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At least 145 records · Page 8Linked to original sources

Ultrastructural alterations in the aqueous outflow pathway of adult buphthalmic rabbits.

The aqueous outflow pathway of adult rabbit eyes with congenital glaucoma (buphthalmos) was examined by light microscopy and by scanning and transmission electron microscopy. The morphology of the buphthalmic rabbit aqueous outflow pathway was markedly abnormal when examined at 6 months, 1 yr, and 2 yr displaying apparent loss and/or compression of the iris pillars, dilation of the intertrabecular spaces, loss of endothelial cell-to-cell association and disorganization of trabecular lamellae, and posterior displacement of the aqueous plexus. In addition, the trabecular meshwork lamellae were observed only adjacent to the sclera and the inner portion of the trabecular meshwork was limited to swirls of collagen with scattered cells. These morphological findings suggest that the disease process in the rabbit principally involves an alteration in the differentiation and maintenance of the structural integrity of the trabecular meshwork. The loss of structural support of the buphthalmic trabecular meshwork may be a factor in the wide variation in intraocular pressure and may allow for compression of the trabecular meshwork against the aqueous plexus.

Animals↗

Buphthalmos in the setting of persistent hyperplastic primary vitreous cataract.

PURPOSE: To report a case of unilateral persistent hyperplastic primary vitreous cataract presenting with ipsilateral buphthalmos. DESIGN: Retrospective case report. METHODS: The patient chart was reviewed, and a relevant literature search was performed. RESULTS: A four-month-old child presented with persistent hyperplastic primary vitreous cataract in the right eye and ipsilateral buphthalmos with no discernable angle abnormalities. He underwent lensectomy, anterior vitrectomy, and limited transscleral diode cyclodestruction in the right eye. CONCLUSIONS: This appears to be the first report of buphthalmos presenting with unilateral persistent hyperplastic primary vitreous. Despite two comorbidities, the patient has thus far responded well to initial treatment and visual rehabilitation.

Anterior Eye Segment↗

Unilateral esotropia after enucleation in infancy.

Five patients developed esotropia in the remaining eye after unilateral enucleation in the first few weeks to months of life. Esotropia was associated with a face turn toward the opposite side and abduction nystagmus with a null point in extreme adduction. Our experience with these patients supports the reflexogenic theory for the development of a type of congenital-infantile esotropia. An intact globe-ocular muscle relationship, even in a blind eye, may have a stabilizing effect on the fellow eye in the first few weeks to months of life, and this should be considered before enucleation is done.

Cataract Extraction↗

Enlargement of the globe with ocular malformations in c-Myc transgenic mice.

PURPOSE: To study the ocular development in transgenic mice carrying the mouse c-myc gene under the control of the Mx gene promoter (Mx-c-myc). METHODS: Transgenic mice were generated by standard techniques. For histological studies, the tissues were fixed with 10% buffered formalin, embedded in paraffin according to the standard procedure and sliced in 4-microm sections. c-Myc expression was investigated by reverse transcriptase-polymerase chain reaction and Southern blot analysis. RESULTS: A line of the Mx-c-myc mice displayed progressive enlargement of the globe with other ocular malformations. Histologically, the enlarged eyes exhibited closed cornea-iris angle, microphakia, corneal epithelial disorders, and attenuation of the inner retinal layers. Developmental analysis of eyes from these Mx-c-myc mice revealed irregular development of the iris and ciliary body at embryonic day 15.5 and the closed angle at 1 week of age. Leaky exogenous c-myc expression was detected in cornea, iris, lens, and retina from the Mx-c-myc mice by reverse transcriptase-polymerase chain reaction and Southern blot analysis. No other developmental abnormalities were observed in the Mx-c-myc mice. The anterior segment of the enlarged eyes showed the closed angle with elongation of the iris and ciliary body. There was no attenuation in the outer retinal layers from the outer plexiform layer to the retinal pigment epithelium. CONCLUSIONS: We conclude that the buphthalmos and accompanying changes were not due to expression of the exogenous c-myc in cornea and retina but may be the secondary changes of elevated intraocular pressure. We suggest that Mx-c-myc mice can serve as a useful model for investigating the development of the anterior segment and the genesis of buphthalmos.

Abnormalities, Multiple↗

[Buphthalmia secondary to congenital pigmented epithelial iris cyst].

We report the case of a 2-day-old child with buphthalmia caused by a congenital pigmented epithelial iris cyst. The use of ultrasound biomicroscopy (UBM) and high-resolution echography (20MHz) specified the diagnosis before general anesthesia for complete examination and treatment. Surgery can control intraocular pressure and the opening of the visual axis; there is, however, relative amblyopia. This case confirms the advantage of UBM in examining tumors of the anterior segment. The early diagnosis, facilitated by use of the UBM, provided quick and well-adapted treatment.

