Nystagmus bei Kindern – eine Übersicht

Zusammenfassung

Nystagmus ist definiert als unwillkürliche, periodische Bewegung, bei der eines oder beide Augen vom Fixationspunkt wegdriften (sog. Drift). Auf die Drift, die langsame Phase, folgt entweder eine schnelle, refixierende Bewegung (sog. Refixationssakkade oder schnelle Phase) oder eine langsamere Bewegung zur Wiederaufnahme der Fixation. In dieser Übersichtsarbeit sollen die wichtigsten Formen von Nystagmus bei Kindern erörtert werden, für eine Übersicht bei Erwachsenen sei auf [1] verwiesen.

Abstract

Nystagmus describes an involuntary, periodic movement of one or both eyes. About 1/600 children and adolescents have nystagmus, most of them idiopathic infantile nystagmus (IIN), also called “congenital nystagmus”, which can be caused by mutations in the FRMD7 gene. Other frequent forms of nystagmus are latent nystagmus, which is usually associated with infantile strabismus, and nystagmus associated with albinism. Sometimes difficult to distinguish in young infants is a sensory nystagmus, where a defect in the visual system reduces vision and causes nystagmus. Causes include retinal dystrophies, congenital stationary night blindness and structural ocular defects including optic nerve hypoplasia or dense bilateral congenital cataracts. Unilateral nystagmus can be the sign of an anterior visual pathway lesion. Seesaw nystagmus may be associated with suprasellar and mesodiencephalic lesions and – rarely – with retinal dystrophies.

The ophthalmology plays a key role in identifying the form of nystagmus. Children with new onset nystagmus, with spasmus nutans, with vertical or unilateral nystagmus and those with seesaw nystagmus require neurologic evaluation including imaging of the brain.

The treatment of nystagmus depends on the underlying cause. Even minor refractive errors should be corrected, contact lenses offer advantages over glasses.

Gabapentin and memantine, possibly also carbonic anhydrase inhibitors, are effective in treating IIN, nystagmus in albinism and sensory nystagmus. Nevertheless, pharmacologic treatment of nystagmus is rarely used in children; the reasons are the limited effects on vision, the need for lifelong therapy, and potential side effects. Eye muscle surgery (Anderson procedure, Kestenbaum procedure) can correct a nystagmus-related anomalous head posture. The concept of “artifical divergence” of Cüppers may help to decrease nystagmus intensity in patients whose nystagmus dampens with convergence. The four-muscle-tenotomy, which involves disinsertion and reinsertion of the horizontal muscles at the original insertion of both eyes, has a proven but limited positive effect on visual acuity.

Kernaussagen

• Nystagmus beschreibt eine unwillkürliche, periodische Bewegung, bei der eines oder beide Augen vom Fixationspunkt wegdriften („Drift“). Auf die Drift, die langsame Phase, folgt entweder eine schnelle, refixierende Bewegung („Refixationssakkade“ oder schnelle Phase) oder eine langsamere Bewegung zur Wiederaufnahme der Fixation.

• Dem Augenarzt kommt die Schlüsselrolle zu, den Nystagmus richtig einzuordnen. Kinder mit neu aufgetretenem Nystagmus, mit Spasmus nutans, mit vertikalem und mit Seesaw-Nystagmus sollten neurologisch abgeklärt werden, inklusive einer Bildgebung des Kopfes.

• Die Behandlung von Menschen mit Nystagmus richtet sich nach der Grunderkrankung.

• Selbst geringe Refraktionsfehler sollten korrigiert werden, Kontaktlinsen haben in mancher Hinsicht Vorteile im Vergleich zur Brille.

• Aufgrund des begrenzten praktischen Nutzens und der potenziellen Nebenwirkungen wird ein Nystagmus selten pharmakologisch behandelt.

• Chirurgisch kann durch eine Umlagerung der Augenmuskeln (Anderson-Operation, Kestenbaum-Operation) eine nystagmusbedingte Kopfzwangshaltung, welche die Patienten zur Verbesserung der Sehfunktion einnehmen, korrigiert werden. Die „artifizielle Divergenz“ nach Cüppers kann die Intensität eines Nystagmus bei Patienten mindern, bei denen dieser durch Konvergenz beruhigt wird. Bei einer 4-Muskel-Tenotomie werden die 4 geraden horizontalen Augenmuskeln am Ansatz durchtrennt und dort wieder fixiert.

Schlüsselwörter

Nystagmus - Kind - Diagnose - Therapie - ophthalmologische Chirurgie - Kopfzwangshaltung

Key words

nystagmus - child - diagnosis - therapy - eye surgery - anomalous head posture

Publication History

Article published online:
24 February 2023

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