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Biomedical subjects

K Strömland

Publications and source records attributed to K Strömland.

At least 37 records · Page 2Linked to original sources

Morphometry of the optic nerve and retinal vessels in children by computer-assisted image analysis of fundus photographs.

BACKGROUND: The retinal fundus in childhood has a different morphology than in adulthood. Existing methods are not suitable for evaluation of fundus photographs from children. Therefore, a new method for quantitative analysis of fundus morphology utilizing a personal computer-assisted digital mapping system was developed. METHODS: A CCD flatbed scanner is used to digitize fundus photographs, producing computer images which are analyzed on an IBM/AT computer. Area measurements of the optic disc, excavation and peripapillary crescent are made, as well as determinations of the length, branching, tortuosity and distribution of the retinal vessels on the fundus surface. RESULTS: Determination of the inter- and intra-observer variability of the computer-assisted image analysis technique demonstrated good reproducibility. The method is demonstrated using fundus photographs of six normal children and six children with the fetal alcohol syndrome. Typical variations in appearance of optic disc and retinal vessels are seen. CONCLUSION: The system is unique in measuring both the optic nerve head and the retinal vessels and is therefore especially useful for detailed studies of normal and abnormal development of these structures in children.

Adolescent↗

Optic nerve hypoplasia: comparative effects in children and rats exposed to alcohol during pregnancy.

Children with the fetal alcohol syndrome often have ocular anomalies. These include abnormalities of the eyes and adnexa (strabismus, blepharoptosis, epicanthus), as well as intraocular defects (cataract, glaucoma, persistent hyperplastic primary vitreous, retinal and optic nerve anomalies). Based on the clinical results in an ophthalmological study of a group of Swedish children with the fetal alcohol syndrome, in which optic nerve hypoplasia was found in up to one-half of the group, an experimental study was designed in rats pre- and perinatally exposed to alcohol by means of a liquid diet. The optic nerve was seriously affected. Macroglial cells and optic axons were ultrastructurally damaged. The diameter of the optic nerve cross section, glial cell nuclear area, axonal diameter, and the total number of optic axons showed significantly lower values in the alcohol-exposed group than in the controls. In addition, the retina from the alcohol-exposed animals displayed significantly lower values of the retinal thickness and ganglion cell nuclear volume, as compared to the controls. Thus, rats exposed to alcohol in utero developed hypoplasia of the optic nerve similar to the findings in children born to alcoholic mothers. This strongly supports the hypothesis that prenatal alcohol exposure may adversely affect the development of the optic nerve.

Animals↗

Autism in thalidomide embryopathy: a population study.

Of a population of 100 Swedish thalidomide embryopathy cases, at least four met full criteria for DSM-III-R autistic disorder and ICD-10 childhood autism. Thalidomide embryopathy of the kind encountered in these cases affects fetal development early in pregnancy, probably on days 20 to 24 after conception. It is argued that the possible association of thalidomide embryopathy with autism may shed some light on the issue of which neural circuitries may be involved in autism pathogenesis.

Adult↗

Thalidomide embryopathy: revisited 27 years later.

A prospective ophthalmological study was done in 86 out of a total of 100 Swedes with established thalidomide embryopathy. Forty-six (54%) of all examined individuals had eye findings, which made the eye the second most commonly affected organ in thalidomide embryopathy only surpassed by upper limbs (81%). Forty-three patients (50%) had ocular motility defects, mostly incomitant strabismus. Facial palsy and abnormal lacrimation each occurred in 17 (20%) individuals. One patient had coloboma of the uvea and optic disc and another two had coloboma of the optic disc. Infrequent anomalies were microphthalmos, congenital glaucoma, lipodermoid, and large refractive errors. The observed ocular motility defects, facial palsy and abnormal lacrimation occurred with early induced defects in thalidomide embryopathy, but not with isolated late occurring anomalies. This suggests that thalidomide exerts its effects on the development of these structures early in the teratogenic period, probably mainly during the fourth week of development.

Abnormalities, Drug-Induced↗

Refractive evaluation in thalidomide embryopathy.

To evaluate the ocular findings associated with thalidomide embryopathy, we examined 86 of 100 Swedes who had a proven correlation between birth defects and the mother's intake of thalidomide during pregnancy. Cycloplegic refraction, keratometry, and axial length measurements were performed. The subjects were divided into four groups according to their physical malformations, giving a time frame for when in gestation the insult occurred (the sensitive phase for thalidomide is 20-36 days after conception). The results indicate a trend toward shorter and longer axial lengths, high refractive errors, and corneal astigmatism in thalidomide embryopathy compared to controls, and in addition there was a tendency for those anomalies to occur in the group with the earliest thalidomide-induced defects. It is suggested that thalidomide disturbs the growth and shape of the eye and that this effect is exerted early in its teratogenic period.

