This review will focus on the mechanisms leading to ROP by exploring factors responsible for poor early weight gain and abnormal vascularisation of the eye of the preterm infant.
In a nation-wide survey of Aicardi syndrome, defined as the onset of epilepsy in the first six months of life, agenesis of the corpus callosum (partial or total) and lacunar chorioretinopathy, 18 patients, all girls, born between 1975 and 2002 were identified in Sweden. Fifteen were definite cases and three were regarded as probable, since they only fulfilled two of three inclusion criteria in addition to other cerebral malformations and/or chorioretinal changes. Calculations based on this survey and population-based studies on epilepsy in retarded children yielded a prevalence rate in the range of 2 - 15 : 100 000 girls. All but one had an ordinary birth weight, length and head circumference for gestational age. One was born preterm, one post term. The age at diagnosis varied from three days to 12 years and decreased during the period reflecting the increased awareness of the syndrome. Eleven came to medical attention because of seizures. Six had myoclonic, four generalized tonic-clonic and eight tonic, clonic or complex partial seizures. One had hypsarrhythmia, five multifocal epileptiform activity, three bilateral independent bursts, two burst-suppression pattern, six other types of spikes and one slowing of background activity. Asymmetrical EEG abnormalities indicating independent hemispheric dysfunction were detected in 13/18 (72 %). Complete absence of the corpus callosum was found in 13/18 (72 %), although not identical with the previous group, a partial defect in 3/18 (17 %), and a thinning in 2/18 (11 %). Of 15 children with definite Aicardi syndrome, 13 had binocular and two monocular lacunae. In one of the latter two, subtle monocular lacunae were found on fundus photographs, but had been missed on repeated clinical examinations. Of three children with probable Aicardi syndrome typical lacunae were reported in one and other kinds of depigmentation in the other two. Most of the children had anomalous optic discs. Neuroimaging in infancy or early childhood combined with ophthalmological examination and ocular fundus photography will facilitate an early diagnosis of Aicardi syndrome. Seizure type and EEG abnormalities may be non-specific at onset.
Purpose The aim of this study was to assess visual perception at school age of children born preterm with known lesions to the posterior visual pathways or with ophthalmologic signs that might indicate such lesions. Methods The study group consisted of 91 patients born before the 37th gestational week. Visual perception was assessed using the TVPS-R (Test of Visual Perceptual SkillsRevised) and a structured interview. In addition, ophthalmologic and orthoptic examinations were performed. Results On the test of visual perception, 67% of the patients had results below the third percentile of the American reference group. This is to be compared with 10% of Swedish full-term controls. Scores below the third percentile were observed in 87% of the patients with known brain lesions, 48% of those with strabismus without known brain lesion, and 86% of those with reduced visual acuity in the absence of strabismus and known brain lesion. Conclusions Reduced visual perception is common among children born preterm who have strabismus and/or reduced visual acuity, as well as in those with known brain lesions. This study emphasises the need to find tools to identify and assess those patients who have visual perceptual problems that may restrict their ability to meet the demands of daily life.
Aim: To assess cognitive ability in a population‐based group of prematurely born school‐aged children and to relate these findings to postnatal morbidity. Method: The study group consisted of a cohort of 51 children born preterm, 43 (26 boys, 17 girls) of whom were available for psychological evaluation At evaluation, their median age was 10 y (range 8–11 y). They were all born between 1988 and 1991, with gestational age less than 29 wk (median 27, range 24–28). Their median birthweight was 1060 g (range 450–1450). The Wechsler Intelligence Scale for Children (WISC‐III) was used, and the test results were compared with those of a standardized, age‐matched, normative group of children. Results: Thirteen children (30%) performed below average [intelligence quotient (IQ) >80] for Full Scale IQ (FSIQ). Thirty‐six children had a Verbal IQ (VIQ) below the mean value of 100 [84%, 95% confidence interval 73–95%], p > 0.0001. The Performance IQ (PIQ) was within the expected range of a normal population, although a large variability was observed. Discrepancies between VIQ and PIQ of more than 15 IQ units were found in 42% of the children. High postnatal morbidity (days with assisted ventilation, number of blood transfusions) and low birthweight standard deviation scores (SDS) were associated with lower PIQ than VIQ, while low postnatal morbidity and high birthweight were associated with higher PIQ than VIQ. Conclusion: This cohort of preterm children had reduced overall verbal capacity independent of morbidity, and a large variability in performance capacity that was associated with postnatal morbidity. The findings suggest that there are different mechanisms influencing the outcome of verbal and performance capacity in preterm children.
Aicardi syndrome has been defined by the triad of agenesis of the corpus callosum, early seizure onset and lacunar chorioretinopathy. In a nation-wide survey a total of 18 Swedish cases were found. Fourteen girls were re-examined by one of the authors at the ages of 1-27 years. One was seizure free following epilepsy surgery operation, 13 were drug resistant. Two were on ketogenic diet. Most of the girls had multifocal EEG discharges. All except one were severely disabled with severe mental retardation and total dependency on helpers for activities of daily life. Communication, nutrition, and motor function were severely affected areas. Visual function was difficult to evaluate because of mental retardation and lack of co-operation and varied from severely impaired to normal. In one case the appearance of the ocular fundus was documented to have changed with time. One girl was exceptional and seizure free with mild mental retardation. An update by March 2006 showed that 12/18 patients were still alive, at a median age of 13.5 years (range: 3-31 years). Six had died between the ages of 3-10 years. They had all suffered from intractable epilepsy and belonged to the most disabled group. Surprisingly two had died from malignant brain tumours.
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