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The position of eyes under general anesthesia was measured in different groups of nonparalytic strabismus. Almost all patients with esotropia or hyperactive inferior oblique muscle showed divergent eye positions while patients with exotropia showed no consistent trend. The eye position of patients with lid ptosis without squint, which served as a control group, were divergent in all cases. Electromyographic observation of the medial rectus muscle under the same condition of anesthesia revealed that muscle discharge disappeared almost completely at the stage at which the eyes were in a well-stabilized position.
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PMID:Eye position and electromyographic observation of squint eyes under general anesthesia. 47 34

Partial trisomy (interchromosomal duplication) of the short arm of chromosome No. 12 was observed in an infant girl with psychomotor retardation, prominent forehead, ptosis of the right eyelid, esotropia/exotropia, flat nose, hypotonia and other anomalies. A comparison of her features with those in five reported cases with a similar chromosomal imbalance shows certain features common to all, but the material is too limited for definitive characterization of a trisomy 12p syndrome.
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PMID:Partial trisomy 12p due to t(12;21)pat translocation. 85 22

From April 1983 to April 1988, 381 botulinum toxin injections for lid spasms were administered to 106 patients. Sixty-nine had bilateral blepharospasm and 37 had hemifacial spasm. Of the 381 injections, 308 had been given to patients who returned for follow-up examinations. No systemic effects were noted at any of these visits; all side effects were temporary; there were no serious complications. Ptosis, the most frequently encountered problem, occurred after 26 (8.4%) of the injections. Other complications included: corneal exposure (after eight injections, 2.59%); face droop (after 11 injections, 3.57%); diplopia (after five injections, 1.62%); and subtle visual blurring (after eight injections, 2.59%). One patient noted jaw tenseness, another mentioned tearing, one reported brow droop, and another complained of crossed eyes. Ten injections had minimal effect; in these cases a repeat injection usually was effective. Only four patients chose surgery after beginning injections. We conclude that botulinum toxin injections are a safe, effective means of treating lid spasms.
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PMID:Side effects of the use of botulinum toxin for treatment of benign essential blepharospasm and hemifacial spasm. 238 55

Ten infants and children who presented with craniofacial dysostosis are discussed; four had Apert's syndrome, four had Crouzon's syndrome, one had Pfeiffer's syndrome, and one had hypertelorism. The follow-up of the patients ranged from 3 months to 7 years, with an average of 19 months. Patients had bifrontal and biparietal craniectomies to correct frontal and temporal orbital retrusion, while two had left unilateral procedures only. One patient (T.S.) had had three similar procedures before he was 3 years old and patient B.B. had two before he was 11 months old due to the complete failure of bony orbital growth. Before the cranial surgery, one patient had a preexisting esotropia with bilateral congenital sixth nerve paresis, one had a V-pattern exotropia, and one had a right intermittent hypotropia due to right superior rectus weakness. In no case was there a change in the ocular alignment after infantile craniectomy. There were assorted ophthalmologic anomalies, such as congenital bilateral sixth nerve paresis, absent superior rectus function, bilateral ptosis in addition to absent superior rectus function, and two patients presented with frank and repeated exorbitism.
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PMID:Strabismus in craniofacial dysostosis. 340 45

The tendinous origins and insertions of the extraocular muscles were studied embryologically by macroscopic and microscopic methods. It is concluded from this investigation that these tendons of origin and insertion arise from mesenchymal tissue similar to that of their respective muscles. These tendon-muscle groups have developed from superior and inferior mesenchymal complexes. The origins of the extraocular muscles are attached to the periorbita by an interlocking of the tendinous and muscular fibers, which allows for mobility of the extraocular muscles in all extreme directions of gaze and also results in a strong mechanical mooring for these muscles. Avulsion at the origins of the extraocular muscles following severe traction or trauma is rare. The additional origin of the superior and medial rectus muscles to the dura of the optic nerve explains the pain that may occur on movement of the eye in optic neuritis. Optic nerve compression and thyroid myopathy is explained by mucopolysaccharide and inflammatory cell infiltration of the muscular interdigitations that extend up to the site of origin of the rectus muscles. Findings of this investigation suggest that the association of ptosis and superior rectus muscle underaction may be due to a persistence of fibrous tissue that has endured from embryologic development between the superior rectus and levator palpebrae superioris muscles. Superior oblique tendon sheath syndrome is explained by embryologic strands remaining between the tendon of the superior oblique muscle and the trochlea. The insertions of the rectus muscles extend from the equator of the eye to the limbus early on in development. By processes of differential degeneration between the sclera and the rectus tendon, posterior recession of the tendon from the limbus, and contemporaneous growth of the anterior segment of the eye, these tendons reach their adult location only between the ages of 18 months and 2 years. In strabismus surgery, measurements for muscle adjustments should be assessed from the limbus rather than from the sites of insertion of these tendons. In the series of patients with esotropia, no mechanical abnormalities were noted in relationship to the insertions of the medial or lateral recti muscles. Furthermore, no correlation was found between the site of insertion of the medial rectus muscle and the degree of esotropia.
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PMID:The origins and insertions of the extraocular muscles: development, histologic features, and clinical significance. 359 Apr 78

