Botulinum toxin in the management of blepharospasm.

Seventy-seven patients with blepharospasm unresponsive to other forms of therapy were treated with botulinum A toxin. The drug was injected into the orbicularis oculi muscle of both upper and lower eyelids and, in some patients, into the brows and upper part of the face as well. A total of 192 treatment sessions were given, with follow-up ranging from four weeks to 18 months. Orbicularis muscle spasms decreased markedly over the first five days following injection, both in patients with essential blepharospasm and in those with hemifacial spasm. Beneficial effect was temporary, however, and repeated injections were required for sustained relief. Fifty-two patients received multiple treatments, ranging from two to eight injections each, with a mean duration of benefit of 11.8 weeks. Results of subsequent injections were similar to the first in rapidity of onset, degree of benefit, and duration of effect. Complications were local, mild, and transient, and no systemic side effects were encountered.

Botulinum toxin for blepharospasm: single-fiber EMG studies.

In four patients who received periocular injections of botulinum toxin for blepharospasm, abnormal neuromuscular transmission was demonstrated by single-fiber EMG in arm muscles. The time course with which the abnormalities developed and cleared, as well as the inverse relationship between the neuromuscular jitter and the firing rate in the abnormal muscles, indicated that the toxin caused the abnormalities in arm muscles. No weakness was detected clinically in muscles distant from the face, but the abnormal neuromuscular transmission indicates that the toxin spread remotely from the site of injection.

Reliability of brightness comparison testing in predicting afferent pupillary defects.

We tested the reliability of brightness comparison testing in predicting relative afferent pupillary defects (RAPDs) in 206 patients. For unselected patients, brightness comparison testing correctly predicted the RAPD in 62% of patients. For subgroups of patients with glaucoma and optic nerve lesions, correct predictions were made in 92% and 83% of patients, respectively. Sensitivity and specificity of brightness comparison testing with a 6-V test light were 85% and 49%, respectively. In subgroups of patients who are strongly suspected of having an RAPD but in whom pupillary testing is not possible, brightness comparison testing can reliably predict presence or absence of an RAPD.

Cataract surgery and intraocular lens implantation in patients with retinoblastoma.

OBJECTIVE: To report the visual outcome and complications after extracapsular cataract extraction with posterior chamber intraocular lens (IOL) implantation in eyes that underwent external beam radiotherapy for the treatment of retinoblastoma. METHODS: Eleven eyes of 8 patients aged 1.5 to 8.0 years at the time of surgery for irradiation-induced cataract were observed for 6 to 39 months (mean [+/-SD], 20+/-10 months). A standard technique of extracapsular cataract extraction, posterior chamber IOL implantation, pars plana posterior capsulotomy, and pars plana anterior vitrectomy was performed in 9 eyes, and secondary IOL placement was performed in 2 eyes. RESULTS: All eyes that underwent primary IOL implantation achieved visual acuities equal to or better than those best ever recorded prior to the development of the cataract. There were no postoperative complications, and all eyes experienced minimal postoperative inflammation. The 2 eyes that underwent secondary IOL implantation achieved their best-corrected aphakic visual acuities but experienced a prolonged course of postoperative inflammation. CONCLUSION: Intraocular lens placement in the posterior chamber of eyes with regressed retinoblastoma and irradiation-induced cataract seems to be a safe and effective method for the correction of aphakia.

Posttraumatic venous obstructive retinopathy associated with enlarged optic nerve sheath.

A case of posttraumatic unilateral enlargement of the optic nerve sheath associated with a venous obstructive retinopathy and progressive visual loss responded with marked improvement in visual function after nerve sheath decompression. Indirect (closed-head) trauma may be related to the progressive nerve sheath enlargement, venous obstructive retinopathy, and progressive visual loss. It is apparent that a venous stasis retinopathy can result from a posttraumatic optic nerve sheath lesion. Prompt recognition by echographic and/or roentgenographic investigation and subsequent decompression may be curative.

The case of the gray optic disc!

A child with interstitial deletion of chromosome 17, right optic nerve hypoplasia, and impaired speech had bilateral congenital optic disc pigmentation, giving the appearance of gray optic discs. This appearance did not change over a period of two-and-a-half years. Previous cases of gray optic discs in neonates have been notable for 1) absence of visible pigmentation within the optic discs, 2) resolution of the gray discoloration over a period of months, and 3) development of albinotic features in several infants. This suggests that congenital optic disc pigmentation is a rare cause of gray optic discs. Congenital optic disc pigmentation is compatible with good vision.

A simple transposition procedure for complicated strabismus.

