Search PubMed⌕ Search

Biomedical subjects

K Strömland

Publications and source records attributed to K Strömland.

At least 19 recordsLinked to original sources

[Fetal alcohol syndrome--unnecessary suffering which has not become rarer. Eyes are affected in up to 90 per cent of the cases].

Fetal alcohol syndrome, FAS, is characterized by pre- and/or postnatal growth deficiency, CNS dysfunction, typical facial features and evidence of prenatal alcohol exposure. FAS is often an unrecognized diagnosis and is based on clinical symptoms alone. The cognitive and behavioral disturbances have a great influence on the children's ability to learn and on their adult life. FAS is to a great extent found in areas with less than satisfactory socio-economic conditions. Since ophthalmologic involvement is frequent, an eye examination may be helpful in making a diagnosis. Prevention is essential since the brain damage prevails, despite comprehensive medical and social supportive efforts being made during childhood and in education.

Adult↗

Autistic spectrum disorders in Möbius sequence: a comprehensive study of 25 individuals.

The prevalence of autistic disorder was analysed in 25 individuals with Möbius sequence, a disorder with brain-stem dysfunction. The sample consisted of 18 males and seven females (20 participants were aged 2 to 22 years, and five were aged 1, 19 and 23 months, and 55 years old). Participants were recruited after a nationwide call and were part of a multidisciplinary study of individuals with Möbius sequence. They were given a meticulous neuropsychiatric examination including standardized autism diagnostic interviews. Ten individuals had an autistic spectrum disorder. Six of these met all diagnostic criteria for autism. In 23 individuals cognitive development could be assessed. Eight of those 23 patients had clear learning disability and six individuals were functioning in the normal but subaverage range. Autistic spectrum disorder and learning disability occurred in more than a third of the examined patients. Considering the hospital-based nature of the sample, these findings may be overestimates. Nevertheless, awareness of this coexistence is important in the diagnosis and habilitation care of children with Möbius sequence. Moreover, the results provide further support for the notion of a subgroup of autistic spectrum disorders being caused by first trimester brain-stem damage.

Adolescent↗

[Fetal alcohol syndrome--unnecessary suffering which has not become rarer. Eyes are affected in up to 90 per cent of cases].

Fetal alcohol syndrome, FAS, is characterized by pre- and/or postnatal growth deficiency, CNS dysfunction, typical facial features and evidence of prenatal alcohol exposure. FAS is often an unrecognized diagnosis and is based on clinical symptoms alone. The cognitive and behavoral disturbances have a great influence on the children's ability to learn and on their adult life. FAS is to a great extent found in areas with less than satisfactory socio-economic conditions. Since ophthalmologic involvement is frequent, an eye examination may be helpful in making a diagnosis. Prevention is essential since the brain damage prevails, despite comprehensive medical and social supportive efforts being made during childhood and in education.

Adult↗

[Eye muscle paralysis can increase the knowledge on autism].

One third of Swedes with thalidomide embryopathy have sixth and seventh cranial nerve palsy, named Möbius syndrome/sequence, and some of them suffer from autism. Further, other patients with Möbius sequence have autism. The teratogenic effect of thalidomide on the development of different organ structures occurs early in pregnancy. By studying the birth defects, information can be obtained concerning when the damage occurred. Erroneous development of the sixth and seventh cranial nerves is induced early in pregnancy. The ensuing palsy may be associated with autism. New research is focusing on genes controlling early cranial nerve development.

Abnormalities, Multiple↗

Children with septo-optic dysplasia - how to improve and sharpen the diagnosis.

The data in this article are based on investigations performed in 25 children with suspected septo-optic dysplasia (SOD). There are many signs and methods that help in the diagnosis of SOD. In particular, the ocular fundus, abnormalities of the hypothalamo-pituitary axis and other midline brain structures should be described. In order to achieve a more holistic and functional diagnosis, the degree of neurological, neuropsychiatric and psychological involvement should also be stated. It has been suggested that SOD is associated with autosomal recessive inheritance, and it can be speculated that it is the result of genetic and environmental influences early in gestation. An early diagnosis can favourably influence the outcome of the affected child.

