Restrictive strabismus after ocular surgery for retinitis pigmentosa in Cuba.
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Biomedical subjects
Publications and source records attributed to R W Hertle.
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PURPOSE: Our purpose was to establish whether comitance was a common or uncommon finding in children with esodeviation associated with a neurologic insult. METHODS: A retrospective chart review was performed of children with acquired esodeviation associated with an identifiable neurologic insult. RESULTS: Examinations of 30 children seen over a 2-year period were analyzed. Twenty-two (73%) had brain tumors. Twelve (40%) had comitant esodeviation, and the other 18 (60%) had incomitant measurements. Of the patients with comitant esodeviation, 6 had normal abduction OU (two after recovery from bilateral sixth nerve palsies), and 6 had mild or minimal abduction deficits. Moderate or severe abduction deficits were associated with incomitant measurements. CONCLUSIONS: Comitant esodeviation can be common in children with identifiable neurologic insults.
Ophthalmic genetics is a rapidly expanding field that includes both clinical and basic sciences. Clinical descriptions of syndromes and new diagnostic testing both reflect the increase in knowledge and technology of molecular biology. Technologic advances have resulted in old diseases being described in new ways and being attributed to new pathophysiologic mechanisms. This review is intended to update the past year's contributions in ophthalmic genetics. Discussion is by disease type, which is familiar to most clinicians.
BACKGROUND: Penetrating keratoplasty in infancy and childhood has traditionally met with limited visual success due to a combination of unique physiology and technical problems in this patient population. With the advances in microsurgical instrumentation, corneal preservation, and visual developmental physiology ophthalmologists are finding increasing indications for penetrating keratoplasty in the childhood population. The long term results of neonatal penetrating keratoplasty in two patients with unilateral congenital corneal opacification are reported. METHODS: Penetrating keratoplasty was performed on one eye in each of two infants within the first 3 weeks of life. Amblyopia treatment and optical therapy have been continued since surgery. RESULTS: After 6 years both grafts have remained clear. One patient developed the infantile esotropia syndrome. Visual development using Snellen optotypes is age normal for both transplanted eyes. CONCLUSIONS: Penetrating keratoplasty when combined with optical correction and amblyopia therapy may restore and preserve vision in selected patients with congenital corneal opacification if performed in the neonatal period.
Although ophthalmic involvement in developmental and genetic abnormalities has been recognized for centuries, it is only recently, due to new discoveries in molecular biology, that our classification and pathophysiology of congenital eye diseases have been advanced. In the next few years these advances will increasingly influence aspects of diagnosis classification, and therapy in ophthalmology. Tests for acquired and inherited diseases in opthalmology are being revolutionized by biotechnical innovations. The molecular genetics of ocular diseases is currently undergoing changes in classification due to intense investigations using advanced biotechnology. The potential for new treatment modalities, such as gene therapy, for molecular eye diseases, once thought to be pure fiction, is now a reality. This paper will outline recent changes in classification, organization, and general knowledge regarding inherited eye diseases.
The differentiation of congenital motor nystagmus (CMN) from sensory defect nystagmus (SDN) in early life is a common diagnostic problem, since their wave forms are indistinguishable. A diagnosis of SDN is made when fundal or ERG abnormalities are present. CMN is generally diagnosed by exclusion. This approach can lead to late or incorrect diagnosis, and to the overlooking of important genetic implications. In addition, the classification of congenital nystagmus into pure motor and sensory types is inadequate. The authors present case reports illustrating this problem and suggest strict criteria for a diagnosis of CMN. They also point out that a miscellaneous group of rare disorders of the macula tend to be overlooked and misdiagnosed.
OBJECTIVE: Iridocyclitis has been identified as a dosage-dependent side effect in patients with the acquired immunodeficiency syndrome (AIDS) who are treated for Mycobacterium avium complex (MAC) infection with systemic rifabutin. We reviewed cases of acute hypopyon uveitis occurring in patients with AIDS to establish whether there was an association. DESIGN: Retrospective case series. SETTING: Outpatient clinic and inpatient hospital-based ophthalmology referral practice and infectious disease specialty service. PATIENTS: Seven patients with AIDS, aged 10 to 40 years, presenting with acute unilateral hypopyon mimicking infectious endophthalmitis. MAIN OUTCOME MEASURES: Findings from complete ophthalmological evaluation and ancillary laboratory testing. RESULTS: At the time of presentation, all seven patients were receiving treatment for MAC infection with rifabutin (dosage range, 300 to 600 mg/d) and clarithromycin. Results of microbiological investigations in five patients were negative. Iridocyclitis became bilateral in all seven patients, and hypopyon developed in the contralateral eye in five of seven patients. Hypopyon resolved rapidly with intensive topical corticosteroid therapy. Residual inflammation responded to topical corticosteroids with or without reduction of the rifabutin dosage. CONCLUSIONS: Concomitant use of rifabutin, clarithromycin, and fluconazole may precipitate hypopyon uveitis in patients with AIDS being treated for MAC infection.
