[Orbito-palpebral reconstruction in severe congenital anophthalmos and microphthalmos].
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The authors analyze 8 cases of congenital anophthalmia (CA). In all the cases this condition has been a component of multiple congenital developmental abnormalities; in 4 cases the defect has been nosologically diagnosed. Developmental defects of the central nervous system have been revealed in all the cases. Histologic studies have made it possible to single out, with due consideration for the normal embryogenesis of the eye, the following variants of CA: (1) complete anophthalmia, (2) partial anophthalmia--(a) purely neuroectodermal, (b) mixed mesodermal/ectodermal, (3) anophthalmia with cysts, (4) anophthalmia with intracranial synophthalmus. The possible mechanisms of teratogenesis acting in every case of CA are discussed.
Both congenital anophthalmia and severe microphthalmia lead to micro-orbit with early appearance of craniofacial hemiatrophy. There is simultaneous lack of development of the lids (which remain small but complete) and of the conjunctival sac. Use of esthetic ocular prosthesis is thus impossible. The conjunctival sac can be dilated with conformators of increasing size but this technique has no effect on the orbit. The results of repeated plastic surgery are unsatisfactory. Stimulation of ocular growth by an expandable prosthesis in early infancy would appear to be the most logical and effective therapy. The authors propose a "new" technique for the expansion of the orbital cavity of the congenital anophthalmia. They have deviced a silicone prosthesis which is progressively expanded in size. The device has now been in use for over eighteen months with really encouraging results (17 cases).
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