Eye retraction syndrome

Introduction to eyeball regression syndrome The disease is a horizontal rectus dyskinesia disease, with the eyeball reversing with the retreat of the eyeball, while the inward or inward deflection, the cleft palate is characterized by small and other congenital dysplasia of the eye or the whole body. The symptoms. That is, muscle, fascia dysplasia, the cause of fibrosis of the extraocular muscles and their fascia may be due to congenital dysplasia, or may be caused by post-natal muscle intrathecal hemorrhage (such as birth injury). basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: ptosis, pigmentation, congenital cataract, cleft palate

Cause of eyeball regression syndrome

Abnormal anatomy (30%):

(1) The lateral rectus muscle is replaced by an inelastic fiber band, so it cannot be rotated. In the internal rotation, the medial rectus muscle contracts and the lateral rectus muscle does not relax correspondingly, thus causing the eyeball to recede and the cleft palate to narrow.

(2) Abnormal muscle attachment point, Spicer found that in one case, the inferior rectus muscle attachment point was inward and close to the medial rectus muscle. Bahr found that one case of the rectus muscle attachment point was 12 mm from the limbus. Two patients with Duane syndrome were reported in China. The upper end of the medial rectus muscle is 4.0mm and 3.5mm, respectively, which is significantly forward; the distal rectus muscle end is 7.5mm from the limbus, which is far away from the limbus. Tracher-Callins is considered to be the fascia and eyelid. In the ligament defect between the fascia, Gobin found that 23 cases had abnormal fibrosis, abnormal muscle attachment point and internal rectus fibrosis in 67 cases of Duane syndrome, and it was considered that the difference in elasticity of the medial rectus was caused by external rotation limitation. The reason can be explained by the reasons of fibrosis. When the rectus muscle contractes, because the lateral rectus muscle is replaced by a fibrous cord, it can not be relaxed at the same time, causing the two to pull the eyeball back to the iliac crest, and the cleft palate The reduction was due to the retreat of the eyeball and the loss of support of the eyelids. However, all cases were found to have fibrotic changes during the operation, so that all cases could not be explained, and the ups and downs of the eyeball retreat could not be explained. .

(3) There is an abnormal fiber band connection between the tip of the ball and the wall of the ball, which limits the movement of the eyeball.

Abnormal peripheral nerve innervation (30%):

In recent years, electromyography (EMG) studies have suggested that abnormal innervation of the rectus muscle of the eye is the cause of all Duane's retrograde syndrome and is used to explain its overall clinical manifestations.

Breinin used EMG to study this syndrome and found that the diseased eye was dominated by the abnormality of the third nerve. When it was turned inside, the inner and outer, the upper and lower rectus muscles and the inferior oblique muscle also had nerve impulses, causing the common contraction of these muscles. The effects of the superior and inferior rectus muscles can cancel each other out. The action of the inferior oblique muscle causes the eyeball to rotate up and turn up. The explanation of the eyeball retreat is that when the internal rectus muscle contracts, the lateral rectus muscle also contracts at the same time, so that the eyeball is pulled. In the sacral retraction, the upper and lower jaws become cleft palate due to loss of support. Therefore, a series of signs of this syndrome can be explained by the abnormal innervation of the nerve (dislocation nerve supply). It was found that the EMG amplitude was only 100V when the external rectus muscle was rotated. When the diseased eye was turned, the external rectus muscle had a strong contraction discharge with a wave amplitude of 280V, indicating that when the inner rectus muscle of the third cranial nerve was excited and contracted, The lateral rectus muscle is also excited and contracted, and the inferior oblique muscle is also excited. Therefore, the eyeball is turned up. When the inferior oblique muscle is cut during treatment, the symptoms of the upturn disappeared when the diseased eye is turned, and 31 cases of type II syndrome are examined. Both the inner and outer rectus muscles have 200 The discharge of V indicates that the third cranial nerve also abnormally innervates the lateral rectus muscle, so that the external rectus muscle is also discharged when the diseased eye is rotated, and the discharge intensity is equal to that of the medial rectus muscle.

Central nervous system abnormalities (20%):

In recent years, some authors have applied electro-oculogram (EOG), nystagmus (ENG) to squint movement, tracking movement, optokinetic nystagmus and vestibular-ocular reflex in patients with ocular retraction syndrome, confirming that the eigengen may be GOURDEAU et al. Quantitative examination of eye movements in 5 patients with unilateral type I using EOG found that there were abnormal saccades in the eyes and healthy eyes, and 3 vestibular-eyes in 4 eyes. Reflex, optokinetic nystagmus has a significant asymmetry, thus suggesting an intrinsic abnormality in the cerebral nervous system.

Genetic (10%):

About 10% of patients have a family history, and their hereditary pattern is autosomal dominant. It has been reported that a 3rd generation and 5 people suffer from this disease, but most of the cases are sporadic. The literature reports the chromosomes of patients with retrograde syndrome. There are abnormalities, more of the 16 and 22 chromosomes have changed, in addition to eye abnormalities, may be associated with congenital malformations of other body systems, such as morning glory syndrome, small eyeballs, optic nerve hypoplasia, wide pupil distance and so on.