Blindness↗

Optic nerve hypoplasia with isolated tortuosity of the retinal veins: a marker of endocrinopathy.

OBJECTIVE: To investigate whether children with optic nerve hypoplasia and pituitary hormone insufficiencies have specific ocular fundus characteristics that may facilitate early diagnosis and treatment. DESIGN: From May 15, 1995, through December 15, 1997, 17 children (8 girls and 9 boys, aged 0.3 to 13 years) with optic nerve hypoplasia were referred to the Department of Pediatric Ophthalmology, Children's Hospital, Göteborg, Sweden, and divided into 2 groups dependent on the presence (n = 8) or absence (n = 9) of pituitary deficiency. Morphological characteristics of the ocular fundus were evaluated by digital image analysis of fundus photographs, and the morphological characteristics of the brain structures were studied by magnetic resonance imaging. RESULTS: An isolated venous tortuosity noted among the children with optic nerve hypoplasia and endocrinopathy was the morphological ocular fundus variable that discriminated between the 2 groups of patients with optic nerve hypoplasia. Both groups of children demonstrated significantly reduced optic disc, cup, and neuroretinal rim area and few vascular branching points. CONCLUSION: Optic nerve hypoplasia with isolated tortuosity of the retinal veins may potentially help the ophthalmologist in identifying children who should undergo a thorough diagnostic workup of endocrine function.

Adolescent↗

The möbius sequence: a relook.

BACKGROUND: The constellations of findings often referred to as Mobius syndrome might be better described as Mobius sequence, because the term sequence defines a cascade of secondary events after an embryonic insult from heterogeneous causes. Classic clinical findings include evidence of sixth and seventh cranial nerve involvement, often with associated malformations of limbs, craniofacial structures, and other cranial nerves. METHODS: A prospective study was undertaken in Sweden of 25 patients who showed characteristic findings of Mobius sequence. RESULTS: Of the patients who did not have strabismus surgery, 10 patients had straight eyes in the primary position, 7 had esotropia, 2 had exotropia, and 1 had hypertropia. All had significant limitation of abduction, except 1 patient with exotropia who showed minimal underaction on abduction but a large limitation of adduction. In the description in these early cases, some patients manifested a clinical pattern resembling a horizontal gaze paresis. Narrowing of the palpebral fissure on adduction similar to that seen in Duane syndrome was observed in a few cases. Two patients had ptosis. Nineteen patients had diminished facial expression bilaterally, often asymmetric, and 6 cases appeared to be unilateral. Seven patients had abnormal tearing. Associated systemic findings included Poland anomaly (2), club feet or other limb anomalies (8), micrognathia (8), tongue malformations (17), cleft palate (5), and speech problems (18). An unusual finding was autism syndrome (6) or autism-like syndrome (1). CONCLUSIONS: The associated findings in Mobius sequence may give further clues to the location and timing of the developmental disturbance. The wide range of ocular motility patterns suggests that the previous concept of a lesion solely in the sixth nerve nucleus is an inadequate explanation for these findings.

Abnormalities, Multiple↗

Visual and ocular outcome in children after prenatal exposure to antiepileptic drugs.

PURPOSE: To prospectively study the incidence of structural and/or functional ophthalmological abnormalities in the offspring to an unselected population of women with epilepsy, subjected to a well controlled antiepileptic drug (AED) treatment during pregnancy. METHODS: Forty-three children prenatally exposed to antiepileptic drugs and 47 controls were included. Blinded ophthalmological examinations including fundus photography were performed at a median age of 7 years and 4 months. RESULTS: No major eye anomalies were found except in one child in the exposed group who had nystagmus and low vision. The visual acuity was lower in the eye with lowest acuity among the exposed children (p < 0.05). No other significant difference was found between the two groups. CONCLUSION: The results suggest that a well-controlled treatment with AEDs, preferably monotherapy, during pregnancy does not have any major adverse effects on the development of the eye and ophthalmological functions.

