inflammation with optic atrophy, deafness and hoarseness. Common morphological features characterized these patients with short stature, head enlargement, saddle back nose and short and thick extremities with clubbing of fingers. The course was that of a chronic inflammatory disease with numerous flare-ups associating fever, splenomegaly and adenomegaly. Except for a high level of eosinophils in blood, CSF and tissues, the biology was non specific and only exhibited features of inflammation. Except for two families, the disease was sporadic. A high frequency of prematurity with features resembling a foetal infection was observed but no proof of a possible causal virus has so far been found so that etiology remains unknown.

Association of adverse perinatal events with an empty sella turcica in children with growth hormone deficiency.

High-resolution computed tomography (HR-CT) of the hypothalamo-pituitary region was performed in 26 consecutive children presenting with growth hormone deficiency (GHD) at one clinic. 58% had an empty sella turcica (ES) and 42% a full sella turcica (FS). There was no difference between the ES and FS groups for mean (+/- 95% confidence limits) presentation age (ES 6.7 (+/- 1.8) years, FS 5.6 (+/- 2.2) years), height standard deviation score (SDS) (ES -3.9 (+/- 0.8), FS -3.3 (+/- 0.5] nor head circumference SDS (ES -1.9 (+/- 1.1), FS -0.7 (+/- 1.1]. There were significant associations between the ES group and a history of adverse perinatal events (p less than 0.001) and multiple pituitary deficiency (p = 0.014). Growth hormone response to an acute growth hormone releasing factor test showed no association with HR-CT diagnosis. Sella turcica volumes were calculated from the HR-CT scans. All sella volumes were small; mean SDS for height was -2.6 (+/- 0.2). There was no difference in sella volume SDS between the ES and FS groups (ES -2.9 (+/- 0.3), FS -2.5 (+/- 0.4]. Adverse perinatal events may cause an ES and GHD by compromising the blood supply to the pituitary gland or infundibulum.