Outcomes after laser therapy for threshold retinopathy of prematurity.

OBJECTIVE: To determine the anatomic outcomes of eyes treated with laser photocoagulation for threshold retinopathy of prematurity and to identify potential risk factors for unfavorable outcomes after treatment. DESIGN: Retrospective, noncomparative case series. INTERVENTION: Photocoagulation of the peripheral avascular retina with an argon or diode laser indirect ophthalmoscope. PARTICIPANTS: One hundred twenty eyes of 81 infants with threshold retinopathy of prematurity treated with laser photocoagulation from 1989 through 1997 with at least 12 months of follow-up after treatment. MAIN OUTCOME MEASURES: The principal outcome was the presence of an unfavorable anatomic outcome defined as a retinal detachment, macular fold, or retrolental tissue. RESULTS: One hundred nine of 120 eyes (91%) had favorable outcomes. Eleven eyes (9%) had retinal detachments, and 1 of the 11 also had retrolental tissue. Zone 1 eyes appeared to be 3.3 times more likely to have an unfavorable outcome compared with zone 2 eyes, but the 95% confidence interval (0.8-14.5) did not support this statistically. Twenty-four of 109 eyes (22%) experienced dragging of the temporal vessels. Those with zone 1 disease were 13.7 times more likely to experience temporal dragging compared with zone 2 eyes (95% confidence interval, 3.3-57.2). CONCLUSIONS: After laser photocoagulation for threshold retinopathy of prematurity, 91% of eyes had a favorable anatomic outcome. Compared with zone 2 eyes, zone 1 eyes may be more likely to have temporal dragging of the retinal vessels. Laser therapy is effective in the treatment of threshold retinopathy of prematurity.

Diode laser photocoagulation for retinopathy of prematurity: a histopathologic study.

Laser photocoagulation has largely supplanted cryotherapy as an effective treatment for retinopathy of prematurity. This case describes the ocular histopathologic findings of a pair of eyes in a severely premature male infant treated with diode laser photocoagulation for bilateral stage 3 retinopathy of prematurity (ROP) for 360 degrees in zone 1 with severe plus disease. The right eye responded to treatment; the left eye developed persistent vitreous hemorrhage and total retinal detachment. The histopathologic examination of laser burns in the right eye disclosed segmental areas of chorioretinal scarring with retinal atrophy and gliosis, loss of RPE and extensive atrophy of the choroid and its vasculature, which involved both the choriocapillaris and larger vessels. The left eye had iris neovascularization, a chronic organized vitreous hemorrhage and a totally detached retina. The histopathologic findings in an eye of a premature infant with threshold ROP treated with diode laser photocoagulation resembled those reported after transsceral cryotherapy. Diode laser photocoagulation may produce less severe chorioretinal damage.

Surgical repair of rhegmatogenous retinal detachment after treatment for retinoblastoma.

OBJECTIVE: This study aimed to evaluate the causes of rhegmatogenous retinal detachment in children with retinoblastoma and to assess the surgical approach and results of retinal reattachment surgery. DESIGN: The study design was a retrospective review of all children in whom a rhegmatogenous retinal detachment developed after treatment for retinoblastoma over a 20-year period. PARTICIPANTS: Nine children, 5 to 33 months of age, participated. MAIN OUTCOME MEASURES: Assessment of the treatment methods for retinoblastoma, apparent cause of the rhegmatogenous retinal detachment, interval from the most recent retinoblastoma treatment until repair of the rhegmatogenous retinal detachment, method of surgical repair, and long-term anatomic and visual results were measured. RESULTS: Rhegmatogenous retinal detachment was diagnosed in nine eyes of nine children with treated retinoblastoma. Prior treatment of retinoblastoma included cryotherapy (n = 5), laser photocoagulation (n = 1), external beam radiation therapy (n = 7), and systemic chemotherapy (n = 4). An atrophic retinal hole was present at the most recent site of cryotherapy in three eyes and of laser photocoagulation in one eye. Extreme retinal thinning with a possible retinal break was noted in two eyes at a cryotherapy site. A dialysis was noted in one eye, and a retinal break could not be detected in two eyes. Treatment of retinoblastoma tumors was performed between 1 week and 15 years before retinal reattachment surgery. The retinal detachment was repaired externally with a scleral buckling procedure in seven eyes, with subretinal fluid drainage in five eyes, and without drainage in two eyes. Cytologic evaluation of subretinal fluid was performed in three eyes and showed no tumor cells. The retinal detachment was repaired by pars plana vitrectomy in one eye, and the vitrectomy specimen showed no tumor cells. In one child, the retinal detachment was not repaired. The retina remained attached after surgery in five eyes with follow-up ranging from 5 to 115 months. In three eyes, retinal redetachment associated with recurrent viable intraocular retinoblastoma was noted after surgery, necessitating enucleation. In no case was orbital or metastatic retinoblastoma detected at follow-up. CONCLUSIONS: Rhegmatogenous retinal detachment in children with treated retinoblastoma generally originates from an atrophic retinal hole at the site of prior cryotherapy or laser photocoagulation. If there appears to be complete regression of the intraocular tumor after treatment, surgical repair of the rhegmatogenous retinal detachment may be approached cautiously.