Anterior Eye Segment↗

Reference values of facial features in Scandinavian children measured with a range-camera technique.

Reference values of facial features in white Scandinavian children were ascertained using a newly developed range-camera technique with a three-dimensional measuring program specifically designed for anthropometric measurements. Seven facial features in 613 healthy subjects (322 girls, 291 boys), aged 1 month to 18 years, and nine subjects with the fetal alcohol syndrome,aged 7 to 18 years, were studied. Data were analysed by multivariate multiple regression and measurements of each facial feature plotted against age and presented as curves, with 95% and 99% univariate prediction limits. All children with fetal alcohol syndrome had shorter palpebral fissures, and in six of them the outer canthal distance was shorter than that in the reference group. The reference values presented may be useful in clinical practice--for example, in the evaluation of children with syndromes that result in dysmorphology of the face and in the planning of plastic and reconstructive surgery.

Adolescent↗

Assessment of facial features with a range camera.

We describe a new method for the quantitative analysis of facial features using a range camera with a three-dimensional measuring program. The range camera consists of a video camera, a light projector, and a computer. The video camera captures a number of images of different light patterns projected on to the face. From these, a "range image" is computed. One fully illuminated intensity image of the face is also taken. By defining anatomical landmarks manually on the intensity image, three-dimensional measurements are automatically obtained from corresponding positions in the range image. The method is highly accurate and precise, and is a simple and reliable way of assessing facial features. We recommend that the system be adapted in clinical practice--for example, in diagnosing syndromes and planning plastic and reconstructive surgery.

Cephalometry↗

The puzzle of autism: an ophthalmologic contribution.

PURPOSE: A previous study of 86 thalidomide-affected subjects with ophthalmic manifestations revealed the unexpected finding of autism in 4 of the 5 severely retarded individuals. The subjects had anomalies associated with an early gestational effect of thalidomide, including facial nerve palsy and incomitant strabismus. Because autism has been observed in a few cases of Möbius sequence (Möbius syndrome), a condition characterized by involvement of the sixth and seventh cranial nerves, the similarity to early thalidomide embryopathy suggested a relation between cranial nerve involvement and autism. The present study was undertaken to further evaluate the association of autism with patients manifesting findings of Möbius syndrome. METHODS: A prospective study of 25 Swedish patients with Möbius sequence was conducted. The patients had a complete multidisciplinary evaluation, including ophthalmologic and psychiatric examinations and standard testing for autism. Findings associated with autism were compared with the ocular and systemic anomalies of the 4 thalidomide-affected subjects. RESULTS: In the Möbius group 6 patients had autism, achieving the criteria for autism according to all the diagnostic manuals that were used. One patient showed autistic-like conditions meeting fewer numbers of the criteria. A few were too young to be meeting evaluated. Incomitant strabismus ranging from primary abduction defects alone to a horizontal gaze paresis pattern was noted in these patients, in addition to characteristic findings of seventh nerve paresis. Aberrant lacrimation was observed in many cases, especially often associated with autism. CONCLUSION: The common group of anomalies noted in both cases of thalidomide embryopathy and Möbius sequence suggests that brain-stem damage probably early in embryogenesis can sometimes be associated with autism.

Abnormalities, Drug-Induced↗

Midline brain lesions in children with hormone insufficiency indicate early prenatal damage.

