Duane syndrome

Duane syndrome (synonyms: Stilling - Türk- Duane syndrome, congenital Retraction Syndrome ) is a congenital (congenital ) eye muscle paralysis. It was by ophthalmologists Jacob Stilling (1887 ), Siegmund Türk ( 1896) and Alexander Duane (1905 ) described as a disease. The proportion of Duane 's syndrome at all Strabismusfällen is about 1%.

Causes

The cause of a defect healing will be accepted after intrauterine damage to the abducens nerve, accompanied by a Fehlinnervation of the lateral rectus muscle by neurons of the oculomotor nerve. A hereditary occurrence is also described. The Fehlinnervation resulting in unintended adduction (ie tension of the rectus medialis) of the affected eye in the simultaneous activity of the rectus lateralis, so its ipsilateral antagonist, which leads to a kind of " reins effect ".

Symptoms

It comes with an intended movement of the affected eye toward the nose ( adduction) to the characteristic retraction ( retraction ) of the eyeball in the eye socket with secondary narrowing of the palpebral fissure. Horizontal eye movements, and the movement of the affected eye towards temples (abduction ) are, depending on the type more or less heavily restricted. The intraocular pressure can be increased. When looking straight ahead is often a squint is present, which can be compensated by an abnormal head posture in general and as binocular single vision is possible.

Special

A special feature is the wide variation of Retraktionssyndroms, inter alia, from the different relative sizes of the three possible portions of the lateral rectus arise, namely that of the abducens normally innervated by the ulnar area of the area that will fail innervated by the oculomotor nerve, and not innervated fibrotic area. In addition, the clinical picture is determined by which Okulomotoriusfasern involved in Fehlinnervation the lateral rectus muscle. For this purpose are in the question that the medial rectus belonged (which is well described as the most common case ) normally, or those who for the innervation of the vertical engines ( superior rectus, inferior rectus, inferior oblique ) were intended. In addition, special features arise depending on whether low-or high-threshold neurons are grown.

Diagnostics

The strabologische test has been done in an eye clinic or relevant department ( Orthoptics ), and it contains accurate Motilitätsanalysen, dual image schemata, quantification of any head postures and differential diagnostic boundaries, such as a sixth nerve palsy. In addition, usually a electromyography is to be performed.

Classification

Duane syndrome which due to its etiology to the group of " congenital cranial Fehlinnervations syndrome ( Congenital Cranial Dysinnervation Disorders - CCDD ).

  • Type I Marked limitation of abduction (no movement about the center line available)
  • Mild limitation of adduction
  • Slight esotropia in primary gaze
  • Moderate retraction and Lidspaltenverengung with increasing test adduction
  • Type II Low restriction of abduction
  • Marked limitation of adduction
  • Significant retraction upon adduction and Lidspaltenverengung
  • Raising or lowering of the eye in adduction possible
  • Type III Marked restriction of both adduction and abduction
  • Retraction occurs with no specific adduction

Type I is much more common than Type II and Type III.

Therapy

Therapeutically may come into question strabismus surgery. The aim here is to improve the horizontal eye movements, as well as an enlargement of the field binocular single vision with reduction of any existing head posture. In general, this carefully dosed rear bearings corresponding muscles ( displacement of the muscle approach backwards in Muskelzugrichtung ) the method of resection ( shortening of the tendon or muscle while maintaining the original muscle approach ) should be employed so as not to increase by as increased tension retraction. Existence of variations in height, is usually taken of interventions on the vertical motors distance, as the cause of this in one, caused by the high voltage, slipping the horizontal engines is to look around the equator. The inter-muscular membrane here is no longer able to keep the muscles in the zero-degree meridian. Tension -reducing wide rear bearings and possibly the implementation of a so-called posterior fixation may offer here.

For some time, the transposition of the straight vertical engines, known as the VRT (Vertical rectus transposition) is used as an operational measure, in some cases, Duane syndrome. The principle is known as Hummelsheim operation already in the surgical treatment of paralysis of the abducens nerve (VI cranial nerve). If this operation is performed early on, a significant improvement of monocular excursion capability of the affected eye is possible.

Jakob Stilling Antagonist (muscle) Torticollis Digital Object Identifier
248258
de