Patterns of uveitis in children at the apex institute for eye care in India: analysis and review of literature.

AIM: To study patterns of uveitis in Indian children and compare with data sets published earlier in the literature. METHODS: Consecutive patients below 16 years of age presenting to the uvea clinic of a tertiary eye care center were included prospectively through the period of July 2009-August 2013. Children with retinal vasculitis, exogenous endophthalmitis and masquerade syndromes were excluded from analysis. Uveitis was classified as per the nomenclature system adopted by the International Uveitis Study Group. Hemogram, Mantoux test and chest X-ray were done for each patient, along with tailored investigations and pediatric review as per clinical profile. Clinical pattern and etiology were the main outcome measures. RESULTS: One hundred and thirty-four children were analyzed. Anterior uveitis (40%) was the commonest pattern followed by intermediate uveitis (25%), panuveitis (18%) and posterior uveitis (17%). Bilateral disease was present in 54%, 15% had infectious uveitis, 10% had granulomatous uveitis and 54% had idiopathic uveitis. Complications were present in half of the patients. Juvenile idiopathic arthritis (22), followed by toxoplasmosis (10) and tuberculosis (5), was the commonest etiology. Intermediate uveitis, non-granulomatous inflammation and older onset of disease had the high odds ratio of having idiopathic disease. CONCLUSION: Patterns of pediatric uveitis can vary between regions from even within the same geopolitical region. Anterior uveitis is commonest, and juvenile idiopathic arthritis and toxoplasmosis are the most frequent etiologies. Diagnosis of pediatric ocular tuberculosis is more difficult than in adults and needs better and well-defined criteria.

Lateral eyelid rotation flap: a novel technique for reconstruction of full thickness eyelid defect.

The purpose of this study was to study anatomical, functional, and cosmetic outcomes of a novel technique, 'Lateral Eyelid Rotation Flap' for reconstruction of full thickness eyelid defect. In this prospective interventional study, 10 patients with full thickness eyelid defect measuring 1/2-2/3rd of eyelid width were included. Eyelid reconstruction was performed by single surgeon, using lateral eyelid rotation flap. Anatomic outcome was assessed by analyzing horizontal and vertical palpebral apertures (HPA and VPA), eyelid contour, and lateral canthus. Functional outcome was assessed by measuring tear film break-up time (TBUT) and Schirmer's test in both the eyes. Cosmetic outcome was evaluated by patients. Median age of patients was 56 years. Nine cases had full thickness defect following the excision of eyelid malignancy. The mean horizontal defect size was 17 ± 4.2 mm. HPA did not change significantly after surgery. VPA was statistically comparable to contralateral eye at 1-month follow-up. Lateral canthus angle recovered by 3rd month after surgery. TBUT and Schirmer's tests were comparable to contralateral eye. Eight patients graded cosmetic outcome as good to excellent. This is a new, single-stage technique for reconstruction of full thickness eyelid defects, with full thickness eyelid tissue including margin.

A new computer-based Farnsworth Munsell 100-hue test for evaluation of color vision.

To evaluate a computer-based Farnsworth-Munsell (FM) 100-hue test and compare it with a manual FM 100-hue test in normal and congenital color-deficient individuals. Fifty color defective subjects and 200 normal subjects with a best-corrected visual acuity ≥ 6/12 were compared using a standard manual FM 100-hue test and a computer-based FM 100-hue test under standard operating conditions as recommended by the manufacturer after initial trial testing. Parameters evaluated were total error scores (TES), type of defect and testing time. Pearson's correlation coefficient was used to determine the relationship between the test scores. Cohen's kappa was used to assess agreement of color defect classification between the two tests. A receiver operating characteristic curve was used to determine the optimal cut-off score for the computer-based FM 100-hue test. The mean time was 16 ± 1.5 (range 6-20) min for the manual FM 100-hue test and 7.4 ± 1.4 (range 5-13) min for the computer-based FM 100-hue test, thus reducing testing time to <50 % (p < 0.05). For grading color discrimination, Pearson's correlation coefficient for TES between the two tests was 0.91 (p < 0.001). For color defect classification, Cohen's agreement coefficient was 0.98 (p < 0.01). The computer-based FM 100-hue is an effective and rapid method for detecting, classifying and grading color vision anomalies.