We combined a recession or resection of recti muscles with a vertical or horizontal transposition to correct a complicated paralytic ocular deviation in eight patients. The transposed muscles were reattached to the globe parallel to the spiral of Tillaux and adjacent to the paralyzed muscle. Postoperatively, seven patients demonstrated fusion in the primary position or required a slight head turn to fuse. There were no surgical complications, and no patient developed symptomatic cyclotropia, diplopia, or anterior segment ischemia.

Fadenoperation for the management of complicated incomitant vertical strabismus.

We reviewed retrospectively the records of 17 patients with incomitant vertical diplopia secondary to an underacting inferior rectus muscle who had been treated by surgically weakening the contralateral inferior rectus muscle with a posterior fixation suture (fadenoperation). This was the sole procedure in four patients with blowout fractures and in two patients with restriction secondary to scleral buckling procedures. It was combined with horizontal or vertical muscle surgery in 11 patients. The procedure was particularly useful in preventing diplopia on downgaze after excessive weakening of a tight inferior rectus muscle in seven patients with thyroid ophthalmopathy. In three patients it was used in anticipation of an iatrogenic incomitancy in downgaze secondary to a large recession of a tight inferior rectus muscle. All patients were markedly improved after surgery and no complications were encountered.

Secondary posterior chamber intraocular lens implantation in pediatric patients.

PURPOSE: To report results of secondary posterior chamber intraocular lens (IOL) implantation in previously aphakic pediatric patients. METHODS: In 19 pediatric patients, 19 aphakic eyes (11 after infantile and eight after traumatic cataract surgery) received secondary sulcus-fixated posterior chamber IOL implants. RESULTS: Visual acuity of 20/40 or better was achieved with IOL implantation and overrefraction in three of 11 infantile (27%) and six of eight traumatic cataract patients (mean follow-ups, 18.1 months [range, 8 to 29 months] and 18.0 months [range, 6 to 28 months]), respectively. Eighteen of 19 patients (95%) demonstrated postoperative vision equal to or better than preoperative levels; 15 of 19 patients (79%) showed improved vision after IOL implantation. The mean +/- SD difference between actual and predicted postoperative refraction at 1 month was -0.97 +/- 0.96 diopter. Average refractive error at last examination was -0.40 +/- 2.43 diopters. Amblyopia therapy was performed in 14 patients. One IOL required repositioning 8 months postoperatively. Strabismus was present in 14 patients before and 13 patients after IOL implantation, requiring surgery in four patients. CONCLUSIONS: Secondary IOL implantation can be performed successfully in carefully selected pediatric patients. Visual acuity results are better in eyes with a history of traumatic cataract and are influenced by patient compliance. The short-term risks of the procedure appear no greater than those of primary IOL implantation, and complications resemble those seen in adults.

Treatment of blepharospasm with botulinum toxin.

In 43 patients (81 eyes) with blepharospasm resistant to other forms of therapy, 149 outpatient injections of botulinum A toxin were given into the orbicularis oculi muscle. Subsequent follow-up periods ranged from ten to 210 days. Orbicularis oculi spasm, eyelid forced closure, and eyebrow spasm all decreased substantially over the first ten days following the initial injection. Patients with essential blepharospasm, hemifacial spasm, or previous surgery all responded in a similar fashion. This beneficial effect was transient, however, and additional treatment was required for sustained relief. Twenty-five patients received multiple injections with a mean interval of 65 days. Results of second and third injections were similar to the first in rapidity of onset and duration of effect. Complications were local, mild, and transient, and no systemic side effects were noted.

Management of the posterior capsule during pediatric intraocular lens implantation.

One of the major obstacles in pediatric intraocular lens implantation has been the subsequent dense opacification of the posterior capsule. We used a modification of the standard pediatric cataract surgical procedure, which involved endocapsular cataract extraction, posterior chamber intraocular lens implantation, pars plana posterior capsulotomy, and pars plana anterior vitrectomy in 20 consecutive patients with unilateral traumatic, radiation-induced, and developmental cataracts. Visual axes were rapidly restored in all patients without further intervention for posterior capsule opacification. Visual acuity returned to 20/40 or better in all patients and 75% of all patients (15 patients) reached maximum improvement by five weeks. No complications attributed to intraoperative removal of the posterior capsule occurred.

Refractive changes after pediatric intraocular lens implantation.

