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

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Ballast Water Control and Management Regulations

Water is commonly used as ballast for vessels that range from smaller sailboats to large tankers, cruise ships and other vessels. In both larger and smaller vessels, ballasts using water are designed to improve the stability of the vessel. Furthermore, the amount of water ballast can be adjusted as needed based on the weight of the vessel and the need for buoyancy and stability. Increasing water ballast generally requires the vessel to take on water from the area where it is located at the time, and decreasing water ballast discharges water from the vessel into the environment to decrease buoyancy. There are ballast water control and management regulations in place in many areas of the world that are designed to minimize the environmental impact associated with this process.

The Potential Issues There are a number of environmental issues that are related to the use of water ballast. Water that is taken into a vessel in one location may contain a number of biological elements that are native to that area, and these can include everything from microorganisms and pathogens to vegetation and animals. These biological elements are picked up by the vessel, transported to another area and discharged into a unique and foreign environment. The introduction of these non-native biological elements can be destructive in a number of ways. It can affect fragile ecosystems, spread pollution and illness and more. Ballast water control and management regulations are in place to minimize the impact of these potential issues related to water ballast.

Unique Regulations Around the Globe It is important to note that the regulations regarding ballast water vary from country to country. When navigating into unfamiliar waters, it is imperative to explore the local regulations in greater detail. Even regulations between neighboring countries like Canada, the United States and Mexico can vary significantly in certain areas.

Common Regulations Regarding Ballast Water While the regulations around the globe regarding ballast water may vary, many of the underlying principles regarding the regulations may be very similar. This is due in large part to the fact that the regulations largely are designed with the same environmental protection purposes in mind. Common regulations regarding ballast water relate to avoiding taking on water that is in known environmental protection areas or known polluted areas. For example, a marine sanctuary may be a protected environment while an area with visible algae growth may be a contaminated area. Water generally should not be taken on for ballast purposes close to sewage areas, dredging areas and other related areas where contamination or pollution may be more likely. In addition, it may be advised or regulated that ballast tanks be cleaned on a regular basis, and the amount of water discharged close to the coast may be regulated.

These are just a few of the common ballast water management regulations that may be in place in many coastal waters around the globe. It is important for anyone who is navigating a vessel to become familiar with ballast water regulations before entering a new area by sea.

Management Buyout

A management buyout (MBO) is a form of acquisition where a company’s existing managers acquire a large part or all of the company.

Management buyouts are similar in all major legal aspects to any other acquisition of a company. The particular nature of the MBO lies in the position of the buyers as managers of the company, and the practical consequences that follow from that. In particular, the due diligence process is likely to be limited as the buyers already have full knowledge of the company available to them. The seller is also unlikely to give any but the most basic warranties to the management, on the basis that the management knows more about the company than the sellers do and therefore the sellers should not have to warrant the state of the company.

Management buy outs are usually brought about because an owner wishes to retire or because a parent company wants to sell a particular part of its business which it no longer sees as central to its future plans.

Selling to the existing managers is often considered a good way of securing the future of the operation and that of its staff because the existing management teams are a known quantity and the current owner trusts them to look after the business.
The existing management teams often have clear strategies of how to grow the company and to make significant personal wealth as part of the process.
The good news is that MBOs have a relatively high success rate as the management team is familiar with the business and can deal with any issues quickly.
Although simple in concept, there is a lot of value at stake in an MBO process, and all of the parties want to maximize their share of the value. Most of the other parties are experts at MBOs and will also employ their own advisers to look after their interests.
A successful management buy out (MBO) needs a combination of factors in place to ensure its success:

1. The team of managers needs to have a spread of skills and talents. It needs someone who understands the ins and outs of a balance sheet (a financial manager or qualified accountant). It needs someone with vision to see what the business could become, given time and investment.
2. The business must be viable. It does not necessarily have to be profitable but it does have to be capable of achieving profit. Often, MBOs take place because managers feel they could do a better job than the existing management.
3. The existing owner of the business must be willing to sell. If he or she won’t sell, there’s no way of taking it over unless the financial backers and shareholders in the business give their approval.
4. At the end of the whole process, an MBO has to achieve a realistic price for the business. If the existing owners are selling because they want to retire, they’ll hold out for the best possible price. So too will most owners except those who are desperate to get some cash in. But bankers and other financial supporters will not pay just any price the agreed valuation has to reflect the potential of the business.