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[A case of brainstem infarction presenting with paralytic pontine exotropia and non-paralytic pontine exotropia].

We reported a patient with brainstem infarction who presented paralytic pontine exotropia (PPE) in acute phase and non-paralytic pontine exotropia (NPPE) during the recovery phase. On March 10, 1991, a 60-year-old man was brought to our hospital with chief complaints of double vision and right hemiparesis. The ocular position of the left eye on forward gaze was fixed at the midline, while the right eye was abducted, and skew deviation was observed. On leftward gaze, neither eye could pass the midline, and on rightward gaze the right eye was abducted and monocular nystagmus was noted in the abducted right eye, which indicated paralytic pontine exotropia. T2-weighted MRI showed high signal intensity lesions in the left paramedian portion of the mid-pontine tegmentum beneath the fourth ventricle, and in the midline of the pontine base extending to the left side. The next day, similar disturbance of ocular movement was seen on rightward gaze, but the limitation of leftward gaze had improved and NPPE was noted to have developed. On the third day, there was no abduction of the right eye on forward gaze, but left MLF syndrome was still present. Seven days later, the disturbances of ocular movement disappeared. The lesion was considered to be due to partial impairment of the left paramedian pontine reticular formation in addition to the MLF. PPE caused by ischemia due to the perforating arteries of the brainstem, different from that due to basilar artery occlusion may recover after progression through NPPE and MLF syndromes. Hence, one-and-a-half syndrome, PPE, NPPE, and MLF syndromes are considered to be analogous to one another.

Brain Stem

Whole-exome sequencing uncovers the genetic basis of hereditary concomitant exotropia in ten Chinese pedigrees.

PURPOSE: To explore possible pathogenic genes for concomitant exotropia using whole-exome sequencing. METHODS: In this study, 47 individuals from 10 concomitant exotropia (including intermittent exotropia and constant exotropia) pedigrees were enrolled. Whole-exome sequencing was used to screen mutational profiles in 25 affected individuals and 10 unaffected individuals. Sanger sequencing and in silico analysis were performed for all participants. Two target genes were used to capture the sequences of 220 sporadic samples. RESULTS: All 10 concomitant exotropia pedigrees presented autosomal dominant inheritance with childhood onset (3.35 ± 1.51 years old). Eleven different missense variants were identified among seven potential pathogenic genes (COL4A2, SYNE1, LOXHD1, AUTS2, GTDC2, HERC2 and CDH3) that cosegregated with pedigree members. All variants were predicted to be deleterious and had low frequencies in the general population. Distinct variants of COL4A2 were present in three pedigrees, and distinct variants of SYNE1 were present in two pedigrees. Fifteen variants in AUTS2 and four variants in GTDC2 were identified in 220 patients with sporadic concomitant exotropia using a target-capture sequencing approach. CONCLUSION: This is the first study to explore the genetic mechanism of concomitant exotropia and identify seven associated genes (COL4A2, SYNE1, LOXHD1, AUTS2, GTDC2, HERC2 and CDH3) that may be candidate genes causing concomitant exotropia. More samples and in-depth studies are needed to verify these findings.

Adult

The use of part-time occlusion for early onset unilateral exotropia.

Eleven patients (9 months to 5 years of age) with intermittent or constant exotropia of predominantly one eye on distant gaze were studied to investigate the effect of part-time occlusion. No patient had constant exotropia on near fixation. Visual acuity was found to be equal in both eyes. All patients were treated by part-time patching of the non-deviating eye from four to six hours a day and tapered as appropriate. If exotropia recurred after conversion to a heterophoria, part-time occlusion was reinstated. With occlusion, all patients converted to hetero- or orthophoria, at least temporarily. Mean follow-up (to last visit or surgical intervention) was 22 months (range 3 to 37 months). Three patients (27%) later developed constant exotropia (mean 28.3 months after beginning occlusion) and underwent surgery. Three patients (27%) became and remain orthophoric without further patching. Part-time occlusion for preschool patients with exodeviations that are predominantly unilateral can postpone surgical intervention and convert exotropia to orthophoria or exophoria.

Child, Preschool

[Disproportionately large communicating fourth ventricle with bilateral exotropia: report of two cases].