PURPOSE: To report refractive changes after cataract surgery and intraocular lens implantation in infants and children. METHODS: In an ongoing prospective study, the refractive errors of all patients younger than 18 years undergoing intraocular lens implantation were determined at 6 weeks, 3 months, 6 months, and 1 year, and at least yearly thereafter. All patients with greater than 6 months of follow-up were included in the study. RESULTS: Eighty-three eyes of 81 patients were identified. Cataracts were traumatic in 32 eyes (38%) and developmental in 42 eyes (50%). At implantation, the mean (+/-SD) age was 6.3 +/- 4.6 years (range, 9 months to 17 years). The mean follow-up was 26.6 months (range, 6 months to 6.6 years). Patients 0 to 2 years old at the time of implantation demonstrated a mean myopic shift of -3.00 diopters during a mean follow-up period of 2.5 years. Patients 2 to 6 years old at the time of implantation demonstrated a mean myopic shift of -1.50 diopters in a similar follow-up period. Children aged 6 to 8 years experienced a mean myopic shift of -1.80 diopters during a mean follow-up period of 3.0 years, while children older than 8 years at the time of intraocular lens implantation experienced a mean myopic shift of -0.38 diopters during a mean follow-up period of 1.8 years. On average, the operated-on eye showed a greater mean myopic shift than the fellow eye. No statistically significant differences in refractive change were found in comparing amblyopic to nonamblyopic eyes, traumatic to nontraumatic cataracts, or primary to secondary intraocular lenses. CONCLUSIONS: Our data demonstrate a trend toward increasing postoperative myopia in pediatric patients undergoing intraocular lens implantation. This myopic shift is greatest in the younger age groups and persists until at least 8 years of age. There is much variability in the postoperative refractive changes, and predicting exactly when and where the refraction will stabilize for an individual patient is difficult.

Magnetic resonance imaging of optic gliomas.

We compared magnetic resonance imaging of the optic nerves and chiasm with computed tomography in four patients (a 17-year-old girl, a 14-year-old boy, a 13-year-old girl, and a 22-year-old woman) with biopsy-proven or suspected optic gliomas. Orbital abnormalities were effectively viewed by both techniques. Gliomatous and normal optic nerves had similar spin-echo magnetic resonance imaging characteristics. Magnetic resonance was superior to contrast-enhanced computed tomography for identification of the intracranial optic nerves, chiasm, and optic tracts, but computed tomography delineated orbital anatomic and spatial relationships better.

Leptomeningeal dissemination of optic pathway gliomas in three children.

We treated three children with optic pathway gliomas who had progressive disease associated with metastatic spread to the leptomeninges. One patient had radiographic resolution of leptomeningeal disease after treatment with intravenous carmustine and oral mercaptopurine but died of progressive pulmonary fibrosis. The second patient was treated with intravenous thiotepa, and the leptomeningeal disease remained stable. The third patient was treated with intravenous vincristine sulfate, cyclophosphamide, cisplatin, and etoposide and had a significant size reduction of the leptomeningeal lesion. Although leptomeningeal dissemination is a seemingly rare event, it is important that all children with optic pathway gliomas be considered for this possibility, particularly after the onset of new, atypical neurologic symptoms.

Use of latanoprost in the treatment of glaucoma associated with Sturge-Weber syndrome.

PURPOSE: To determine if latanoprost reduces intraocular pressure in eyes with glaucoma associated with Sturge-Weber syndrome. METHODS: We conducted a prospective study in which eyes with uncontrolled intraocular pressure associated with Sturge-Weber syndrome were treated with latanoprost 0.005% once daily. All eyes were already receiving at least two other antiglaucoma medications. Intraocular pressure was measured at baseline and after treatment for at least 1 month. All intraocular pressure measurements were taken within 24 hours of drug instillation. RESULTS: Six eyes of six patients received latanoprost. Two (28%) of the six eyes demonstrated an intraocular pressure decrease that averaged 8.8 mm Hg. These two responders had juvenile onset glaucoma, whereas the four nonresponders had congenital onset glaucoma. CONCLUSIONS: Latanoprost may significantly reduce intraocular pressure in selected patients with glaucoma associated with Sturge-Weber syndrome.

New and recurrent tumors in germinal retinoblastoma: is there a treatment effect?

Patients with germinal retinoblastoma (those with bilateral disease or positive family history) have a mutation which puts them at risk for developing new tumors. It is unclear whether the frequency of new tumor development is effected by the type of treatment employed. It may be hypothesized that external beam radiation "sterilizes' the whole retina, and thus decreases the risk of new and recurrent tumors. We reviewed our experience with 66 eyes in 47 patients over the past ten years. We did not find a significant difference in the incidence of new and recurrent retinoblastoma among eyes treated with external beam radiation versus focal modalities.

Optotype acuity and re-operation rate after unilateral cataract surgery during the first 6 months of life with or without IOL implantation.