Clinicopathologic review of epithelial tumors of the lacrimal gland.

OBJECTIVE: To study the clinicopathologic features of epithelial tumors of the lacrimal gland diagnosed at a tertiary care center in India during a 10-year period. METHODS: Medical records of cases with histopathologically proven epithelial tumors of the lacrimal gland were reviewed for demographic details and clinical features at the time of presentation. For histopathologic findings, slides with hemotoxylin and eosin-stained sections of the tumors were reviewed. Special stains, including histochemical and immunohistochemical stains, and additional sections were studied, whenever needed. RESULTS: Of 66 cases with epithelial tumors of the lacrimal gland identified during the study period, 50 (76%) cases were benign and 16 (24%) cases were malignant tumors. Histopathologic examination was consistent with the diagnosis of pleomorphic adenoma (PA) in all 50 cases of benign tumors. The mean age at diagnosis in PA cases was 37.7 years. Microscopic examination of PA tumors showed cystic degeneration in 18 (36%) cases, squamous metaplasia in 16 (32%) cases, calcification in 6 (12%) cases, and ossification in 1 (2%) case. Among malignant tumors, adenoid cystic carcinoma (ACC) was the most common tumor (n = 12, 18%), with an average age at diagnosis of 32.4 years. Microscopic examination of ACC revealed that most cases were grade I tumors. Solid areas were noted in 4 (33.3%) cases and perineural invasion was identified in 2 (16.7%) cases. Other malignant tumors included 1 case each of carcinoma ex-PA (1.5%), lacrimal duct carcinoma (1.5%), mucoepidermoid carcinoma (1.5%), and basal cell adenocarcinoma (1.5%). CONCLUSIONS: As far as the authors are aware, this is the largest series on clinicopathologic features of epithelial tumors of the lacrimal gland from South Asia. The incidence of PA was significantly higher in this study than reported in the Caucasian population. Among malignant tumors, ACCs were the most common, although rare tumors were also identified.

Correlation between clinical features, magnetic resonance imaging, and histopathologic findings in retinoblastoma: a prospective study.

OBJECTIVE: To correlate clinical features with histopathology findings in advanced intraocular retinoblastoma and to determine the diagnostic accuracy of magnetic resonance imaging (MRI) in detecting tumor invasion. DESIGN: Prospective, nonrandomized case series. PARTICIPANTS: We included 75 patients with group E retinoblastoma. METHODS: Demographic and clinical features were recorded at presentation. Contrast-enhanced MRI was performed to study tumor characteristics and extent of invasion. Primary enucleation was performed and histopathologic features noted. Statistical analysis was done using the Kruskal-Wallis test to determine correlation between clinical features and histopathology. Sensitivity, specificity, and accuracy of MRI in detecting tumor invasion were determined. MAIN OUTCOME MEASURES: Significant associations between clinical findings at presentation and high-risk histopathology, and correlation between MRI results and histopathologic evidence of tumor invasion. RESULTS: A significant association was found between iris neovascularization and choroidal invasion (P = 0.032), intraocular pressure and optic nerve invasion (P = 0.034), and shallow anterior chamber and iris invasion (P = 0.021). Corneal diameter did not show any significant correlation with high-risk histopathology. On MRI, tumor volume showed a significant association with optic nerve invasion (P = 0.023). The accuracy of MRI in detecting choroidal invasion was 68% (sensitivity, 60%; specificity, 80%). Prelaminar invasion was correctly identified in 9 out of 15 eyes (accuracy, 84%; sensitivity, 60%; specificity, 90%), whereas the accuracy of MRI in detecting postlaminar invasion was 76% (sensitivity, 61.9%; specificity, 81.5%). Ciliary body invasion was correctly identified in 5 out of 7 eyes (accuracy, 93.3%; specificity, 95.6%) and scleral invasion in 5 out of 6 eyes (accuracy, 98.7%; specificity, 100%). CONCLUSIONS: As far as we are aware, this is the first prospective study on the correlation of clinical features and MRI findings with histopathologic risk factors in eyes primarily enucleated for retinoblastoma. Neovascularization of iris, intraocular pressure, shallow anterior chamber, and tumor volume correlated well with high-risk histopathology. Because MRI has limitations in reliably predicting microscopic infiltration of the choroid and optic nerve, decision in favor of neoadjuvant chemotherapy on the basis of suspected postlaminar invasion on MRI is not justified in the absence of histopathologic evidence of disease.

