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Q U I N T E S S E N C E I N T E R N AT I O N A L

Prosthodontic treatment of hypohidrotic ectodermal dysplasia with complete anodontia: Case report

Karlla Almeida Vieira, DDS, MS1/Milena Schaaf Teixeira, DDS, MS1/ Cecília Gatti Guirado, DDS, MS2/Maria Beatriz Duarte Gavião, DDS, MS2

The hereditary condition known as ectodermal dysplasia is characterized by the absence or defect of 2 or more ectodermally derived structures. A case of a 6-year-old child with hypohidrotic ectodermal dysplasia with complete anodontia is presented. Common dental, oral, and physical conditions were taken into consideration. Clinical management consisted of removable complete dentures to improve psychologic development and to promote better functioning of the stomatognathic system. (Quintessence Int 2007;38:75­80)

Key words: child, complete anodontia, hypohidrotic ectodermal dysplasia, prosthetic treatment

Ectodermal dysplasia (ED) represents a large and complex group of diseases comprising more than 170 different clinical conditions.1,2 ED syndrome affects both males and femaies of all races and ethnic groups. Prevalence is estimated to be 7 cases in 10,000 births. The mortality rate is 30% in infancy or early childhood because of intermittent hyperpyrexia.3,4 ED consists of clinically and genetically heterogeneous groups of disorders, characterized by absence of or incomplete or delayed development of 1 or more of the appendages derived from epidermal tissue (hair, sweat gland, teeth, skin, and nails) or of oral ectodermal origin during embryogenesis.3 When at least 2 types of abnormal ectodermal features occur--for example, malformed teeth and extremely sparse hair--the person is identified as being affected by an ED syndrome.5,6

Two different forms are clinically distinguished: an autosomal inherited form (Clouston syndrome) and a hypohidrotic form (Christ-Siemens syndrome).7­9 Hypohidrotic ectodermal dysplasia (HED) is usually an Xlinked recessive genetic trait, in which case the disorder is fully expressed in men only. However, women who carry a single copy of the disease gene (heterozygote carriers) may exhibit some of the symptoms and findings associated with the disorder. Spontaneous gene mutation is also possible.10 Many individuals with HED also have characteristic facial abnormalities, including a prominent forehead, a sunken nasal bridge (so-called saddle nose), thick lips, and/or a large chin. The skin on most of the body may be abnormally thin, dry, and soft with an abnormal lack of pigmentation (hypopigmentation). However, the skin around the eyes (periorbital) may be darkly pigmented (hyperpigmentation) and finely wrinkled, appearing prematurely aged. In many cases, affected infants and children may also exhibit underdevelopment (hypoplasia) or absence (aplasia) of mucous glands within the respiratory and gastrointestinal tracts and, in some cases, decreased function of certain components of the immune system (eg, depressed

1

Master's degree student, Department of Pediatric Dentistry, Piracicaba Dental School, State University of Campinas, São Paulo, Brazil.

2

Professor, Department of Pediatric Dentistry, Piracicaba Dental School, State University of Campinas, São Paulo, Brazil.

Reprint requests: Dr Karlla Almeida Vieira, Faculdade de Odontologia de Piracicaba (Odontopediatria), Av. Limeira 901, Areião, Piracicaba - SP, Brasil, 13414-903. Fax 55 19 3412 5218. E-mail: [email protected]

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Fig 1

Frontal view of the patient.

Fig 2

Profile of the patient.

lymphocyte function, cellular immune hypofunction), potentially causing an increased susceptibility to certain infections and/or allergic conditions. Many affected infants and children experience recurrent attacks of wheezing and breathlessness (asthma); respiratory infections; chronic inflammation of the nasal passages (atrophic rhinitis); scaly, itchy (pruritic) skin rashes (eczema); and/or other findings.11 In these patients the appearance of the teeth is extremely important because it can affect the patients' self-esteem,12 which creates treatment challenges for the clinician.13 Since oligodontia or complete anodontia leads to atrophy of the alveolar bone, prosthetic treatment is of great value to these patients, from the functional standpoint as well as for psychologic and psychosocial reasons. The most frequent prosthetic treatment for the dental management of ED is removable prosthodontics.12 The need for partial or complete dentures is critical during the preschool years and continues into adulthood. Since alveolar bone development is dependent on the presence of teeth, children with ectodermal dysplasia have little or no bone ridge upon which to construct dentures. Hence, the problems involved in attempting to restore function and appearance are greater than usual.14 The present report describes the prosthetic management of a young child manifesting HED with complete anodontia and

shows the importance of prosthetic therapy to the short-term welfare of the patient.