We determined laser parameters and delivery system requirements for successful use of the potassium-titanyl-phosphate (KTP) laser for strabismus surgery and its histopathologic effect on extraocular muscle, tendon, sclera, and cornea of human and rabbit eyes. Enucleated cadaver eyes and exenterated rabbit orbital contents were used. Using a 200-microns fiberoptic tip delivery system, varying laser energies were used to perform muscle insertion site and corneal damage, tenotomies, and myotomies. Pulse durations of 0.5 second and energies above 0.8 watt were needed for tenotomy or myotomy. No damage to the surrounding tissues or sclera was observed with laser tenotomy or myotomy performed 0.5 mm from the insertion site. Energy above 0.8 watt applied directly caused full thickness disruption. In comparison to scissor myotomy, the laser provided charring of the superficial tissues. This study shows that the KTP laser can be safely and accurately used for extraocular muscle tenotomy or myotomy.
We studied ten children with central visual impairment with a known neurologic defect and an abnormal visual-evoked response who had results of repeat electrophysiologic testing evaluated at the Children's Hospital of Philadelphia from December 1989 through July 1991. Central visual impairment is defined as poor visual function with a normal anterior visual pathway. Age at first examination ranged from 5 to 48 months with a followup of two to 31 months. Repeat visual-evoked response testing showed improvement in seven patients and no change in three. Grating acuity as measured by the Teller acuity card procedure, performed in nine of ten patients, improved in seven, showed no change in one, and declined in one. We found a potentially favorable prognosis for those infants with central visual impairment, despite an initially abnormal visual-evoked response. We used a clinical approach to this disorder to improve diagnostic categorization and prognostic capabilities in central visual impairment.
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The ocular findings and visual prognosis were reviewed in 24 patients with the Treacher-Collins syndrome who were evaluated in the craniofacial clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between 1980 and 1991. All patients had some abnormality. Vision loss was present in 37% of patients. Amblyopia was present in 33%, significant refractive errors were present in 58%, and anisometropia was documented in 17%. Strabismus was present in 37% and significant lid and adnexal abnormalities were seen in 96%. The prognosis for normal vision in at least one eye is good but vision loss secondary to amblyopia is more resistant to treatment owing to other medical problems and social concerns.
The authors report four cases of the rarest form of the congenital fibrosis syndrome. This disorder is exhibited in infancy as unilateral blepharoptosis, strabismus, limited ductions, globe displacement (enophthalmos and blepharoptosis), and decreased vision, usually due to amblyopia. Forced ductions are positive and surgical exploration confirms anomalous muscle structure. Computed tomography and magnetic resonance imaging studies in these four patients were diagnostically beneficial, showing extraocular muscle and tendinous insertion involvement, and poorly defined intraconal and extraconal masses that had the appearance of scar or inflammatory tissue. All patients had globe displacement. The opposite eye and intracranial contents were normal in all of our patients. Results of histopathologic examination obtained at surgery in three of these patients show replacement of affected structures by fibrous tissue and included the extraocular muscles, orbital fat, Tenon's capsule, and conjunctiva.
The authors reviewed the ocular findings in 49 patients with facial microsomias not considered part of a known syndrome. Five patients had bifacial microsomia, and 44 patients had hemifacial microsomia. These patients were evaluated in the Craniofacial Clinic in the Division of Pediatric Ophthalmology at Children's Hospital of Philadelphia between the years 1979 and 1989. Ocular or adnexal abnormalities were present in 67% of patients. Visual loss was present in 8% of eyes. Amblyopia was present in 16% of patients. Significant refractive errors were present in 27% of patients, and anisometropia was documented in 8%. Strabismus was present in 22% of patients, and significant lid and adnexal abnormalities were seen in 41%. Because ocular or adnexal findings present in a significant percentage of patients with more mild forms of facial microsomias, regular examinations to uncover and treat these abnormalities are recommended.
Chorioretinal folds have been reported as a result of many intraocular and extraocular inflammatory processes or tumors. Visual loss is usually secondary to a combination of the underlying process and chorioretinal folds involving the macula. We report a patient who developed decreased vision, metamorphopsia, chorioretinal folds, and a lamellar macular hole secondary to global compression by a bone fragment. The chorioretinal folds regressed and his vision stabilized following surgical decompression. Chorioretinal folds and lamellar macular hold formation are previously unrecognized complications of reconstructive craniofacial surgery.
We used electrooculography to study the saccadic velocities, smooth pursuit, and vestibular ocular reflex in a patient with an acute thalamic hemorrhage. Our findings confirmed what others have shown in that there were hypometric saccades contralateral to the lesion, impaired smooth pursuit ipsilaterally, and a preserved vestibular ocular reflex. In addition, we demonstrated an asymmetry with the contralateral eye being more affected. It is also shown that the "convergence" movements seen on attempted upgaze are typical of saccades and not vergence movements. A discussion of possible pathophysiologic mechanisms with review of other studies is presented.
We report a 10-year-old white girl who developed a pinealoblastoma 2 years before presenting to us with metastatic spread to the left optic nerve. She was treated for the original tumor with irradiation and chemotherapy. She presented to us with decreased vision in the left eye, unilateral disk edema, and diffuse left optic nerve swelling without increased intracranial pressure. Biopsy of the posterior optic nerve sheath was required for diagnosis. Vision was lost in the left eye, but the optic nerve swelling diminished after chemotherapy and additional radiation therapy.
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