Cysts↗

Cataract extraction and intraocular lens implantation in anterior megalophthalmos.

This report describes the implantation of a standard posterior chamber intraocular lens (IOL) in a patient with bilateral cataract and anterior megalophthalmos. After extracapsular cataract extraction, the IOL was sutured to the posterior surface of the iris and anterior capsule. Different types of IOLs were used in each eye, and the surgical technique was adapted to the characteristics of the IOL. No complications were noted. Visual rehabilitation was successful. Extracapsular cataract extraction with a posterior chamber IOL sutured to the posterior surface of the iris and anterior capsule is a useful option in patients with anterior megalophthalmos and cataract.

Adult↗

The effect of buphthalmos on orbital growth in early childhood: increased orbital soft tissue volume strongly correlates with increased orbital volume.

PURPOSE: Our purpose was to evaluate the effect of increased orbital soft tissue volume on orbital growth. METHOD: Patients with unilateral or significantly asymmetric bilateral buphthalmos as determined by axial computed tomography scan were recruited. Volumetric determinations of the bony orbit with use of axial 1.5 mm sections on computed tomography were undertaken. Statistical analysis of the paired ocular length measurement and bony orbital volume measurements for each patient were performed. RESULTS: Eight patients (mean age 41 months) with a 15% or greater difference in axial length were enrolled. The mean axial length of the buphthalmic globes was 23% greater than that of the contralateral globes. Orbits harboring a buphthalmic globe had an orbital volume 11% greater than on the contralateral side. CONCLUSION: Increased orbital soft tissue volume as evidenced by buphthalmos was significantly associated with enlarged bony orbital volume. This indicates that soft tissue volume is a determinant of orbital volume and suggests that orbital tissue expanders might enhance bony development in patients with anophthalmos or microphthalmos and after early enucleation.

Bone Development↗

Retinal detachment and giant retinal tears in aniridia.

Clinical findings in aniridia may include corneal epitheliopathy, glaucoma, lens subluxation and cataract, and hypoplasia of the fovea and optic nerve. We report the occurrence of retinal detachment due to giant tears in four eyes of three children with aniridia. All eyes were buphthalmic and none had undergone lens or posterior segment surgery. All operated eyes underwent vitreolensectomy and silicone oil injection; useful vision was restored in two eyes. The pathogenesis and management of this previously unreported complication are discussed.

Aniridia↗

Childhood blindness and low vision in Uganda.

PURPOSE: To examine the causes and outcome of subnormal vision starting in childhood in Uganda, to aid in planning for its avoidance and for rehabilitation. METHODS: All those seen having subnormal vision with onset from birth to 15 years (total 1135) (schools 1983-97; community 1988-97) were included in the study. Clinical examination was performed and a World Health Organisation (WHO) form completed, with analysis by its computer program. Main outcome measures were visual acuity, signs of eye pathology, outcome of treatment and refraction and low vision assessment. RESULTS: By WHO category 14.8% had visual impairment, 6.5% severe visual impairment, 63.2% were blind and 15.2% were too young to test. The acuities and causes were similar in school and community groups, excepting cortical visual impairment and multiple impairment, which are much commoner in the community. Cataract was the largest cause of visual impairment (30.7%) and surgical outcome was unsatisfactory. Visual loss following corneal ulceration was the second commonest cause of subnormal vision (22.0%). CONCLUSIONS: Cataract and corneal damage cause half of all subnormal vision, which is avoidable for both. Cataract surgery needs to be upgraded. To prevent corneal visual loss, primary health care should continue to be expanded, especially measles immunisation and nutrition care; rubella immunisation should be added. Special education needs to be greatly expanded. Public perceptions need changing if results are to be improved and help offered to more than the present minority.

Adolescent↗

Congenital unilateral buphthalmos in Walker-Warburg syndrome: a clinicopathological study.

BACKGROUND AND PURPOSE: Walker-Warburg syndrome is a congenital autosomal recessive oculocerebral disorder characterised by hydrocephalus, brain agyria, microphthalmos and retinal dysplasia with or without meningoencephalocele. We describe an unusual finding of congenital unilateral glaucoma and buphthalmos in one eye and microphthalmos in the fellow eye of two neonates with Walker-Warburg syndrome. PATIENTS: Two neonates with Walker-Warburg syndrome and unusual findings of buphthalmos in one eye and a microphthalmic fellow eye are presented. RESULTS: Histological examination of the buphthalmic eyes revealed the presence of mesenchymal tissue in the anterior angle covered by endothelium. No anterior chamber angle was identified in the microphthalmic fellow eye and the iris was adherent to the corneal periphery. CONCLUSIONS: Congenital buphthalmos may also appear in Walker-Warburg syndrome. The buphthalmos may result from later embryonal ocular developmental arrest than that of the microphthalmic eye.

Abnormalities, Multiple↗