Two cases of disproportionately large communicating fourth ventricle (DLCFV) accompanied by consciousness disturbance and bilateral exotropia are reported. Case #1 was a 21-year-old male who suffered from consciousness disturbance and bilateral exotropia due to malfunction of the ventriculoperitoneal shunt (VPS) which had previously been operated on twice for a left parietal arteriovenous malformation, which had caused ventricular hemorrhage several times. The last hemorrhage was massive and made ventricular casting, including the fourth ventricle. Both bilateral exotropia and the fourth ventricular dilatation were well controlled by the reconstruction of the VPS. Case #2 was a 66-year-old female, semicomatous because of massive subarachnoid hemorrhage with ventricular casting hematoma due to rupture of the right middle cerebral aneurysm. Though an improvement of the consciousness disturbance was obtained by continuous ventricular drainage (CVD), bilateral exotropia and consciousness deterioration appeared after lumboperitoneal shunt followed by the removal of the CVD. Another CVD was then carried out and some improvement was obtained again. However, the same symptoms appeared again after the VPS, followed by the removal of the CVD. The patient finally died despite a third CVD. Autopsy revealed a markedly dilated fourth ventricle and massive subarachnoid clots particularly around the foramen of Magendie and Luschka. The pathogenesis of DLCFV and bilateral exotropia are also discussed.

Adult

Optometric management of optically induced consecutive exotropia.

A 5-1/2 year old black female initially presenting with a moderate angle esotropia and latent hyperopia developed a large angle constant exotropia 2 years after final correction of her refractive error. The occurrence of consecutive exotropia as a result of optical correction of hyperopia has been documented infrequently in the ophthalmologic literature and has rarely been mentioned in the optometric literature. While the overall risk for occurrence of this complication from correction of hyperopia may be small, it is a problem which may occur and can be avoided. Unfortunately, there is only limited information about the various risk factors that should be monitored to avoid the occurrence of an optically induced consecutive exotropia. What is available with regard to evaluation and management is scant, and there are no case reports emphasizing optometric management which includes the use of lenses, occlusion and vision therapy. The purpose of this paper is to present a case report of optically induced consecutive exotropia followed by a summary of the available information from the optometric and ophthalmologic literature. This combined information will aid optometrists managing these patients to avoid the occurrence of this problem and better understand the various management aspects when it does occur.

Accommodation, Ocular

Monofixational intermittent exotropia.

The surgical correction of intermittent exotropia occasionally results in a monofixational sensory pattern. Stereopsis as a measure of bifixation was studied preoperatively and postoperatively in 39 cases of intermittent exotropia. Of 26 patients with bifixation, 25 remained so after surgery. Of 13 patients with monofixation, 11 remained unchanged by surgery, one improved, and one lost all binocularity. The presence of preoperative monofixational intermittent exotropia, to explain a monofixation result from intermittent exotropia surgery, is proposed.

Adolescent

[Congenital nystagmus and intermittent exotropia. Suppression of nystagmus by fusional convergence].

We report on a patient with a combination of congenital nystagmus and intermittent exotropia. When the exotropia became manifest, a gross nystagmus appeared. When, however, the exotropia was overcome by fusinal convergence, the nystagmus was largely suppressed. The angle of squint was surgically reduced by such an amount that the exodeviation was just compensated, and the fusional convergence required to overcome the remaining angle sufficed to suppress the nystagmus.

Convergence, Ocular

[A case of hemi-hyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction].

A case of hemihyperhidrosis and non-paralytic pontine exotropia due to brainstem infarction is reported. A 55-year-old hypertensive man developed right hemiparesis with slight dysarthria and nausea upon awaking. The right side of his face and right upper limb and trunk to the level of the Th8-9 territory showed hyperhidrosis, which disappeared in a week. Ocular motor examination revealed that during forward gaze with the left eye fixing, the right eye deviated outward. The patient was able to adduct the right eye to midposition with the right eye fixing. Rightward gaze elicited full abduction and right-beating nystagmus of the right eye, but the left eye did not adduct. When he attempted to gaze leftward, both eyes made the full excursion, but saccades were slow in that direction. Convergence was intact. Vertical gaze was full, and he did not show Horner's sign. This ocular sign, non-paralytic pontine exotropia, disappeared three days later. T2-weighted spin echo magnetic resonance imaging disclosed a small lesion with high intensity in the inner side of the left middle pons. This hyperhidrosis was thought to be caused by destruction of inhibitory fibers thermoregulating sweating. These findings suggest that at the level of the middle pons inhibitory fibers descend along the inner side of facilitatory fibers thermoregulating sweating, which are speculated to descend the dorso-lateral part of the pontine tegmentum. These findings also suggest that lesions of non-paralytic pontine exotropia may be located in the paramedian pontine reticular formation rostral to the abducens nucleus with ipsilateral medial longitudinal fasciculus lesion, but further investigation is necessary.