Outcome, pathologic findings, and compliance in orbital retinoblastoma (International Retinoblastoma Staging System stage III) treated with neoadjuvant chemotherapy: a prospective study.

PURPOSE: The present study prospectively evaluated outcome, pathologic findings, and compliance in orbital retinoblastoma patients (International Retinoblastoma Staging System [IRSS] stage III). DESIGN: Prospective study and case series. PARTICIPANTS: Twenty-eight consecutive IRSS stage III retinoblastoma patients were enrolled prospectively in the study after ethics committee approval and written informed consent. METHODS: Planned therapy for patients included treatment with a uniform protocol consisting of neoadjuvant chemotherapy followed by enucleation, adjuvant radiotherapy, and chemotherapy. Prospective computerized data entry and telephone contact were undertaken to improve compliance, a major issue in treatment of orbital retinoblastoma. MAIN OUTCOME MEASURES: Event-free survival (EFS) and overall survival (OS) were calculated using Kaplan-Meier survival analysis. The difference between groups was analyzed using the log-rank test. RESULTS: Median follow-up was 14.75 months (range, 2.23-26.3 months). The OS for all patients was 40.4% and the EFS was 33.33% at a follow-up of 26.3 months. The median EFS and OS were 10.37 months and 15.73 months, respectively. At baseline, bilateral retinoblastoma predicted inferior EFS (P = 0.0006) and OS (P = 0.0081). Twenty-two of 28 patients underwent enucleation, and viable tumor was present in 21 of 22 enucleated specimens (95%). Neoadjuvant chemotherapy prevented orbital exenterations. Presence of tumor in the cut end of the optic nerve significantly correlated with inferior EFS (P = 0.012) and OS (P = 0.0098). Central nervous system metastasis was the most common site of relapse and death. The overall rate of compliance with treatment was 67.8% (19/28), which was twice the compliance rate at the authors' center before initiation of the present study. CONCLUSIONS: There is a paucity of information in the literature on outcomes and pathologic findings after neoadjuvant chemotherapy in IRSS stage III retinoblastoma treated with a uniform treatment protocol, and as far as the authors are aware, there has been no prospective study. Neoadjuvant chemotherapy was able to avoid exenteration in all operated patients; however, residual viable tumor was present in 95% of enucleated specimens. Bilaterality and tumor in the optic nerve cut end after neoadjuvant chemotherapy were associated with inferior outcome. Compliance could be improved with computerized data entry and regular telephone contact.

Clinical predictors of high risk histopathology in retinoblastoma.

BACKGROUND: Previous studies show that clinical features at presentation, in retinoblastoma patients, like glaucoma and neovascularization of iris are associated with a higher incidence of high risk histopathology findings (HRF) in enucleated eyes. Herein, we analyze association between clinical features at time of enucleation and occurrence of HRF including invasion of anterior chamber, iris, ciliary body, choroid (massive), sclera, extrascleral tissue, optic nerve beyond lamina cribrosa, and optic nerve cut end, in a large series of eyes enucleated for retinoblastoma. PROCEDURE: We retrospectively studied demographic, clinical, and histopathology findings in all retinoblastoma patients who underwent primary enucleation at our center, over a 5 years duration. Statistical analysis was done to find any association between clinical features at presentation and the presence of HRF. RESULTS: Three hundred twenty-six eyes were studied. Median age of presentation was 2 years. Glaucoma was the most common clinical finding at presentation apart from leucocoria. Out of 326 enucleated eyes, 28 (8.6%) had extrascleral and/or optic nerve transection invasion. Among remaining 298 eyes, with completely resected tumor, 115 (38.6%) had massive choroidal invasion, 54 (17%) had retrolaminar optic nerve invasion, and 24 (7%), 29 (9%), and 23(7%) had anterior chamber, iris, and ciliary body invasion, respectively. Age more than 2 years, lag period more than 3 months, hyphema, pseudohypopyon, staphyloma, and orbital cellulitis were associated with occurrence of three or more HRF on univariate analysis. CONCLUSIONS: Clinical variables including older age, longer lag period, hyphema, pseudohypopyon, staphyloma, and orbital cellulitis were strongly associated with occurrence of HRF in this study.

