Extraocular retinoblastoma in Indian children:clinical, imaging and histopathological features

AIM: To study eyes with extraocular dissemination (EORB), with the following aims:first to establish the mean lag period and to understand various reasons for delayed presentation, second to study their imaging profiles and third to analyze histopathological features of eyes enucleated after neoadjuvant chemotherapy.METHODS: Prospective study of clinical and imaging features of EORBs (stage Ⅲ and Ⅳ International Retinoblastoma Staging System) presenting to a tertiary eye care centre. Histopathological features of eyes enucleated after receiving neoadjuvant chemotherapy were analyzed. A pictorial illustration of the varied imaging profile of EORB was also presented.RESULTS: Over a period of one year, 97 eyes were diagnosed with retinoblastoma; 32 children (36 eyes) (37.1%) had EORB. Mean age 3.6±1.9 years, 71.9% males, 71.9% unilateral, 3.1% with positive family history and 40.6% with metastasis. On imaging, there was extrascleral involvement in 22.2%, involvement of orbital part of optic nerve in 33.3%, involvement of central nervous system in 27.8% and orbital wall involvement in 2.9% eyes. On histopathological analysis of eyes enucleated after neoadjuvant chemotherapy, 25.0% had no residual viable tumour tissue and rest all tumours were poorly differentiated.CONCLUSION:There are very few human malignancies where definitive treatment is started without any confirmed histopathological diagnosis and imaging plays an important role in diagnosis and appropriate staging of the disease. Chemotherapy has a variable effect on EORB, 75.0% of eyes with EORB had residual viable tumour tissue when enucleated after receiving neoadjuvant chemotherapy

Giant congenital facial melanocytic nevus

Giant Congenital Melanocytic Nevus (GCMNs) is mostly reported in area of trunk followed by limbs and head. Their incidence is <1:20,000 newborns It derives attention due to its association with malignant melanoma.The risk of developing malignant melanoma is between 5 to 10%.We report a case of twelve year old boy with hyperpigmented lesion on face

Outcomes of canalicular trephination versus canaliculodacryocystorhinostomy in common canalicular blocks

AIM: This study aimed to compare the outcomes and efficacy of canalicular trephination with monocanalicular stenting and canaliculodacryocystorhinostomy (canaliculoDCR) with silicone intubation. MATERIALS AND METHODS: A prospective comparative study was done in 30 patients (30 eyes) with common canalicular blocks who were randomized into two groups. Fifteen patients underwent canalicular trephination with monocanalicular stenting and 15 patients underwent canaliculoDCR with silicone intubation. Common canalicular obstruction was diagnosed by preoperative syringing and the location of block was confirmed on probing. Stents were kept in both the groups for 3 months and followed up till 6 months postoperatively. Success was defined based on both anatomical and functional outcomes. Anatomical success was defined by the free passage of fluid on syringing with the fluid felt in the throat. Functional success was defined in terms of relief from epiphora based on the subjective opinion and its categorization by Kraft and Crawford's grading and the fluorescein dye disappearance test (FDDT). RESULTS: Eighty percent of eyes in canalicular trephination group and 73.3% of eyes in canaliculoDCR group were anatomically patent on syringing at the final follow-up. Sixty-six percent of eyes in trephination group and 53.3% in canaliculoDCR group were reported to have absent epiphora (complete recovery). Ten eyes in both groups had Grade 0 and 1 FDDT indicating a functional success of 66.6% in both groups. CONCLUSIONS: Canalicular trephination can produce results comparable to canaliculoDCR with silicone intubation in cases of common canalicular blocks. The average duration of surgery is significantly less in canalicular trephination which gives this procedure an added advantage

Ultrasound biomicroscopy image patterns in normal upper eyelid and congenital ptosis in the Indian population

Purpose: To study the features of upper eyelid in healthy individual and different types of congenital ptosis in the Indian population using ultrasound biomicroscopy (UBM). Methods: This was a prospective observational study at a tertiary care center. Eyelid structure of healthy individuals with no eyelid abnormalities (n = 19); simple congenital ptosis (n = 33) cases; Marcus Gunn jaw-winking ptosis (MGJWP, n = 7) cases, and blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, n = 20) cases were studied on a vertical UBM scan using 50-MHz probe. Lid-thickness, tarsal-thickness, orbicularis oculi and levator-Muller-orbital septum-conjunctival (LMSC) complex were measured in primary gaze. Comparison was made between four groups and results were statistically analyzed using ANOVA test. In normal individuals, LMSC measurements were repeated in down-gaze imaging. Results: Skin with subcutaneous tissue, LMSC complex and pre-aponeurotic fat-pad appeared echodense while orbicularis oculi and tarsus appeared echolucent. In primary gaze, mean thickness (± standard deviation) of the eyelid, tarsus, orbicularis oculi and LMSC, respectively, were: 1.612 ± 0.205, 0.907 ± 0.098, 0.336 ± 0.083, and 0.785 ± 0.135 mm in normal individual. LMSC showed 46.64% increase in thickness on down-gaze. The mean eyelid thickness and LMSC were thicker in MGJWP and BPES as compared to normal. In different types of congenital ptosis cases, various patterns of UBM imaging were observed. Conclusion: UBM allows noninvasive imaging of eyelid structures with good anatomical correspondence in normal eyelids and study the structural alterations of eyelids in different types of congenital ptosis. UBM can be used to highlight the anatomical difference in normal eyelids that may help modify the surgery for better cosmetic outcomes. Furthermore, it has the potential to be used in preoperative evaluation and operative planning in certain types of acquired ptosis, which needs to be evaluated

Clinical profile of the patients with pediatric epiphora in a tertiary eye care center

Purpose: To study the clinical profile of children aged <10 years presenting with epiphora at a tertiary eye care center, to compare the clinical profile between the early onset (<3 years) and the late-onset (≥3 years) group, and to study the success of different treatment modalities. Materials and Methods: A prospective nonrandomized observational study was conducted in 209 eyes of 167 patients (42 bilateral cases). The main outcome measure was postoperative relief of presenting symptoms and signs at 3 months follow-up. Results: Fifty-five percent cases (92 of 167) were early-onset cases, and 45% (75 of 167) were late onset. The male:female ratio was 1.9:1. Seventy-five percent cases were unilateral. The etiological profile was –76% cases of congenital nasolacrimal duct obstruction (NLDO), 18% traumatic/surgical, 4% acquired NLDO, and 2% punctal causes. The overall success rate of all the treatment modalities in our study was 80% (167/208) –82% for sac massage, 77% for probing, 79% for intubation dacryocystorhinostomy, and 100% for punctal surgery. A significant association was noted between the treatment outcome and laterality (P = 0.04), presence of infection (P = 0.032), symptom severity (P = 0.027), history of previous treatment (P = 0.024), and age. No significant association was found between the treatment outcome and gender (P = 0.73), socioeconomic status (P = 0.43), etiology (P = 0.45), and treatment modality (P = 0.33). Conclusion: This study describes the complete range of causes and treatment modalities for pediatric epiphora and highlights the etiology, signs and symptoms, treatment, and the comparative outcome between the early versus the late-onset group and analyses the factors predictive of the outcome