Complete Denture Prosthodontics in Children with Ectodermal Dysplasia: Review of Principles and Techniques

Made by- Dr. Amit PG Prosthodontics 1st year Bidra AS, Martin JW, Feldman E. Complete denture prosthodontics in children with ectodermal dysplasia: review of principles and techniques. Compend Contin Educ Dent. 2010 Jul-Aug;31(6):426-33

ECTODERMAL DYSPLASIA

Hereditary ectodermal dysplasia Ectodermal dysplasia syndrome

Ectodermal dysplasia represents a group of inherited conditions in which two or more ectodermally derived anatomic structures fail to develop. Depending on the type of ectodermal dysplasia. hypoplasia or aplasia of tissues such as skin, hair. nails, teeth, or sweat glands may be seen.

. etiology &pathogenesis
Aberrant development of ectodermal derivatives in early embryonic life. Genes responsible for the varied syndromes are located on different chromosomes and may be mutated or deleted. The various types of this disorder may be inherited in anyone of several genetic patterns including autosomal dominant, autosomal recessive and X-linked .

clinical features
The best known of the ectodermal dysplasia syndromes is

Hvpohidrotic (anhidrotic) ectodermal dysplasia

Hidrotic ectodermal dysplasia (Clouston syndrome)

In most instances, this disorder seems to show an X-linked inheritance pattern; therefore, a male predominance is usually seen.

 The following structures may be affected: Skin-soft,smooth,thin &dry Sweat glands-partial or complete absence-hyperpyrexia Hair- scalp&eyebrowsfine,scanty&blond Facial features-bridge of the nosedepressed Supraorbital ridges, frontal bosses-pronounced Lips- protuberant

Oral Manifestations
Anodontia Altered shape of teethtruncated/cone High arched palate Cleft lip may be present Hypoplasia of salivary glandsxerostomia

.histopathology
Decreased number of sweat glands and hair follicles . The adnexal structures that are present are hypoplastic and malformed.

PROSTHODONTIC AND PSYCHOLOGICAL FACTORS

The treatment of patients with congenital craniofacial defects presents psychosocial as well as technical challenges.

In the general population, physical attractiveness contributes to a positive self-concept and social wellbeing.
Unusual facial features exacerbate the social challenges of meeting new people and getting along with others, particularly inschool years. Lowered self-esteem, speech defects, decreased academic performance, and social isolation may result from merely looking different from ones peers.

These factors can contribute to inappropriate acting out and impaired social interactions.
Hickey AJ, Salter M Prosthodontic and psychological factors in treating patients with congenital andcraniofacial defects. .J Prosthet Dent. 2006 May;95(5):392-6.

PROSTHODONTIC AND PSYCHOLOGICAL FACTORS

A significant percentage (30%-40%) of children with craniofacial abnormalities, experience difficulties with internalizing (shyness, depression, and social isolation) and externalizing (disobedience, fighting, and impulsive behavior) problems, learning disorders, and social competence.

These individuals may develop coping mechanisms to reduce social interaction and avoid scrutiny, including teasing, belittling, and other negative social contacts.

Hickey AJ, Salter M Prosthodontic and psychological factors in treating patients with congenital andcraniofacial defects. .J Prosthet Dent. 2006 May;95(5):392-6.

 When medical and dental interventions improve the appearance and function of a patient with congenital and craniofacial defects, this can have a profound effect on the individuals happiness and productivity. Implant-supported fixed and removable prostheses, overdentures, and traditional fixed and removable prostheses can provide more normal facial contours, an improved smile line, improved arch relationships, and improved function. Implant-supported prostheses can enhance stability, retention, function, and bone preservation.

 The authors have observed that patients with congenital craniofacial defects often feel more positive about themselves after prosthetic treatment. Patients embarrassed by their teeth and facial appearance are frequently less motivated to maintain good oral hygiene or seek regular dental care, resulting in increased tooth loss and destruction of oral tissues; this exacerbates an existing problem. Early intervention can be extremely beneficial for the patients well-being.

FIXED PROSTHODONTICS

TREATMENT CONSIDERATIONS

Fixed prosthodontic treatment is seldom used - because many afflicted individuals have a minimal number of teeth. In addition, ED patients are often quite young when they are first treated, and fixed partial dentures (FPDs) with rigid connectors should be avoided in young, actively growing patients. This is because rigid FPDs could interfere with jaw growth, especially if the prosthesis crosses the midline. Hogeboom presented a case that dramatically demonstrated the occurrence of jaw growth in an individual treated for ED in which the two segments of a detachable fixed prosthesis separated at the midline because of transverse jaw growth.
Pigno MA, Blackman RB, Cronin RJ Jr, Cavazos E Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent. 1996 Nov;76(5):541-5.