Between July 1984 and March 1985, 51 injections of type A botulinum toxin were given to 39 patients aged 11 to 81 years with various types of strabismus, including paralytic strabismus, sensory exotropia, consecutive and residual postsurgical deviations and partially accommodative esotropia. All deviations were documented by orthoptic assessment and photography. After treatment the patients were followed for up to 3 years. The beneficial effects of a single injection were modest, and a second injection was often necessary to achieve satisfactory ocular alignment. Thirteen of the 26 patients (50%) with nonparalytic strabismus and 10 of the 13 patients (77%) with paralytic strabismus had a good outcome (final deviation 12 prism dioptres or less). Adverse side effects included transient ptosis and diplopia and inadvertent vertical deviation. Twenty-one consecutive cases of nonparalytic horizontal strabismus treated with adjustable sutures were also reviewed. The results in these patients were more predictable and longer lasting than those in the patients who received botulinum toxin.
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PMID:Injection of type A botulinum toxin into extraocular muscles for correction of strabismus. 360 94

A 17-year-old woman with a left congenital ptosis and an alternating esotropia is presented. During horizontal gaze to the right and left, the eyelid of the abducting eye elevated and the eyelid of the adducting eye lowered. There were no synkinetic eyelid movements with contraction of the pterygoid, sternocleidomastoid, or facial muscles. Synkinetic eyelid movements due to aberrant regeneration were unlikely due to absence of previous third nerve palsy. A supranuclear innervational abnormality is proposed to explain the clinical findings.
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PMID:Unilateral blepharoptosis with synkinetic movements of the eyelids on horizontal gaze. 374 96

Malignant hyperthermia is an autosomal dominant disorder with variable expressivity that is caused by a membrane defect in the sarcolemma of myofibrils. A patient with strabismus (esotropia) had tachycardia and masseter muscle rigidity on exposure to succinylcholine chloride and halothane, but because of rapid recognition of the condition and discontinuation of the procedure, the potentially lethal complications of malignant hyperthermia did not develop. A serum creatine phosphokinase level showed a substantial increase above normal. Two weeks later, the patient underwent successful correction of the strabismus under general anesthesia, using morphine sulfate and thiopental sodium without complication. This condition is of interest to ophthalmologists because it occurs with increasing frequency in patients with strabismus and ptosis, and it may be triggered by certain local anesthetic agents often used by ophthalmologists.
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PMID:Suspected malignant hyperthermia in a strabismus patient. A case report. 725 98

The prognosis for 45,X/46,XX mosaicism diagnosed prenatally has yet to be established. We report our experience with 12 patients in whom prenatal diagnosis of 45,X/46,XX mosaicism was detected by amniocentesis for advanced maternal age or decreased maternal serum alpha-feto protein and compared them with 41 45,X/46,XX patients diagnosed postnatally. The girls in the prenatal group range in age from 3 mo to 10 years. All have had normal linear growth. Four had structural anomalies including: ASD (n = 1); ptosis and esotropia (n = 1); labial fusion (n = 1); and urogenital sinus, dysplastic kidneys, and hydrometrocolpos (n = 1). Gonadotropins were measured in seven; one had elevated luteinizing hormone/FSH at 3 mo of age. One has developmental delay and seizures as well as ophthalmologic abnormalities. None would have warranted karyotyping for clinical suspicion of Turner syndrome. The prevalence of 45,X/46,XX mosaicism is 10-fold higher among amniocenteses than in series of postnatally diagnosed individuals with Turner syndrome, which suggests that most individuals with this karyotype escape detection and that an ascertainment bias exists toward those with clinically evident abnormalities. The phenomenon of a milder phenotype for the prenatal group is similar to that observed for 45,X/46,XY diagnosed prenatally. Prenatal counseling for 45,X/46,XX in the absence of such ultrasound abnormalities as hydrops fetalis should take into account the expectation of a milder phenotype (except, possibly, with respect to developmental delay) than that of patients ascertained postnatally. The same does not hold true for 45,x diagnosed prenatally.
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PMID:Prenatal diagnosis of 45,X/46,XX mosaicism and 45,X: implications for postnatal outcome. 766 95

Congenital synkinetic movement of the upper eyelid is rare. In this case report, we present a 4-year-old patient with a left upper eyelid ptosis that improved on abduction and became worse on adduction. The patient also had congenital esotropia and superior rectus palsy. Strabismus surgery was performed, and excellent surgical results were achieved. A 17-mm left levator aponeurectomy and myectomy was later performed with satisfactory results. Pathology studies of the levator muscle and aponeurosis showed no evidence of inflammation, degeneration, or atrophy. Electron microscopy showed no skeletal muscle abnormality. We postulate that this congenital synkinetic movement reflects an incomplete nerve cell dying process that occurs during the embryonic phase of development.
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PMID:Congenital synkinetic movements associated with elevation of the upper eyelid. 819 70


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