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The management of secondary glaucoma in nanophthalmic patients.

PURPOSE: To investigate the clinical characteristcs, management of secondary glaucoma in nanophthalmos, and the prevention of its complications. METHODS: Retrospectively, 9 cases (17 eyes) with nanophthalmic glaucoma were studied. RESULTS: The axial length of the eyes ranged (14.36 - 19.33) mm; All of the cases combined with hyperopia ranged (+7.00 +/- 16.00) D. All 17 eyes had the manifestation like angle-closure glaucoma. The glaucoma was controlled in 9 of 17 eyes at the early stage, which underwent laser iridotomy (4 of 9 eyes also underwent laser iridoplasty). 1 eye underwent ciliary photocoagulation because its visual acuity was lost and the patient complained of pain. The other 7 eyes underwent filtration surgery and 3 of them had permanent loss of vision caused by disastrous complications after the surgery. CONCLUSIONS: Management of secondary glaucoma in nanophthalmos is complicated. The laser iris surgery is safe and effective in glaucoma at the early stage. Vortex vein decompression, sclerotectomy or anterior sclerotomy may be performed to reduce disastrous complications.

Adolescent↗

Effect of prenatal exposure to diagnostic ultrasound on the development of mice.

Pregnant Swiss albino mice were exposed to diagnostic ultrasound (3.5 MHz, approximately 65 mW) for 10 min on Day 3.5 (preimplantation period), 6.5 (early organogenesis period), or 11.5 (late organogenesis period) of gestation. Sham-exposed controls were maintained for comparison. Exposed as well as control fetuses were dissected out on the 18th day of gestation, and changes in total mortality, body weight, body length, head length, brain weight, sex ratio, and microphthalmia were recorded. Exposure on Day 3.5 of gestation resulted in a small increase in the resorption rate and a significant reduction in fetal body weight. A low fetal weight and an increase in the number of growth-retarded fetuses were produced by exposure on Day 6.5 postcoitus. A statistically nonsignificant increase in the incidence of microphthalmia was induced in fetuses exposed on Day 6.5 or Day 11.5 of gestation. These results indicate that ultrasound may have some adverse effects on the mouse embryos depending on the developmental stage at which the exposure occurred.

Animals↗

[Severe visual impairment of children with hydrocephalus and concomitant diseases].

PURPOSE: The aim of this study is to assess the condition of the sight organ among small children suffering from hydrocephalus and accompanying diseases. PATIENTS AND METHODS: The results of the examination done upon fifteen children (five girls and ten boys) suffering from hydrocephalus and accompanying diseases have been analysed. The accompanying diseases were as follows: cerebral palsy, epilepsy, retrolental fibroplasia, microphthalmia and congenital cataract. The average age of a child was 3,7 months. The examination concerned: visual acuity, the setting of the eyeballs, ocular fundus and flash visual evoked potentials (FVEP). Evans' Index was calculated from MRI or TK examinations. RESULTS: The following distortions have been noticed: bilateral optic atrophy (14), bilateral nystagmus (4), bilateral strabismus (5), bilateral loss of pupils' reaction (2). The latency of FVEP was like in control group, but the amplitude was markedly lowered. CONCLUSIONS: Optic atrophy is the main cause of low vision in children with hydrocephalus. Severe visual disorders might be a serious problem of rehabilitation of those children. Objective examination in patient, who do not co-operate, is FVEP. Evans' Index is not prognostic value about blindness in children with hydrocephalus.

Cataract↗

Familial cataracts and concurrent ocular anomalies in chow chows.

A closely inbred line of Chow Chows affected with congenital cataracts was studied. Sixteen dogs were examined including 1 adult male, 2 adult females, and 13 pups. Twelve of the pups were from 6 different litters, out of 6 different bitches, all sired by 1 adult male. The exact relationship of the thirteenth pup was undetermined. Clinical evaluation included slit-lamp biomicroscopy, biomicroscopic photography, and indirect ophthalmoscopy. Clinical appearance of the cataracts was variable, ranging from incipient nuclear or capsular lesions to advanced cortical opacity. The lens nucleus was most consistently affected, with variable involvement of the lens cortex. Concurrent ocular anomalies of some eyes included wandering nystagmus, entropion, microphthalmia, persistent pupillary membrane remnants, and multifocal retinal folds. A correlation was not apparent between the character or severity of the cataracts and the finding of the other anomalies. Histologic examination of 12 lenses revealed posterior displacement of the lens nucleus, retained lens epithelial cell nuclei in the nuclear and cortical lens, anterior capsular irregularity and duplication, anterior lens epithelial duplication, and posterior subcapsular migration of epithelium. The high incidence of cataract in this family of Chow Chows suggested an inherited defect, although the inheritance pattern was undetermined.