Molecular & phenotypic characterization of Staphylococcus epidermidis in implant related infections.

BACKGROUND & OBJECTIVES: The discrimination between the Staphylococcus epidermidis colonizing the deep seated indwelling devices and those which are mere commensals has always been a challenge for the clinical microbiologist. This study was aimed to characterize the S. epidermidis isolates obtained from device related infection for their phenotypic and molecular markers of virulence and to see whether these markers can be used to differentiate the pathogenic S. epidermidis from the commensals. METHODS: Fifty five S. epidermidis isolates from various device related infections such as endophthalmitis following intra-ocular lens (IOL) implantation, intravascular (IV) catheter related sepsis and orthopaedic implant infections, were studied for slime production, biotyping, antibiotic sensitivity; and mec A and ica positivity by the recommended procedures. RESULTS: Twenty three (41.8%) isolates were multi-drug resistant, 26 (65.2%) were slime producers, 30 (54.5%) were adherent, 23 (41.8%) possessed the intercellular adhesin (ica) gene, and 28 (50.9%) harboured the mec A gene. Biotypes I and III were the commonest, most members of which were multi- drug resistant. Twenty two (73.3%) of the 30 adherent bacteria were slime producers as opposed to only 4 (16%) of the 25 non-adherent bacteria (P<0.001). A vast majority i.e. 21 (91.3%) of the 23 ica positive organisms were adherent to artificial surfaces in contrast to only 9 (28.1%) of the 32 non-ica positive organisms (P<0.001). Twenty (86.9%) of the 23 ica positive bacteria were slime producers, as opposed to only 6 (18.7%) of the 32 ica negative bacteria (P<0.001). Of the 23 multi-drug resistant isolates, 19 (82.6%) carried the mec A gene. INTERPRETATION & CONCLUSIONS: The present findings showed that ica AB and mec A were the two important virulence markers of S. epidermidis in implant infections and slime was responsible for the sessile mode of attachment on the devices.

Understanding the postmortem changes in concentrations of methanol in ocular matrices in rabbits.

Death due to abuse and accidental ingestion of methanol is widely known around the globe. The paper presents the postmortem changes in concentrations of methanol in the vitreous humor, aqueous humor, and ocular tissues in a rabbit model in methanol intoxication. Rabbits were intoxicated with methanol at a dose of 6.3 ml/kg through oral gavage. After 3.5 h of methanol administration, the rabbits were sacrificed. Vitreous humor, aqueous humor, and ocular tissues were collected both, perimortem (immediately) and postmortem (17 h post-death). Whole blood and plasma samples were also collected to explore the correlation between levels of methanol in whole blood/plasma and ocular fluids/ocular tissues if any. All the samples were analyzed by Headspace Gas Chromatography. The analysis revealed a decrease in methanol levels at postmortem for all the matrices, except for retina-choroid than its perimortem value. For retina-choroid, no significant change in methanol levels at postmortem was found.

Development of novel in silico model to predict corneal permeability for congeneric drugs: a QSPR approach.

This study was undertaken to determine in vivo permeability coefficients for fluoroquinolones and to assess its correlation with the permeability derived using reported models in the literature. Further, the aim was to develop novel QSPR model to predict corneal permeability for fluoroquinolones and test its suitability on other training sets. The in vivo permeability coefficient was determined using cassette dosing (N-in-One) approach for nine fluoroquinolones (norfloxacin, ciprofloxacin, lomefloxacin, ofloxacin, levofloxacin, sparfloxacin, pefloxacin, gatifloxacin, and moxifloxacin) in rabbits. The correlation between corneal permeability derived using in vivo studies with that derived from reported models was determined. Novel QSPR-based model was developed using in vivo corneal permeability along with other molecular descriptors. The suitability of developed model was tested on ß-blockers (n = 15). The model showed better prediction of corneal permeability for fluoroquinolones (r(2) > 0.9) as well as ß-blockers (r(2) > 0.6). The newly developed QSPR model based upon in vivo generated data was found suitable to predict corneal permeability for fluoroquinolones as well as other sets of compounds.

