A randomised comparison of bilateral recession versus unilateral recession-resection as surgery for infantile esotropia.

OBJECTIVE: Infantile esotropia, a common form of strabismus, is treated either by bilateral recession (BR) or by unilateral recession-resection (RR). Differences in degree of alignment achieved by these two procedures have not previously been examined in a randomised controlled trial. DESIGN: Controlled, randomised multicentre trial. SETTING: 12 university clinics. PARTICIPANTS AND INTERVENTION: 124 patients were randomly assigned to either BR or RR. Standardised protocol prescribed that the total relocation of the muscles, in millimetres, was calculated by dividing the preoperative latent angle of strabismus at distance, in degrees, by 1.6. MAIN OUTCOME MEASURE: Alignment assessed as the variation of the postoperative angle of strabismus during alternating cover. RESULTS: The mean preoperative latent angle of strabismus at distance fixation was +17.2 degrees (SD 4.4) for BR and +17.5 degrees (4.0) for RR. The mean postoperative angle of strabismus at distance was +2.3 degrees (5.1) for BR and +2.9 degrees (3.5) for RR (p = 0.46 for reduction in the angle and p = 0.22 for the within-group variation). The mean reduction in the angle of strabismus was 1.41 degrees (0.45) per millimetre of muscle relocation for RR and 1.47 (0.50) for BR (p = 0.50 for reduction in the angle). Alignment was associated with postoperative binocular vision (p = 0.001) in both groups. CONCLUSIONS: No statistically significant difference was found between BR and RR as surgery for infantile esotropia.

[Ocular neuromyotonia after radiation therapy]. / Okuläre Neuromyotonie nach Strahlentherapie.

Ocular neuromyotonia is a rare disease that is diagnosed mainly in patients treated with radiation. All such patients described in the literature presented with temporary diplopic images as a common symptom. In our case, the patient described an abducens paresis of the right eye combined with a sporadic exotropia half a year after radiation treatment of an epipharynx carcinoma. An adduction deficit on the right side could be triggered by holding the gaze to the right over a longer period of time, leading to exotropia in the primary position and gaze to the left. Symptoms were reduced with carbamazepine.

[Choroidal effusion syndrome after embolization of an indirect cavernous sinus fistula via the superior ophthalmic vein]. / Chorioidales Effusionssyndrom nach Embolisation einer indirekten Sinus-cavernosus-Fistel über die V. ophthalmica superior.

Embolization of a cavernous sinus fistula (SCF) via the superior ophthalmic vein (SOV) was reported to be an almost uncomplicated procedure, even after ligature of the vein at the end of the procedure. We report about a complication of this therapy. A 58-year-old female had a successful embolization of a right indirect cavernous sinus fistula via the SOV. At the end of the operation the SOV was ligated because of the danger of bleeding. Directly after surgery she experienced general worsening of the right eye with signs of venous congestion and marked effusion syndrome. The patient underwent total heparinization to achieve an opening of venous collaterals. Under local therapy with atropine 1% eye drops a decrease of the intraocular pressure was observed. The effusion syndrome was completely resolved within 1 month. If embolization of a cavernous sinus fistula is performed via the SOV, the ligature of the vein at the end of the procedure leads to thrombosis, which can reduce the venous stream from the eye and orbit. A secondary effusion syndrome with ocular hypertension because of a ciliolenticular block situation is possible and requires appropriate therapy. It is not possible to assess the capacity and time of opening of the venous collateral system before surgery. Therefore a transient outflow disturbance should be considered.

[Is congenital superior oblique strabismus a paretic disorder?--A magnetic resonance tomographic study]. / Ist der einseitige Strabismus sursoadductorius eine paretische Störung? -- Kernspintomographische Untersuchungen.

