IgG4‑related intraocular inflammation masquerading as ciliary body melanoma in a young girl.

Immunoglobulin G4 (IgG4‑related diseases) affects various tissues and organs of the human body. Orbital, adnexal, and scleral inflammations were already reported in the medical literature. To the best of our knowledge, we report the first case of intraocular IgG4‑associated inflammatory mass in the ciliary body mimicking as a melanoma in a 23‑year‑old female from Northeast India. Characteristic histopathology, immunohistochemistry in the tissue, protein chemistry, and raised serum IgG4 were supportive for the diagnosis. As this newly diagnosed disease has multi‑organ affection and little is known about its pathogenesis particularly in eye and adnexa, the present case will open many challenges in clinico‑pathological diagnosis and research in the future.

Comparison on testability of visual acuity, stereo acuity and colour vision tests between children with learning disabilities and children without learning disabilities in government primary schools.

Context: Children with learning disabilities might have difficulties to communicate effectively and give reliable responses as required in various visual function testing procedures. Aims: The purpose of this study was to compare the testability of visual acuity using the modified Early Treatment Diabetic Retinopathy Study (ETDRS) and Cambridge Crowding Cards, stereo acuity using Lang Stereo test II and Butterfly stereo tests and colour perception using Colour Vision Test Made Easy (CVTME) and Ishihara’s Test for Colour Deficiency (Ishihara Test) between children in mainstream classes and children with learning disabilities in special education classes in government primary schools. Materials and Methods: A total of 100 primary school children (50 children from mainstream classes and 50 children from special education classes) matched in age were recruited in this cross‑sectional comparative study. The testability was determined by the percentage of children who were able to give reliable respond as required by the respective tests. ‘Unable to test’ was defined as inappropriate response or uncooperative despite best efforts of the screener. Results: The testability of the modified ETDRS, Butterfly stereo test and Ishihara test for respective visual function tests were found lower among children in special education classes (P < 0.001) but not in Cambridge Crowding Cards, Lang Stereo test II and CVTME. Conclusion: Non verbal or “matching” approaches were found to be more superior in testing visual functions in children with learning disabilities. Modifications of vision testing procedures are essential for children with learning disabilities.

Single stage surgery for Blepharophimosis syndrome.

Purpose: The purpose of this study was to report the functional and cosmetic outcome of single stage surgical procedure for correction of the classic components of Blepharophimosis syndrome. Materials and Methods: We report a retrospective case file review of 11 patients with Blepharophimosis syndrome operated between July 2004 and April 2008. Each patient had undergone the correction of epicanthus inversus, telecanthus, palpebral phimosis, and bilateral ptosis as a single-stage surgical procedure. Patients were examined and photographed before and after surgery. The mean follow-up was 3 years (range 2-6 years). Results: A total of 11 patients (8 males, 3 females) with a mean age of 9 years (range 6--22 years) were reviewed. The surgical outcome was assessed both functionally and cosmetically. The mean preoperative visual acuity was 0.729 ± 0.316 SD and the mean postoperative visual acuity was 0.856 ± 0.277 SD (P <0.0428). There was a statistically significant decrease of astigmatism following ptosis correction (P<0.05), improvement of telecanthus (P<0.0001) in terms of IICD (inner intercanthal distance), and HPFL (horizontal palpebral fissure length) (P=0.019) along with improvement of the superior visual field. The mean preoperative and postoperative IICD was 3±0.33 SD and 2.418 ± 0.189 SD, respectively. There was also a significant postoperative improvement of ptosis (P< 0.01), as measured by IPFH (vertical interpalpebral fissure height). All the patients had a stable functional and cosmetic result after a mean follow-up period of 3 years. Conclusion: Single-stage surgical correction of the classic anomalies of Blepharophimosis syndrome provides stable and successful long-term results.

Primary primitive neuroectodermal tumor of the orbit.

Primitive neuroectodermal tumor (PNET) is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS). PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET). This tumor which expresses MIC-2 gene (CD99) seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.

Ocular adnexal lymphoma in the Northeast Indian population.

We present the clinical profile of biopsy and immunohistochemistry-proven ocular adnexal lymphomas in the Northeast Indian population. Nineteen patients between October 2004 and June 2006 with ocular adnexal lymphoma were analyzed retrospectively. Histopathological classification was done according to international working formulation. Twelve patients were male and seven were female. All were diagnosed as non-Hodgkin's lymphoma and the majority were B cell type (89%). Most of the cases (42%)were treated with radiotherapy followed by chemotherapy.

Clinical spectrum of spontaneous carotid-cavernous fistula.

A carotid-cavernous fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. A CCF is divided into two categories, direct and indirect. Direct fistulas usually account for 70 to 90% of all CCF. Spontaneous, low-flow fistulas are usually associated with atherosclerosis, hypertension and collagen vascular disease or may develop in females during peripartum period. The elderly age group, especially women are at increased risk. We report three cases of spontaneous CCF presenting with ocular manifestations and hypertension, without any collagen vascular disease. One case was a direct variety and the other two were of indirect variety.