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 11,717 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

Significant alcohol ingestion during pregnancy can cause a spectrum of malformation of various degrees of severity in offspring. The full expression of "fetal alcohol syndrome" includes reduced growth, facial anomalies, and mental retardation. Affected infants are usually of near-term gestation, but small in weight and length. They continue to exhibit decreased growth postnatally. Mental retardation appears to be related to the degree of dysmorphic severity of appearance. It is primarily caused by central nervous system pathology rather than social environment. The most prevalent ophthalmologic finding in our series of a short horizontal palpebral fissure appears to be due primarily to a marked increased in intercanthal distances between the medial canthi (primary telecanthus) and to less extent mild displacement of the lateral canthi. Ptosis, often asymmetric, was noted in a number of patients. Comitant convergent strabismus was present in about 50% of our cases; a few had amblyopia. An important observation was the frequent and often high degree of myopia in these children. Low-incidence anomalies include corneal opacities (Peters anomaly in one), cataract, tortuosity of retinal vessels, and long eyelashes. Our findings plus many observations in the literature establish that children with fetal alcohol syndrome are at considerable risk for a variety of eye problems.
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PMID:Fetal alcohol syndrome. 726 59

Two hundred twenty-six consecutive surgical ptosis cases were carefully evaluated to establish the incidence of strabismus in ptosis. The overall incidence of strabismus in surgical ptosis patients was found to be 36%. Most remarkable were the findings in 113 congenital ptosis patients; 32% of these patients were found to have strabismus. Only 44% of these patients had entities previously described associating strabismus with ptosis. It is postulated that in four of the congenital ptosis patients (3.5%), strabismus developed as a result of their ptosis. In one of these patients, strabismus and amblyopia developed while the patient was being observed prior to ptosis surgery. This study demonstrates the need for careful ocular motility evaluation in all patients with ptosis. Ocular motility evaluation and follow-up is especially important in congenital ptosis, where the ptotic lid may, in fact, precipitate strabismus and amblyopia.
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PMID:Strabismus in ptosis. 738 9

Amblyopia can result from strabismus, anisometropia, media opacities, and congenital disorders such as nystagmus. Complicated forms of ptosis (ie, associated with neurofibroma or hemangioma) are also known to cause amblyopia. A previously unconfirmed cause of amblyopia is uncomplicated ptosis. We carefully examined 123 consecutive surgical ptosis patients with uncomplicated congenital or early acquired ptosis. Twenty-five cases of amblyopia (20%) were seen. Four cases (3.2%) were thought to be caused by the ptosis. In two of these cases (1.6%), the amblyopia was directly attributed to the ptosis. In one of the remaining two cases, exotropia and amblyopia developed on the ptotic side while the patient was being observed for the ptosis. In another patient, a progressively increasing cylindrical refractive error and amblyopia developed, which were attributed to the ptosis. We recommend careful evaluation of cases of congenital ptosis for the detection and treatment of amblyopia.
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PMID:Amblyopia in ptosis. 738 10

The primary goal in mismanaged as well as untreated cases of combined double elevator muscle palsy and ptosis is alleviation of the paretic ocular motor imbalance to correct pseudoptosis, followed, if necessary, by levator resection to correct any residual true ptosis component. The great hypotropia often found in double elevator muscle palsy should be corrected, preferably by a muscle transposition procedure combined, in certain cases, with inferior rectus muscle recession if the inferior rectus muscle has contracted. Only in young patients can these two surgical procedures be safely combined, particularly if it is desirable to decrease the number of general anesthetics that the patient must take. Only after proper management of the paretic strabismus should the levator be resected, because, in certain cases, extraocular muscle surgery will completely abolish the upper lid ptosis.
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PMID:Surgically mismanaged ptosis associated with double elevator palsy. 745 35

Cardio-facio-cutaneous (CFC) syndrome consists of heart defects, a characteristic facial appearance, ectodermal abnormalities, growth retardation and developmental delay. The authors report two children with this condition, drawing particular attention to the neurological manifestations. The neurological features are neuro-opthalmological findings such as strabismus, ptosis and nystagmus, cortical atrophy, ventriculomegaly, mental retardation, seizures and hypotonia. These manifestations may allow differentiation of CFC from Noonan syndrome, which shares many of the physical features, but not these neurological features.
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PMID:Cardio-facio-cutaneous (CFC) syndrome: neurological features in two children. 768 72

