[Pathological findings in the eye of children with facial nevus].

Background: Port-wine stains distributed to the trigeminal area may be associated with eye and/or central nervous system complications. Visual loss may be prevented with screening for eye pathology at an early age with adequate intervention.

Material And Methods: 45 children with port-wine stains in the trigeminal area were examined by an ophthalmologist under general anaesthesia.

Ocular findings in allogeneic stem cell transplantation without total body irradiation.

Objective: To evaluate the ophthalmologic complications in hematologic patients after allogeneic stem cell transplantation (ASCT) without total body irradiation.

Design: Retrospective noncomparative case series.

Participants: A total of 150 ASCT patients.

[Leukocoria (white pupil) among children--mother is always right].

Leucocoria is a rare, but serious symptom in early childhood. It may be present at birth or develop during infancy. As in adults, infants usually display a black pupil, and upon shining a bright light into the pupil with the ophthalmoscope, an orange or red reflex emerges.

[Retinopathy of prematurity. Experiences with a routine examination].

Retinopathy of prematurity continues to be an important cause of serious visual impairment and blindness in children. We have previously suggested a schedule for ophthalmoscopic examinations to identify the serious cases where there may be indication for cryotherapy. We present our experience from this schedule, which covered premature infants with gestational age below 31 weeks and/or birth weight below 1,500 g.

Incidence of registered visual impairment in the Nordic child population.

A collaborative, population based, prospective register study on the incidence of visual impairment in children during the year 1993 was carried out in five Nordic countries with a total population of 17 million inhabitants. The child population was 3.8 million individuals aged 0-17 years.

Aplasia cutis congenita associated with limb, eye, and brain anomalies in sibs: a variant of the Adams-Oliver syndrome?

Aplasia cutis congenita (ACC) may occur in isolation or with other congenital malformations. Peripheral limb anomalies and ACC are major elements of the Adams-Oliver syndrome, which is usually inherited as an autosomal dominant disorder. We report on a sister and brother with ACC and brain, eyes, and transverse limb anomalies.

[Retinopathy of prematurity. Cryotherapy can reduce the risk of severe visual impairment].

Retinopathy of prematurity is a main cause of severe visual impairment and blindness in infancy. It is reported that the incidence is increasing along with the growing survival of infants with an extremely short gestation period. Transscleral cryotherapy has been shown to be effective in arresting the progression of the disease into the sight-threatening fibrovascular stages.

Apple peel intestinal atresia in siblings with ocular anomalies and microcephaly.

Two siblings who presented shortly after birth with signs of upper intestinal obstruction were successfully operated for apple peel jejunal atresia. In addition to intestinal malformations, both siblings exhibited severe microcephaly and ocular abnormalities not previously reported in this condition.

Visual impairment in Nordic children. V. X-linked juvenile retinoschisis.

In a study on 2,527 visually impaired children from four Nordic countries X-linked juvenile retinoschisis was diagnosed in 35 male children. Striking differences in frequency between the four countries were found, with 26 cases reported from Finland, 5 cases from Denmark, and 4 cases from Norway. None was reported from Iceland.

Visual impairment due to retinopathy of prematurity in Nordic children.

The registers of visually impaired children in Denmark, Finland, Iceland and Norway have been compiled into a common database by a Nordic study group of ophthalmologists, NORDSYN. The database contains information on 2527 children aged 0-17 years. The total number of children with visual impairment due to retinopathy of prematurity (ROP) is 247.

Cryotherapy for retinopathy of prematurity. International perspectives and some recommendations for treatment.

Retinopathy of prematurity (ROP) is a leading cause of severe visual impairment and blindness in infancy. Transscleral cryotherapy has been shown to be effective in arresting the progression of ROP into the sight-threatening fibrovascular stages of the disease. It is currently recommended that all eyes reaching 'threshold' ROP should be treated.

Visual impairment in Nordic children. IV. Sex distribution.

