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Results for “ANOPHTHALMOS”

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PubMed1985

Anophthalmos and first branchial arch defects.

The association of unilateral mandibulofacial dysostosis and anophthalmos at the same side is reported. The proband presented at the age of six months with: right anophthalmos and hypoplasia of the orbit at the same side, hypoplasia of the right mandible and maxilla, right external ear deformity, cyanotic heart disease, hemivertebrae, cataract and corneal opacity in the left eye. This case shows the maximal effect of the abnormal development of the first branchial arch (the mandible, maxilla, and ears) on the globe and the orbit. The other end of the spectrum in which the minimal effect on the globe was present (unilateral absence of choriocapillaris and retinal pigment epithelium inferiorly) was reported by Cotlier & Alghadyan in 1981. This supports the concept that abnormal development of the mandible may influence the development of the orbit and the globe. The extent of the influence of the abnormal developing first branchial arch on the eye is discussed.

Abnormalities, Multiple↗

PubMed2005

Bilateral congenital anophthalmos and agenesis of the optic pathways.

This report presents a rare example of a bilateral congenital anophthalmos and an agenesis of the optic pathways. The MR imaging studies revealed that the eyeballs, optic nerves, optic chiasm, optic tracts and optic radiation were absent. The chromosomal examination was normal. Mild mental retardation was also observed. Apart from the rarity of the anophthalmos and the total absence of the optic pathways, no etiologic reason for this pathology could be detected, which makes this case more significant.

Abnormalities, Multiple↗

PubMed2000

Regional variation in blindness in children due to microphthalmos, anophthalmos and coloboma.

BACKGROUND: The prevalence and causes of blindness in children vary widely between regions. Few epidemiological data are available on the relative importance of the major congenital anomalies of the globe (i.e., microphthalmos, anophthalmos, coloboma) as causes of blindness in children. The aim of this study was to determine the re-gional variation in the proportion of severe visual impairment and blindness due to congenital abnormalities of the globe in children in schools for the blind and in those identified through Community Based Rehabilitation programs. Other objectives were to estimate the prevalence of blindness due to major congenital abnormalities, and to investigate their etiology. METHODS: Data on the causes of blindness in children were collected between 1990 and 1998 using standard methods, definitions and reporting form in 26 countries. Children were examined in schools for the blind and in Community Based Rehabilitation programs. RESULTS: Of 7,113 children aged 3-15 years with severe visual impairment and blindness examined, 762 (10.7%) had microphthalmos, 161 (2.3%) had anophthalmos, and 96 (1.3%) had coloboma. There are large regional differences in the proportion of severe visual loss in blind school children, ranging from 1.4% in Cuba to 33.2% in Sri Lanka. Severe visual loss due to congenital abnormalities of the globe is estimated to affect between 0.4 and 16.2/100,000 children in the countries studied. An underlying cause could not be identified in 84.2%. CONCLUSIONS: Major congenital abnormalities of the globe are important causes of severe visual loss in children, particularly in Asian countries. Further research into etiology is warranted in order to plan prevention programs.

Adolescent↗

PubMed2000

Bilateral Tessier no. 4 facial cleft with left eye anophthalmos: a case report.

Craniofacial clefts are very rare and manifest in a variety of patterns. Tessier classified these clefts in 1973 and numbered them 0 to 14. Tessier No. 4 Facial cleft is a rare variant of craniofacial cleft. Not more than 50 cases are reported in world literature, amongst which only 5 cases are true bilateral in nature. However, combinations of two different variants are not very uncommon. A case of male Indian child aged 4 years with a true bilateral Tessier No. 4 Facial cleft is reported. He also had anophthalmos of the left eye. This is probably the first case in which true bilateral Tessier No. 4 Facial cleft with anophthalmos of one eye is noticed.

Anophthalmos↗

PubMed1992

Orbito-palpebral reconstruction in anophthalmos and severe congenital microphthalmos.

In patients with congenital anophthalmos and severe microphthalmos, a tiny orbit and socket exist with little eyelids, frequently preventing retention of a standard conformer or prosthesis. Socket expansion is sometimes impossible with microorbitism; the retention of a prosthesis is also difficult when malformations of the eyelids exist. The treatment of these difficult cases includes three stages. The first stage is orbital expansion that depends on the cephalometric studies of the patient: transverse osteotomy on the maxilla and the zygomatic bone with lateral bar by extracranial route, vertical osteotomy on the roof of the orbit by intracranial route. In some cases, the osteotomy includes expansion in the transverse and vertical diameter with bone grafts in the defects and on the lateral and superior rims. Simultaneously, socket expansion is performed by incision of the conjunctival sac circumferentially, with mucosal or split skin grafts on a conformer. The second stage includes eyelid reconstruction by different flaps. A third stage is frequently needed for correction of eyelid malposition on the prosthesis: ptosis, entropion surgery. Two cases of congenital anophthalmos are reported. Methods and indications of treatment are discussed.