Two siblings with typical manifestations of whistling face (Freeman-Sheldon) syndrome (WFS) born to unaffected parents are presented. In Case 1, deep-set eyes, epicanthus, blepharophimosis, right lid ptosis, strabismus, anti mongoloid slant, small mouth, mask-like face, high-arched palate, nasal speech, dysphagia, kyphosis and minimal scoliosis were noted, while Case 2 displayed blepharophimosis, mask-like face, long philtrum, high-arched palate, scoliosis, bilateral post-axial polydactyly of the feet and pes varus. We corrected the blepharophimosis in Case 1 by bilateral canthotomy and canthoplasty. This syndrome is usually inherited as an autosomal dominant trait; however, some authors have reported an autosomal an autosomal recessive form of this syndrome similar to our cases. Nevertheless, this could be explained by genetic expression of the mutant gene.
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PMID:Whistling face (Freeman-Sheldon) syndrome in two siblings. 782 40

Botulinum toxin A induced ptosis (BTXAP) has become an established method for producing a temporary ptosis for corneal protection. Adams et al1 reported from an initial series of 15 patients and observed that the ptosis lasted for a mean period of 2.5 weeks and that full recovery was achieved after a mean of 8.1 weeks. They noted that in 80% of cases there was a temporary superior rectus weakness which lasted for a mean of 6 weeks. We present three cases in which the superior rectus weakness was permanent and required corrective strabismus surgery. We believe that these are the first cases reported. We propose two possible mechanisms which may be acting together: firstly that a prolonged period of occlusion may have led to a breakdown of fusion, and secondly that following botulinum toxin induced superior rectus weakness there was contracture of the ipsilateral antagonist muscle further disrupting fusional mechanisms.
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PMID:Persisting hypotropias following protective ptosis induced by botulinum neurotoxin. 783 43

A 7 year old girl with intrachromosomal triplication 46,XX,-15,+der(15)(pter-->q13::q13-->q11::q11-->qter) resulting in tetrasomy of 15q11-q13 is reported. Fluorescence in situ hybridisation confirmed that the tetrasomic region included the entire segment normally deleted in Prader-Willi and Angelman syndrome patients, and breakpoints were similar to those reported in two tandem duplications of 15q11-q13. The middle repeat was inverted, suggesting a possible origin through an inverted duplication intermediate. Microsatellite analysis showed that the rearrangement was of maternal origin and involved both maternal homologues. Clinical findings included multiple minor anomalies (a fistula over the glabella, epicanthic folds, downward slanting palpebral fissures, ptosis of the upper lids, strabismus, a broad and bulbous tip of the nose, and small hands and feet), motor and mental retardation, a seizure disorder, and limited verbal abilities. In addition, immunological examination disclosed a selective immunodeficiency. The overall phenotype did not clearly resemble that of cases with tetrasomy 15pter-q13 associated with an extra inv dup(15)(pter-->q13:q13-->pter) chromosome. The latter aberration causes more severe mental deficit and intractable seizures, but less marked phenotypic alterations, although some overlap in mild facial dysmorphic features is present. A number of features common to Angelman syndrome were also observed in the patient.
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PMID:Intrachromosomal triplication of 15q11-q13. 783 57

As an alternative to conventional medical and surgical modalities that have met little success in the treatment of paralytic strabismus and essential blepharospasm, we explored the use of botulinum toxin as a treatment of choice in these two disorders. We used botulinum toxin in three patients with paralytic strabismus and in nine patients with essential blepharospasm. In three patients with paralytic strabismus, the botulinum toxin was injected into the ipsilateral antagonist of the paralysed muscle. The preinjection deviations ranged from 18 to 60 prism diopters. Two of these three patients achieved orthotropia around the thirtieth day and thereafter maintained it. The third patient became orthotropic on the eighteenth day, but deviation recurred and therefore required another injection of toxin. In nine patients with essential blepharospasm, botulinum toxin was injected into the orbicularis oculi muscles. Both objective and subjective improvement occurred in all nine patients within seven days and the effect lasted 12 to 15 weeks. Further injection of the toxin produced extremely beneficial results. However, the only significant complication that we encountered in both groups of strabismus and blepharospasm was ptosis, which was usually partial and temporary. From our experience, we advocate the use of botulinum toxin in the treatment of essential blepharospasm.
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PMID:Botulinum toxin in the treatment of paralytic strabismus and essential blepharospasm. 812 43


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