Benign paraspinal ganglioneuroma with paraneoplastic opsoclonus myoclonus syndrome.

Opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare immune-mediated movement disorder occurring as a paraneoplastic manifestation of neuroblastic tumours (NTs), especially neuroblastoma in infancy. Ganglioneuroma (GN), the benign tumour in the spectrum, is rarely associated with OMAS. We report the case of a child in her second year of life presenting with acute onset of progressive paraplegia and OMAS. MRI showed diffuse and infiltrating left paraspinal mass from T3-T9 levels with differentials of neuroblastoma or ganglioneuroblastoma. Histopathological and immunohistochemistry examination of the excised tumour showed maturing GN. The OMAS was managed with intravenous immunoglobulin and steroids. In the 6-month follow-up, the child has a residual motor weakness with myelomalacia in neuroimaging. The case report substantiates the occurrence of OMAS as paraneoplastic manifestation in NTs, including benign, in children younger than 2 years with a female predilection.

A Rare Case of Primitive Neuroectodermal Tumor of Tongue.

Primitive neuroectodermal tumor of the periphery is a rare entity and belongs to Ewing sarcoma family of tumors. It comprises 1% of all sarcomas and majority of these cases occur in the age group of less than 35 years. It is highly aggressive and patient often presents with distant metastasis leading to poor prognosis. Its characteristic features include t(11:22) translocation. A young female patient presented with growth in the tip of the tongue. Biopsy was done and histopathology using H&E and PAS staining revealed primitive neuroectodermal tumor. Immunohistochemistry helped to confirm the diagnosis. There was no evidence of distant metastasis at the time of presentation. The most accepted modality of treatment is wide surgical excision followed by chemoradiotherapy. The growth was excised and patient was treated with radiotherapy. The patient succumbed to liver metastasis following 10 months of treatment. Peripheral PNET usually affects the long bones like femur and in head and neck region, mandible is most frequently involved. This is a rare case of PNET of tongue with very few references from literature and hence makes it an interesting read.

Tubercular Uveitis with Ocular Manifestation as the First Presentation of Tuberculosis: A Case Series.

Tuberculosis is very common disease in India. It is one of the most common causes of Granulomatous Uveitis in our Country even today. So the strongest suspicion in our mind when we are treating a case of Uveitis, should be TB. We reviewed all the cases of clinically suspected ocular tuberculosis attending the Ophthalmology OPD of Sri Siddhartha Medical College between December 2012 and December 2014 who were refractory to routine uveitis management and later on responded to anti-Tubercular treatment. History of TB contact, Ocular manifestation, Demographic Profile of the patients, Diagnostic test, Treatment regime were looked into. Here by we present a case series of 15 cases of refractory uveitis that later were detected to be of tuberculous origin. We studied the characteristic features, complications and correlation of mantoux test, ESR and Koch's contact with these cases.

Study of Variation in Intraocular Pressure Spike (IOP) Following Nd- YAG Laser Capsulotomy.

INTRODUCTION: Posterior Capsular Opacifications (PCO) is a frequent complication of cataract surgery following posterior chamber intraocular lens implantation. Nd -Yag laser capsulotomy is the treatment of choice for PCO and is known to be associated with complications like Raised Intraocular Pressure (IOP), Intraocular lens pitting, intraocular lens cracks, cystoid macular oedema, retinal detachment, corneal burns. Raised IOP is the most common complication and prescribing anti-glaucoma drugs post capsulotomy is a common practise. Our study helps us to anticipate the post procedural IOP rise in specific patients and treat only selected group of patients with anti- glaucoma medications. AIM: To study and correlate the effect of energy used and number of shots with post procedural IOP spike following Nd-YAG laser capsulotomy cases. MATERIALS AND METHODS: All patients with PCO presenting to Ophthalmology Out Patient Department at Sri Siddhartha Medical College between November 2014 to November 2015 were included. All the patients with glaucoma, uveitis and high myopia were excluded from the study. Data relevant to history, ocular examination and IOP were recorded. RESULTS: Significant correlation of IOP spike with the number of Nd- YAG Laser shots delivered was found by One-way ANOVA Post-Hoc Tukeys Test. The p-value was significant for shots more than 40, provided the energy was restricted to 20 mJ and below. Correlation of energy with IOP spike was not significant as found by One-way ANOVA, Post-Hoc Tukey test. Predictability of 2 hours post-procedure IOP regarding persistent IOP rise was significant. CONCLUSION: It was observed that all pseudophakic patients may not require anti-glaucoma medication pre, or post Nd YAG laser capsulotomy. Only patients who required more than 40 shots during the procedure would need a close observation and if persistent rise is documented, ocular hypotensives may be advised.