Phthisis bulbi in retinoblastoma.

BACKGROUND: Phthisis bulbi is a relatively uncommon and atypical clinical presentation of retinoblastoma. DESIGN: Retrospective study conducted at a tertiary care hospital. PARTICIPANTS: Eighteen consecutive retinoblastoma patients with primary phthisis bulbi. METHODS: Retrospective analysis of clinical, imaging and histopathological features of all retinoblastoma patients with primary phthisis bulbi, treated at our centre between January 2005 and December 2009. MAIN OUTCOME MEASURE: Clinical and histopathology features. RESULTS: Eighteen (3.5%) retinoblastoma patients developed primary phthisis bulbi. The median age of presentation was 1.5 years. The median duration of symptoms before presentation was 6 months. In total, 15 out of 18 (83%) cases had bilateral disease. Among these, 80% (12/15) had advanced intraocular disease in the fellow eye. Most common first symptom was white reflex. History of orbital inflammation was present in 12/18 cases. Computed tomographic scan of orbit showed intraocular mass with calcific densities in 16 eyes. In two cases, hyperdense mass was seen without any calcification. On histopathology, residual viable tumour cells with characteristics of poorly differentiated retinoblastoma were found in 67% (12/18) eyes. High-risk factors were present in six cases with microscopic residual disease in three cases. CONCLUSIONS: This is the largest case series of retinoblastoma patients with primary phthisis bulbi. Phthisis bulbi in retinoblastoma may be associated with bilateral disease in most cases and advanced intraocular disease in the fellow eye in a significant number of cases. Regression is incomplete in majority of these cases; therefore, enucleation must definitely be done in all cases of retinoblastoma presenting with phthisis bulbi.

Clinical and pathologic profile of angiomyxomas of the orbit.

PURPOSE: To describe clinical, imaging, and histopathologic features of angiomyxomas of the orbit, which are extremely rare tumors in the orbit. METHODS: A retrospective review of clinical case records and imaging findings of histopathologically diagnosed cases of angiomyxoma over a period of 8 years (2001-2008). The histopathologic features were studied by routine hematoxylin-eosin staining, special stain (Alcian blue), and immunohistochemistry. RESULTS: Four cases (2 male and 2 female) with a mean age of 35 years (range, 28-40 years) were diagnosed as angiomyxoma. Two of these were angiomyxomas, and 2 were aggressive angiomyxomas. All patients presented with gradual progressive proptosis. The mean duration of symptoms was 10.5 months (range, 5-24 months). There was associated reduction in the visual acuity in 2 cases. The superior orbit was involved in all 4 patients. CT scan showed a heterogeneously enhancing irregular mass confined to the superior orbit. Surgical removal of the mass was performed in all 4 cases. On follow-up, there was recurrence at 6 months in 1 case, which was histopathologically diagnosed as an angiomyxoma. CONCLUSIONS: Angiomyxoma is an extremely rare, locally aggressive orbital tumor, occurring in the third to fourth decade of life. Complete excision is the treatment of choice. These tumors are likely to have a recurrence due to their infiltrative growth and require long-term follow-up.

Recurrent bilateral dermatofibrosarcoma protuberans of eyelids.

A 26-year-old man had a history of multiple nodules involving the upper and lower eyelids bilaterally, for which he had undergone wide local excision along with eyelid reconstruction. Three years later, he presented to the authors with recurrence of the tumor masses. Tumor debulking surgery was performed bilaterally, with eyelid reconstructive surgery. Histopathology of the excised mass showed spindle-shaped cells in the dermis, which were arranged in a storiform pattern. Immunohistochemically, the tumor expressed vimentin diffusely and CD-34 focally. Histopathologic findings of storiform pattern of tumor cells and positive immunohistochemical stains for CD-34 led to the diagnosis of dermatofibrosarcoma protuberans. The clinical course was marked by repeated local recurrences following multiple debulking procedures. Local control could not be achieved in this case and at last follow up, the tumor was surgically unresectable.

Effect of fluoroquinolones on the expression of matrix metalloproteinase in debrided cornea of rats.