Early treatment of posterior retinopathy of prematurity: a controlled trial.

OBJECTIVE: The purpose of the study is to assess the possible benefits of early laser treatment for posterior retinopathy of prematurity (ROP) and to provide data concerning the natural history of posterior ROP. DESIGN: The study design was a prospective, multicenter, randomized trial. PARTICIPANTS: A total of 19 infants with prethreshold posterior ROP were studied. INTERVENTION: Randomization to immediate indirect laser photocoagulation or observation, with application of laser photocoagulation for those control eyes reaching threshold disease, was performed. MAIN OUTCOME MEASURES: Patients were assessed at 3 months and the anatomic outcome recorded along with any adverse treatment effects. RESULTS: An unfavorable structural outcome developed in 3 (16%) of 19 early treatment eyes compared with 3 (18%) of 17 for those treated only if threshold disease was reached. Of the 17 control eyes, 15 (88%) reached threshold disease. Progression to threshold occurred within 1 week in all but two eyes. All 12 control eyes with posterior ROP and any amount of extraretinal fibrovascular proliferation progressed to threshold disease. CONCLUSIONS: Although the number of patients studied is too small to reach statistical significance, the likelihood of a favorable outcome for eyes with prethreshold posterior ROP treated immediately with laser photocoagulation is comparable to that obtained by withholding treatment until threshold disease is reached. There is a high probability of progression from prethreshold to threshold disease, usually within 1 week or less.

Peripapillary staphyloma.

PURPOSE: To report a rare case of peripapillary staphyloma. METHOD: Case report. A 5-month-old infant was examined for esotropia associated with a retinal and optic nerve abnormality of the left eye. RESULTS: Detailed clinical examination along with diagnostic ultrasonography and electroretinography established a diagnosis of a peripapillary staphyloma of the left eye measuring 9.77 mm in depth. The patient's right eye was entirely normal, and she showed no systemic abnormalities. CONCLUSION: Although extremely rare, the peripapillary staphyloma may cause severe visual impairment of the affected eye in an otherwise healthy individual unassociated with systemic abnormalities.

Familial exudative vitreoretinopathy associated with nonneovascular chronic angle-closure glaucoma.

PURPOSE: Cases of angle-closure glaucoma in patients with familial exudative vitreoretinopathy have been reported secondary to neovascularization of the anterior segment. Cases secondary to nonneovascular mechanisms have not been previously reported. METHODS: Two cases are presented of angle-closure glaucoma as a result of a nonneovascular mechanism. RESULTS: Neovascularization was found to be a very unlikely explanation for the angle closure in these two cases. CONCLUSION: There may be an association between familial exudative vitreoretinopathy and angle-closure glaucoma as a direct result of a retrolental process or more likely a relative lens-iris pupillary block with a large lens.

PCR assay confirms diagnosis in syndrome with variably expressed phenotype: mutation detection in Stickler syndrome.