Abnormalities, Drug-Induced↗

Goldenhar syndrome and autistic behaviour.

Two girls with concomitant Goldenhar syndrome (oculo-auriculovertebral spectrum disorder) and autistic disorder are described. One was diagnosed as having Goldenhar syndrome in the first few weeks of life and as having autistic disorder in her fifth year; the other was diagnosed as having Goldenhar syndrome when she was referred for evaluation of autistic symptoms at seven years of age. The type of physical abnormalities encountered in Goldenhar syndrome suggests damage to neural structures in the second or late stages of the first trimester. The two cases described in this report suggest that autistic disorder sometimes can result from neural damage during the second trimester.

Autistic Disorder↗

[Alcohol during pregnancy damages eye and vision development].

Maternal alcohol abuse during pregnancy causes malformations of the eyes with serious consequences to the vision of the affected children. A high percentage (up to 90 per cent) of children suffering from the fetal alcohol syndrome have eye abnormalities of different kinds. The most typical are hypoplasia of the optic nervehead and increased tortuosity of the retinal vessels, especially of the arteries, both occurring in up to half of the cases. Visual acuity is often reduced. Considering the developmental timing of different tissues of the eye, one can presume that there is a risk for deleterious effects of alcohol on eye structures at any point of time from early gestation until development is completed.

Alcoholism↗

The study of malformations "by the company they keep".

Our study of individuals with thalidomide embryopathy reaffirms many of the principles of clinical teratology concerning sensitivity of developing structures and specificity of action. It also highlights important features of ocular teratology that differ from those of other organ systems. Because malformations of the eye and associated structures do not significantly threaten the survival of the embryo, fetus, or neonate, any variation in prevalence in older individuals is not due to the nature of the ocular malformation but may be significantly modified by "the company they keep," the systemic malformations occurring at the same time as the original insult, whether it be an environmental, a genetic, or a local disturbance. Additionally, the presence of clusters of malformations caused by a teratogen at a specific time supports the concept of a common pathogenic mechanism and provides suggested avenues for further research into the cause of these malformations.

Abnormalities, Drug-Induced↗

Full-field electroretinograms in patients with the carbohydrate-deficient glycoprotein syndrome.

We examined five patients who had carbohydrate-deficient glycoprotein syndrome with full-field electroretinograms. Only two of the patients showed fundus changes typical for retinitis pigmentosa, whereas abnormal electroretinograms were seen in all patients. There was no recordable rod response; however, a delay in the cone b-wave implicit time was noted. All patients had nyctalopia. These observations suggest that patients with the carbohydrate-deficient glycoprotein syndrome have a progressive tapetoretinal degenerative disorder of the retinitis pigmentosa type with defined alterations in the electroretinogram.

Adolescent↗

Teratoma in the pterygopalatine fossa extending into the orbit: a case report.

An unusual case of intraorbital teratoma is presented. It was observed for the first time after 4 months when it caused increasing swelling of the maxillary region and proptosis of the eye. The diagnosis was difficult to establish. Repeated computed tomography scans were very helpful in evaluating the progress of the tumor. Surgical intervention with an infratemporal approach was undertaken and proved to be very successful.

Journal Article↗

Contribution of ocular examination to the diagnosis of foetal alcohol syndrome in mentally retarded children.

An ophthalmological study was conducted on a series of 28 Swedish children suffering from mild mental retardation without an obvious aetiological diagnosis. The eye examination supported a diagnosis of foetal alcohol syndrome in five out of six suspected cases and revealed two additional not previously suspected (25%). Eye anomalies were observed in 16 patients (57%) with fundus abnormalities (optic nerve hypoplasia, retinal dystrophy) being most frequent, occurring in 11 cases (39%). Four patients had substantially reduced visual acuity in both eyes. Eight children had abnormal features of the face and outer eye region. Ocular motility disturbances (strabismus or nystagmus) were seen in five patients. The ophthalmological study disclosed that at least 10% of this group of children with mild mental retardation suffered from the foetal alcohol syndrome. It is concluded that, in children with mental retardation, an ocular examination is a valuable diagnostic tool for revealing prenatal origin.

Child↗

Ocular pathology in disialotransferrin developmental deficiency syndrome.