Matrix metalloproteinases (MMPs) are implicated in regenerative and healing processes in corneal injuries. Based upon reports that topical fluoroquinolones (FQs) may cause perforations during corneal healing by modulating MMPs, this study evaluated the comparative effects of commercially available FQs eye drops on the expression of MMP-2 and MMP-9 in the cornea after ethanol injury. Uniform corneal epithelial defects were created using 70% ethanol in the right eye of the rats (n = 6). The groups studied were (I) sham, (II) normal saline with benzalkonium chloride (NS-BKC), (III) norfloxacin 0.3%, (IV) ciprofloxacin 0.3%, (V) lomefloxacin 0.3%, (VI) sparfloxacin 0.3%, (VII) gatifloxacin 0.3%, and (VIII) moxifloxacin 0.5%. Each treatment was instilled six times/day up to 48 h and rats were sacrificed using excess of anesthesia. The corneas were excised to study the expression of MMP-2 and MMP-9 using gelatin zymography and real-time PCR. All the FQs significantly increased the expression of MMP-2 and MMP-9 as compared to the sham and NS-BKC-treated group. NS-BKC did not show a significant effect on MMPs expression compared to the sham group. Among the studied FQs, ciprofloxacin was observed to exhibit maximal induction of MMP-2 and MMP-9, whereas lomefloxacin exhibited an equivocal effect on both MMP-2 and MMP-9 expression. Findings of the present study demonstrate that topical application of FQs may induce the expression of MMP-2 and MMP-9 in debrided corneal epithelium and, therefore, may delay corneal wound healing. Thus, it can be concluded that selecting a FQ for ophthalmic use having minimal effect on MMPs may impact wound healing in injured or vulnerable cornea.

Postmortem redistribution of ketamine in ocular matrices: A study of forensic relevance.

The present study evaluates the postmortem redistribution of ketamine in ocular matrices, such as vitreous humor, aqueous humor, and ocular tissues in an animal model. To understand the redistribution of ketamine and its metabolite (norketamine) in the ocular matrices, an in vivo study was performed in rabbits. The rabbits were divided into two groups: perimortem and postmortem. The postmortem samples were collected at 17 h after the administration of ketamine (40 mg/kg) intravenously. For a better understanding of the metabolism of ketamine in eyes, an ex vivo study was conducted in goat eyes after administration of ketamine intravitreally. The samples were analyzed by LC-MS/MS and the levels of ketamine and norketamine in these matrices were compared with that of whole blood and plasma. The results of the in vivo study showed a decrease in ketamine levels in whole blood and plasma while an increase in ocular matrices at postmortem. Though, in most cases, this increase/decrease was statistically insignificant. Moreover, there was an increase of norketamine level in ocular matrices. Ex vivo study also shows the presence of norketamine in ocular matrices of goat eyes. The presence of norketamine in goat eyes may be indicative of the metabolism of ketamine in the eyes.

Evaluation of the impact of P-glycoprotein (P-gp) drug efflux transporter blockade on the systemic and ocular disposition of P-gp substrate.

PURPOSE: The impact of P-glycoprotein (P-gp) blockade on the intravenous (i.v.) pharmacokinetics of rhodamine-123 (Rho-123), and the subsequent effect on its disposition in ocular and nonocular tissues, was studied by using rabbits. METHODS: Three (3) control rabbits received only an i.v. bolus dose of Rho-123 (1.52 mg/kg). Three (3) blocker-pretreated rabbits received an i.v. dose of GF120918 (3.5 mg/kg) 30 min before the i.v. bolus of Rho-123. The plasma concentration of Rho-123 at different time points was subjected to a pharmacokinetic compartmental analysis, using WinNonlin (Scientific Consultants, Lexington, KY). For tissue-distribution study, a drug treatment similar to the i.v. kinetic study was followed by having 5 rabbits in each group. The animals were sacrificed at 30 min with an excess of anesthesia. Plasma and tissues samples were analyzed by using a validated high-performance liquid chromatographic IV method with a fluorescent detector. RESULTS: The method validated was sensitive enough to estimate Rho-123 up to 1.94 ng/mL in plasma. I.v. Rho-123 data fitted well into the three-compartment model, and P-gp blocker treatment changed it into a two-compartment model. The P-gp blockade significantly increased the mean tissue concentrations in the lungs and spleen, whereas the rise in mean tissue levels in the heart, liver, and kidney and in all ocular tissues were found to be statistically insignificant. CONCLUSIONS: Increasing the ocular concentration of systemically given drugs may not be possible with the degree of P-gp blockade achieved when using GF120918 at the studied concentration after an i.v. administration.

