[Pathological findings in the eye of children with facial nevus]. / Patologiske funn i øyet hos barn med fødselsmerke i ansiktet.

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. RESULTS: Seven of 30 patients with one or both eyelids affected had a choroidal vascular anomaly. Four patients had glaucoma. These four patients had both eyelids affected. Two children with a vascular anomaly had only upper eyelid involvement. INTERPRETATION: Patients with facial port-wine stains affecting the eyelids should be screened for eye pathology at an early age.

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. INTERVENTION: Ophthalmologic examinations of 130/150 patients were made, with particular attention to the occurrence of graft-versus-host disease (GVHD). RESULTS: One hundred thirty patients with a mean age of 35.5 (SD 10.6) years at the time of ASCT were followed up for a mean of 12 months (range 3-60 months). GVHD developed in 73 patients (56.2%). Of 130 patients, 52 (40.0%) had ocular complications, and 29 (22.3%) of those had GVHD. Keratoconjunctivitis sicca was diagnosed in 13 (10.0%) patients, and 12 (9.2%) had different stages of pseudomembranous conjunctivitis. Cataract developed in 3 patients (2.3%) in the observation period, and 7 (5.4%) had keratitis. Six (4.6%) patients had uveitis, and 8 (6.2%) had retinal hemorrhages. No ischemic retinopathy was found. Bilateral optic disc edema developed in 10 (7.7%) patients. CONCLUSIONS: Fewer ocular complications were observed in this series than in earlier studies, and the visual outcome was favorable. Cyclosporine has been linked to the development of optic disc edema and ischemic retinal lesions. The latter condition was not observed in the study patients, and the optic disc edema resolved within 1 year in all patients without any detectable visual impairment, in spite of continuation of the drug. Seven patients had acute GVHD at the time of optic disc edema, which is considered to be another manifestation of acute GVHD.

[Leukocoria (white pupil) among children--mother is always right]. / Leukokori (hvit pupill) hos barn--mor har alltid rett.

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. The first person to see that something is wrong, is often the mother, but she cannot give a precise description. When the infant is crying it can be difficult to examine the red reflex, and the necessity of admitting the infant to an ophthalmologist, when the mother is worried, is discussed. In this paper we discuss two cases of retinoblastoma and one case of congenital cataract where the treatment was delayed due to late diagnosis of the leucocoria. The main conclusion is always to listen to the relatives when they are worried about the infant's eye, and to make sure that the infant is properly examined.

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. The phalanges of the hands and feet were either short or absent. The girl also had absence of right patella, was severely mentally retarded and blind with retinal nonattachment. The boy had a falciform fold in the left eye. He died at age one week and autopsy showed partial agenesis of corpus callosum. The findings in the sibs may represent a severe variant of the Adams-Oliver syndrome, or a previously unrecognized syndrome involving vascular disruption.

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. If both eyes of an infant reach 'threshold', both eyes should be treated. For centers that handle a limited number of infants with ROP it is suggested that the cryotherapy should be done under general anesthesia.

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. Cataract was treated in 2 of the 3 eyes in which it developed. Vitrectomy was performed in 2 eyes with vitreous haemorrhage and persisting vitreous haze. Secondary enucleation has been avoided in all eyes 3-9 years after irradiation. One patient developed metastases in the temporal region at the enucleated side. Visual acuity is 6/12-6/6 if the macula has not been destroyed by tumour or focal therapy. Sightsaving therapy is presently also offered to unilateral cases and worst affected eyes in bilateral cases if the eyes belong to Reese-Ellsworth groups I-IV.