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At least 19 recordsLinked to original sources

Clinicopathologic correlation of microphthalmos with cyst.

Three patients had microphthalmos with cyst in one orbit and contralateral congenital cystic eye, microphthalmos alone, or microphthalmos with cyst. Four eyes were examined histopathologically. The microphthalmic eye demonstrated a spectrum of anterior segment abnormalities, retinal disorganization and gliosis, and a choroidal and scleral colomboma. The cyst connected to the colobona consisted of an outer fibrovascular layer and inner gliotic neuroectodermal layer. The cyst probably originated from proliferation of neuroectodermal tissue at the edge of a persistently open embryonic fissure. Treatment consisted of multiple aspirations of the cyst, excision of the cyst alone, and excision of both the microphthalmic eye and cyst.

Abnormalities, Multiple

Complicated colobomatous microphthalmos in the BW rat: a new form of inherited retinal degeneration.

A new model of inherited retinal degeneration has been found in the rat. It is inherited in association with a number of other ocular defects, including microphthalmos, coloboma, retinal dysplasia, optic nerve hypoplasia and/or aplasia, as well as medullation of the nerve fiber layer of the retina. Together, these abnormalities constitute a condition referred to as complicated colobomatous microphthalmos. This condition was originally discovered in the Bmn strain of rats but subsequently transferred to a new genetic background in the Bmn-wys strain of rats (BW). This facilitated the histological evaluation of both the developmental and degenerative ocular defects in the adult animals. A well defined pattern emerged relating eye size, optic nerve size and retinal histology. Normal-sized eyes had normal-sized optic nerves and normal retinal histology while intermediate-sized eyes with no optic nerves had uniformly thin retinas. In contrast, intermediate-sized eyes with small optic nerves had areas of both normal thickness and thin retina. All of these eyes developed retinal degeneration characterized by a late onset and slow progression associated with normal phagocytic activity in the pigment epithelium and a tendency for the rod outer segments to fragment into very thin structures rather than accumulate as lamellar debris. This indicates that the retinal degeneration in the BW model differs in many respects from the well studied RCS model.

Animals

Bilateral microphthalmos without microcornea associated with unusual papillomacular retinal folds and high hyperopia.

A 23-year-old man had bilateral microphthalmos without microcornea. The eyes measured about 15 mm in length by ultrasound while the corneas were 11 mm in diameter. A distinctive, elevated, funnel-shaped retinal fold stretching between the disk and the macula was present bilaterally. Other unusual aspects were the deep anterior chambers and uncompromised chamber angles. This combination of anomalies is believed to be the result of a failure of growth of the posterior outer coats of the eye. These were of insufficient size to accommodate the excess sensory retina which folded to conform to its outer structural shell. Other cases of high hyperopia may in some instances be cases of microphthalmos, unrecognized because of normal-sized corneas.

Adult

Birth order and parental age in microphthalmos and other ocular diseases.

We compared the distribution of birth order and maternal and paternal ages of blind school children throughout Japan with that of the total Japanese population of the corresponding age groups and with that of a subgroup of children with acquired blindness. The number of first-born children with microphthalmos was smaller, and the number of second-, third-, or fourth-born children was larger, as compared with the control groups. The differences were highly statistically significant by chi-square test. There was a less pronounced indication of birth order effect in amblyopia, congenital cataract, and optic nerve atrophy, which involved more first-borns than in the controls. The distribution of maternal age was also different from the control group in microphthalmos, congenital cataract, corneal opacity, and optic nerve atrophy. Less mothers in their 20s and more in their 30s produced children with these conditions. We believe this finding may be partly related to the rapid decline in infant mortality and in the incidence of congenital blindness in Japan.

Adult

Incipient histopathological lesions in citral-induced microphthalmos in chick embryos.

