Abstract
We report on a male patient with exophthalmos of unclear etiology, basilar impression, syringohydromyelia and type II Arnold-Chiari malformation. Two diseases involving the orbital region were to be considered in differential diagnostic terms: endocrine orbitopathy and osseous orbit dysplasia. The typical physical appearance associated with basilar impression as well as suppurative keratitis in Lagophthalmos was striking. Tetraspasticity with pareses, bulbar symptoms, proximally pronounced muscular atrophy as well as a left hemihypesthesia was shown neurologically. Although the orbit CT was normal, sonography revealed thickened ocular muscles. There was euthyroidism in diffuse goiter with negative thyroid autoantibody findings. Because of lack of definitive detection of muscular swelling, tumor, or vascular anomaly in the various images, orbital dysplasia in the context of a malformation syndrome affecting several organs is suggested as the cause of the exophthalmos.
Similar content being viewed by others
References
Char DH: Thyroid Eye Disease. Williams and Wilkins, Baltimore—London—Los Angeles—Sidney 1985.
Jones Is, Fa Jakobiec: Diseases of the orbit. Haper and Row, New York—San Francisco—London 1979.
Kahaly G, J Beyer: Die Differentialdiagnose der endokrinen Ophthalmopathie. In:Beyer J, G Kahaly (ed): Endokrine Ophthalmopathie. PMI-Verlag, Frankfurt 1987.
Klaus, E: Die basilare Impression. Hirzel, Leipzig 1969.
Klaus E: Die familiäre Form der basilaren Impression. Schw Arch Neur Neuroch Psychiat 104 (1969) 31–42.
Lieb W: Opthalmologische Diagnostik der endokrinen Ophthalmopathie. In:Beyer J, G Kahaly (ed): Endokrine Ophthalmopathie. PMI-Verlag, Frankfurt 1987.
Mummenthaler M, M Eichenberger: Skelettmißbildungen am craniocervikalen Übergang. Schweiz Med Wochenschr 93 (1963) 1830–1836.
Pfeifer G: Angeborene Fehlbildung des Gesichtes, der Kiefer und der Mundhöhle. In:Opitz H, FR Schmid (ed): Handbuch der Kinderheilkunde. Bd 9 Springer, Berlin 1968.
Reinwein D, CR Pickardt, O Fischedick, K Schürmann, K Ullerich: Therapie der endokrinen Ophthalmopathie. Dtsch med Wschr 104 (1979) 792–794.
Schürmann K, D Voth: Die Bedeutung der transfrontalen Orbitotomie für die operative Behandlung der intraorbitalen raumfordernden Prozesse. Fortschr Augenheilk 25 (1972) 188.
Uhlenbrock D, HJ Fischer: Computertomographie bei endokriner Ophthalmopathie mit malignem Exophthalmus. Dtsch med Wschr 110 (1985) 495–499.
Ullerich K, O Fischedick: Endokrine Ophthalmopathie. Klinische Symptomatik, Klassifikation, diagnostische Einordnung, Behandlungsindikation. Röntgen-Bl 29 (1976) 331.
Waller RR, DH Jacobson: Endocrine ophthalmopathy: Differential diagnosis. In:Gorman CA (ed): The eye and orbit in thyroid disease. Raven Press, New York 1984.
Wende S, E Kazner, TH Grumme: The diagnostic value of computed tomography in orbital diseases. A cooperative study of 520 cases. Neurosurg Rev 3 (1980) 43–49.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kahaly, G., Müller-Forell, W., Tettenborn, B. et al. Exophtalmos and basilar impression. A contribution to differential diagnosis of endocrine orbitopathy. Neurosurg. Rev. 11, 287–292 (1988). https://doi.org/10.1007/BF01741424
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01741424