RARE AND INTERESTING CASE OF GOLDENHARS SYNDROME IN A 3 YEARS OLD MALE

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It was An Austrian OPHTHALMOLOGIST Maurice Goldenhar Who Was The first In The Year 1850 To Have Described A Syndrome Complex Charsterised By Cong Presence of a Preauricular Skin Tag Or Appendage Some Times Presence Of Squint Anoohthalmos Coloboma Of Upper Lid Iris Retina Astigmatism Microphthalmos Blephrophymosis Syndrome May Also Be Seen However It Is Very Rare G H S Is Also Termed As Occulo Auriculo Vertebral Syndrome Or Dysplasia And Cranio Facial Dysplasia 80 To 85 Percent Cases Of G H S Syndrome Are Normal From Visual And Mental Facality Point Of View It Is Only In 5 To 15 Percent Cases Of G. H S Their Are Additional Cong Anomolies lWhich Are Due To Incomlplete Development of Ist And Second Branchial Arch Due To Detect in the Genes Not Inhereted Autosomal Dominant And Recessive Or May Be Due To Maternal Gestational D M Or Exposure To Rubella And Influenza Viruses Or Intake Of Drugs Like Cocaine Thalidomide Retinioc Acid Antibiotics One Has To Look For These Additional Cong Anomolies lWhich Are Facial Asymetry High Arched Palate Hare Lip Cleft palate Defects In Kidney like Double Ureters And Hydronephrosis Defects In Spine And Limbs Cong Heart Underlying Orbital Involement Dental Anamolies Hearing Impairment C N S Involvement 7th Nerve Involement Ipselateral Involement Of Trigemenal Nuclii And Trigeminal Anasthesia Impairment of Memory Mental Facality And Intelegence So We Have To Do Following Investegations Ultrasound Abdomen X-rays Spine And Limbs ECHOCARDIOGRAPHY M R I Orbits Dental examination Hearing Test E. E G Scenerio Of Dermiod s Usually Unilateral Can Be Bilateral Though Rare Can Involve Entire Cornea Or May Be Confined To Conjuctiva Only Prevalence 1 In 10 000 Inferotemporal Site Is The Commonest About 70 Percent Male Female Ratio Is 3 To2 Case Report A 3Years Old Make Child Was Seen By Me In My Office Few Years back With Parents Having Noticed Palish White LImbal Lesion At The Inferotemporal site Reye since Birth With Associated Cong Presence of A Preauricular Skin Tag left Ear This was Diagnosed Of The Child Having G H S F T Delivered Male Child From Non Cousin Married Parents No History Of Exposure To Oxygen Or Jaundice Breast Fed Normal Mile Stones Vision Mydriatic Refraction FUNDII All Norma No Other Associated Cong Anamolies So This Child With Grade 1 Limbal Dermiod Normal Intelligence No Other Associated Cong Anamolies Only needed Parentral Counseling And Observations However If Limbal Dermiod Involves Pupillary Area and Thretens Vision Surgery Is The Modality Of Treatment Which Is Both Visual And Cismotic They Are Lamellar Kerataplasty Autograft Stem Cell Graft Aminiituc Membrane Graft Smile Lenticule Tatooing And Fibrin Glue Refrences Their Are Goldenhas Support Groups Their Are Families Of G H S 17 Such Families Are Seen In Greece Children Born In Middle East During Gulf War Born in the Different Military Hospitals Had High Incedence Of G H S Mittal et All Reported 3 Cases Of Optic Nerve Drusens Associated With G H S If Patients Of G. H S Have Facial Asymetry Hare Lip Cleft Palate And Preauricular Skin Tag Or Appendage They Should be Referred To Plastic And Facio Maxillary consultant's For Plastic Repair

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Goldenhars Syndrome is genetic disorder and patience environment and patience friends circle and depression and drugs addicted patience some patients intelligence taking drugs depending on your mind activities like bad or good thoughts, Childhood behaviour ya angree behaviour, love problem, family problem, money problem, this bad habits improve than treatment first meditation and second medicine.. patients slowly slowly bheviour changes improve eyes sight with spects and eyes exercise, diet vitamin And protein fully diet.. and care.. sleeping 8 hours.. better results seen patients behaviour and eyes sight and eyes drops uses .. for eyes nourishment..

Sorry It Should Read As Case

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