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CASE REPORT OF RARE CASE IF GOLDENHAR SYNDROME IN A 3 YEARS OLD MAKE CHILD TITLE PROF TITLE PROFILE CASE REPORT AFFILIATION AUTHOURS NAME DR GOWHAR AHMAD SR CON OPHTHALMOLOGIST FLORENCE HOSPITAL CHANAPORA SRINAGAR KASHMIR UNIVERSITY OF J AND K COUNTRY INDIA EMAIL ADDRESS gowhar.ahmad1948@gmail.com KEY WORDS CONG LIMBAL DERMIOD PREAURICULAR SKIN TAG PREAURICULAR APPENDAGE SQUINT ANOPHTHALMOS COLOBOMAS OF LID IRIS RETINA ASTIGMATISM MICROPHTHALMOS BLEPHROPHMOSIS SYNDTOME FACIAL ASYMETRY HIGH ARCHED PALATE HARE KIO CLEFT PALATE DEFECTS IN KIDNEY DOUBLE URETERS HYDRONEPHROSIS DEFECTS IN LIMBS SPINE CING HEART UNDERLYING ORBITAL INVOLEMENT DENTAL ANAMOLIES HEARING DEFECT IMPAIRMENT OF MEMORY MENTAL FACALITY 7TH N INVOLVEMENT TRIGEMINAL ANASTHESIA ABASTRACT IT WAS MAURICCE GOLDENHAR AN AUSTRIAN OPHTHALMOLOGIST IN THE YEAR 1850 WAS THE FIRST TO DESCRIBE THE SYNDROME COMPLEX CHARETERISED BY CONG PRESENCE OF LIMBAL DERMIID WITH CONG ASSOCIATED ORESENCE OF PREAURICULAR SKIN TAG OR APPENDAGE SOMETIMES PRESENCE OF SQUINT ANOPHTHALMOS COLOBOMAS OF THE U LID IRIS RETINA ASTIGMATISM MICROPHTHALMOS BLEPHROPHMOSIS SYNDROME MAY ALSO BE SEEN HOWEVER IT IS VERY RARE G H S IS ALSO TERMED AS OCULO AURICULO VERTEBRAL SYNDROME AND CRANIIFACIAL SYNDROME INVOLVIMG HEAD FACE EAR NOSE SOFT PALATE AND MANDIBLE 80 TO 85 CASES OF G H S ARE NORMAL from.visual AND MENTAL FACALITY POINT OF VIEW IT IS OMLY IN 10 TO 15 PERECENT CASES OF G H S THEIR SRE ADDITIONAL CONG ANAMOLIES WHICH ARE DUE TO INCOMPLETE DEVOLPMENT OF 1ST AND SECOND BRANCHIAL ARCH DUE TO DEFECTS IN GENES NOT INHERETED AUTOSOMAL DOMINANT AND RECESSIVE ALSO DUE TO MATERNAL GESTATIONAL D M IR EXPOSURE TO RUBELLA HEAMOPHILUS CYTOMEGALIC INC VIRUS OR INTAKE OF THALIDOMIDE COCAINE AND RETINIOC ACID IN THE FIRST TRIMESTER OF PREGNANCY ONE HAS TO WORK UP FOR THESE ANAMOLIES IN THE FIRM OF USG ABDOMEN XRAY LIMBS SPINE ECHOCARDIOGRAPHY MRI ORBITS OPHTHALMIC EXAMINATION DENTAL EXAMINATION HEARING TESTS E E G CONG DERMIODS ARE USUALLY UNILATERAL CAN BE BILTERAL THOUGH RARE CAN INVOLVE ENTIRE CORNEA BUT MAY BE CONFINED TI CONJUCTIVA ONLY INCIDENCE 1 IN 10 000 INFEROTEMPORAL SITE IS TGE COMMONEST 70 PERCENT MALE FEMALE RATIO 3 TO 2 GRADED ACCORDING TO CONEAL INVOLVEMENT GRADE 1 EPETHELIAL INVOLVEMENT GRADE 2 DESMETS MEMBRANE INVOLEMENT GRADE 3 INVOLVEMENT OF ENTIRE ANT SEGMENT CASE REPORT 3 YEARS OLD MALE CHILD WAS SEEN BY ME SOME TIME AGO WITH PARENTS HAVING NOTICED SMALL PALISH WHITE LESION IN THE INFEROTEMPRAL SITE IF LIMBUS R EYE WITH ASSOCIATED CONG PRESENCE OF L SIDED PREAURICULAR SKIN TAG SYNDROMECOMPKEX DIAGNOSTIC OF G H S FT DELIVERED CHILD FROM NON COUSIN. MARRIED COUPLE FOLLOWING LSCS NO.HISTORY OF EXPOSURE TO OXYGEN JAUNDICE BREAST FED NORMAL MIKE STONES VISION MYDRIATIC REFRACTION AND FUNDII NORMAL NO OTHER ASSOCIATED CONG ANAMOLIES THIS CHILD HAD LIMBAL DERMIOD NOT INVOLVING VISUAL AXIS NEEDED ONLY REASSURANCE FOR THE PARENTS AND OBSERVATION HOWEVER IF THE LIMBAL DERMIOD INVOLVES VISUAL TREATMENT IS SURGICAL WHICH IS BOTH VISUAL AND COSMOTIC SURGICAL MODALITIES ARE LAMELLAR KERATAPLASTY AUTOGRAFT STEM CELL GRAFT SMILE LENTICULE TATOOINING FIBRIN GLUE HERE AFTER EXCITON OF DERMIOD.ITNIS TATTOED TO GET ORIGINAL COLOUR THEN CORNEAL LENTICULE IS OUT IN SITU HELD IN POSITION WITH FIBRIN GLUE SINCE NO STICHES ARE APPLIED SO PIST OPERATIVE PAIN AND ASTIGMATISM IS LESS DISCUSSION G H S IS A GENETIC DISORDER CHARETERISED BY CONG PRESENCE OF CONG LIMBAL DERMIOD WITH CIG ASSOCIATED PRESENCE OF PREAURICULAR SKIN TAG OR APPENDAGE WAS DESCRIBED FIRST BY AN AUSTRIAN OPHTHALMOLOGIST MAURICCE GOLDENHAR IN THE YEAR 1850 80 TO 85 PERCENT CASES OF G H S ARE NORMAL FROM VISUAL AND MENTAL FACALITY POINT OF VIEW ONLY 10 TO.15 PERCENT CASES OF ADDITIONAL CONG ANAMOLIES REFENCES WE GAVE G H S SUPPORT GROUPS THEIR ARE FAMILIES OF G H S 17 DUCH FAMILIES IG G H S ARE SEEN IN GREECE CHILDREN BORN IN MIDDLE EAST IN GULF WAR BORN IN DIFFERENT MILIATRY HOSPITALS HAD HIGH INCEDENCE OF G H S MITTAL ETAL IN YEAR 1968 IN INDIAN JOURNAL OF OPHTHALMOLOGY REPORTED 3 CASES OF OPTIC NERVE DRUSENS ASSOCIATED WITH G H S

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