Eyeball syndrome prevention

As the specific etiology is currently unknown, the disease temporarily lacks effective preventive measures, timely detection and treatment, timely correction of strabismus, weakness, avoiding the impact of visual acuity is further a focus of prevention, while paying attention to reasonable use of the eye Pay attention to eye hygiene and avoid eye fatigue to improve immunity and avoid eye infections.

Complications of retrograde syndrome Complications, ptosis, pigmentation, congenital cataract, cleft palate

Intrinsic often combined with congenital anomalies in the eye and the whole body, the incidence of congenital anomalies is about 20 times that of the normal population. It has been reported that 30% to 50% of patients are accompanied by congenital defects of the eyes, bones, ears and nerve tissues.

1. Eye complications: gusto-lacrimal reflex or congenital crocodiletears, ptosis, valgus valgus, pigmented nevus, corneal dermoid cyst, keratoconus, small eyeball , pupils are not equal, permanent pupil residual membrane, iris heterochromia, iris defect, congenital cataract, permanent vitreous artery, optic disc displacement, myelinated nerve fibers, insufficient convergence function, nystagmus, etc.

2. Systemic complications: hearing impairment, external ear malformation, facial nerve palsy, cleft palate, malformation, abnormal spinal column, deformity of hands and feet, Golden-hal syndrome, also known as eye-ear-spinal dysplasia (conjunctival epithelioid cyst, ear with attachment , spinal dysplasia), Horner syndrome, etc.

Symptoms of eyeball regression syndrome Common symptoms Strabismus diplopia anisometropic amblyopia eyeball deflection eyeball protrusion

1. Visual function: Most patients with intrinsic vision have good visual acuity. They have good binocular monocular function in the first eye position or in the abnormal head position, and 10% to 20% of patients have different degrees of amblyopia, and 2/3 is anisometropic amblyopia, and about 7% is strabismic amblyopia. It can be seen that intrinsic amblyopia is related to anisometropia and strabismus, and only a few patients complain of diplopia.

2. Eye position deviation: The first eye position can be expressed as a positive position, an internal oblique or an external oblique, and the internal oblique is generally considered to be the most common.

3. Abnormal eye movement: The typical Duane regression syndrome is characterized by a significant or no external rotation.

Examination of eyeball regression syndrome

1. General examination: It can be seen that the eye position is skewed, the eye is turned to the obstacle or the whole eye can not be turned outwards, and there are different degrees of rapid upswing and/or down rotation, cleft palate reduction, and eyeball retreat.

2. Vision examination: Some scorpions appear weak, and a few have double vision.

3. Electroencephalogram and extraocular muscle electromyography: According to different types, electro-oculogram (EOG) and extraocular muscle electromyography (EMG) tracing will have different abnormalities.

Diagnosis and differentiation of retrograde eye syndrome

Diagnostic criteria

The diagnosis of typical Duane syndrome is not difficult. In addition to type I to II, rare cases of retrograde and reversal of the eyeball are reported. In addition, the syndrome is usually congenital, but there is also acquired congenital eye fall syndrome. The diagnostic points have the following 7 points:

1. Eye position deviation: Observe whether the first eye position has esotropia, implicit strabismus, exotropia and external strabismus, and there is no up and down deflection during internal rotation.

2. Abnormal eye movement: There is no obstacle in the internal or external rotation, and how limited is it.

3. Eyeball retreat: Check the first eye position with the eyeball protrusion meter, the eyeball protrusion degree when the internal rotation position and the external rotation position, such as the difference between the eye protrusion degree and the external protrusion when the internal rotation and the external rotation are greater than 2mm.

4. Splitting state: The cleft palate is reduced when the inner rotation is turned, and the cleft palate is large when the outer rotation is turned. If the cleft palate height or the difference between the two eyes is greater than 2 mm, the intrinsic should be considered.

5. When a single eye is sick, it should be compared with healthy eyes.

6. Pulling test: Anatomical abnormalities (such as fibrosis) can be confirmed, and confirmed if necessary during surgery.

7. EMG examination: whether it is abnormal innervation, the clinical classification of some cases does not match the EMG classification, but based on EMG classification (Table 1), in addition, the EME examination can confirm Duane syndrome and A, V And the innervation of the X phenomenon.

Differential diagnosis

The intrinsic should be identified with the following diseases.

1. External rectus paralysis: Although there is limited external rotation, there is no small reflex and cleft palate in the internal rotation. EMG examination has no abnormal innervation of the lateral rectus muscle, and the external rectus paralysis has a normal saccade speed during internal rotation. Or slightly more normal, and the eyeball regression syndrome has a different degree of slowing, the traction test is negative in the external rectus paralysis, and the eyeball back syndrome is positive.

2. The congenital absence of the nerve or the congenital hypoplasia of the external rectus muscle: in these two cases, the EMG showed no electrical activity of the lateral rectus muscle, and the retrograde rectus muscle had electrical activity when the retrograde eye syndrome was transferred.

3. Abnormal regeneration after oculomotor nerve palsy: EOG examination, rapid external sacral movement and slow internal sacral movement similar to ocular regression syndrome type II, but the latter generally have no vertical movement limitation or abnormal downward vision The eyelids are raised (pseudo Greafe sign).

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