BACKGROUND: Recent reports postulate that the concomitant vertical deviation found in congenital superior oblique palsy is due to mechanical abnormalities rather than a congenitally paretic muscle, and is overcome in most patients by fusion. On the basis of the clinical characteristics alone a primary paresis is indeed unlikely. Although intraoperatively a different elasticity of the superior oblique tendon exists in congenital versus acquired cases of superior oblique palsy, preoperatively performed MR imaging shows that the clinical findings in congenital superior oblique muscle malfunction could nevertheless be of paretic origin. MATERIALS AND METHODS: Seventeen consecutive patients (males: n = 13; females: n = 4) were examined. The vertical deviation in adduction was concomitant in vertical versions, the excyclotropia was small and concomitant in all directions of gaze and was less than 10 degrees even after diagnostic occlusion. All patients showed a positive Bielschowsky head tilt phenomenon and large fusional ability. We performed preoperative MR imaging of both orbits in high resolution 3 mm sections in coronal and axial orientations with and without contrast enhancement. RESULTS: In sixteen patients we found a significant reduction in muscle volume or even total aplasia of the superior oblique muscle of the affected side in comparison to the sound muscle on the other side. In contrast, two patients had a full blown clinical picture of a congenital superior oblique palsy but showed symmetrical muscle volumes on both sides in all coronal sections. CONCLUSIONS: Hypoplasia or aplasia of the superior oblique muscle on magnetic resonance imaging provides evidence for a primary paretic cause for the vertical squint found with congenital superior oblique dysfunction. It is not clear, however, whether this is caused by a primary hypoplasia or is of neurogenic origin. Our data together with the consistent difference in tendon morphology of the congenital and acquired forms of superior oblique palsy seem to exclude a purely neurogenic cause for the affection.

[Interocular inhibition in strabismus]. / Interokulare Hemmungsvorgänge bei Strabismus.

Many patients with acquired strabismus do not suffer from diplopia and confusion after an individually and age-dependent interval. They inhibit the image of the deviated eye by binocular rilvary and particularly by the physiological ability to disregard visually disturbing stimuli. In strabismus with early onset, binocular rivalry is also demonstrable, even for stimuli that do not normally lead to suppression. On the basis of anomalous retinal correspondence, this rivalry occurs between retinal points onto which the same object projects. The retinal area with the lesser eccentricity receives the dominance. The fovea of the deviated eye is therefore not suppressed. In small-angle strabismus with smaller functional differences between anomalous corresponding retinal points anomalous fusion and even stereopsis can be possible as long as strong suprathreshold stimuli are presented. Strabismic amblyopia as a consequence of interfoveal suppression can only develop before anomalous retinal correspondence dominates in the strabismic child.

[Long-term outcome of optic nerve sheath fenestration in pseudotumor cerebri]. / Langzeitergebnisse nach Optikusscheidenfensterung bei Pseudotumor cerebri.

BACKGROUND: Chronic papilledema may lead to irreversible damage of optic nerve fibers. To preserve visual functions, a decompression of the optic nerve is recommended by means of a fenestration of the optic nerve sheath. In this study long-term results after optic nerve sheath fenestration in patients with idiopathic pseudotumor cerebri are reported. PATIENTS AND METHODS: 14 patients were re-examined 15 to 145 months (mean 62 months) after a fenestration of the retrobulbar optic nerve sheath on 23 eyes by a transconjunctival approach. Surgery was done to treat progressive visual loss or in severe obscurations. The patient's symptoms, visual acuity, visual fields, and ophthalmoscopic findings of the optic nerve head and the central fundus were compared to the preoperative status. RESULTS: Re-examination revealed improvement or stabilisation of objective and subjective findings in 17 eyes, one of them was operated on the more involved contralateral side only. Six eyes showed a recurrence of the papilledema without a functional change for the worse after an interval of 7 to 121 months. Three eyes of two patients ended up with optic atrophy and extensive visual loss. Preoperatively, these eyes had shown cotton wool spots in the optic nerve head and a rapid deterioration of vision. CONCLUSIONS: Fenestration of the retrobulbar optic nerve sheath can prevent further visual loss in most patients with pseudotumor cerebri, unless the eye has already become nearly blind. Postoperatively, ophthalmological controls are necessary at regular intervals because relapses after successful surgery can occur after months or years.

[Conjunctival cysts of the orbits: clinical aspects and histology in 4 patients]. / Konjunktivale Zysten der Orbita: Klinik und Histologie bei vier Patienten.