Stickler syndrome is an autosomal dominant disease with ocular (severe myopia, vitreal degeneration, and retinal detachment) and other systemic manifestations (hearing loss, cleft palate, epiphyseal dysplasia, and premature osteoarthritis). As with other dominantly inherited conditions, the clinical phenotype of Stickler syndrome varies considerably. To date, all mutations have been located in the type II procollagen (COL2A1) gene. Analysis of a C-->T mutation we had identified previously, in COL2A1 gene in exon 40, in a three generation pedigree showed the loss of a cleavage site for the TaqI restriction enzyme. We designed a rapid PCR based restriction enzyme assay to detect this mutation and used it to establish the diagnosis in a neonate from the same pedigree, presenting with the first occurrence of the Pierre-Robin sequence in the family and minimal ocular findings. These results underline the potential diagnostic value of many as yet undetected DNA mutations in families affected with Stickler syndrome, since the variability of the phenotype can impede accurate diagnosis, appropriate genetic counselling, and effective intervention and prophylactic treatment for affected people.

Stickler syndrome. A mutation in the nonhelical 3' end of type II procollagen gene.

BACKGROUND: All of the mutations in the type II procollagen (COL2A1) gene that have been identified in families affected with Stickler syndrome have been located primarily in the triple helical region of the gene. We report what we believe is the first premature stop codon in the globular C-propeptide region encoded by the COL2A1 gene, in a family affected with Stickler syndrome. DESIGN: Genomic DNA from affected and unaffected family members of this three-generation family was amplified using the polymerase chain reaction. The polymerase chain reaction products were directly sequenced for DNA analysis. RESULTS: Direct sequencing showed a single base deletion in exon 50, resulting in a premature stop codon in exon 51 in the globular C-propeptide of COL2A1 gene in all affected members. CONCLUSIONS: These results implicate premature stop codons as a common cause of Stickler syndrome. The location of this premature stop codon in the far end of the nonhelical 3' end of the gene indicates that a truncated C-propeptide of at least 84 amino acid residues is inadequate for the functional gene product.

Diabetic papillopathy. Patient characteristics and fundus findings.

OBJECTIVE: To better define the spectrum of patient characteristics and fundus findings in patients with a presumably unique clinical entity referred to as diabetic papillopathy. DESIGN: Retrospective series. SETTING: A university-based referral practice. METHODS: We retrospectively reviewed the medical records of all diabetic patients with benign, transient disc swelling who were evaluated at our institution from 1986 to 1992. Data pertaining to patient demographics, visual acuity and symptoms, disc and retinal findings, ancillary test results, and clinical course were collected. RESULTS: Twenty-seven eyes of 19 patients met the study definition of diabetic papillopathy. Patients were generally older (mean age, 50 years) and of a broader age range (19 to 79 years) compared with those in prior reports. Two thirds of patients had type II diabetes. Disc swelling was consistently hyperemic and, on average, resolved within 3.7 months. Macular edema was a frequent associated finding (70% of eyes) as was significant capillary nonperfusion (52% of tested eyes). Only four eyes (15%) had final visual acuities of less than 20/50 and each had prominent macular edema. Cup-disc ratio analysis of uninvolved eyes revealed a significantly small physiologic cup. CONCLUSIONS: The clinical profile of diabetic papillopathy can be expanded to include people who are older or have type II diabetes and that affected eyes commonly have macular edema or retinal vascular changes that can adversely affect the visual outcome. Last, a small physiologic cup may represent an anatomic predisposition to the condition.

Postoperative endophthalmitis in association with diabetes mellitus.