Atypical synophthalmos with arhinencephaly unilateralis.

This article describes a rare case of a 4-month-old infant with atypical synophthalmos with ipsilateral arhinencephaly unilateralis, ethmoid sinus, and lacrimal apparatus and brain abnormalities--diagnosed on the basis of the clinical picture and imaging findings.

Atypical oculo-orbital complex choristoma in organoid nevus syndrome.

We report organoid nevus with left oculo-orbital choristoma fixing the globe in hypotropia, precanthal skin tag, and upper eyelid coloboma and right limbal dermoid. Computed tomography revealed a dilated lateral ventricle and cerebral atrophy. No neurologic deficit existed. The coloboma was repaired, choristoma debulked, and precanthal tag excised.

Nasal endoscopy in congenital dacryocystitis.

PURPOSE: Controversy exists regarding the relationship between nasal pathology and congenital dacryocystitis. The recent advent of nasal endoscopes has greatly improved visualization of deep and hidden areas of nasal anatomy and has led to the elaboration of nasal anatomical variants and pathologies that were previously unknown. The current study aimed to evaluate for associations, if any, between anatomical abnormalities or variations in the lateral nasal wall and the presence and resolution of congenital dacryocystitis. PATIENTS AND METHODS: Phase I was a case-control study that compared the incidence of a predefined set of nasal endoscopic variations in infants with congenital dacryocystitis versus normal infants. Phase II was a cohort study that evaluated the impact of nasal endoscopic variations on the effectiveness of conservative treatment for congenital dacryocystitis. "Failure" to respond to conservative treatment measures was defined as the persistence of symptoms at the first birthday. RESULTS: Inferior turbinate hypertrophy and inferior meatal narrowing were significantly more frequent in the case group than in the control group. Conservative treatment measures resulted in 70 of 83 resolved cases on subsequent follow-up. The occurrence of inferior turbinate hypertrophy or inferior meatal narrowing at final examination was associated with non-resolution. The relative risk of non-resolution with inferior turbinate hypertrophy or inferior meatal narrowing was 7.7 (confidence interval, 2.32 to 25.72) and 12 (confidence interval, 3.7 to 39.2), respectively. CONCLUSION: Inferior turbinate hypertrophy and inferior meatal narrowing are more frequent in cases of congenital dacryocystitis. They are predictive of a poor outcome when congenital dacryocystitis is treated with conservative measures. This information should be considered when making clinical decisions for patients with congenital dacryocystitis.

Clinical outcome and regression patterns of retinoblastoma treated with systemic chemoreduction and focal therapy: A prospective study.

PURPOSE: To prospectively study the clinical outcome and regression patterns of early retinoblastoma (Groups A and B) after systemic chemotherapy and focal consolidation in Indian children. MATERIALS AND METHODS: Group A eyes were treated with focal therapy (transpupillary thermotherapy/cryotherapy) and Group B with systemic chemoreduction and focal therapy. Outcome measures were efficacy and safety of treatment, risk factors for treatment failure, regression patterns, and factors predictive of regression patterns. RESULTS: Of 119 eyes (216 tumors), 14 (11.8%) were Group A and 105 (88.2%) were Group B eyes. The mean follow-up was 22.6 months. Tumor control was achieved in 111/119 eyes (93.3% overall, 100% Group A, 92.4% Group B). Eight Group B eyes (6.7%) had treatment failure. No serious systemic side-effects were noted. Risk factors for failure included larger tumors (P = 0.001) and proximity to posterior pole (P = 0.014). Regression patterns were Type 4 (50.2%), Type 3 (31.7%), Type 1 (11.1%), and Type 2 (7%). Factors predictive of Type 4 regression were smaller tumors, anterior location, younger age; Type 3 regression was associated with larger tumors, macular location, and older age. CONCLUSIONS: Systemic chemoreduction and focal therapy provided effective tumor control in Indian children. Factors predictive of regression patterns included age, tumor size and its location, and the modality of treatment.