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PubMedDate not supplied

Eyelid switch flap technique for the management of congenital anophthalmos associated with contracted socket.

Congenital anophthalmos with contracted socket can occasionally present in the second or third decade for cosmetic correction of the deformity. Placement of ocular prosthesis is not possible due to reduced dimensions of the palpebral aperture and contracture of the bony socket. Horizontal widening of the palpebral aperture using an eyelid switch flap technique followed by placement of ocular prosthesis anterior to the hypoplastic orbit achieves good cosmetic correction of this deformity.

Adult↗
PubMed1987

Clinical anophthalmos in a family.

Monolateral or bilateral anophthalmos recurring in the absence of other associated defects in six members of a family is reported. The malformation appears to be inherited as a dominant (autosomal or X-linked) trait with incomplete penetrance. Implications for genetic counselling are briefly discussed.

Adolescent↗
PubMed1997

Bilateral clinical anophthalmos.

We report on the risk factors, associations and outcome of 5 children with bilateral clinical anophthalmos. Our study showed no gestational, environmental or hereditary association but confirmed strong association with multiple systemic abnormalities.

Abnormalities, Multiple↗
PubMed1991

X-linked clinical anophthalmos. Localization of the gene to Xq27-Xq28.

A Northern Ireland family is reported on, in which there is X-linked inheritance of clinical anophthalmos. Multi-point linkage analysis suggests that the gene is localized to the Xq27-28 region (Z = 1.9, Theta = 0.08), though not between the DNA markers DX13 and Factor VIII.

Anophthalmos↗
PubMedDate not supplied

Limb-body wall complex with anophthalmos and choroidal coloboma.

A 23-year-old primigravida underwent induced abortion in the 17th week of pregnancy because ultrasound fetometry had indicated possible encephalocele/hygroma of the skull and deformities of limbs and abdominal wall. The fetus delivered showed a deep facial cleft, absence of nose and maxilla, absence of parietal and frontal bones, missing right upper extremity, and a large thoracoabdominal defect with herniation of internal organs. The placenta was large, with an amniotic fold across the fetal surface. The brain was holoprosencephalic with a midline monoventricle and with focal brain wall dysplasia. The right orbit and eye were absent. The left eye was microphthalmic, with an abnormal cornea, a large choroidal coloboma inferiorly, a dysplastic retina, and a cataractous spherical lens. Theories discussed are pathogenesis of the limb-body wall complex (LBWC) and ocular malformations, particularly uveal coloboma. Previous cases of anophthalmos and uveal coloboma are listed. Microscopic examination of the eye was performed in only one of these. The present case is the sixth case published of choroidal coloboma in association with the LBWC.

Abnormalities, Multiple↗
PubMed1975

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↗
PubMed1992

The ocularists' management of congenital microphthalmos and anophthalmos.

Early socket stimulation is crucial for management of congenital anophthalmos and microphthalmos among infants. Progressive sized hard conformers and lid expansion devices can expand the small socket in these patients. The ocularists' management of these two conditions is discussed and techniques are introduced.

Anophthalmos↗
PubMedDate not supplied

Congenital unilateral absence of orbit with anophthalmos and partial arhinia.

Congenital unilateral absence of orbit is a very rare clinical entity. An 18 month old female child with an unilateral absence of orbit with clinical anophthalmos and partial arhinia is presented. X-ray of the skull revealed an absence of the bony orbit on the left side. Unlike previously described cases of absence of the orbit, this maldevelopment is unilateral and present in an otherwise normal thriving child.

Abnormalities, Multiple↗
PubMed1990

[Bilateral congenital anophthalmos].

The author describes the rare occurrence of congenital bilateral anophthalmos in a child with unilateral microphthalmus in the mother. The grandmother worked for a long time as an X-ray laboratory assistant without adequate protection against ionizing radiation. The cause of the development of pathological conditions in our proband and his mother is most probably a dominant "de novo" mutation in the germinal cells of the grandmother exposed to radiation. The transmission is according to our findings autosomal dominating with a variable expressivity.