Microphthalmos was indeed experimentally in 3-day-old chick embryos by intraamniotic injection of citral. Various degrees of this particular microphthalmic syndrome were observed. Severe cases were always accompanied by destructive changes in the ipsilateral part of the head. The corneal epithelium lost its continuity and the lens showed, even in the early stages, severe degenerative changes with spherophakia. The neural retina seemed to be affected later and showed hyperplasia and fold formation, while the retinal pigment epithelium was only mildly affected. The mesenchyme behaved differently according to its location: the superficial corneal and ciliary mesenchyme underwent hyperplasia, while the deeper choroidal mesoderm showed edematous changes only. This temporal and spatial distribution of pathological changes in the eye structures favors the assumption that citral acts locally. The experimental microphthalmic syndrome is not only due to the direct effect of the exogenous teratogenic factor on the ocular anlagen but is also the result of subsequent adaptive changes of these structures to the new environmental condition.

Acyclic Monoterpenes

Reconstruction of the lower lid in congenital microphthalmos and anophthalmos.

In congenital microphthalmos and anophthalmos, the socket and lids are often underdeveloped. Progressive dilation of the socket often does not increase the horizontal lid aperture or permit the use of a larger prosthesis. The authors present two cases in which a modified Mustarde cheek flap, lined with a tarsal-conjunctival graft, was used to reconstruct and lengthen the lower lid. This procedure results in a larger horizontal lid aperture and permits a larger prosthesis to be contained in the socket.

Anophthalmos

Microphthalmos with orbital cyst: a clinicopathological report.

The clinical and histopathological findings in a case of microphthalmos with orbital cyst is described. The clinical interesting feature of this case was the bilateral coloboma which represented interference in the development of the eye at different stages of embryonic life.

Child, Preschool

[Uveal effusion--microphthalmos--severe hypermetropia].

Report of a case of uveal effusion with nanophtalmus and hypermetropia in a 40-year-old woman. This case is similar to those described in the literature as uveal effusion and nanophthalmus. It shows that the syndrome may be unilateral and not accompanied by glaucoma. The most characteristic traits of the syndrome are thickening of the sclera and peripheral retinal vascular anomalies. The syndrome seems to be secondary to a lesion of the vorticose system and to poor circulation conditions in the intrascleral part of this system.

Adult

Congenital retinal disinsertion syndrome.

The congenital retinal disinsertion (CRD) syndrome refers to cases of retinal detachment with disinsertion and may be divided into two groups. Group 1 reported by Hovland and co-workers includes healthy children with bilateral detachments and giant tears nasally, lens coloboma, and center anterior and posterior cortical lens opacities. Group 2 includes healthy children with a unilateral detachment often associated with microphthalmos and catatract. The opposite eye may show a combination of changes including small central anterior and posterior cortical lens opacities, lens colobomas, and paving-stone degeneration. The patients may have an increased risk of developing a detachment in the second eye, and some of these eyes have been treated with cryopexy. Seven patients belonging to group 2 have been described and four of these had unilateral cataract and microphthalmos. Two other patients had some degree of unilateral microphthalmos. Small central anterior or posterior cortical lens opacities were found in five eyes, and lens colobomas were found in two eyes. Six out of seven patients had varying degrees of paving-stone degeneration temporally in the nondetached eye. The CRD syndrome was familial in two patients who were sisters. Pathologic studies were done in the eyes of three patients.

Child

Multiple ocular anomalies associated with maternal LSD ingestion.

Severe ocular malformations, including microphthalmos, intraocular cartilage, cataract, persistent hyperplastic primary vitreous, and retinal dysplasia, occurred in a premature baby girl. The mother had ingested LSD during the first trimester of pregnancy. To our knowledge, this is the third case reported of ocular teratogenesis associated with maternal LSD ingestion. Further cases must be documented to establish an actual cause and effect relationship between the drug and the induced malformations.

Abnormalities, Drug-Induced

[Enzymatic studies in lenses of different breeds of mice (author's transl)].

Leucinaminopeptidase (LAP), lactatedehydrogenase (LDH) and glucose-6-phosphatdehydrogenase (GPDH) were analysed in the lenses of two different breeds of mice. Compared to the lenses of a control stock (Agnes Blum, Jena), in the healthy seeming, heterozygous mice the same activities of LAP, LDH, and GPDH were found. But in the breed with evident microphthalmos and cataract LDH was increased. Possible connections are discused.

Animals