Adolescent↗

PubMedDate not supplied

Unilateral anophthalmos and absence of right cerebral hemisphere.

A case is presented of a new eight-month-old infant with congenital anophthalmos and ipsilateral absence of cerebral tissue. Various central nervous system anomalies are described as associated with anophthalmos. Therapy is directed toward enlargement of the socket by expansion and/or surgical techniques. Early expansion therapy within the first year of life appears warranted in view of differential orbital growth rates.

Anophthalmos↗

PubMedDate not supplied

[Clinical anophthalmos].

The authors present a 14-year-old patient with congenital clinical unilateral anophthalmos, hospitalized for an conjunctival benign tumour, under ocular prosthesis. The case was included in embryopathic anophthalmos.

Adolescent↗

PubMed1993

Congenital cataract in the right eye and primary clinical anophthalmos of the left eye in a patient with cerebellar hypoplasia.

We examined a 26-year-old man who had an opaque lens OD and clinical anophthalmos OS. Low densities in the left cerebellar hemisphere and vermis and dilated ventricles were noted on a computed tomographic scan of his brain. We believe that the association of developmental cataract OD, clinical anophthalmos OS, and cerebellar hypoplasia in our patient may be rare.

Adult↗

PubMedDate not supplied

Bilateral congenital anophthalmos. Report of two cases.

Two cases of bilateral congenital anophthalmos in Nigerian children are reported. Both cases are sporadic, and belong to the primary type of anophthalmos. The first case has associated polydactyly of both hands. There is no history of ingestion of any teratogenic drugs during pregnancy in the two cases.

Anophthalmos↗

PubMed1977

Anophthalmos. Report of two cases.

Two infants were found to have bilateral anophthalmos and other congenital anomalies. Secondary anophthalmia was diagnosed in one patient who showed malformations of the brain and an absence of ocular tissues including the optic nerves. The other patient had degenerative (consecutive) anophthalmos; fragments of ocular tissues including the optic nerves were found in the orbits.

Abnormalities, Multiple↗

PubMed1979

[Influence of an unilateral anophthalmos on the ophthalmo-dynamography (author's transl)].

In 30 patients with unilateral postoperative anophthalmos, by means of the ophthalmodynamograph model ODG 22 (Boucke) a bilateral ophthalmodynamography (Hager) was performed. The systolic and diastolic pressure of the ophthalmic artery showed no significant difference between the sides without and with the eyeball. The pulsation volume was, however, on the side of anophthalmos (97.9 mm3 in average) slightly, but significantly (p less than 0.05) decreased than the opposite side (103.7 mm3).

Adolescent↗

PubMed1984

Sex-linked hereditary bilateral anophthalmos. Pathologic and radiologic correlation.

A 27-year-old man had X-linked true anophthalmos. No evidence of optic globe, nerves, or chiasm was found. Rudimentary structures suggesting optic tracts were present. Lateral geniculate nuclei were present but gliotic. Calcarine cortex was thinner but had usual lamination. The normal patches of cytochrome oxidase activity in layers II and III of visual cortex were absent.

Adult↗

PubMed1996

Intraorbital tissue expansion in the management of congenital anophthalmos.

Seven cases of intraorbital tissue expansion for the treatment of congenital anophthalmos or microphthalmos are presented. The ages of the patients at insertion of the expander ranged from 4 months to 8 years. A 4 ml spherical tissue expander with a remote injection port was inserted into the affected orbit via a bicoronal approach. Expansion periods ranged between 4 months and 3 years and are continuing in 2 patients. Results were assessed by clinical examination, comparison of photographs, 3D CT scans and orbital measurements taken from axial CT scans which were compared with established normal values. Results confirmed enlargement of the orbit with expansion. Long-term expansion over several years established near normal bony growth patterns. Placement of the expander within the orbital soft tissue cone resulted in more symmetrical expansion than subperiosteal placement. An osteotomy releasing the lateral orbital wall in older children allows expansion of the orbit and may reduce the incidence of expander extrusion. Although intraorbital tissue expansion successfully induces orbital growth, improvement in the form and size of the congenitally deficient eyelids is less marked.

Anophthalmos↗

PubMed1999

Treatment of congenital anophthalmos with self-inflating polymer expanders: a new method.