FIXED PROSTHODONTICS
Individual crown restorations have no age restrictions related to jaw growth, but larger pulp sizes and shorter crown heights may cause concerns. Recently, direct composite restorations have become the more desirable method of restoring normal morphology to hypoplastic teeth . Crowns and direct composite restorations are often used in combination with removable partial dentures (RPDs). Also, orthodontic treatment may be needed to align the teeth into acceptable positions before RPD fabrication.

Pigno MA, Blackman RB, Cronin RJ Jr, Cavazos E Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent. 1996 Nov;76(5):541-5.

IMPLANTS PROSTHODONTICS
Implant-supported restorations can improve physiologic and psychosocial function when compared with complete dentures, but their placement in growing jaws can cause complications. When implant placement in young ED patients is being considered, their dental and skeletal maturity, not their chronological age, should be the determining factor. An individuals growth curve can help in this determination.

 Regarding placement of osseointegrated implants in a growing jaw guckes et al discuss this issue and recommend that implant placement be postponed until age 13 because of possible implant movement caused by jaw growth, the expense of frequent remakes, and the lack of clinical experience in placing implants in young children. A 1989 Consensus Conference on Implantology concluded that implants should not be placed until maximum jaw growth has occurred, which was reported as up to 15 years of age.

 According to Cronin et al and Oesterle et al, possible consequences of early implant placement include implant submergence because of jaw growth, implant exposurebecause of bone resorption associated with jaw growth, implant movement because of jaw growth, and limitation of jaw growth if the implants are connected by a rigid prosthesis that crosses the midline.

REMOVABLE PROSTHODONTICS
Removable prosthodontics is the most frequent treatment modality used for dental management of ED. Although complete dentures are an acceptable form of treatment, overdentures or RPDs supported by natural teeth are desirable for preservation of alveolar bone.

When removable prostheses are fabricated for ED patients, the dentition stage depicted should be appropriate.
In addition, it may be necessary to fabricate and deliver one denture at a time in young patients with no denture experience,to facilitate accommodation.

COMPLETE DENTURE PRINCIPLES IN CHILDREN DIAGNOSTIC IMPRESSIONS MAKING

preliminary diagnostic impressions is challenging due to limited mouth opening . Make the mandibular impression first - decrease anxiety. Use irreversible hydrocolloid material mixed to a higher viscosity prevent aspiration of the impression material and also facilitate patients' comfort as it sets faster.

Kaakko T, Horn MT, Weinstein P, et al. The influence of sequence of impressions on children's anxiety and discomfort. Pediatr Dent. 2003;25(4):357-364.

FINAL IMPRESSIONS
The authors favor vinyl siloxane or polyether for border molding cleaner and have the required accuracy, better working time, and a fast setting time. Limitation of green stick compound - time consuming, patient discomfort related to the procedure, and potential risk of thermal injury.
Vergo TJJr. Prosthodontics for pediatric patients with congenital/ developmental orofacial anomalies: a long-term follow-up.j prost Dent. 2001;86(4):342-347. Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal

JAW RELATION RECORDS

Heat-processed record bases are recommended - superior stability, confirmation of the final retention, and easier adjustment of occlusion performed in the Iaboratory. Obtaining jaw relation records may be a challenge in overdenture patients - Reducing these teeth below the anticipated occlusal plane may be required in conjunction with elective endodontic therapy. A heat-processed base can aid in this procedure by allowing it to be trimmed to a minimal thickness while maintaining its strength.
Vergo TJJr. Prosthodontics for pediatric patients with congenital/ developmental orofacial anomalies: a long-term follow-up.j prost Dent. 2001;86(4):342-347. Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal dysplasia. J Prostbet Dent 2001 ;86(4):.?64-36

 The OVD - collapsed and needs to be restored to a clinically acceptable position.

In a long-term follow-up of these patients, no temporomandibular joint disorder complications resulted

Conventional methods of analysis using esthetics, phonetics, freeway space, and swallowing have been suggested.
It is desirable to use a fast-setting material to obtain this record in a child due to the limited attention span and cooperation. The authors recommend the use of a silicone-based material in children because of its simplicity of use and a faster setting time

Teeth Selection and Teeth Arrangement

Pediatric denture teeth molds should be used rather than filing adult denture teeth to match the size of the primary teeth. Sometimes adult incisor teeth can be incorporated to simulate a mixed dentition. A technique of preparing customized primary teeth using casts from a child with normal dentate has also been described. Appropriate posterior teeth molds are selected based on the arch size and space availability.

NaBadalung DP. Prosthodontic rehabilitation of an anhidrotic ectodermal dysplasia patient: a clinical report. j Prosthet Dent. 1999;81(5):499-502.

 The authors recommend a monoplane occlusion due to its simplicity and freedom of mandibular movement for the growing child. Considerations in teeth arrangement include arranging the teeth to harmonize with the patient's age, face, and oral cavity. Lip projection is an important consideration in patients with ED because they often have protuberant lips - the record base should not be excessively thick in the labial fiange region, and the arrangement of denture teeth should not increase lip projection. Attention must be paid to incorporate the spacing to make the prosthesis look natural and age appropriate. Incorporating an orthodontic arch wire in the denture prosthesis to simulate a"normal" appearance has also been advocated.

