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At least 145 records · Page 8Linked to original sources
PubMed2005

[Microphthalmos and hypertelorism as diagnostic index in ultrasound diagnosis of Fraser syndrome].

Fraser syndrome is a rare genetic syndrome with abnormalities of the head, lungs, kidneys, and limbs. A prenatal diagnosis of FS can be done in families with risk, using foetal ultrasonography. However, a wide qualitative and quantitative variability of possible abnormalities makes the diagnosis in utero notably questionable. We present the results of foetal ultrasonography in a tertigravida, had delivered two children with FS. Signs of foetal hypertelorism and microphthalmia, both traits typical for FS, were detected based on outer and inner orbital diameters and ocular diameters in 28 and 32 weeks of pregnancy. The clinical and pathological examinations after birth confirmed the diagnosis of FS. Our observation suggests that eye anomalies may prompt the diagnosis of FS even if characteristic lung and kidney abnormalities are absent. Therefore, we propose to regularly assess eye dimensions and distance, when performing any foetal ultrasonography in families with of FS.

Abnormalities, Multiple↗
PubMedDate not supplied

[Clinical characteristics and results of microsurgical treatment of bilateral congenital cataracts associated with microphthalmos].

Examinations of 146 children (267 eyes) with bilateral congenital cataracts and microphthalmia have revealed complete microphthalmia in 66 percent of cases, anterior microphthalmia (microcornea) in 34 percent, and various solitary and multiple abnormalities of the anterior section of the eye: microcornea, ectopic pupil, no pupil, a pupil membrane, iridal hypoplasia and aplasia, iridocorneal adhesions, etc. A total of 224 cataract extractions by the aspiration irrigation method were carried out with the use of microsurgical equipment. In cases with abnormalities of the anterior segment of the eye and with some abnormalities of the posterior segment cataract extraction was coupled with reconstructive surgery (creation and center adjustment of the pupil, adhesion dissection, removal of the pupil and hyaline membranes, etc.). Good effect was achieved in 98.2 percent of cases, vision acuity improved in 9.2 percent of cases. Vision acuity of 0.05-0.3 diopters (with eyeglass correction) was achieved in 74.1 percent of cases, that of 0.1-0.3 diopters in 29.9 percent. Anatomical and functional inadequacy of the eye, abnormalities of the fundus oculi, and deprivation amblyopia are responsible for poor functional results. The results evidence a possibility of rehabilitation of children with grave congenital conditions, bilateral congenital cataracts with microphthalmia.

Cataract↗
PubMed1991

[Congenital microphthalmos. Orbital and palpebral expansion].

Ocular atrophy in prime infancy leads to lack of orbital growth. Current treatment of plastic surgery in severe deformities is unsatisfactory, whereas tissue expanding techniques using intraorbital balloon are effective on orbital cavity growth. This procedure makes use of conjunctival shells or aesthetic ocular prosthesis easier, and allows an improved reconstruction. Description of implants and surgical technique of insertion used.

Child↗