Congenital anophthalmos is a rare malformation in which the optic vesicle fails to develop. This leads to a small bony orbit, a constricted mucosal socket, short eyelids, reduced palpebral fissure and malar hypoplasia. The treatment includes both aesthetic and functional aspects. Therefore, a two-step procedure is described using a new self-inflating hydrogel expander. A lens-shaped expander with a diameter of 8 mm expands the lids and the mucosal socket to allow insertion of an eye prosthesis. As a second step, orbital expansion is performed with a spherical device. The expanders absorb lacrimal fluid from the mucosal socket or tissue fluid and start swelling when implanted in the orbital tissue. The insertion of an expander into the orbit as well as into the conjunctival pocket including its fixation by a single suture took only a few minutes and was an easy procedure. The expansion of the small conjunctival sockets was successfully completed in all cases within a period of 2-4 weeks. The weight (= volume in ml) of devices increased from 0.15-1.5 g (lens-shaped expander; weight in grams = volume in ml) respectively, 0.3-3.5 g (spherical device). The expanders inserted in orbital tissue increased from 0.4-4.4 g. This is equivalent to a 10 to 11 fold increase in their water-free volumes. Orbital expansion with spherical devices in combination with the inserted eye prosthesis enlarges the lid and palpebral fissures also. In contrast to conventional silicon balloon expanders, the procedure using self-inflating hydrogel expanders is simple and highly efficient.

Absorption↗

PubMed2000

[Clinical anophthalmos. Cosmetic outcome after 2 years therapy with an orbital expander for stimulating orbital growth].

BACKGROUND: Congenital anophthalmos is a rare condition in which intervention in an early age can stimulate orbital expansion. The therapeutic goal was to allow retention of a suitable prosthesis and to maximise facial symmetry. METHODS AND PATIENTS: We report on 3 anophthalmic newborn male patients, of which one presented with microphthalmos in the other eye as well as associated systemic disorders. Solid moulded shapes were adapted and manufactured out of methylmethacrylate to increase expansion of orbital soft tissue and bone. The moulding of the orbita was performed by silicone made of two components under general anaesthesia during the first two years of life. The following day the solid shapes were fitted in the outpatient department. They were increased in size when they started to rotate within the orbit or when the eyelids were relatively loose around the solid shape. The initial insertion of the orbital expander was performed in the first weeks after birth and was repeated every 3 to 6 weeks within the first year of life. The degree of orbital expansion was determined by measurements of the horizontal eyelid length, the volume of the solid shapes, by measuring the volume in cm3 of water displaced by the shape after submerging it in a cylinder of water and assessment of the cosmetic aspect. RESULTS: Within 8 months of therapy a horizontal eyelid lengthening to 10 mm and an increase of the volume of the orbital expander from 1.5 cm3 to 6 cm3 was achieved in patient 1. After 20 months of therapy patient 2 showed symmetry of the face and the horizontal eyelid length. In patient 3 therapy started 3 years late and after 2 years only a horizontal eyelid lengthening of 3 mm and a suboptimal symmetry of the face was achieved. CONCLUSION: Our own experience suggests that early prosthetic fittings are necessary for an ideal cosmetic outcome. Motivation and cooperation of the parents is an important factor to achieve optimal results.

Anophthalmos↗

PubMed1987

[Hema socket expander in primary conservative treatment of congenital anophthalmos].

In anophthalmos the forces which stimulate development and growth of the bony orbit are absent. As a result these cases have not only a tiny socket but also an underdeveloped bony orbit, which cannot retain an artificial eye. The small conjunctival sac and bony orbit can be mechanically enlarged by means of Hema socket expanders. This hydrophilic material can be manufactured in different forms and sizes and expands the socket and orbit by increasing in volume after hydration. After expanding the conjunctival sac to a certain size the next-larger form is fitted to the socket; the procedure is repeated until an artificial eye can be worn.

Acrylates↗

PubMed1980

[Unilateral congenital anophthalmos with Trabecular agenesia and hydrophthalmos of the other eye (author's transl)].

In a one-year-old female patient, unilateral anophthalmos was ascertained by clinical, echographic and X-ray examination. In addition, the cranial computer tomogram showed trabecular agenesia. The globe of the other eye was existent and was enlarged by hydrophthalmos. Pediatrically this case represents a syndrome with multiple anomalies of etiologically unknown origin; apart from the ocular malformation there are a severe cerebral developmental disturbance, an apallic syndrome, hypsarrhythmia, peculiar disproportional bodily structure with skeletal anomalies as well as an abnormal susceptibility to infections of the respiratory system and a marked, isolated IgA-deficiency. Neither chromosomal anomaly nor familiarity was discovered.

Anophthalmos↗

PubMed1991

A modified tissue expander for socket enlargement in clinical anophthalmos.

Early and rapid expansion of the tiny socket is desirable in clinical anophthalmos. This paper reports three cases utilizing an expandable conformer made of silicone. Socket enlargement adequate for fitting of a satisfactory cosmetic prosthesis was attained rapidly in each case. The design of the conformer allows the greatest expansion toward the fornices.

Anophthalmos↗