Thalassemia results from defects in normal hemoglobin production, and represents the most common inherited anemia worldwide. Diabetes is a complication of b-thalassemia major. We report a case of Diabetes mellitus in a known case of beta thalassemia major. Patient had undergone Splenectomy 1 year back. Patient is taking chelating agent Defasirox 1000mg orally once a day in the morning. Family history reveals, born through third degree consanguineous marri age. The patient was then subjected for laboratory examination reveals BSL was high, urine ketone 2+,urine sugar 3+, ABG was normal, HbA1c was 13 & 3 month old report of  serum ferritin 1200 ng/dl. Multidisciplinary management was instituted. Blood sugar level got controlled over subcutaneous insulin. Patient may have landed in Diabetic ketoacidosis but was promptly diagnosed & treated. This case is presented for its rarity. As the life expectancy of patients with thalassaemia increases, this will also expose our patients potentially to many more years of hyperglycaemia and diabetes. Sustaining metabolic control and controlling cardiovascular risk factors will be critical in the future for preventing complications due to diabetes.

Primary Congenital Glaucoma is an inherited condition which occurs during development of eye. There is defect of trabecular meshwork as well as anterior chamber angle of eye, with consequent high intra ocular pressure of eye.Buphthalmos (Greek for ox eye) was coined in 4th century BC by Hippocrates, this name suggests large eyeballs arising from chronic IOP elevation. Primary congenital glaucoma (PCG) is most common non-syndromic glaucoma occurring in infancy, which can lead to blindness or can offer a lifetime of vision if diagnosed and treated properly. Congenital glaucoma presents with classic trio of symptoms i.e. photophobia, epiphora, blepharospasm and typical signs comprising of corneal clouding, megalocornea, and buphthalmos.[1] Delayed diagnosis, limited treatment and inadequate follow up leads to a heavier burden on the person and community in developing world.[2] Most of the primary congenital glaucoma(PCS) cases are sporadic, while 10-40% familial association, Autosomal recessive with incomplete or variable penetrance pattern and infrequently autosomal dominant. PCG is more common in populations with a larger prevalence of consanguinity and shows variable expressivity and phenotypes in association with CYP1B1 gene mutations. Definitive treatment is early surgical intervention.[3] The purpose of this study is to report three cases of congenital glaucoma presenting to a tertiary care hospital. First case is a neonate with incidental finding of buphthalmos which was then investigated, second case is about a month-old infant with congenital glaucoma and the third case deals with the detailed examination, treatment given for the same. Keywords: Blue eye, Buphthalmos, Congenital glaucoma, Intra-ocular pressure, Primary Congenital glaucoma

Sturge Weber syndrome (SWS) is a rare neurocutaneous disease which affects primarily the brain (pial angiomata), skin (facial port-wine-stained birthmark) and eyes (glaucoma). [1]. SWS is a congenital, nonfamilial and sporadic condition caused due to somatic mutation in GNAQ gene located on the long arm of chromosome 9. The incidence of SWS is 1 in every 20,000 to 50,000 live births.[2]. Symptoms of this syndrome are commonly detected in infancy. The most common somatic mutation is activating R183Q GNAQ somatic mutation, recent studies also show association of GNA11 and GNB2 somatic mutations related to SWS.[3] Sturge–Weber Syndrome (SWS) also known as encephalotrigeminal angiomatosis, is a rare congenital, non-hereditary disorder affecting the brain, orofacial, eyes and skin. Involvement is prominently unilateral but can also be bilateral. The pathogenesis of this syndrome is the incomplete regression of Embryonic blood vessels at the appropriate time of development. Therefore, it leads residual blood vessels to form angiomas on face, in ipsilateral side of meninges, and in the ipsilateral eye [1]. Port wine stains are the cutaneous angioma occurring along dermatomes which the ophthalmic and maxillary division of the trigeminal nerve, the fifth cranial nerve supplies. Recent investigations include Brain magnetic resonance imaging, and adjunctive electroencephalography. However, there are insufficient studies about the sensitivity, specificity, negative and positive predictive value of magnetic resonance imaging and electroencephalography and whether screening improves seizure recognition.[4]. There is lack of evidence that a presymptomatic Sturge-Weber syndrome diagnosis with magnetic resonance imaging has better neurodevelopmental outcomes. In Sturge-Weber syndrome, neurodevelopmental outcomes hugely vary upon prompt recognition of neurologic red flags as well as early seizure control.[5] In this case 26 Day old female baby was referred to our hospital i/v/o convulsion with sepsis with aspiration pneumonia and fever & was investigated for Sturge Weber syndrome. Keywords: Convulsion, Developmental delay, Dystrophic calcification, Facial Naevus, Port wine stain, Tram track appearance, Neurocutaneous marker, Seizures, Sturge-Weber Syndrome.