Illica crest bone graft Ectodermal DysplasiaProsthodontic Management of Ectodermal Dysplasia with Iliac bone graft and endosseous implants : A case report
Dr Neeraj Nagpal , Dr K L Gupta, Dr Ruchi Nagpal, Dr Dhruv Sharma
ABSTRACT : Ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures . Persons suffering from ectodermal dysplasia may have various manifestations of the disease that differ in severity and may or may not involve teeth, skin, nails , seat and sebaceous glands. The most common form of ectodermal dysplasia syndrome is hypohidrotic ectodermal dysplasia and is usually inherited as an X- linked recessive trait .Female carriers have a variable degree of clinical manifestations . This case report discusses prosthodontic management of an 18 year old female suffering from ectodermal dysplasia . Clinical management consisted of iliac crest autogenous bone graft to augment premaxilla . Later 2 endosseous implants placed in premaxilla and 2 endosseous implants placed in parasymphysis region of the mandible. After Osseointegration of the implants a four unit fixed partial denture placed in maxilla and an implant supported over denture fabricated for mandibular arch. Keywords : Ectodermal dysplasia , Christ Siemens syndrome ,Hypohidrosis , Hypotrichosis , Hypodontia . Introduction : Ectodermal dysplasia is a hereditary disorder manifested as dysplasia of tissues of ectodermal origin primarily nail, teeth skin and occasionally dysplasia of mesoderm derived tissues . As defined by Freire – Maia the nosologic group of Ectodermal dysplasia is any syndrome that exhibits at least 2 of the features ,that is ,abnormal hair (trichodysplasia ) , abnormal dentition , abnormal nails (onchodysplasia ) and abnormal or missing seat glands (dyshidrosis ) [1]. More than 150 different variants of Ectodermal dysplasia have been described [2]. Hidrotic and hypohidrotic are the 2 forms of Ectodermal dysplasia . In both types teeth and hair are similarly affected , but manifestations in nails and s eat glands and the hereditary pattern tend to differ [3] . The X- linked hypohidrotic form or Christ Siemens syndrome is characterized by clinical triad of hypohidrosis , hypotrichosis and hypodontia . Hidrotic form is inherited as an autosomal dominant trait and affects teeth , hair , and nails but usually spares the s eat glands .Prosthodontic rehabilitation is of paramount importance in patients suffering from Ectodermal dysplasia for functional , physiologic and psychologic reasons [1]. Case Report : An 18 year old female named Reena reported with multiple missing teeth since birth. Her parents reported in difficulty in getting her married due to poor look resulting from several congenitally missing teeth. After clinical and radiographic examination the case diagnosed as Anhidrotic Ectodermal Dysplasia . Family history of Ectodermal dysplasia as negative . The young lady as moderately built with hypotrichosis , scarce eyebrows and scarce eyelashes , dry anhidrotic skin , depressed nasal bridge , thin lips ,dark pigmented skin around periorbital area and nose , facial height as reduced ( fig . 1 ) She reported absence of eat and that her lips and tongue remain dry in all climates . There was no relevant effect on the nails . Intraoral examination revealed presence of partially erupted four teeth in the region of 13, 16, 23 and 26 ( fig 2, 3 ). The edentulous ridge as atrophic with decreased height . the palate as shallow , oral mucosa as normal and dry due to less saliva , the tongue as relatively large . OPG X ray depicted poorly developed premaxilla and completely edentulous mandible .( fig 4 ) Surgical and Prosthodontic Management Initially the impacted canine in the mandibular arch as surgically removed ( seen in the OPG fig 1 ). The ridge augumentation of the premaxilla was done with the help of an autogenious bone graft taken from the patients iliac crest( fig 2 ). Hence, the residual alveolar ridge made suitable for the implant placement .Three implants were placed in the mandibular arch in C,B and D positions , followed by the fabrication of an implant supported mandibular overdenture .