Animals↗

Periventricular neuronal heterotopia, oro-facio-digital anomalies, and microphthalmia. A new syndrome?

Congenital malformations involving the face, oral cavity, and digits are the main findings in the oro-facio-digital (OFD) syndromes. Various eye anomalies and central nervous system involvement have also been reported in this condition. Here we report on a Brazilian boy with features belonging to the clinical spectrum of the OFD syndromes. He also had additional findings of periventricular nodular heterotopia (PVNH), asymmetric limb involvement, and microphthalmia. This unusual pattern of anomalies has not been reported previously and appears to be unique.

Abnormalities, Multiple↗

Embryology and morphology of microphthalmia in transgenic mice expressing a gamma F-crystallin/diphtheria toxin A hybrid gene.

BACKGROUND: Transgenic mice in which elongating lens fiber cells were ablated resulting in microphthalmia have been reported, however, their embryology and detailed morphology have not. EXPERIMENTAL DESIGN: The morphology of homozygous and hemizygous CDI, transgenic mice carrying the gamma FDT-A gene was studied by light microscopy on different days of gestation as well as postpartum. The findings were compared with normal CD-1 wild type controls. RESULTS: The earliest changes in mouse embryos transgenic for the gamma F-crystallin/diphtheria toxin A transgene are seen on day 12, when apoptotic cells appear in the area of elongation. In hemizygous embryos, ocular development is relatively normal until day 17 when the lens and eye are slightly smaller than normal and the lens vesicle is filled with abnormal lens material. At this time, the posterior capsule of the lens may rupture, releasing abnormal lens material which disperses throughout the eye, perturbing growth and other ocular structures. Additional breaks may subsequently occur and the ultimate morphology of the hemizygotes correlates with when the posterior capsule ruptures, how much lens material is released, and where it disperses. In homozygous embryos, due to extensive ablation of lens fiber cells, the "lens" becomes a diminutive mass of abnormal lens material, posteriorly located within the eye, and otherwise unable to fulfill its mechanical or inductive role in the development of the cornea, anterior chamber, iris, ciliary epithelium, and zonules with the result that all of these structures are markedly abnormal or absent. In addition, the lens is necessary for the accumulation of vitreous which in turn is required for the growth of the eye as a whole. In homozygous animals, vitreous does not accumulate and severe microphthalmia results. CONCLUSIONS: This study confirms and extends previous observations and conclusions on the central, orchestrating role of the lens in the development of the eye and illustrates the power of transgenic technology to elucidate the finer points of mammalian ocular development.

Animals↗

Intracorneal positioning of the lens in Pax6-GAL4/VP16 transgenic mice.

PURPOSE: The purpose of this study was to establish a GAL4/VP16-based binary transactivation system that was active in the lens and corneal epithelium of transgenic mice. METHODS: We generated transgenic mice with the transcriptional transactivator GAL4/VP16 driven by a modified Pax6 promoter that is active in lens and corneal epithelial cells. We also generated and tested UAS-lacZ reporter mice. Wild type and transgenic mice were analyzed by histological, in situ, and Southern hybridization techniques. RESULTS: Five families (OVE1931, OVE1934, OVE1935, OVE1936, and OVE1937) that carry the Pax6-GAL4/VP16 transgene were generated. Unexpectedly, mice from three of the transgenic lines showed ocular abnormalities. In the family OVE1936, cataracts were seen in the heterozygous mice at the time of eyelid opening and homozygotes showed microphthalmia. Transgenic mice in families OVE1931 and OVE1937 appeared normal. Histological analysis of ocular sections of OVE1934, OVE1935, and OVE1936 homozygous transgenic mice showed intracorneal positioning of the lens. The corneal stromal cells were disorganized and there was no distinctive corneal endothelial layer. In situ hybridizations showed robust expression of the GALVP16 transgene in the lens and corneal epithelial cells of the OVE1934, OVE1935, and OVE1936, but not in OVE1931 or OVE1937 families. Bigenic embryos generated by mating the Pax6-GAL4/VP16 mice to the UAS-lacZ mice showed that the GAL4/VP16 transgenic protein is functional and can induce eye-specific expression of a UAS-lacZ reporter gene. CONCLUSIONS: Our data suggest that (1) expression the GAL4/VP16 transgene induces changes in gene expression in lens cells, (2) that developmentally important genes are affected, and (3) that bigenic phenotypes will need to be interpreted with caution.

