Duane Syndrome
| Classification according to ICD-10 | |
|---|---|
| H50.8 | Other specified strabismus - Stilling-Türk-Duane syndrome |
| ICD-10 online (WHO version 2019) | |
The Duane syndrome (Synonyms: Retraktionssyndrom by Stilling-Türk-Duane , Stilling-Türk-Duane syndrome , congenital Retraktionssyndrom ) is a congenital (congenital) eye muscle paralysis . It was described as a clinical picture by the ophthalmologists Jakob Stilling (1887), Siegmund Türk (1896) and Alexander Duane (1905). Duane syndrome accounts for around 1% of all strabismus cases.
causes
The cause is assumed to be congenital damage to the abducens nerve , accompanied by a misinervation of the lateral rectus muscle by neurons of the oculomotor nerve . A hereditary occurrence is also described. In the event of intended adduction (tensioning of the medial rectus muscle ) of the affected eye, the incorrect innervation leads to simultaneous activity of the lateral rectus muscle , that is, its ipsilateral antagonist , which leads to a kind of “rein effect”.
Symptoms
It comes at an intended motion of the affected eye towards the nose (adduction) to the characteristic retraction ( retraction ) of the eyeball in the orbit with secondary constriction of the palpebral fissure. Horizontal eye movements, including movement of the affected eye towards the temple (abduction), are more or less severe, depending on the type. The intraocular pressure may be increased. When looking straight ahead, there is often a squint position , which can usually be balanced by a forced head posture and thus binocular simple vision is possible.
particularities
A special feature is the wide range of variation of the retraction syndrome, which results from the different size ratio of the three possible parts of the lateral rectus muscle, namely the area normally innervated by the abducens nerve, the area that is incorrectly innervated by the oculomotor nerve, and the fibrotic area not innervated at all. In addition, the clinical picture is determined by which ocular motor fibers are involved in the malinervation of the lateral rectus muscle. For this purpose, those that usually belong to the M. rectus medialis (which is probably described as the most common case), or those that are responsible for the innervation of one of the vertical motors ( M. rectus superior , M. rectus inferior , M. obliquus inferior ) come into question ) were intended. In addition, there are special features depending on whether low-threshold or high-threshold neurons have grown in.
Diagnosis
The strabological diagnosis is carried out in an eye clinic or a corresponding specialist department ( orthoptics ) and includes, among other things, precise motility analyzes, double-image schemes, quantification of any head postures and differential diagnostic delimitations, e.g. B. a palsy of the abdomen . In addition, electromyography will usually be performed.
Classification
Due to its aetiology, Duane syndrome belongs to the group of congenital cranial misinnervation syndromes ( Congenital Cranial Dysinnervation Disorders - CCDD).
- Type I.
- Pronounced restriction of abduction (no movement over the midline possible)
- slight restriction of adduction
- slight internal squint when looking straight ahead
- moderate retraction and narrowing of the eyelid gap with increasing attempts at adduction
- Type II
- Little restriction in abduction
- Pronounced limitation of adduction
- significant retraction and narrowing of the eyelid gap with adduction
- Raising or lowering of the eye in adduction is possible
- Type III
- Pronounced restriction of both adduction and abduction
- Retraction takes place without any discernible adduction movement
Type I is much more common than Type II and Type III.
therapy
A squint operation can be considered therapeutically . The aim here is to shift the field of binocular simple vision in the direction of the primary position (head straight posture) with a reduction in the existing forced head posture. In general, carefully dosed repositioning of the corresponding muscles (shifting the muscle attachment backwards in the direction of muscle pull ) is preferred to a resection (shortening the tendon or muscle while maintaining the original muscle attachment) so as not to increase the retraction due to the increased tension. If there are deviations in height, interventions on the vertical motors are usually refrained from, as the reason for this is the fact that the horizontal motors slide off in the area of the equator due to the high voltage. The intermuscular membrane is also no longer able to keep the muscles in the zero-degree meridian. Tension- reducing , wide backward bearings and, if necessary, the implementation of a so-called thread operation can be useful here, but the latter only if a large, disturbing vertical deviation remains with normal backward positioning of the horizontal motors.
For some time now, the transposition of straight vertical motors, the so-called VRT (Vertical Rectus Transposition), has also been used as an operative measure in some cases of Duane Syndrome in the USA. The principle has long been known as the Hummelsheim operation in the surgical treatment of a paralysis of the abducens nerve (VI. Cranial nerve) . Such interventions were already carried out in Belgium in the 1970s, but led to significantly unsatisfactory results, including in the form of secondary vertical deviations, which is why muscle transpositions are not carried out in this country for this indication and are considered contraindicated.
See also
Web links
literature
- Herbert Kaufmann (Ed.): Strabismus. 4th fundamentally revised and expanded edition. Georg Thieme Verlag, Stuttgart / New York 2012, ISBN 978-3-13-129724-2 .
- RA Mehendale, LR Dagi, C. Wu, D. Ledoux, S. Johnston, DG Hunter: Superior rectus transposition and medial rectus recession for Duane syndrome and sixth nerve palsy. In: Archives of ophthalmology (Chicago, Ill.: 1960). Volume 130, number 2, February 2012, pp. 195-201, doi: 10.1001 / archophthalmol.2011.384 , PMID 22332212 , PMC 3753366 (free full text).
- Arthur L. Rosenbaum: The efficacy of rectus muscle transposition surgery in esotropic Duane syndrome and VI nerve palsy. In: Journal of American Association for Pediatric Ophthalmology and Strabismus. Volume 8, No. 5, October 2004, ISSN 1091-8531 , pp. 409-419, doi: 10.1016 / j.jaapos.2004.07.006 .