The relationships between midline brain morphology, anterior visual pathway morphology and hormonal status in children with impaired growth were studied. Intracranial morphology was studied by magnetic resonance imaging in 47 children (14F, 33M), median age 9.7y (range 2.6-18.7y) undergoing growth hormone treatment (GH; 0.1 U/kg/d). They were chosen to represent various birth sizes and a spectrum of hormone insufficiencies. There was a relationship between GH secretion and the morphology of the neurohypophysis, the pituitary stalk and the anterior visual pathways, i.e. the greater the GH insufficiency, the more abnormal were these structures. The children with anterior visual pathway abnormalities had the lowest GH levels and the smallest adenohypophysis. The association between abnormalities of the anterior visual pathways and the hypothalamo-pituitary structures may reflect a common prenatal neural damage in embryologically and anatomically closely related structures.

Adolescent↗

Eye size in healthy Swedish children and in children with fetal alcohol syndrome.

PURPOSE: This study set out to collect reference data for normal ocular growth and to study the teratological effects of alcohol on eye development. METHODS: Eye size was studied in 92 healthy Swedish children (age 1 month to 16 years) as reference and in 13 children (age 1.4 months to 17 years) with fetal alcohol syndrome (FAS) using ultrasonographic axial length measurements. Another three children with FAS were evaluated by clinical examination only. RESULTS: The control group demonstrated a marked increase in total axial length during the first 2 years of life. Girls with FAS had a shorter total axial length (p = 0.045) than their controls. Both boys and girls with FAS demonstrated a relatively smaller vitreous body than the controls, p = 0.015 and 0.068, respectively. Three children with FAS had severe structural anomalies. CONCLUSION: The observations support previous studies indicating that alcohol has an adverse effect on growth and configuration of the eye.

Adolescent↗

Paediatric and ophthalmologic observations in offspring of alcohol abusing mothers.

The offspring of nine women who had abused alcohol and drugs during pregnancy were studied. Of the 30 children, 10 had fetal alcohol syndrome (FAS) or fetal alcohol effects (FAE). Ophthalmological impairments associated with FAS--impaired vision, optic nerve hypoplasia, cataract, increased tortuosity of retinal vessels--were common. The severity of teratogenic lesions varied among the children and was often related to the level of abuse during pregnancy. Most women were unable to take care of their children during periods of substance abuse. All children with FAS/FAE had learning difficulties. Four mothers abstained from alcohol or drugs during some of their pregnancies and gave birth to children without birth defects.

Adult↗

Fetal alcohol syndrome--an ophthalmological and socioeducational prospective study.

BACKGROUND: The eye is a sensitive indicator of adverse effects of prenatal alcohol exposure. Anomalies of the eyes and their adnexa are known to be associated with the fetal alcohol syndrome (FAS), although long-term effects of these malformations are unknown. DESIGN: A prospective ophthalmologic follow-up (median, 11 years; range, 4 to 19 years) was performed in 25 children with FAS. Their social situation and educational status were also investigated. RESULTS: All but one of the children had ophthalmologic abnormalities. Fundus anomalies were observed in 23 children, of whom 19 had optic nerve hypoplasia. Thirteen children had concomitant strabismus. Microphthalmos, buphthalmos, phthisis, microcornea, coloboma of the iris and uvea, blepharoptosis, cataract, persistent hyperplastic primary vitreous, and nystagmus were observed in single cases. The dysmorphology of the eyes remained unchanged during the follow-up period. In 2 children with severe mental retardation and, initially, very poor vision, the severe visual handicap persisted. Seventeen children had an initial visual activity > or = 20/70, which remained unchanged in 10 children and improved in 7 children, despite the presence of optic nerve hypoplasia in 14 of the children. Ten mothers died, 8 of them because of alcohol-related diseases, and only 4 of the mothers were able to take care of their children. Sixteen children went to schools for the mentally retarded, and only 3 children had a normal school education without extra teaching assistance. CONCLUSIONS: In children with FAS, the major sequela, ie, brain, damage, remains despite extensive medical, educational, and social support. The presence of ophthalmic signs, which persisted but did not deteriorate during the follow-up period, strengthens the diagnosis of FAS, and the high frequency of ocular involvement indicates the importance of a complete ophthalmologic evaluation in children with FAS.

Adolescent↗

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↗