Anophthalmos is a complete absence of the tissues of the eye. Embryologically, anophthalmos denotes those cases wherein there is complete failure in the outgrowth of the primary optic vesicle. When essential structures of the eye are present, the aonormality is microphthalmos, no matter how small the eye may be. Clinical anophthalmos represents the majority of cases in which the eye appears to be absent. Congenital anophthalmos is one of the rare ophthalmic anomalies, only 5 cases were reported in Korea and about 100 cases in the world. We report this case with a brief review of literatures.
Anophthalmos* ; Korea ; Microphthalmos

Congenital anophthalmos is fairly rare malformation in which the eyeball is apparently absent. We experienced a 10-day-old boy, who had a congenital anophthalmos of his left eye, a microphthalmos of the other eye and short palpebral fissure of both eyes(right: 12mm, left: 5mm). The absence of left eyeball within a small orbit was confirmed by C-T scanning. We report this interesting case with a brief review of literatures.
Anophthalmos* ; Humans ; Male ; Microphthalmos ; Orbit

PURPOSE: We evaluated the morphologic change of the hydroxyapatite(HA)-coated porous alumina as a new orbital implant when it was used in rabbit. METHODS: After evisceration of 18 eyes of 18 New Zealand white rabbits, we implanted 12 mm-diameter-sized HA-coated porous alumina with different pore sizes; 300, 500, and 800 micrometer, and then the implants were extracted back to evaluate their morphologic change according to different time interval; 2, 4, and 12 weeks. RESULTS: Fibrovascularization was noted at periphery of the implant 2 weeks after implantation in all of the three groups, and to the center of the implant 4 and 12 weeks after implantation. This fibrova-scularization was most predominant in 500 micrometerpore-sized group compared to the rest two groups. CONCLUSIONS: HA-coated porous alumina showed similar appearance of fibrovascularization with relatively more affordable price and easy manufacture, compared to the other porous implants.
Aluminum Oxide* ; Anophthalmos ; Durapatite ; Orbit* ; Orbital Implants* ; Rabbits

PURPOSE: To evaluate the effect on the growth rate of orbital volume of those who received enucleation or evisceration with or without implants, according to different sizes and operation periods. METHODS: The subjects comprised 47 patients who had previously undergone evisceration or enucleation. The volumes of both anophthalmic and normal orbit were measured from CT scans of patients and compared. The relationship between the orbital volume growth rate and the operation at young age with different size implants or operation without implants was evaluated. RESULTS: Five groups, divided by age at operation, were evaluated. Four groups comprising under 12 years of age, had a significant delay in orbital volume growth development (p<0.05). Three groups, divided by size of implants, were evaluated and 17 mm or smaller sized orbital implants showed significant orbital volume growth retardation (p<0.05) CONCLUSIONS: The effect of early enucleation on orbital volume growth should be considered in patients younger than 12 years old. Furthermore, during the operation, implants with sizes larger than 18mm should be used to prevent the retardation of orbital volume growth.
Anophthalmos ; Child ; Humans ; Orbit* ; Orbital Implants ; Tomography, X-Ray Computed

Anophthalmos associated with optic nerve aplasia in the contralateral eye is extremely rare. To our knowledge, this is the first case report in Korea. A 3-day-old girl had anophthalmos in the right eye and optic nerve aplasia in the left. Except for the ocular findings, she was relatively healthy. Her sibling had also anophthalmos and expired 4 days after birth. But we could not take her siblings history in detail. We report this case with a brief review of literatures.
Anophthalmos* ; Female ; Humans ; Korea ; Optic Nerve* ; Parturition ; Siblings

Authors observed clinically for congenital anophthalmos in two cases. One case was a boy, aged 6 years, having congenital anophthalmos of right eye with bifid uvula and another case was newbron baby, aged 4 days, having congenital anophthalmos and blepharophimosis of left eye and was born to undernutciented mother from chronic hepatitis.
Anophthalmos* ; Blepharophimosis ; Hepatitis, Chronic ; Humans ; Male ; Mothers ; Uvula

INTRODUCTION: Congenital anophthalmos is a very rare malformation. In terms of embryology, congenital anophthalmos is caused by the lack of developing of eye bulb that leads to the absence of vital component of eyeball. In Korea, about 5 cases have been reported. CASE: It was a female newborn who had congenital anophthalmos in the left eye with cleft palate associated with the marks of an eyelid with stricture and sinking in left orbit. We have observed conduction defects on visual evoked potential(VEP) pathways while the right eye responded properly on the photosensitivity test, and showed normal state on the chromosome test. RESULT: We experienced a case of congenital anophthalmos with cleft palate. But we have not found the cause through systemic laboratory or chromosomal study results, the parent's past medical history.
Anophthalmos* ; Cleft Palate* ; Constriction, Pathologic ; Embryology ; Eyelids ; Female ; Humans ; Infant, Newborn ; Korea ; Orbit