AIMS: To compare optotype acuities and re-operation rates in children corrected with a contact lens (CL) compared with an intraocular lens (IOL) following unilateral cataract extraction during infancy in a non-randomised, retrospective case series. METHODS: 25 infants with a unilateral congenital cataract underwent cataract surgery with (IOL group, n = 12) or without (CL group, n = 13) IOL implantation when <7 months of age. Optotype acuities were assessed in 19 of these children at a mean age of 4.3 years (range 3.3-5.5 years). The number of re-operations were assessed in 21 children. RESULTS: The visual acuity results were similar in the two treatment groups (p = 0.99); however, two of the four (50%) children in the IOL group compared with two of the seven (28%) children in the CL group undergoing surgery during the first 6 weeks of life had 20/40 or better visual acuity. The children in the IOL group had more re-operations than the children in the CL group (mean 1.1 v 0.36). Most of the re-operations in the IOL group were membranectomies performed during the first year of life (median 8.0 months) whereas all of the re-operations in the CL group were the implantation of a secondary IOL later in childhood (mean 2.2 years). CONCLUSION: Optotype acuities were similar for the children corrected with a CL compared with IOL, while the children in the IOL group underwent more re-operations .

Strabismus after retinal detachment surgery: etiology, diagnosis, and treatment.

Between 5% and 25% of patients may experience persistent diplopia after surgery for retinal detachment. The complexity of the presentation poses a distinct challenge to both the retinal and the strabismus surgeon. Careful evaluation to determine factors contributing to the strabismus and assessment of fusional capabilities are essential before treatment. A combination of the appropriate surgical approach with nonsurgical adjuncts such as prisms or botulinum toxin is often successful in relieving symptoms.

Scleral fixated (sutured) posterior chamber intraocular lens implantation in children.

BACKGROUND: Optical rehabilitation of unilateral aphakia in eyes with no capsular support is problematic in pediatric patients who cannot tolerate contact lenses. Possible options include a unilateral aphakic spectacle, an anterior chamber intraocular lens (IOL), or a scleral fixated posterior chamber IOL. Of these choices the posterior chamber IOL is the most physiologic. Experience in adults shows increased complications with this technique. OBJECTIVE: The objective of this study was to report the short-term results and complications of unilateral scleral fixated posterior chamber IOLs in the pediatric population. METHODS: All patients with scleral fixated lenses younger than 16 years were retrospectively reviewed. Nine patients aged 12 months to 15 years underwent unilateral scleral fixated posterior chamber lens implantation using buried polypropylene fixation sutures. Follow-up averaged 24 months. RESULTS: Postoperative visual acuity improved in all patients. Refractive goals were achieved in all but 1 patient. Complications included elevated intraocular pressure controlled with medications (1 patient), anterior uveitis (1 patient), and mild IOL decentration (1 patient). CONCLUSIONS: Although short-term visual results appear encouraging, this procedure is technically more difficult and has an increased incidence of postoperative complications when compared with secondary sulcus-fixated IOLs supported by capsular remnants. Caution should be exercised when recommending this procedure for pediatric patients because long-term risks are unknown.

Topical versus oral carbonic anhydrase inhibitor therapy for pediatric glaucoma.

PURPOSE: Our purpose was to compare, in a crossover design,the hypotensive effect of oral acetazolamide (Diamox) and topical dorzolamide (Trusopt) in patients with pediatric glaucoma. METHODS: All patients less than 18 years old who were switched from acetazolamide to dorzolamide without other intervention were reviewed. Intraocular pressures were obtained with either a Tono-Pen (Mentor Ophthalmics, Santa Barbara, Calif.) or applanation tonometer. Minimum follow-up times on acetazolamide and on dorzolamide were 1 month (mean 12.2 +/- 19.7 months) and 2 months (mean 8.2 +/- 5.1 months), respectively. The average dose of acetazolamide was 9.9 +/- 1.8 mg/kg/day. RESULTS: Eleven eyes (11 patients) were included. Indications for crossover from oral to topical carbonic anhydrase inhibitor (CAI) therapy were intolerance to acetazolamide (6 eyes) and surgical intervention in the fellow eye (5 eyes). The mean age at the time of crossover was 7.4 +/- 3.0 years. A comparison of intraocular pressure (IOP) before addition of a CAI was made in 8 eyes. The mean IOP off of a CAI was 27.8 +/- 4.9 mm Hg. The mean 10P was reduced to 18.5 +/- 4.3 mm Hg on acetazolamide (mean percent IOP reduction 35.7% +/- 15.6%, p < 0.01) and to 22.2 +/- 5.4 mm Hg on dorzolamide (mean percent IOP reduction 27.4% +/- 17.1%, p < 0.01). All 11 eyes showed an increase in IOP when switched from acetazolamide to dorzolamide, with a mean increase of 3.7 +/- 2.5 mm Hg (20.2% -/+ 13.7%, p < 0.01). Five eyes have remained controlled on dorzolamide and a topical beta-blocker. Five eyes required further intervention for the control of glaucoma. One eye was switched back to acetazolamide for better IOP control. CONCLUSION: Although not as effective as oral acetazolamide, topical dorzolamide causes a significant IOP reduction in this group of pediatric glaucoma patients and appears to be well tolerated.