Zur operativen Optikusdekompression bei Marmorknochenkrankheit (Albers-Schönbergsche Krankheit)

Abstract

This contribution describes the performance of a transethmoidal decompression of the optic nerve in a 5 1/2-year-old boy with marble bones (osteopetrosis, Albers-Schönberg disease). As a result of the operation the function of the eye still with vision was maintained in an unvarying good state over a follow-up period of some 5 years. In the literature only one other case of this diagnosis has been reported in which such an operation was successfully performed, though in that case with craniotomy. The procedure reported here was the first in which the transethmoidal approach was used, which puts less strain on the patient. In addition to the description of the case the problems involved are discussed.

Optic nerve decompression in osteopetrosis

“…2'29 Successful optic nerve decompression with resulting visual stabilization or improvement is reported rarely. 10,14,22,27,32 Recently, retinal degeneration has been described in cases of osteopetrosis and is hypothesized to be the etiology of the visual loss.~5,2~ These reports thus challenge the theory of optic nerve compression and the value of surgical decompression. We are reporting our experience with six patients suffering from osteopetrosis and severe loss of vision.…”