DELIVERY AND FOLLOW-UP

If heat-processed bases were used, the delivery appointment is simplified because the fit would have been verified at the jaw relation appointment and the occlusion refined in the laboratory on the remount casts. The parent and child should be educated about the changes in enunciation, mastication, and swallowing. The patient should be encouraged to use denture adhesives especially for the mandibular denture because its retention is generally insufficient due to the underdeveloped alveolar ridges. Delivering one denture at a time has been advocated to allow the child to adjust to each prosthesis.
Ramos V, Giebink DL, Fisher JG, et al. Complete dentures for a child with hypohidrotic ectodermal dysplasia: a clinical report. J Prosthet Dent 1995;74(4):329-33

COMPLICATIONS AND MANAGEMENT

Caries: Overdenture patients with existing abutment
teeth underneath the dentures need closer monitoring because of oral hygiene issues and dental caries risk.' Use of topical fluoride and the dentures themselves as fluoride carriers have been recommended."

'The authors recommend prescribing a 0.4% stannous fiuoride gel as an anticaries agent for overdenture pdiatrie patients. Patients and parents should be cautioned to avoid the swallowing of excess fluoride.

Toolson LB, Taylor TD. A 10-year report of a longitudinal recall of overdenture pztienu. J Prosthet Dent. 1989;62(2):179-181.

Tissue changes
: Periodontal disease has not been reported as a complication of the abutment teeth. Dead spaces or the space beneath an ill-fitting denture and growing tissues, have been reported to cause inflammatory hyperplasia and epulis formation.

Depending on the severity, this can be managed surgically and pharmaceutically.

After treating the tissues, new dentures should be fabricated. The existing dentures will require a tissue conditioning material in the interim to restore tissue health. The patient should be encouraged to maintain proper oral hygiene.Vergo TJJr. Prosthodontics for pediatric patients with congenital/ developmental
orofacial anomalies: a long-term follow-up.j prost Dent. 2001;86(4):342-347.

DENTURE COMPLICATIONS
the fracture of the mandibular denture at the midline is of particular importance due to its shape to accommodate the narrow anterior mandibular ridge. The acrylic resin in this region needs to be thick enough to resist fractures. The child's dentures will need to be revised periodically to accommodate changes in growth or eruption of occasional abutment teeth. Based on a long-term follow-up of pdiatrie complete denture patients for 2 to 25 years, Vergo recommends relining a denture every 2 to 4 years and making a new denture every 4 to 6 years.

Vergo TJJr. Prosthodontics for pediatric patients with congenital/ developmental orofacial anomalies: a long-term follow-up.j prost Dent. 2001;86(4):342-347.

CONCLUSION
Complete dentures are a simple, inexpensive, and reversible prosthodontic option.
There is a lack of evidence of one technique being superior thus, a simplified approach is necessary. This will perhaps encourage more dentists to treat these patients. The principles described in this article can assist the clinician in using this simple therapeutic option to provide esthetic, functional, and psychological benefits for children and thus contribute to their overall development and well being.

REFERNCES
Bidra AS, Martin JW, Feldman E.Complete denture prosthodontics in children with ectodermal dysplasia: review of principles and techniques. Compend Contin Educ Dent. 2010 JulAug;31(6):426-33 Hickey AJ, Salter M Prosthodontic and psychological factors in treating patients with congenital andcraniofacial defects. .J Prosthet Dent. 2006 May;95(5):392-6. Pigno MA, Blackman RB, Cronin RJ Jr, Cavazos E Prosthodontic management of ectodermal dysplasia: a review of the literature. J Prosthet Dent. 1996 Nov;76(5):541-5.

Kaakko T, Horn MT, Weinstein P, et al. The influence of sequence of impressions on children's anxiety and discomfort. Pediatr Dent. 2003;25(4):357-364.
Vergo TJJr. Prosthodontics for pediatric patients with congenital/ developmental orofacial anomalies: a long-term follow-up.j prost Dent. 2001;86(4):342-347. Hickey AJ, Vergo TJ Jr. Prosthetic treatments for patients with ectodermal dysplasia. J Prostbet Dent 2001 ;86(4):.64-36 NaBadalung DP. Prosthodontic rehabilitation of an anhidrotic ectodermal dysplasia patient: a clinical report. j Prosthet Dent. 1999;81(5):499-502. Ramos V, Giebink DL, Fisher JG, et al. Complete dentures for a child with hypohidrotic ectodermal dysplasia: a clinical report. J Prosthet Dent 1995;74(4):329-33 Toolson LB, Taylor TD. A 10-year report of a longitudinal recall of overdenture pztienu. J Prosthet Dent. 1989;62(2):179-181.