Zusammenfassung
Hintergrund: Eine Amyloidose wird durch fehlgefaltete Proteine verursacht. Diese Proteine lagern sich in Geweben und Organen ab und können dadurch Funktionen beeinträchtigen. Material und Methoden: Es werden 2 Patienten vorgestellt und eine Übersicht über die bislang in der Literatur veröffentlichten rein orbitalen lokalisierten Fälle dargestellt. Ergebnisse: Der erste Fall ist eine 50-jährige Patientin mit einer seit einem halben Jahr zunehmender Ptosis, einem seit 3 Monaten zunehmenden Exophthalmus und einer Motilitätseinschränkung mit Bulbusdeviation des linken Auges. Der zweite Fall ist ein 68-jähriger Patient mit einer seit 4 Jahren zunehmenden Ptosis und Beteiligung der subtarsalen Bindehaut des rechten Auges. Intraoperativ zeigte sich makroskopisch jeweils ein gelb-bräunliches gallertartiges, leicht bröckliges Material. Schlussfolgerung: Der histologische Nachweis der Amyloidse gelingt mit der Polarisationslichtmikroskopie nach Anfärbung mit Kongorot, die bei Verdacht immer durchgeführt werden sollte. Da im Rahmen einer systemischen Form der Amyloidose die Orbita ebenfalls betroffen sein kann, sollte dies internistisch ausgeschlossen werden. Die lokalisierte orbitale Amyloidose hat im Gegensatz zu den systemischen Formen keinen Einfluss auf die Überlebenszeit. Eine unbehandelte systemische Amyloidose kann jedoch teilweise eine Prognose von nur 9 – 13 Monaten haben.
Abstract
Background: Amyloidosis is a disorder caused by a misfoulding of proteins. The deposition of these proteins in tissues and organs can affect the normal function of those tissues and organs. Materials and Methods: Two patients are presented and an overview over the so far published cases with a localised orbital amyloidosis is given. Results: The first case is a 50-year-old woman with progressive ptosis since half a year, progressive proptosis since three months and deterioration of motility and deviation of the left globe. The second case is a 68-year-old man with progressive ptosis since four years and with affection of the subtarsal conjunctiva of the right eye. Macroscopically a yellow-brown, gelatinous, easily crumbled material was seen during operation. Conclusion: the histological proof of amyloidosis can be made visually in intense unidirectional polarised light after congo red staining. This should be done in suspected cases every time. The orbita can also be involved in systemic forms of amyloidosis, so a systemic form should be excluded. The localised amyloidosis has no effect on the survival time in contrast to the systemic forms does have an effect. An untreated systemic form may be associuated with a prognosis of only 9 to 13 months.
Schlüsselwörter
orbitale Amyloidose - Exophthalmus - Lidschwellung - Ptosis
Key words
orbital amyloidosis - proptosis - lid swelling - ptosis
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Dr. Sabine Naxer
Augenheilkunde; Bereich Strabologie, Neuroophthalmologie und okuloplastische Chirurgie, UMG Göttingen
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