Christ–Siemens–Touraine–Syndrome : Anhidrotic Ectodermal Dysplasias Presenting in ENT as Atrophic Rhinitis With Nasal Myiasis–3 Case Reports

Dr.Kavitha.P.Palled; DR.Raxith Sringeri R

Volume : III, Issue : VII, July - 2014

Christ–Siemens–Touraine–Syndrome : Anhidrotic Ectodermal Dysplasias Presenting in ENT as Atrophic Rhinitis With Nasal Myiasis–3 Case Reports

Dr. Kavitha. P. Palled, Dr. Raxith Sringeri R

Abstract :

Ectodermal Dysplasia represents a complex group of pathologies containing over 170 clinical features with an incidence of 7 out of 1, 00,000 live born children. CASE 1: 3 yr, male presented with foul smelling discharge and falling of crusts from nose from 2 and half years. Case 2: 4 yr, male child with foul smelling discharge and falling of crusts from nose since 2 yrs. He also had a history of Delayed development of teeth and eathing difficulties since his first month of life. History of upper air pathway infections and recurrent otitis since he was 2 months old. CASE 3: 13 months old female child presented with complains of nasal obstruction and presence of crusts, mucopurulent rhinorrhea, nasal fetidness and eathing difficulties since her first month of life. All cases treated with regular alkaline nasal douches and glucose glycerine packs. The ears were regularly suction cleaned and ofloxacin–dexamethasone–clotrimazole ear drops was applied and adequate dietary rehabilitation with vitamin and mineral supplementation. Antibiotics in the form of ampicillin and cloxacillin for two weeks were administered. Atrophic rhinitis is a type of disease associated with Genetic and molecular alterations of ectodermal dysplasia. Anhidrotic dysplasias can present as Atrophic Rhinitis in ENT. The full–blown presentation of CST syndrome in a female patient is rare.