BACKGROUND: Only a few reports concerning congenital conjunctival cysts of the orbit (primary nonkeratinized epithelial cysts) have been published. PATIENTS: We describe four further cases of conjunctival cysts of the orbit, which were observed between 1995 and 1997 in Tübingen and Vienna. Histologically, all cysts were lined by nonkeratinizing epithelium with goblet cells. Epidermal appendages were found in the wall of one cyst. The patients' age was between 1 and 32 years. Clinically, the prominent symptom was swelling of the upper eyelid, persisting in three patients for more than several years. In one patients diplopia and restriction of eye movements developed within several weeks. CONCLUSIONS: The cases we described are in accordance with the cases reported in the literature. Often a swelling of the upper eyelid persisted for several years, before the cyst has become symptomatic predominantly in an adult patient. In contrast to dermoid or epidermoid cysts, there is no bony erosion and the cysts are located in the superior to nasal orbit. These cysts are probably more common as it is represented in the literature.

[Insufficiency of a frontalis loop]. / Insuffizienz einer Frontalis-Schlinge.

After 13.5 years in vivo a 2-0 Terylene frontalis loop was excised because of an insufficiency. Histology showed a persisting giant cell reaction while lymphocytes were almost totally absent. The fibrils of the suture seemed to be intact. However, they were separated by ingrowing connective tissue which, on theoretical grounds, should improve the function of the frontal muscle on the upper eyelid. Therefore, insufficiency of the loop was probably caused by the (subclinical) foreign body reaction or the long-lasting mechanical stress leading to tissue damage.

Ocular myasthenia gravis: response to long-term immunosuppressive treatment.

OBJECTIVE: Ocular myasthenia gravis is a subtype of myasthenia gravis that causes relatively mild disability, but may convert into severe generalised muscle weakness. A universal management plan for ocular myasthenia gravis has not been established. This study was performed to determine the outcome of ocular myasthenia gravis with the currently available therapeutic options. METHODS: Retrospective analysis of 78 patients with ocular myasthenia gravis with a mean disease duration of 8.3 (range 0.5-58.3) years. RESULTS: In 54 patients (69%) symptoms and signs remained confined to the extraocular muscles during the observation period. The remaining 24 patients (31%) developed symptoms of generalised myasthenia gravis; 50% of them within two years, 75% within four years after onset. A somewhat reduced risk of generalisation was found in those with mild symptoms, normal repetitive nerve stimulation test, and low or absent antiacetylcholine receptor (AChR) antibodies at the time of diagnosis. Patients receiving immunosuppressive treatment (corticosteroids and/or azathioprine) rarely developed generalised myasthenia gravis (six of 50, 12%). Those without such treatment, usually due to uncertain diagnosis and late referral, converted into generalised myasthenia gravis significantly more often (18 of 28, 64%). CONCLUSIONS: The prognosis of ocular myasthenia gravis is good. A conventional scheme with short-term corticosteroids and long-term azathioprine seems adequate to achieve remission in most patients. The proportion of patients developing generalised myasthenia gravis was smaller in this population compared with previously published groups (usually 50%-70%). Early immunosuppressive treatment is at least partially responsible for this finding. Thymectomy (performed here in 12 patients with an abnormal chest CT) also correlated with a good outcome, but had no apparent advantage over medical treatment alone.

[Pathogenesis of eso- and hypotropia in high myopia]. / Zur Pathogenese der Eso- und Hypotropie bei hoher Myopie.

BACKGROUND: In myopia characteristic types of strabismus and defects of ocular motility can occur. One of these myopia related disturbances shows a gradually increasing eso- and hypodeviation in progressively myopic eyes, caused by a corresponding deficit of abduction and elevation. Different pathogenetic factors have been described for this long known clinically uniform entity. PATIENTS AND METHODS: In eight patients with pathologic myopia a marked eso- and hypotropia was operated on in the last 15 years. The deviation was infantile in two and acquired in six cases. We performed a routine orthoptic examination and looked for anatomic variations of the rectus muscles during surgery. RESULTS: In seven of the eight cases the anterior portion of the lateral rectus muscle was not directed straight dorsaly but obliquely into the lower temporal quadrant of the orbit. A supraposition of the horizontal recti muscles in addition to a recess-resect procedure provided satisfactory results in five of six cases. CONCLUSIONS: Scleral ectasia in high myopia can lead to a downslip of the lateral rectus muscle relative to the globe, giving this muscle a depressing effect at the cost of its physiological action. As in Duane's syndromes with up- or down-shoot, a repair of the deviation is possible by combining horizontal surgery with vertical transposition of the horizontal recti muscles. Additional operations on the vertical recti muscles can thus be avoided.

