Benjamin H. Ticho

Branch Retinal Vein Occlusion and Quadratic Variation in Arteriovenous Crossings

To explore further the origin and clinically observed regional variation of branch retinal vein occlusion, we studied fluorescein angiograms of 42 patients (42 eyes) with branch retinal vein occlusion and a control population of 126 consecutive patients. In a statistically significant percentage of crossings, the artery was anterior to the vein in those areas of the retina clinically predisposed to branch retinal vein occlusion. Thirty-nine of the 42 patients with branch retinal vein occlusion sites had artery-anterior-to-vein crossings (P = .002), whereas 183 of all 266 arteriovenous crossings in these same eyes were similarly positioned. The artery lay anterior to the vein in significantly more temporal retinal crossings (337 of 457) than nasal retinal crossings (89 of 149; P = .002). Similarly, significantly more superotemporal quadrant crossings (164 of 209) than inferotemporal quadrant crossings (173 of 248) had the artery anterior to the vein (P = .0045). These results suggested that variation in the pattern of arteriovenous crossings may have a role in the clinical distribution of branch retinal vein occlusion.

Primary treatment of nasolacrimal duct obstruction with nasolacrimal duct intubation in children younger than 4 years of age.

PURPOSE To report the outcome of nasolacrimal duct intubation as the primary treatment of congenital nasolacrimal duct obstruction (NLDO) in children younger than 4 years of age. METHODS A total of 182 eyes of 139 children receiving intubation with planned tube retention for 2 to 5 months were enrolled in a prospective, nonrandomized observational multicenter study (19 sites). Children were ages 6 months to <45 months at the time of surgery, with no previous nasolacrimal surgical procedures and had at least one of the following clinical signs of NLDO: epiphora, mucous discharge, and/or increased tear lake. RESULTS Treatment success was defined as absence of epiphora, mucous discharge, and increased tear lake at the outcome visit, 1 month after tube removal. The surgical outcome was assessed in 150 eyes (82% of cohort). The proportion of eyes treated successfully was 91% (95% CI: 86%-95%). The outcome dye disappearance test was normal in 125 (86%) eyes, indeterminate in 13 (9%), and abnormal in 7 (5%) of the 145 eyes tested. Monocanalicular tubes were used in 74% of cases. The tube was removed before the planned minimum retention time of 2 months in 61 eyes (41%). For 23 eyes, the early removal was attributed to inadvertent displacement by the patient. CONCLUSIONS In children 6 months to <45 months of age, nasolacrimal duct intubation in a nonrandomized and noncomparative trial was a successful primary treatment of NLDO in about 90% of cases not lost to follow-up.

Resolution of congenital nasolacrimal duct obstruction with nonsurgical management

OBJECTIVE To determine how often nasolacrimal duct obstruction (NLDO) resolves with 6 months of nonsurgical management in infants aged 6 to less than 10 months. METHODS As part of a randomized trial evaluating the cost-effectiveness of immediate office probing vs observation with deferred probing for unresolved cases, 107 infants aged 6 to less than 10 months who had NLDO and no history of nasolacrimal duct surgery were prescribed 6 months of nasolacrimal duct massage and topical antibiotics as needed. Resolution of the NLDO was assessed 6 months after study entry and was defined as the absence of all clinical signs of NLDO (epiphora, increased tear lake, or mucous discharge) and not having undergone NLDO surgery. Exploratory analyses assessed whether baseline characteristics, including age, sex, laterality, and prior treatment, were associated with the probability of NLDO resolving without surgery. RESULTS At the 6-month examination, which was completed for 117 of the 133 eyes (88%), the NLDO had resolved without surgery in 77 eyes (66% [95% CI, 56%-74%]). None of the baseline characteristics we evaluated were found to be associated with resolution. CONCLUSIONS In infants 6 to less than 10 months of age, more than half of eyes with NLDO will resolve within 6 months with nonsurgical management. Knowledge of the rate of NLDO resolution in infancy without surgery will help clinicians and parents effectively discuss treatment options.

Laser Photocoagulation at Birth Prevents Blindness in Norrie's Disease Diagnosed Using Amniocentesis

OBJECTIVE To report the first case of prophylactic laser treatment to prevent blindness in a patient who was diagnosed with Norries disease by genetic testing with amniocentesis. DESIGN Case report. PARTICIPANTS A 2-year-old white boy with Norries disease. METHODS A 37-week gestational age male with a family history of Norries disease was born via Cesarean section after the mother had undergone prenatal amniocentesis fetal-genetic testing at 23 weeks of gestation. A C520T (nonsense) mutation was found in the Norries disease gene. After examination under anesthesia confirmed the diagnosis on the first day of life, laser photocoagulation was applied to the avascular retina bilaterally. The patient was followed closely by ophthalmology, pediatrics, and occupational therapy departments. MAIN OUTCOME MEASURES Functional outcome, as documented by Teller visual acuity and formal occupational therapy testing, and anatomic outcome, as documented by Retcam photography and fluorescein angiography. RESULTS Complete regression of extraretinal fibrovascular proliferation was observed 1 month after laser treatment. No retinal detachment had occurred to date at 24 months. Teller visual acuity at 23 months of life was 20/100 in both eyes. The patients vision and developmental milestones were age appropriate. CONCLUSIONS Pre-term genetic diagnosis with immediate laser treatment after birth may preserve vision in individuals affected with Norries disease.

