Prosthodontic Management of Ectodermal Dysplasia with Iliac bone graft and endosseou

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 .

In the maxillary arch ridge the ridge augumentation was done with the help of a iliac crest graft Henceforth , a tooth supported fixed prosthesis i.r.t 24,25,26 as fabricated . Followed by the placement of 2 implants in the premaxillary segment .After three months of osseointegration , a four unit implant supported fixed prosthesis replacing 11 ,12 ,21 and 22 as fabricated .

An iliac crest bone graft as harvested ( fig 3 ) and stabilized to augment the premaxilla ( fig 5 ) .The iliac crest is preferentially selected because it is an autogenous cancellous bone graft and is easily harvested and has predictable acceptance and biointegration . After 2 months of bone augmentation 2 endosseous implants were placed in the augmented premaxilla( fig 6 ). Osseointegration of the implants as allowed for four months , followed by fabrication of four unit fixed partial denture prosthesis in 11, 12, 21and 22 regions. Impression as made using elastomeric impression material by putty ash technique .Abutment analogues and implant analogues were placed in the impression . The impression as poured in die stone by split cast technique. Later three endosseous double stage implants were placed in the mandibular arch ( fig 7 ). After four months of Osseointegration , the implants were uncovered , the abutments ere attached and a customized impression as made , after border molding , in rubber base impression material. The mandibular arch rehabilitated with implant supported overdenture ( fig 8 ) due to economical constraints and ease of rehabilitation .The denture as fabricated from heat cured P.M.M.A. (Trevalon powder and liquid , Dentsply ) .The intaglio surface of the denture as relieved and the female housings of the implant attached using autopolymerising acrylic resin P.M.M.A. (Dentsply ) There as marked improvement in speech and the facial esthetics . Discussion The treatment for a patient of Ectodermal dysplasia varies and generally depends on the persons age , dental agenesis , degree of malformation of teeth , the growth and development of the stomatognathic system of the patient and patient’s motivation . According to Nowak [1] treating the ectodermal dysplasia patient requires the clinician to be knowledgeable in growth and development , behavioral management , techniques in fabrication of prosthesis , the ability to motivate the patient in the use of the prosthesis and the long term follow -up for the modification and/ or replacement of the prosthesis . Prosthodontic treatment for ectodermal dysplasia includes removable partial denture or complete denture , overdenture and implants .These approaches may be used either individually or in combination to provide optimal results . Early prosthodontic treatment is generally recommended from the age of 5 years . This early restoration of facial appearance is essential for normal psychological development . [1] . Rapid growth in early life dictates the use of removable partial or complete dentures for these patients. When full growth is reached, treatment planning may include dental implants to retain, support, and stabilize prostheses. Osseointegrated implants offer an alternative that will provide major improvement in the long-term prognosis for oral rehabilitation. In treatment planning for implant dentistry in these patients, extra care must be taken to determine whether adequate bone level to receive the implants is present and whether there is adequate vertical dimension of bone to support the implants. Diminished bone volume may limit the success of implants, especially in the maxilla . The problems associated with complete denture placement in an ectodermal dysplasia case are mainly associated with periodic adjustment due to growth changes and difficulties in achieving good retention and stability [5] .Difficulties in achieving adequate resistance to lateral and anteroposterior displacement of the denture in hypohidrotic ectodermal dysplasia patient are due to dryness of oral mucosa and underdevelopment of maxillary tuberosities and alveolar ridges .[6] If few teeth are present in the mouth, overdentures are the most desirable treatment options [1,3].Overdentures help in preservation of the alveolar bone . Due to decreased number of abutments conventional fixed prosthodontic treatment needs to be altered . Dental implants may be placed in specific areas of the alveolar ridge to gain support for the FPD . For adult patients with ectodermal dysplasia , dental implants are the treatment of choice because growth has stabilized and implants may be used to support , retain and stabilize the prosthesis [1]. In this case of an 21 year old female and the premaxilla being poorly developed . Hence, an iliac crest autogenous bone graft used to augment the premaxilla followed by placement of 2 endosseous implants . The implants were later rehabilitated with a four unit porcelain fused to metal fixed partial denture . The treatment not only improved the patient- s functional and esthetic status (fig 7 ) but also improved the psychological wellbeing and the social life. Conclusion The study discusses the management of a young lady with Anhidrotic Ectodermal Dysplasia having multiple missing teeth and atrophic partially edentulous maxilla and edentulous mandibular ridge . A prosthodontic rehabilitation consisted of bone augmentation of premaxilla with iliac crest autogenous bone graft . Subsequently , 2 endosseous implants were placed to support a four unit fixed partial denture . An implant supported overdenture fabricated for completely edentulous mandibular arch . Hence , physical , physiological and psychological status of the patient as optimally restored . References 1.Pigno MA , Blackman RB ,Cronin RJ ,Cavazos E (1996 ) Prosthodontic management of ectodermal dysplasia :a review of literature . J Prosthet Dent 2.Pinheiro M ,Freire-Maia N (1994 ) Ectodermal dysplasias : a clinical classification and a casual review . Am J Med Genet 53 : 153- 162 3.Bonilla ED , Guerra L , Luna O (1997 ) Overdenture prosthesis for oral rehabilitation of hypohidrotic ectodermal dysplasia : a case report . Quintessence Int 28 (10): 657-661 Tarjan I , Gabrir K , Rozsa N (2005 ) Early prosthetic treatment of patient with ectodermal dysplasia : a clinical report J Prosthetic Dent 93 : 419-424 REFRENCES

1.Hickey A, Vergo JR TJ: Prosthetic treatments for patients with ectodermal dysplasia. J Prosthet Dent 2001;86:364- 368. 2.Guckes AD, Scurria MS, King TS, et al: Prospective clinical trial of dental implants in persons with ectodermal dysplasia. J Prosthet Dent 2002;88:21-25. 3.Dhanrajani PJ, Jiffry AO: Management of ectodermal dysplasia: a literature review. Dent Update 1998;25:73-75 4.NaBadalung DP: Prosthodontic rehabilitation of an anhidrotic ectodermal dysplasia patient: a clinical report. J Prosthet Dent 1999;81:499-502 5.Pigno MA, Blackman RB, Cronin RJ, et al: Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent 1996;76:541-545 6.Blattner RJ: Hereditary ectodermal dysplasia. J Pediatr 1968;73:444-447 7.Davarpanah M, Moon JW, Yang LR, et al: Dental implants in the oral rehabilitation of a teenager with hypohidrotic ectodermal dysplasia: report of a case. Int J Oral Maxillofac Implants 1997;12:252-258 8.Bolender CL, Law DB, Austin LB: Prosthodontic treatment of ectodermal dysplasia. A case report. J Prosthet Dent 1964;14:317-325 9.Snawder KD: Considerations in dental treatment of children with ectodermal dysplasia. J Am Dent Assoc 1976;93:1177-1179 10.Guckes AD, Brahim JS, McCarthy GR, et al: Using endosseous implants for patients with ectodermal dysplasia. J Am Dent Assoc 1991;122:59-62 4. Sha RM (1990 ) Prosthetic management of hypohidrotic ectodermal dysplasia with anadontia : case report . Aust Dent J 35 : 113-166