How useful is anomalous correspondence?

In anomalous retinal correspondence (ARC), retinal points in the right and left eyes which receive stimuli from one object in space have the same visual direction despite a manifest motor deviation. The mode of cooperation of these anomalously corresponding retinal points depends on their relative eccentricity or their relative functional level. If there is a great difference, as for the most part in large angle strabismus, the retinal point with less eccentricity and better resolution will prevail, while the other will be suppressed. This leads to a second area of high resolution in the binocular visual field with a possibly irritating effect. In microtropia, anomalous corresponding retinal points have functionally different levels only in the very centre of the visual field. ARC provides in this case a binocular cooperation very similar to normal fusion. To an individually different degree, ARC can adapt to new squint angles, which makes squint surgery possible without postoperative diplopia, but a change to normal binocularity with stable interocular connections can not be achieved. ARC enables the heterotropic patient to see with both eyes simultaneously without diplopia by a regionally changing use of inhibition and anomalous fusion in the interest of the best possible perception.

[The value of the prism adaptation test in determining the degree of squint surgery]. / Zum Wert des Prismenadaptationstests bei der Dosierung einer Schieloperation.

The angle of squint deviation was measured preoperatively before and after a prism adaptation of 1 day's duration in 50 esotropic and 45 exotropic patients. All had a deviation of 17 degrees or more under prismatic correction and were operated on by a recess-resect procedure of 10 mm on one eye. Postoperative measurements were done about 4 days and 6 weeks after surgery. In 58% of the esotropic and 37% of the exotropic patients, the initial deviation increased under prisms. This was found most often when normal retinal correspondence was present. There was a correlation between the effect of surgery with the change of deviation under prisms, particularly in normosensory esotropia and intermittent exotropia. Accordingly, the standard deviation of the surgical effect was smaller in this group, when it was calculated from the deviation under prisms in comparison to a calculation from the primarily measured angle. The influence of the preoperative deviation on the effect of a 10-mm recess-resect procedure was also found for the deviation under prisms and must therefore additionally be mentioned in the dosage of surgery. This retrospective study shows that preoperative prism adaptation improves predictability of the effect in squint surgery, at least in normosensory esotropia and intermittent exotropia. In patients with early-onset strabismus prism adaptation does not improve predictability, but higher dosage seems to be useful in cases with increased deviation under prisms and did not lead to over corrections. In this group the test represents an additional method for evaluation of the binocular sensory status.

Neuroepithelial adenocarcinoma of the eye.

A 29-year-old male patient with a microphthalmos on the right side since birth was seen in June 1987 in the University Eye Clinic because of a massive exophthalmus of the former microphthalmic eye. Computer tomography revealed a diffuse, infiltrating tumor of the right orbit. The bulbus was not demarcated, and the whole orbita had to be eviscerated. Histologically, we found a solid, undifferentiated tumor with clear signs of malignancy. It consisted of epithelial cells, partly in nest formation, which were keratin positive and vimentin negative, and of more spindle-shaped formations which were keratin negative and vimentin positive. In some areas, the tumor showed a tubular or an adenoid proliferation. CEA or S-100 could not be demonstrated. The histopathological diagnosis was neuroepithelial adenocarcinoma of the eye. The differential diagnosis and the genesis of the tumor are discussed.

[Perception of the blind spot with Rauschfeld campimetry. An additional diagnostic criterion in papillary changes]. / Die Wahrnehmung des Blinden Flecks bei der Rauschfeldkampimetrie. Ein zusätzliches diagnostisches Kriterium bei Papillenveränderungen.