Brain Stem

[Intermittent exotropia associated with accommodative esotropia].

Intermittent exotropia associated with accommodative esotropia is a rare particular type of strabismus. 8 cases of XT-ET were presented here. The clinical features of them were summarized as follows: 1. The age of onset was early. 2. The initial symptom was esotropia, exotropia was observed by doctor. 3. The most patients (6/8) associated high or medium hyperopia. 4. The AC/A rate were normal or lower than normal. 5. The angle of deviation varied with the examination, exotropia 30 degrees-15 degrees<-->orthophoria<-->esotropia 15 degrees-30 degrees. 6. The esotropia can be corrected with spectacles. The visual-vestibular interaction examination for eye movement were examined in 3 cases, but have no regular expression yet. Finally, the incidence (0.4%, 8/21 58) and treatment for this disease were discussed.

Accommodation, Ocular

[A case of non-paralytic pontine exotropia due to pontine tegmentum lesion confirmed by magnetic resonance imaging and electronystagmography].

A case with non-paralytic pontine exotropia (NPPE) due to brainstem infarction is reported. A 77-year-old hypertensive man suddenly developed dizziness, double vision, dysarthria, and right ataxic hemiparesis. Oculomotor findings on admission consisted of: (1) full right exotropia in the primary position; (2) complete adductive paralysis of the left eye with slight preservation of convergence; (3) tonic deviation of the right eye to the full abducting position with right-beating nystagmus after an immediate forward gaze. The leftward saccades showed multiple saccades with slow velocity on electronystagmography (ENG). The right exotropia disappeared and the slight adductive paresis of the left eye remained with right monocular nystagmus seven weeks after the onset. Magnetic resonance imaging (MRI), which was performed nine weeks after the onset, disclosed a small lesion with high intensity involving the left medial longitudinal fasciculus (MLF) on T2-weighted spin echo image. The leftward saccades showed multiple saccades with normal velocity eleven weeks after the onset. The hypofunction of unilateral PPRF with ipsilateral MLF lesion probably causes the contralateral NPPE.

Aged

[Personal experience with consecutive exotropia].

This article presents a detailed analysis of sensor and surgical exotropias. Of the 669 operated esotropias 20 patients (2.98%) had surgical consecutive exotropia. Of the 1264 esotropias treated conservatively throughout several years, 7 patients (0.55%) showed sensor consecutive exodeviation. In both groups exotropia appeared in more than 60% of children after the age of 10 years. In 45% of the cases, surgical exodeviation appeared within 6 months of surgical treatment what suggests a wrong estimation of the situation. In the age from 2 to 5 years, 75% of the children were surgically treated. In 65% of the operated patients, several factors were present at the same time, such as amblyopia, anisometropia and vertical component.

Child

[Postoperative secondary divergent strabismus. Statistical analysis of 160 consecutive exotropias].

Exotropias secondary to convergent squint surgery are fully different from primary exotropias. Their frequency appears to be largely variable according to the operative procedures. The computerized analysis of 160 consecutive exotropias reveals the compound nature of their motor impairments. In each case it is necessary to carry out a careful check up of various causal parameters, but the authors find they are not bound by simple correlations. In all cases an elongation reducing one or several muscles is noticed. Such anomalies are much more frequent and heavy than those observed in primary esotropias. Their iatrogenic origin is unquestionable. In 37% of the cases heavy capsular scars have been observed; their consequences on passive forces are obvious, but it is difficult to carry out their exact evaluation by the usual clinical tests. In that series the resulting surgical planning was made according to the various abnormal parameters had allowed the achievement of a good motor result in 89% of the cases. In 86% of the cases, only one additional operative procedure was necessary.