Bilateral Diffuse Iris Nodular Nevi: Clinical and Histopathologic Characterization

BACKGROUND Diffuse nodular nevus of the iris is an uncommon condition that presents with multiple verrucous excrescences distributed diffusely on the iris surface. METHODS The authors describe 30 patients with bilateral diffuse iris nodular nevi and report associations with bilateral congenital cataract, neurofibromatosis, oculodermal melanocytosis, congenital ptosis, morning glory anomaly, Axenfeld anomaly, or Peters anomaly. RESULTS Iris nodules were uniform in size and distribution and were brown, as was the surrounding iris. Light and electron microscopy of iridectomy specimens from one patient showed elevated plaques composed of aggregates of plump, lightly pigmented nevoid cells interwoven with mature, densely pigmented spindle-shaped uveal melanocytes. CONCLUSIONS The authors report the largest clinical series and first ultrastructural description of bilateral diffuse iris nodular nevi, which represents a variant of neural crest development. No ocular complications could be attributed to the iris nodules, which should be differentiated from Lisch nodules and other pathologic iris lesions.

Diffuse Iris Nevus in Oculodermal Melanocytosis: A Light and Electron Microscopic Study

We studied by light and electron microscopy the diffuse iris nevus of an eye in a 16-year-old patient with oculodermal melanocytosis and choroidal malignant melanoma. The nevus cells in the anterior border layer of the iris appeared to be poorly differentiated, showing moderate infolding of the nuclear membrane with heterochromatin clumping, watery cytoplasm, abundant mitochondria, fine filaments, rough endoplasmic reticulum, and numerous pinocytotic vesicles. Small scattered immature melanosomes and occasional giant melanosomes were observed in these cells. Deep in the iris stroma, however, nevus cells were found singly or in small groups, and were associated with an increasing number of melanized melanosomes and cytoplasmic filaments and reduced numbers of other cytoplasmic organelles, such as mitochondria and free ribosomes. Differentiation of the iris nevus cells appeared to progress from the anterior border layer toward the iris stroma. This observation suggests that intrastromal nevi may be more benign than nevi with surface plaque. The ultrastructural characteristics of the diffuse nevi of oculodermal melanocytosis were compared with those of other iris nevi.

Mucoepidermoid carcinoma of the eyelid skin

PURPOSE Mucoepidermoid carcinoma involving the eye or its adnexa generally arises from the conjunctiva. We describe a nodular lesion of the lower eyelid skin with histologic features consistent with low-grade mucoepidermoid carcinoma. METHOD Case report. RESULTS By histology, the tumor demonstrated areas of squamous cell carcinoma and scattered islands of mucin-secreting cells. Histochemistry showed hyaluronidase-resistant mucin and intense immunoreactivity with an antibody against carcinoembryonic antigen suggesting that the tumor originated from acrosyringeal structures. CONCLUSIONS To our knowledge, this is the first report of a mucoepidermoid carcinoma arising from sweat glands in the eyelid skin.

Is The Convergence Insufficiency Symptom Survey Specific for Convergence Insufficiency? A Prospective, Randomized Study.

Background and Purpose The Convergence Insufficiency Symptom Survey (CISS) is a questionnaire used as an outcome measure in treatment of convergence insufficiency. The current prospective randomized trial evaluates the diagnostic specificity of the CISS. Patients and Methods Surveys were completed by 118 adolescent patients who presented for routine eye examinations. Scores were compared between patients who could be classified as having convergence insufficiency (CI) or normal binocular vision (NBV). In addition, a comparison was done between self- and practitioner-administered CISS scores within these groups. Results The mean CISS score did not differ significantly between NBV patients (14.1 ±11.3, range of 0 to 43) and CI patients (12.3 ±6.7, range of 3 to 28); P = 0.32. Mean CISS scores were lower when physician-administered (11.4 ±7.9) than when self-administered (16.3+11.4); P = 0.007. Conclusion CISS scores tend to be higher when self- vs. practitioner-administered. This study suggests that the CISS questionnaire is not specific for convergence insufficiency.

Iris Transillumination Defects Associated With Pallister–Killian Syndrome

This report describes a new finding of iris transillumination defects in Pallister-Killian syndrome, a rare multi-system disorder resulting from mosaic tetrasomy of the short arm of chromosome 12.