In conventional test point perimetry the optic disc is detected as an absolute scotoma (blind spot). However, in white noise campimetry the blind spot usually cannot be perceived: the white noise is continuous over the whole field if presented to a normal eye. This is true not only for the blind spot but also for all scotomas caused by congenital damage to the retina or the visual pathway (e.g. colobomas). On the other hand, the blind spot is always perceived in white-noise campimetry if acquired damage to the peri-cecal region is present. In such cases this method can give important information concerning the etiology of an enlarged blind spot detected with conventional test point perimetry. In addition during the follow-up of a papilledema, fading of the perception of the blind spot in the white-noise field indicates when the edema is resolved. Results of white-noise campimetry in papilledema, glaucomatous atrophy of the optic disc and anterior ischemic optic neuropathy are presented and compared with findings of conventional test point perimentry.

The diagnostic value of serum autoantibodies in endocrine orbitopathy and idiopathic ocular myositis.

Sera of patients with Graves' orbitopathy (GOP) often contain antibodies against retroorbital tissue components. The presence of such autoantibodies has been considered to indicate an autoimmune pathogenesis of the disease. However, their specificity has not been conclusively tested, because studies demonstrating autoantibodies used controls with no lesion in the extraocular eye muscles. Although ocular myositis (OM) is clinically distinct from GOP, damage to the retroorbital muscles is a common histopathological finding in both conditions. Using an immunoblot technique, reactions of sera from patients suffering from either disease were compared. Sera from both groups contained antibodies against a variety of antigenic determinants recognized by both sera. These autoantibodies may have been induced secondary to tissue damage and should be considered to be nonspecific. Because the role of tissue damage was not accounted for in previous studies, evidence concerning antigens supposedly specific for GOP should be reevaluated. The reaction patterns of OM and GOP sera were slightly different. These differences were specific enough to suggest that sera from patients with GOP contain antibodies against eye muscle components that are not present in the sera of patients with idiopathic OM. These findings support the assumption that GOP is an autoimmune disease. However, the major autoimmune targets remain to be identified and their pathogenic relevance is still unclear.

Infantile exotropia with homonymous hemianopia: a rare contraindication for strabismus surgery.

A homonymous hemianopia can be compensated by a strabismus in the direction of the visual field defect if the deviated eye has a harmonious anomalous localization. This compensation is only partial; its extent corresponds to the angle of deviation. We report on two patients with a hemianopia to the left. Under binocular conditions the visual field defect was reduced by an exotropia of the left eye with panoramic vision. Unfortunately, up to now it has not been possible to induce such a sensory-motor compensation in cases of homonymous hemianopia with normal retinal correspondence in an adult visual system.

[Cystic teratoma of the orbit]. / Das zystische Teratom der Orbita.

Teratomas are rare cystic tumors of the orbit which lead to excessive congenital exophthalmos. They increase rapidly in size after birth and therefore require immediate surgical treatment. Initial puncture of the cyst under sonographic control prevents progressive mechanical damage to the globe and the optic nerve, providing sufficient time for the preoperative measures required prior to final surgical removal. The characteristic clinical features make it easy to identify a teratoma provided it has been taken into consideration in the differential diagnosis. It is shown with reference to one of the authors' own cases that a good functional result is possible after removal of the encapsulated tumor with preservation of the globe, even when the eye has been excessively displaced by the tumor.

Objective and subjective evaluation of cyclovergence and cyclofusion.

There is a marked dispersion in the measured results of the angle formed by the intersection of the optic disc-center-meridian with the horizontal meridian, when the cycloposition of the eyes is determined through evaluation of the position of the optic disc (with fundus photographs or indirectly with perimetry). Values of the angle ranging from 0 degree at 12 degrees are still physiological. Inside this dispersion, individual right-left asymmetries of less than 4 degrees with photography and of less than 7 degrees with monocular perimetry are still normal as well. Positional differences of less than 4 degrees in sequential testings are also in a physiological range. The motor behaviour of both eyes during cyclofusion is evaluated by means of a perimetrical analysis of the blind spot at the phase-difference haploscope. Various sensory and motor adaptation phenomena are detected by comparing objective and subjective findings in patients with cyclotropia. These phenomena are discussed in detail.