Esotropia

Binocularity and photophobia in intermittent exotropia.

The relationship between photophobia and binocular function in intermittent exotropia was examined in 42 subjects (21 patients and 21 controls). In all subjects fusional amplitude and strength of binocular sensory status were tested before and during exposure to bright light. An explanation of photophobia in intermittent exotropia was suggested as impairment in binocular cooperation, since the indicators of binocular function were significantly altered in the patients, particularly during light exposure.

Adolescent

Infantile exotropia in healthy children.

Constant exotropia is uncommon during the first year of life. We retrospectively identified 13 patients with large, constant exotropia during the first year of life which required surgery. Bilateral lateral rectus muscle recessions were done in all 13 cases. Only two patients have required reoperation. The clinical characteristics and management of this unusual condition are similar to those of infantile esotropia.

Exotropia

The surgical overcorrection of intermittent exotropia.

Many strabismus surgeons recommend an initial surgical overcorrection for intermittent exotropia. Others caution against overcorrection because of possible nasal suppression and amblyopia in children, or because of possible diplopia in adults. We reviewed the records of 69 patients who were initially overcorrected following surgery for an intermittent exotropia. The mean postoperative follow-up was 3.1 years. Eight patients (11.6%) had a persistent overcorrection of 3 prism diopters or more and three patients (4.3%) had persistent diplopia. Patients with a persistent overcorrection had a greater mean age (P less than .02) and a greater mean initial overcorrection (P less than .005) compared with the patients who were not overcorrected 3 delta or more. No child lost stereoacuity or developed amblyopia due to the overcorrection.

Adolescent

Excessive single lateral rectus muscle recession in the treatment of intermittent exotropia.

Ten patients with small angle intermittent exotropia of 14 to 16 prism diopters were treated by unilateral lateral rectus muscle recession of 11.5 to 12 mm on the nondominant eye. In the immediate postoperative phase, overcorrection of 4 to 6 delta gave a very good functional result. Abduction deficiency was minimal for recession up to 12 mm. This procedure should be considered as an alternative approach in the treatment of small angle intermittent exotropia showing a "basic" pattern.

Child

Lateral incomitance in exotropia: fact or artifact?

The prevalence of significant lateral incomitance in patients with nonparetic exotropia is reported to be 22%. We speculated that measurement artifact may be the cause for some cases of apparent lateral incomitance. We measured the effective power of plastic ophthalmic prisms using a helium-neon laser in the frontal plane position and at 10 degrees, 20 degrees, and 30 degrees of rotation from the frontal plane. The rotated prisms represented the situation in which a neutralizing prism rotates with the head during measurement of lateral gaze positions. For prisms of 35 prism diopters or more, even 10 degrees of rotation produced significant artifactual incomitance. For smaller prisms, 20 degrees or more of rotation was necessary to induce significant lateral incomitance. We prospectively measured 40 consecutive patients with exotropia. Only three patients (9%) had true incomitance greater than 5 delta, and only one had incomitance in both directions of gaze. Significant lateral incomitance could be induced in every patient examined by improperly positioning the neutralizing prism. Because the detection of lateral incomitance causes most strabismus surgeons to reduce the amount of surgery they perform, special care is necessary when measuring deviations in lateral gazes.

Diagnostic Errors

An alternative approach to the surgical management of exotropia--the unilateral lateral rectus recession.

Fifty-five patients with moderate angle exotropia of 15 to 20 prism diopters were surgically treated with a unilateral lateral rectus recession of 7.0, 7.5, or 8.0 mm. The mean age at surgery was 14.5 years. The mean preoperative deviation was 18.0 delta. The mean lateral rectus recession was 7.6 mm. At the initial postoperative follow up, all patients were either a small-angle exotropia or esotropia, or orthophoric. Twenty-eight of the 55 patients (51%) were orthophoric at their most recent follow up. Twenty-four patients (44%) were undercorrected, with 22 of these having residual exodeviations less than 8 delta. Three patients (5%) were overcorrected.

Adolescent