A modification of the correction of enophthalmos. Association with anophthalmos by implantation of connected siliconized rubber beads.
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Four children with severe congenital eye anomalies are described of which three had related symptoms. Two had bilateral anophthalmia, the optic nerves not detectable by computed cranial tomography and magnetic resonance imaging, and the third child had bilateral microphthalmia and coloboma iridis. The fourth patient had bilateral cryptophthalmia as part of Fraser syndrome. All four patients were of small stature. In three of them growth hormone deficiency was demonstrated which was of hypothalamic origin as shown by growth hormone releasing hormone tests. In the fourth child hypogonadotropic hypogonadism and tertiary thyroid deficiency were diagnosed which responded well to thyroxine treatment. Pathogenetically the described disorders are due to congenital defects of midline structures as a common "developmental field".
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PURPOSE OF REVIEW: Rehabilitation of the congenitally anophthalmic orbit is frustrating to both the parents and physician. Traditional methods involve using progressively enlarging static acrylic conformers to expand the conjunctival socket, followed by placement of conventional static spherical orbital implants, dermis-fat grafts, or inflatable balloon expanders for orbital enlargement. Limitations of these methods typically result in less-than-optimal cosmetic outcomes, with retardation of bony orbital and overlying soft tissue growth adversely affecting midfacial growth and symmetry. Recent advances in tissue expansion technology may offer additional, novel alternatives to conventional therapies. RECENT FINDINGS: Hydrogel tissue expanders were recently adapted for use in congenital anophthalmia. The expanders are placed in their dry, contracted states, and expand gradually to their full size via osmosis of surrounding tissue fluid, with up to a 10-fold increase in volume. Offering the benefit of predictable and controllable self-expansion, hydrogel expanders may offer yet another alternative or adjunctive therapy to the early rehabilitation of the contracted socket. Separate appliances are used for conjunctival and orbital reconstruction. Initial results appear promising. Tempering the enthusiasm for their use, however-particularly in terms of implanted orbital expanders-is the recent spate of long-term complications reported from previous uses of hydrogels as scleral buckling material. SUMMARY: Self-expanding hydrogel tissue expanders appear to offer an intriguing reconstructive alternative to the frustrating condition of congenital anophthalmia. Long-term safety of the material as an orbital implant has not yet been demonstrated, but early results are promising.
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