Animals↗

Choroidal effusion following laser peripheral iridotomy for the treatment of angle closure glaucoma in a patient with nanophthalmos.

We report a case of Nanophthalmos with choroidal effusion and retinal detachment following a peripheral laser iridotomy for the treatment of angle closure glaucoma. Angle closure glaucoma was diagnosed in the right eye of the patient after finding an elevated intraocular pressure and narrow angles on gonioscopy. The glaucoma was treated by lowering the intraocular pressure medically followed by laser peripheral iridotomy. The first day after a laser peripheral iridotomy, choroidal effusion was noticed that was followed by an exudative retinal detachment within a week. The medical and laser therapies for the lowering of intraocular pressure for the treatment of angle closure glaucoma in patients with nanophthalmos can be associated with choroidal effusion, retinal detachment and loss of vision.

Adult↗

Microphthalmia due to congenital varicella infection: a case report.

The authors report the clinical case of a newborn affected by congenital varicella syndrome, occurred about the 12th gestational week, with ophthalmic involvement. The ocular anomalies consisted in right microphthalmia, with lens opacities and atrophic chorioretinitis, without any involvement of other pathologies. The ophthalmic lesions and the sierological data confirmed that the infection occurred during the first weeks of gestation. Although the manifestation limited to the eyes is extremely rare, the authors point out the necessity of an appropriate prevention to avoid irreversible involvements of important organs.

Chickenpox↗

[Townes-Brocks syndrome. Case report and review of the literature].

We report on a 14 months old Turkish girl with bilateral triphalangeal thumb and preaxial hexadaktyly on her left hand, bilaterally missing third toes and "Satyr-ears" as typical features of Townes-Brocks Syndrome. Besides reviewing all previous publications we discuss frequency and significance of all features--especially of anorectal malformations and the third missing toe.

Abnormalities, Multiple↗

[Persistent hyperplastic primary vitreous--developmental anomaly of the eye in children].

Persistent hyperplastic primary vitreus in children continues to be a diagnostic and therapeutic challenge for ophthalmologists. It can occur in isolation, in association with other ocular disorders and rarely as a part of systemic disorder. Characteristic features include microphthalmic eye, white vascularized retrolental tissue with or without a persistent hyaloid artery, centrally dragged ciliary processes, an anteriorly shifted and (or) swollen lens, and varing degrees of lenticular opacification. PHPF is the most common associaton with unilateral cataracts. Differential diagnosis and functional effect of treatment are discussed.

Abnormalities, Multiple↗

[The structure of the superior colliculus in the microphthalmic mouse strain 944].

The structure of the superior Colliculus (CS) in congenitally blind mice was compared with that in healthy control mice. Thus Nissl staining and degeneration experiments were done resulting in differences in the Stratum opticum (SO) of the CS between microphthalmic and control mice. In microphthalmic mice the volume of SO decreases in 14% and packing density of neurons increases in 20% in comparison with controls. Terminal degeneration after lesion of the ipsilateral visual cortex is seen in the surface laminae only. This connection seems independent of visual signals.

Animals↗

The use of tissue expanders for induction of facial skeletal growth.

We present a case of successful orbital expansion with a tissue expander in 1 patient. This method will be valuable in preventing the facial deformities of anophthalmia and microphthalmia. The method was successful from a technical standpoint because of scleral investment of the expander and because of relative fixation of the expander by passage of the filling tube through the lateral orbital wall. The early experience of this patient confirms our expectations of successful orbital expansion based on the natural history of patients with orbital tumors or congenital glaucoma and on the experimental work of other investigators.

Child, Preschool↗