Disialotransferrin developmental deficiency (DDD) syndrome is a recently described disease consisting of hepatopathy, mental retardation and neuropathy. The biochemical findings indicate a defect in the assembly of the carbohydrate moiety that is common to the secretory glucoproteins. It is believed to be of autosomal recessive inheritance. An ophthalmological examination of ten children suffering from this syndrome showed that all had ocular involvement. Esotropia (and deficient abduction) was found in all ten patients. Seven children had retinitis pigmentosa which was verified by an ERG in three. One patient had retinal signs suggestive of retinitis pigmentosa. The high incidence of ocular findings in the DDD syndrome, which are reported for the first time, indicate that an ophthalmological examination is a helpful diagnostic tool in this disease.

Adolescent↗

Eye findings in partial trisomy 2q.

Two cases with mental retardation, kyphosis, microcephalus, typical facial features, and eye anomalies had a previously undescribed recombinant chromosome change with duplication 2(q34; qter) secondary to paternal inversion 2(pter; q34). Eye findings consisted of reduced vision, myopia, nystagmus, iris defects and fundus lesions. Deformed optic discs were found in both patients and the retinal nerve fiber layer was thinner than normal.

Abnormalities, Multiple↗

Ocular abnormalities in the fetal alcohol syndrome.

Thirty children suffering from the fetal alcohol syndrome (FAS) were compared with 22 matched controls regarding malformations of the eyes and the visual function. All mothers to the FAS children had a documented abuse of alcohol during pregnancy. It was not possible to get reliable, detailed data on drinking habits, nor could it be ruled out that some mothers might have used psychopharmaceutic drugs during periods of the pregnancy. Defects of the outer eye region, or intraocular abnormalities, or both, were found in 27 FAS children (90%). Anomalies of the outer region like ptosis and strabismus were frequently found in the FAS children but were not common in the controls. Abnormalities in the anterior segments and media were found in six eyes. Malformations of the fundus were the most frequently occurring abnormalities in the FAS children. Forty-eight per cent of the eyes showed hypoplasia of the optic nervehead and 49% had an abnormal tortuosity of the retinal arteries. These variables were studied quantitatively by a new photogrammetric method. There were serious consequences to the vision of the FAS children. Nineteen per cent of the eyes has a visual acuity of 0.2 (20/100) or less, and 46% had 0.3-0.6 (20/60-20/30). Almost all controls had normal vision. As maternal alcohol abuse during pregnancy was the only variable which could be traced in every case of FAS, it is appears very likely that alcohol caused the abnormalities of the eyes, possibly through non-specific, intra-uterine growth retardation. It that were the case, ocular abnormalities should be common in children with non-FAS growth retardation. A control study did not support this possibility.

Abnormalities, Multiple↗

Eyeground malformations in the fetal alcohol syndrome.

The fetal alcohol syndrome (FAS) occurs in children born by alcoholic mothers. The syndrome consists of growth deficiency of prenatal onset, central nervous system effects with retarded mental and motor development and malformations of various organs, the most striking being the facial abnormities. There is a small head circumference and midfacial deformities. Typical eye features are telecanthus, epicanthus, short palpebral fissures, a flat nose bridge, ptosis, microphthalmus and strabismus. 30 children with FAS were examined for defects of the inner parts of the eyes. Malformations of the optic discs and retinal vessels were the most frequent findings, occurring in 16 children. Another five children had borderline findings. Nine had bilateral hypoplasia of the optic discs, eight of them also with changes of the retinal vessels, most often increased tortuousity. Four children showed bilateral optic atrophy. One of them also had highly tortuous retinal vessels. One girl had very tortuous retinal vessels without other findings in her fundi. One boy had pronounced malformations of both eyebulbs and another boy had bilateral changes of his eyeground structures. Various intraocular malformations were observed in 53% of the children with FAS.

Adolescent↗

Ocular involvement in the fetal alcohol syndrome.

Maternal alcohol abuse during pregnancy causes malformations of the eyes with serious consequences to the vision of the affected children. A high percentage (up to 90%) of children suffering from the fetal alcohol syndrome have eye abnormalities, including malformation in the outer eye region, disorders of motility, and defects of different intraocular structures. Two kinds of malformations stand out as most typical--hypoplasia of the optic nervehead (up to 48%) and increased tortuosity of the retinal vessels, especially of the arteries (up to 49%). Visual acuity is often moderately or severely reduced. Considering the developmental timing of different tissues of the eye, one can presume that there is a risk for deleterious effects of alcohol on eye structures at any point of time from early gestation until development is completed.

Adolescent↗