Anophthalmos↗
PubMed1978

Congenital anophthalmos. Problems in management.

The management of congenital anophthalmos is difficult under the best of circumstances. Therapy is directed towards enlargement of the sorbet by expansion and/or surgical techniques. Early expansion therapy within the first year of life appears to be warranted in view of the differential orbital growth rates.

Anophthalmos↗
PubMed1993

Experimental effects of intraorbital tissue expansion on orbitomaxillary growth in anophthalmos.

A skeletal growth study evaluating an intraorbital tissue expander as a globe replacement in anophthalmos was conducted in an infant animal model. Twenty-one 21-day-old kittens were equally divided into the following three groups: a control, a unilaterally enucleated, and an enucleated-expanded group. Postoperative three-dimensional computed tomographic scans were obtained at 5 months of age immediately before study termination. Linear orbital, orbitomaxillary, and zygomaticomaxillary dimensions were measured on the scans and compared at necropsy with orbital volume assessment on the dry skulls. Control animal orbits averaged 21.5 mm in height, 21.0 mm in width, and 5.1 cm3 in orbital volume, and an orbitomaxillary-zygomaticomaxillary height-to-width ratio of 31.0: 24.5. The control side, its contralateral side, and the contralateral orbits of the treated sides were not significantly different. By study completion, enucleation resulted in a significant loss of orbital volume (mean, 1.9 cm3; p < 0.001) confirmed by deficient orbitozygomaticomaxillary measurements. The expanded orbits showed no loss of volume (mean, 5.2 cm3; p > 0.1) and normalization of all linear midfacial structures. This study demonstrates that early expansion emanating from within the orbital soft tissue positively affects both the skeletal and lid anomalies associated with congenital globe loss or early enucleation.

Animals↗
PubMedDate not supplied

Cyclopian monster with anophthalmos.

A cyclopian monster with anophthalmos is a rare occurrence. Such a case is presented along with a case of a cyclops with synophthalmos. The many abnormalities result from an anomalous development of structures derived from the prosencephalon.

Abnormalities, Severe Teratoid↗
PubMed1995

Anophthalmos-syndactyly (Waardenburg) syndrome without oligodactyly of toes.

We report on 2 brothers from a consanguineous family from a small city of southeast Turkey. Both have bilateral anophthalmia, soft tissue syndactyly of the feet, bilateral partial synostosis of metatarsals IV and V, and basal synostosis of the fourth and fifth toes on the right in the older sib only, thus differing from all previously reported cases of anophthalmos-syndactyly syndrome.

Child↗
PubMed1995

Coralline hydroxyapatite spheres as secondary orbital implants in anophthalmos.

BACKGROUND: Secondary reconstruction of the anophthalmic socket is frequently challenging. A number of surgical techniques are described to improve the cosmetic and functional outcome. Recently, the use of hydroxyapatite as a primary orbital implant after enucleation has gained popularity and yielded encouraging surgical results. There are no published reports concerning the use of hydroxyapatite as a secondary orbital implant in anophthalmos. METHODS: The authors reviewed the charts of 17 patients who received a secondary hydroxyapatite orbital implant to rehabilitate the anophthalmic socket. Each patients' preoperative and postoperative photographs, socket appearance, implant and prosthetic motility, and overall impressions of the surgical outcome were analyzed. Complications of surgery and secondary procedures were reviewed. RESULTS: All except one patient attained an improved prosthetic appearance postoperatively and 76% achieved improved implant and prosthetic motility. Four patients undergoing surgery early in the series had worsening of pre-existing ptosis. In two patients, implant exposure developed; a socket infection with subsequent loss of the implant developed in one of these patients. Eight patients underwent additional eyelid and orbital surgery at the time of secondary implant surgery, whereas nine patients required additional staged procedures. CONCLUSION: Secondary orbital implantation with the coralline hydroxyapatite sphere is an encouraging alternative to traditional secondary orbital implant surgery. Surgical outcomes in this study were excellent with few complications and high patient satisfaction.

Adolescent↗
PubMed1965

ANOPHTHALMOS.

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Anophthalmos↗