CASE REPORT

A 6-year-old Caucasian boy was presented to the Department of Pediatric Dentistry of Piracicaba Dental School, State University of Campinas, in São Paulo, Brazil, because of the lack of teeth, speech problems, and mastication difficulties, which resulted in a very restricted diet and esthetic concerns. The child himself seemed anxious to improve his appearance, and his attitude enhanced his compliance with the treatment. The patient is an only child. An evaluation by a pediatrician resulted in the diagnosis of HED with hypotrichosis, hypohidrosis, and bronchial asthma. There were no signs of mental retardation. His mother exhibited mild features of the syndrome. During the extraoral examination a facial physiognomy typical of HED was observed, exhibiting sparse scalp hair, missing eyelashes and eyebrows, prominent forehead and ears, saddle nose, and protuberant and everted lips. The skin was soft, thin, and dry with linear wrinkles and a hyperpigmentation around the eyes and the mouth. The nails were normal. A diminished lower facial height contributed to a senile facial expression (Figs 1 and 2).

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Fig 3 (left)

Intraoral view of the patient. Panoramic radiograph of the patient.

Fig 4 (above)

The intraoral examination revealed complete absence of primary teeth. The oral epithelium was normal in appearance. The palate was shallow and the oral mucosa was healthy with a slight dry appearance. The tongue was relatively large. Evaluation of the diagnostic casts showed underdeveloped alveolar ridges with minimal height (3 mm in both the mandibular and maxillary incisal regions, 4 and 3 mm, respectively, in the mandibular and maxillary regions of the supposed second primary molar) and width (2 mm in both the mandibular and maxillary anterior and 2.0 and 2.5 mm at the left and right sides, respectively) (Fig 3). There was no clinical evidence of tooth development, and complete anodontia was later confirmed by radiographic finding (Fig 4). To improve the child's appearance, mastication, and speech, he was provided with removable complete maxillary and mandibular dentures. The basic principles of removable prosthodontic treatment for children have been reported12,15,16 and were followed in the case presented here. Initial impression with infant trays was done with addition-cured silicone as diagnostic casts. The glabella bearing was adapted to the depressed nasal bridge with a silicone material. Then custom trays were prepared for functional impression. On the master casts, acrylic bases with wax rims were made to establish maxillomandibular relations, and then they were mounted on an articulator. The

maxillomandibular relation was based on fullness of the lower face, appearance of the lips, height of the lower face compared to the upper, occlusal plane relative to the tongue, retracted mandibular position, freeway space, and phonetic tests. Placing marks on the nose and chin and measuring the distance between these marks when the patient hummed in a monotone determined the rest position. An increase of 1 mm in the occlusal vertical dimension was performed for better static and dynamic occlusion, which improved the balance of the dentures. The maxillomandibular recordings were carefully verified at the try-in stage (Fig 5). After the final insertion routine, hygiene instructions for the dentures were given to the patient and his parents. Initially, the patient had some difficulty in keeping the mandibular denture in his mouth. To improve and facilitate its adaptation, he was advised to use a denture adhesive paste for the first few days. A week later, he was completely at ease with his new denture and loathed parting with it, even at bedtime. At recall appointments some adjustments were made to eliminate a little interference (Fig 6). The child began to feel the effect of his appearance, and his parents reported a significant improvement in speech and mastication. Further recalls have taken place every 3 months. As with all removable appliances, there is potential for misplacement. The dentures were replaced after 12 months

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Fig 5

Intraoral view showing the complete prostheses.

Fig 6

Facial view of the patient after treatment.

Fig 7 Prosthetic dentures showing dimensional changes in arch length and width after 1 year.

because of skeletal growth, so new ones were made. A continued conservative approach is still indicated to fit the vertical growth of the mandible (Fig 7).

DISCUSSION

There is little information in the literature, other than clinical reports, with regard to the dental management of the young patient with ED.12 In the case of this patient, the diagnosis of HED was made in the first 2 years of life, as this child suffered repeated bouts of fever that required medical attention.

The most common dental treatment in cases of ED with complete anodontia is removable dentures, as used in the present case. Patients with ED present a characteristically thin and underdeveloped residual ridge, covered by thin mucosa and topped by a cord of movable connective tissue. These features, along with the decreased quantity of saliva, are the main problems encountered in treating such cases.17 In the present case, the dentures had to be constructed very flat, and the amount of saliva was sufficient for the dentures to adhere. There is no definitive time to begin treatment, but Till and Marques18 recommend that an initial prosthesis could be delivered when the child starts school, so that the child could have a better appearance and have time to adapt to the prosthesis. Usually, by the time the child reaches school age, he or she is old enough to recognize the esthetic handicap and is willing to cooperate. In this case, the initial molding was a key stage in determining the patient's cooperation, as he wanted to have a better appearance. The main concern of the prosthodontist treating a patient about 5 years old is the enlargement of facial height and depth. Both are not 90% complete until the age of 14, while facial width and hence dental arch width are 80% complete at 5 years and 90% complete at 11 years.19 Indeed, when dentures are fitted in children of 5 years or older, the first discrepancies to be observed are those resulting from adaptation in the posterior region of the prosthesis.17