A Nordic study group of ophthalmologists, NORDSYN, has compiled registers in Denmark, Finland, Iceland and Norway of 2527 visually impaired children aged 0-17 years. This paper is concerned with the sex-distribution in the registers and has documented a statistically significant excess of males in two of the registers (Denmark and Finland). The dominance of males seems to be related to two main conditions: 1.

Visual impairment in Nordic children. III. Diagnoses.

The diagnoses, according to type and site and the degree of visual impairment, responsible for severe visual impairment in children below the age of 18, were analyzed in a material compiled from the national registers of visually impaired in Denmark, Finland, Iceland and Norway. Among 2527 children the predominant causes of visual impairment are ascribed to congenital malformations, neuro-ophthalmological diseases and retinal diseases. Optic atrophy is the leading single cause of severe visual impairment when all diagnoses are compared, and this also applies when all categories of visual impairment are included.

Visual impairment in Nordic children. II. Aetiological factors.

Careful clinical-aetiological assessment of visually impaired children is one of the prerequisites for prevention of future, 'unavoidable' cases of visual impairment of children in the industrialized part of the world. In a collaborative study (NORDSYN) between four Nordic national registers of visual impairment, we analysed and classified some of the factors considered to be essential components for the development of low vision or blindness in children. We discuss the conceptual basis for aetiological classification of eye disorders and visual impairment.

Visual impairment in Nordic children. I. Nordic registers and prevalence data.

A Nordic study group of ophthalmologists, NORDSYN, has compiled data from registers in Denmark, Finland, Iceland and Norway of 2527 visually impaired children. Each record contains the following information: sex, year of birth, year of registration, classification of visual impairment, ocular diagnosis, systemic diagnosis, aetiology and evt. additional impairments.

Sightsaving therapy in retinoblastoma. Experience with external megavoltage radiotherapy.

In Norway, sightsaving therapy in retinoblastoma has only been given to least affected eyes in bilaterally affected children. The results in 8 eyes given external high energy radiotherapy are reported. Additional focal therapy was given to 3 eyes for residual tumours and to one eye with new tumour at the ora serrata.

Changes in the juxtapapillary retinal pigment epithelium following intravenous injection of sodium iodate. A light and electron microscopic study using horseradish peroxidase as a tracer.

The effect of an intravenous injection of sodium iodate on the retinal pigment epithelium immediately surrounding the optic nerve head has been investigated using horseradish peroxidase as a morphological tracer. A sodium iodate injection leads to a necrotic reaction of practically the complete retinal pigment epithelium. The juxtapapillary pigment epithelium, however, showed only attenuation and depigmentation without any necrotic reaction 4 and 12 days after the injection.

Demonstration of a diffusional pathway between the subretinal space and the juxtapapillary connective tissue. An in vitro experiment using horseradish peroxidase as a tracer.

A defect in the blood-retina barrier in the optic nerve head region has been demonstrated. In order to show the exact localization of this defect, an in vitro experiment has been performed on rabbit eyes, using horseradish peroxidase as a tracer. From a subretinal depot the tracer diffused between the innermost retinal pigment epithelial cell and the cells of Kuhnt intermediary tissue into the border tissue of Elschnig and the juxtapapillary choroid.

A defect in the blood-retina barrier in the optic nerve head region in the rabbit and the monkey.

The permeability properties of the tissues in the optic nerve head region have been investigated in monkey and rabbit using horseradish peroxidase as an histochemical tracer. Following intravenous administration, the tracer diffused from the peripapillary choroid into the different parts of the optic nerve head region, and also into the receptor layer of the sensory retina adjacent of Kuhnt intermediary tissue. This observation demonstrates a defect in the blood-retina barrier in this region.

Refsum's disease. Eye manifestations in a patient treated with low phytol low phytanic acid diet.

The cardinal eye symptoms of Refsum's disease are night blindness, retinal pigmentary degeneration and constriction of the visual fields. Similarities with or differences from retinitis pigmentosa are discussed. A 39-year-old male has had manifestations of Refsum's disease from the age of 7 years and has been on a low phytol low phytanic acid diet for the last 13 years.