PURPOSE: We describe our experience with the Permacol graft in anophthalmic socket reconstruction, and compare it to the autologous buccal mucosal graft, emphasizing the postoperative vascularization and contraction of each graft. METHODS: This was a retrospective comparative study. We measured the time necessary for the graft surface to be completely vascularized, as well as the fornix depth of the conjunctival sac in anophthalmic patients. RESULTS: Ten patients underwent Permacol graft reconstruction, with 44 undergoing buccal mucosal graft reconstruction. Seven eyelids (70%) in the Permacol group had a good outcome, with improvement in lower eyelid position and prosthesis retention. Nine out of 10 eyelids (90%) in this group showed complete vascularization of the graft at 2.6 ± 1.9 months postoperatively, while the grafted buccal mucosa was fully vascularized at 1.1 ± 0.3 months postoperatively (p < 0.01). Postoperative fornix depth in the Permacol group was 9.1 ± 2.2 mm, compared to 14.9 ± 4.5 mm in the buccal mucosal graft group (p < 0.01). Mean increases in fornix depth were 33.1% and 67.9% of the mean vertical length of the implanted graft. CONCLUSIONS: The Permacol graft can be useful as spacer graft material in anophthalmic socket patients. It takes longer to vascularize, and undergoes greater graft shrinkage with time, compared to the buccal mucosal graft.
Acellular Dermis* ; Anophthalmos ; Eyelids ; Humans ; Lacrimal Apparatus ; Mouth Mucosa* ; Prosthesis Retention ; Retrospective Studies ; Transplants*

PURPOSE: We report a case of orbital pseudotumor developed in an anophthalmic socket, presenting no typical symptoms or signs. METHODS: A 67-year-old woman was referred for treatment of necrotizing scleritis of her left eye and painful orbital pseudotumor of her right eye. The right eye had been removed 10 years previously. There was an irregular mass in superomedial portion of anterior orbit. Orbital MRI showed poorly defined T1 iso and low T2 signal intensity of a lesion in the medial anterior portion of the right orbit. Because systemic steroid administration was limited, 2 mg of betamethasone was injected locally, after which the size of the lesion was decreased and the orbital pain improved. CONCLUSIONS: The diagnosis of an orbital pseudotumor developed in an anophthalmic socket may be difficult because of the lack of typical eyeball-related signs or symptoms such as proptosis, red eye, or decreased vision. But, the easy access to the lesion can provide early and effective treatment through direct injection of glucocorticoid.
Aged ; Anophthalmos* ; Betamethasone ; Diagnosis ; Exophthalmos ; Female ; Humans ; Inflammation* ; Magnetic Resonance Imaging ; Orbit* ; Orbital Pseudotumor ; Scleritis

PURPOSE: To evaluate the effects of X-irradiation on prenatal deaths, i.e., preimplantation deaths, embryonic deaths, and fetal deaths, and on external malformations in precompacted preimplantation ddy mice. MATERIALS AND METHODS: Pregnant mice (n=85), obtained by limiting the mating time to from 6 to 9 A.M., were segregated into 11 groups. The first five groups (n=26) were irradiated with X-ray doses of 0.1, 0.5, 0.75, 1.5, and 3 Gy, respectively, at 24 h post conception (p.c.) of the preimplantation period. The second five (n=27) groups were irradiated at the same X-ray doses, respectively, but at 48 h p.c. of the preimplantation period. The last group (n=32) was the control group. The uterine contents were examined on the 18th day of gestation for prenatal deaths and external malformations. RESULTS: 1) A statistically significant increase in preimplantation deaths with increasing dose was observed in the experimental groups irradiated at 24 h p.c. and in the groups irradiated at 48 h p.c., as compared to the control group. The threshold dose was close to 0.05 Gy and 0.075 Gy for the irradiations at 24 h p.c. and 48 h p.c. respectively.2) A statistically significant increase in embryonic deaths with increasing dose was observed in all irradiation groups, except the group irradiated with a dose of 0.1 Gy at 48 h p.c..3) No fetal deaths were found in any experimental group.4) In the experimental groups irradiated at 24 h p.c., anomalies increased with statistical significance, as compared with the control group: 2 exencephalies, 2 open eyelids, 3 anophthalmias, 2 cleft palates, 2 gastroschisis, 1abdominal wall defect, 1 leg defect, and 2 short tail anomalies; the threshold dose for external malformations was close to 0.2 Gy at 24 h p.c.. In the groups irradiated at 48 h p.c., 1 open eyelid and 2 short tail anomalies were observed, but there was no statistical significance in those malformations. CONCLUSION: The results of this study reveal that X-irradiation of precompacted preimplantation ddy mice causes not only preimplantation deaths and embryonic deaths but also external malformations. In addition, external malformations were observed in our experiments at diagnostic doses, including 0.1 and 0.5 Gy. For this reason, we recommend that irradiation should be avoided during the preimplantation period by applying Rugh's 10-day rule.
Animals ; Anophthalmos ; Cleft Palate ; Eyelids ; Fertilization ; Fetal Death ; Gastroschisis ; Leg ; Mice* ; Neural Tube Defects ; Pregnancy ; Tail