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As learning and reinforcement of articulation keeps on until 8 years of age, premature loss of several primary teeth may result in speech abnormalities.20 At the beginning of therapy the patient was quiet and withdrawn. He became communicative and his speech skills improved after the insertion of the complete maxillary and mandibular dentures. Difficulty with mastication has been referred to as a major problem arising from loss of teeth.21 With complete dentures, adequate mastication ability as well as esthetic standards were observed in the patient. Development of a good psychologic self-image was achieved through the esthetic improvement. The cosmetic effect alone seemed to transform the patient into a socially acceptable individual. The vertical dimension of occlusion increase of 1 mm was very well tolerated, in accordance with suggestions from the literature.19,22 Periodic recalls of young patients with ED are also important, because prosthesis modification or replacement will be needed as a result of continuing growth and development.23 The patient is now on a 3-month recall schedule, to allow the necessary adjustments of his dentures. Dental treatment as a phase in the management of ectodermal dysplasia is essential. The first goal of the prosthodontic device is to meet the immediate needs of the young patient, which include mastication, esthetics, speech development, and improvement of psychologic factors. To obtain a successful outcome, there needs to be good communication between the professionals in an interdisciplinary team and the parents/caregivers.

3. It helps the patient develop a good psychologic self-image. 4. It starts at an early age and will necessarily continue for many years. New alternatives for rehabilitation for children with hypohidrotic ectodermal dysplasia, such as the use of implants, must be carefully considered, taking into account the presence of underdeveloped, thin alveolar bones and age.

ACKNOWLEDGMENTS

The authors wish to thank the Postgraduate Program in Pediatric Dentistry, Piracicaba Dental School, for the support to perform this study.

REFERENCES

1. Pinheiro M, Freire-Maia N. Ectodermal dysplasias: A clinical classification and a causal review. Am J Med Genet 1994;53:153­162. 2. Priolo M, Silengo M, Lerone M, Ravazzolo R. Ectodermal dysplasias: Not only "skin" deep. Clin Genet 2000;58:415­430. 3. McLaughlin WS. Congenital absence of all primary and permanent lateral incisors in a carrier of Xlinked hypohidrotic ectodermal dysplasia. Int J Paed Dent 1991;2:99­103. 4. Ziada H, Holland T. Ectodermal dysplasia: A case report. J Irish Dent Assoc 1997;43:127­130. 5. Gorlin RJ, Cohen MM, Hennekam RCM. Hypohidrotic Ectodermal Dysplasia. In: House J (ed). Syndromes of the Head and Neck. Oxford: Oxford University Press, 2001:540­542. 6. Freire-Maia N. Ectodermal dysplasias. Hum Hered 1971;21:309­312.

CONCLUSION

Prosthetic management of children with complete anodontia associated with hypohidrotic ectodermal dysplasia is important because: 1. It provides good esthetics, phonetics, and masticatory comfort. 2. It maintains healthy supporting tissues throughout a lifetime of denture-wearing experience.

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Kupietzky A, Houpt M. Hypohidrotic ectodermal dysplasia: Characteristics and treatment. Quintessence Int 1995;26:285­291.

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Crawford PJM, Aldred MJ, Clarke A. Clinical and radiographic dental finding in X linked hypohidrotic ectodermal dysplasia. J Med Genet 1991;28: 181­185.

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Pigno MA, Blackman RB, Cronin RJ, Cavazos E. Prosthodontic management of ectodermal dysplasia: A review of the literature. J Prosthet Dent 1996; 76:541­545.

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Pinkham JR. Pediatric Dentistry: Infancy through Adolescence, ed 2. Philadelphia: Saunders, 1994: 59­68, 236­237, 401­404, 407, 616­623.

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Bedi R, Devlin H. Full dentures for young children. Dent Update 1982;9:87­92.

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Bakre H, Rapp R, Hadeed G. Clinical management of ectodermal dysplasia. J Clin Pediatr Dent 1995;19: 167­172. 21.

Paul ST, Tandon S, Kiran M. Prosthetic rehabilitation of a child with induced anodontia. J Clin Ped Dent 1995;20:5­8.

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Bolender CL, Law DB, Austin LB. Prosthodontic treatment of ectodermal dysplasia: A case report. J Prosthet Dent 1964;14:317­325. 22.

Al-Sehaibany F, White G. Posterior bite raising effect on the length of the ramus of the mandible in primary anterior cross-bite: Case report. J Clin Ped Dent 1996;21:21­26.

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Walsh JF. Pedodontic prostheses. J Prosthet Dent 1976;36:13­16.

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Laird WRE. Dentures for children. Br Dent J 1996; 121:385­386.

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Sarnat BG, Brodie AG, Kurbacki WH. Fourteen-year report of facial growth in case of complete anodontia with ectodermal dysplasia. AMA Am J Dis Child 1953;86:162­169.

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Shaw RM. Prosthetic management of hypohidrotic ectodermal dysplasia with anodontia: Case report. Aust Dent J 1990;35:113­116.

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