BACKGROUND: Endophthalmitis continues to be a potentially devasting complication of ocular surgery, despite advances in microsurgical technique and infection-preventing measures. Patients with diabetes have altered immunity at various levels and may be more susceptible to infection after ocular surgery. The authors evaluate the associations between diabetes mellitus and postoperative endophthalmitis. METHODS: The records of 162 consecutive patients treated over a 5-year period for endophthalmitis occurring within 2 weeks of ocular surgery were retrospectively reviewed. RESULTS: Twenty-one percent of this consecutive series of patients with endophthalmitis after surgery had diabetes mellitus. Both the diabetic and nondiabetic groups were similar with respect to age, type of primary surgery, duration from surgery to onset of symptoms, presenting visual acuity, and management of endophthalmitis. Seventy-nine percent of the patients with diabetes and 68% of those without diabetes had culture-proven endophthalmitis. Staphylococcus was responsible for 74% and 71% of the culture-positive cases, respectively. The patients with diabetes were more likely to have endophthalmitis secondary to a gram-negative organism (P < 0.001) than those without diabetes (18.5% versus 5.7%). Visual outcome was worse in the diabetic group, although this may be related to preoperative visual status. CONCLUSIONS: Twenty-one percent of this consecutive series of patients with endophthalmitis after surgery had diabetes mellitus. The patients with diabetes mellitus were more likely to have endophthalmitis caused by gram-negative organisms and appear to have a poorer visual prognosis after treatment for endophthalmitis.

Exudative retinopathy of adults: a late sequela of retinopathy of prematurity.

The cases of 11 patients with a unilateral, retinal detachment associated with hard exudate and other late sequelae of retinopathy of prematurity are described. The abnormality was first noted in the second to fourth decades of life. Ten of the patients were women and one was a man. The source of the hard exudate was thought to be telangiectatic retinal vascular abnormalities. Among the ten treated eyes, four demonstrated an improvement in visual acuity. In two cases a mass lesion was also noted in the fundus.

Surgical management of complications associated with X-linked retinoschisis.

OBJECTIVE: To evaluate the role of surgical intervention in cases with severe, vision-threatening complications of X-linked retinoschisis. DESIGN: A retrospective survey of consecutive patients with X-linked retinoschisis who underwent surgery at our institution during a 16-year period. SETTING: A tertiary-care eye hospital. PATIENTS: Six eyes of four patients were identified. The mean age of the patients at the time of the first surgical procedure was 4.9 years (range, 18 months to 9 years). INTERVENTION: Scleral buckling procedure or pars plana vitrectomy. MAIN OUTCOME MEASURE: Surgical indications and long-term anatomic and visual outcome. RESULTS: Patients were initially operated on for rhegmatogenous retinal detachment (three eyes), exudative retinal detachment (one eye), and vitreous hemorrhage (two eyes). The surgical approach was scleral buckling for retinal detachment and vitrectomy for vitreous hemorrhage or proliferative vitreoretinopathy. Anatomic success and ambulatory vision (20/400 or better) was achieved in five of the six eyes with a mean follow-up of 3.8 years (range, 1 to 6 1/2 years). An average of 1.8 procedures per eye were performed. Two of the four eyes approached by primary scleral buckling eventually required vitrectomy. Proliferative vitreoretinopathy with retinal detachment was the major reason for reoperation. CONCLUSIONS: Surgery for X-linked retinoschisis-associated retinal detachment and vitreous hemorrhage can yield favorable anatomic and functional results. Multiple operations and the use of advanced vitreoretinal techniques to manage proliferative vitreoretinopathy-related complications, however, were necessary for ultimate success in certain cases.

Reoperation following diabetic vitrectomy.

A review of 484 consecutive eyes that were undergoing an initial pars plana vitrectomy for the sequelae of proliferative diabetic retinopathy disclosed that 41 eyes (8.5%) required one or more additional vitrectomy operations. The primary causes for reoperation included rhegmatogenous retinal detachment in 18 (44%) of the 41 eyes, recurrent vitreous hemorrhage in 21 eyes (51%), and glaucoma in two eyes (5%). The visual prognosis was worse in the group with rhegmatogenous retinal detachment, with 10 (56%) of 18 eyes progressing to no light perception (P = .003). Severe preretinal and subretinal fibrous proliferation, as demonstrated histopathologically, accounted in large part for the poor result. The preretinal membrane formation appeared to occur secondary to a combination of diabetic extraretinal vascular growth and proliferative vitreoretinopathy. Among the total group of 41 eyes that required subsequent surgery, the retina eventually remained detached in 18 eyes (44%), and phthisis bulbi occurred in 13 eyes (32%). Rubeosis iridis developed in 17 (94%) of 18 eyes in which the retina remained detached.

Management of late-onset angle-closure glaucoma associated with retinopathy of prematurity.

