Corneal dystrophies are defined as a group of inherited corneal disorders characterized by opacification of the cornea. Initial classification of corneal dystrophies was based on the layer of cornea involved but with the advent of better technology, a larger picture has evolved that includes both phenotypic and genotypic variants. With the evolving knowledge, a revised classification has been proposed by the International Committee for Classification of Corneal Dystrophies (IC3D). This classification has taken into account the clinical, histologic and genetic basis of the disease, integrating them into one. Our understanding of corneal dystrophies has reached new heights with mutations identified in at least 14 genes. Even though there has been a vast addition of information to the database, we still come across new variants which may seem enigmatic phenotypically. With the additional new information refining the molecular basis, there is a need to explore further the underlying molecular pathology so as to enable the possibility of better treatment modalities to the affected patients and their families. In view of the recent advances, we hereby review the dystrophies with an aim to provide updated information on the clinical and molecular aspects of corneal dystrophies which will aid in their differential diagnosis and management.
AIMS AND OBJECTIVES To know various causes, sex distribution, socioeconomic status, onset, severity, visual prognosis of paediatric strabismus. METHODOLOGY The present study was undertaken for a period of 18 months. All the patients were selected from those attending the outpatient department. 50 cases of squint were evaluated thoroughly to know the various factors responsible for squint and how it was caused. RESULTS Total Number of Patients who attended OPD were 72,431 (4000 per month). Total Number of Paediatric squint cases among them were 121. So, Incidence of Paediatric squint in general OP was-0.16%. In the present study of total 121 paediatric squint cases, 21 cases did not fulfil the inclusion criteria and in the remaining 100, cases were included by taking every 2 nd patient. Esotropia is seen in 38 children contributing to 76% of total and exotropia is seen in 12 children contributing 24% of total cases. Most common cause of esotropia is Essential Infantile Esotropia contributing to 31% of total esotropias. Males are affected more commonly than females with an incidence of 60%. Esotropia is seen in 38 children contributing to 76% of total and exotropia is seen in 12 children contributing 24% of total cases. In this study, males are affected more commonly than females contributing to 60% of total and females constitute 40% of the total. In this study, incidence of paediatric squint is more commonly seen in lower socioeconomic population contributing 60% of the total. 15 cases are of congenital in onset constituting 30% of total and 35 cases are of acquired in onset constituting 70% of total. Small angle Esotropia and large angle Esotropia: In this study out of total 38 cases of esotropia, large angle esotropias are more common and contributing to 89% of the total. Out of total 50 cases, 16% had an angle of <15⁰ and 76% of total had an angle of 15⁰-30⁰ and 8% of total had an angle of >30⁰. Out of 50 cases, significant family history was present in 15 cases contributing to 30% of total cases. Following early recognition and prompt treatment, the prognosis is usually good. 58% of cases showed good visual improvement. CONCLUSION Parents are naturally worried about the child with squint eyes. So if we know the common aetiological factors, it will be easy for us to counsel the parents towards the prevention and management of this condition.
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