Late-onset angle-closure glaucoma secondary to retinopathy of prematurity (ROP) occurred in ten eyes of ten patients. The age at presentation ranged from 12 to 45 years (mean, 32 years). Eight eyes had nonneovascular mechanisms for the angle closure while two had neovascular angle closure. Treatment of eyes with this form of secondary angle closure included medical management alone, peripheral iridectomy, trabeculectomy, lensectomy, alloplastic tube shunt implantation, and cilioablative procedures. Three eyes required more than one of these treatments. The choice of therapy was based on the initial intraocular pressure and vision, degree of lens opacity and intumescence, presence of anterior segment neovascularization, and the gonioscopic appearance of the anterior chamber angle. The clinical features of this condition are described and a proposed mechanism and therapeutic approach are presented.

Inadvertent globe perforation during retrobulbar and peribulbar anesthesia. Patient characteristics, surgical management, and visual outcome.

The authors report a series of 20 eyes from 20 patients in whom inadvertent perforation of the globe occurred during local anesthesia for ocular surgery. Perforation resulted from retrobulbar anesthesia in 18 eyes and from peribulbar anesthesia in 2 eyes. Nine (45%) of 20 eyes had an axial length greater than or equal to 26.00 mm. Combining this figure with axial length data for the general population and estimates for the risk of globe perforation during local anesthesia yields an approximate incidence of perforation in eyes with axial length greater than or equal to 26.00 mm of 1 in 140 injections. Proliferative vitreoretinopathy (PVR) developed in 8 of the 20 eyes (40%) in this series. Overall, 15 (75%) of the 20 eyes were successfully repaired, and, in five eyes (25%), the final visual acuity was 20/70 or better.

Proliferative retinopathy: absence of white matter lesions at MR imaging.

One form of retinopathy associated with diabetes is a proliferative small vessel process thought to be mediated by biochemical, hemodynamic, and endocrinologic factors. The authors conducted a prospective study to determine whether patients with diabetes who had proliferative retinopathy had evidence of intracranial microangiopathy visible at magnetic resonance (MR) imaging. Twenty-five patients under 40 years of age with proliferative retinopathy and insulin-dependent diabetes mellitus and 10 age-matched control subjects were studied with MR imaging. Axial images were reviewed by two neuroradiologists for the presence of white matter foci of high signal intensity. No patients demonstrated evidence of these foci. There was no evidence of ischemic foci in any of the patients (all patients were neurologically asymptomatic). The vasculopathy associated with proliferative retinopathy does not appear to affect the intracranial circulation to the extent detectable with MR imaging. The presence of white matter foci of high signal intensity or ischemic changes in the brains of insulin-dependent diabetic patients under 40 years of age should not be attributed to diabetic vasculopathy. Other causes should be considered.

Systemic complications associated with retinal cryoablation for retinopathy of prematurity.

Eighty infants with proliferative retinopathy of prematurity (ROP) were treated with peripheral retinal cryoablation. Among the serious systemic complications encountered were three instances of respiratory arrest and one of cardiorespiratory arrest. Recommendations that may help prevent these adverse systemic effects in premature infants include: (1) avoidance of excess subconjunctival anesthetic doses, (2) preoperative administration of systemic atropine to minimize the oculocardiac reflex, (3) consideration of an analgesic agent to decrease the pain and exhaustion, and (4) cardiorespiratory monitoring in a hospital setting, with an intravenous line in place, at the time of treatment.

Re-operation following pars plana vitrectomy for the sequelae of proliferative diabetic retinopathy.

A review of 306 eyes undergoing pars plana vitrectomy for the complications of proliferative diabetic retinopathy revealed that 26 (8.5%) required a second vitrectomy operation. Among the eyes that required repeat vitrectomy, the eventual visual acuity ranged from 20/20 to 20/400 in 35% and was no light perception in 31%. Reasons for subsequent surgery included recurrent vitreous hemorrhage in 54% of eyes, retinal detachment in 42% and neovascular glaucoma in 4%. Twelve eyes (46%) remained with a permanent retinal detachment despite attempts at surgical repair, and eleven of these twelve eyes subsequently developed rubeosis iridis.