Get documents about "Treatment Considerations for a Patient with Ectodermal Dysplasia
Document Sample
J. Int Oral Health 2010 Case Report
All right reserved
P- ISSN
Treatment Considerations 0976 – 7428
for a Patient with
E- ISSN
Ectodermal Dysplasia: A 0976 – 1799
Case Report
Journal of
#
Patel Jayantilal R* Sethuraman Rajesh** Naveen Y G
Patel Nandini ## International
*M.D.S, Professor and Head, **M.D.S, Reader, #Senior Oral Health
Lecturer, ## Post Graduate Student, Department of
Prosthodontics, K.M Shah Dental College, Vadodara, Gujarat,
India. Contact: drjrpatel33@yahoo.com Prosthodontics
Abstract: Case Report
Ectodermal dysplasia is a hereditary disease characterized by
congenital dysplasia of one or more ectodermal structure and
other accessory appendages. The oral manifestations are
anodontia and poor bony foundation which impairs both Received: Aug, 2010
aesthetic as well as the masticatory function. The
Accepted: Nov, 2010
Prosthodontic management of patients with such dysplastic
condition necessitates a multidisciplinary approach. However
the definitive treatment can only be rendered after the
completion of growth period, and till then a provisional
treatment can be given to enhance the aesthetic and functional
requirement of the patient. This case report describes the
Prosthodontic provisional oral rehabilitation of a 12 year old
male pediatric patient with ectodermal dysplasia.
Bibliographic listing:
Key words: Ectodermal dysplasia, Anodontia, Provisional EBSCO Publishing
Prosthodontic Rehabilitation.
Database, Index
Introduction: Copernicus, Genamics
Journalseek Database
Ectodermal dysplasia (ED) is a hereditary disorder that
can affect several ectodermal structures. These structures may
include: skin, hair, nails, teeth, nerve cells, sweat glands, parts
of the eye and ear, and parts of other organs.
JIOH, December 2010, Volume 2 (Issue 4) 73
74
ED syndromes have been described as a group of available to improve appearance, mastication, and
disorders of morphogenesis displaying two or more speech (12).
of the following signs and symptoms.(1,2,3)
Children with ED usually have a normal
1. Trichondysplasia (abnormal hair) mentality and life expectancy, and their facial
appearance warrants professional concern for their
2. Abnormal dentition emotional well being and social progress (7, 13).
Tanner (14) states ectodermal dysplasia with an
3. Onchondysplasia (abnormal nails)
abnormal appearance may affect normal social and
4. Dyshidrosis (abnormal or missing sweat glands) psychological development in young patients.
Functional needs also must be considered since the
The most reported ED syndrome is x-linked difficulty these children experience in masticating
hypohidrotic (anhidrotic) ED (Christ-Siemens- may cause nutritional problems (7, 13). Therefore,
Touraine syndrome) which affects one to seven dental care for ED patient is important.
Individuals per 10,000 with males afflicted more
frequently than, females(1,2,4,5-8). Patients with This clinical case report describes the oral
hypohidrotic ED generally have prominent rehabilitation of a 12 year-old male ED patient
supraorbital ridges, frontal bossing, and a saddle diagnosed with hypodontia
nose. The maxilla may be under developed and the
lips are thick and prominent. The nose may appear Case report:
pinched and the aleque nasi appears hypoplastic. A 12-year-old male patient with ectodermal
The patient may resemble like an old edentulous dysplasia was referred by his physician to the K.
person (7, 8). The skin is usually dry, scaly, and M. Shah Dental College & Hospital, Piparia for
easily irritated as a result of poorly developed or an examination due to the non-eruption of his
absent sebaceous glands. Sweat glands may also be permanent teeth [Figure-1].
absent or few in number or nonfunctioning which
may result in a increased body temperature. Scalp
hair may be absent, sparse, very fine pigmented, or
abnormal in texture. Eyebrows, eye lashes, and
other body hair may also be sparse or absent. When
hairs are present, they may be fragile, dry, and
generally disorderly because of the lack of
sebaceous glands. Finger and toe nails are usually
normal (8). Orofacial characteristics of this
syndrome include anodontia or hypodontia,
hypoplastic conical teeth, underdevelopment of the
alveolar ridges, frontal bossing, a depressed nasal
bridge, protuberant lips, and hypotrichosis (9, 10).
Teeth in the permanent dentition are frequently
small, conical, tapered (peg shaped), and widely
spaced [Figure – 2]. Lack of alveolar growth may
be associated with this condition and frequently
results in increased interocclusal distance which Fig-1: Preoperative patient photo
allows optimum artificial tooth placement (11).
Patients may present with a marked mandibular
protrusion. Depending on the severity of the His parents consulted a general dentist
condition, various prosthodontic treatments are when the boy was 4 years old, because his milky
JIOH, December 2010, Volume 2 (Issue 4) www.ispcd.org
75
teeth were not erupted and he had difficulty in
speaking and chewing. Treatment was not
possible at that time due to behavioral problems
at that age. At age six, the boy became
uncomfortable with the appearance of his teeth
and very reluctant to smile. He was now
motivated to seek help from a dentist.
Fig-3: A panoramic radiograph revealed six
permanent teeth
Fig-2: Patient photo showing peg shaped central and
lateral incisor Since patient was only 12 year of age with still
A clinical examination revealed only growth period left and also pulp horns were
permanent teeth present in the maxilla. The four significantly high, the treatment plan was
existing teeth were extremely tapered (peg- formulated and executed to give maxillary
shaped) and widely spaced [Figure-2]. All removable partial denture and mandibular
Mandibular teeth were missing. The color of complete denture with composite build up for
alveolar mucosa and gingiva was normal, but maxillary central and lateral incisors for the
the alveolar ridges were rather atrophic except patient’s aesthetic concerns related with the peg
in the areas where teeth were present. shaped teeth.
The patient exhibited typical
characteristics of hypohidrotic ED, including a
saddle nose, fine sparse hair, everted lips in
profile, and hypodontia. The skin of the body
was dry and atrophic. However, the shape of the
fingernails and toenails appeared normal.
During the physical examination he showed no
signs of mental problems and his vital signs
were normal. A panoramic radiograph revealed
six permanent teeth (central incisors, lateral
incisors and molar) in the maxilla & completely
Fig-4: Composite resin build up for maxillary central
edentulous mandibular arches [Figure-3]. and lateral incisors
Treatment plan and procedure Diagnostic impressions were made with
alginate impression material and diagnostic
mounting was done. Maxillary Central &
Lateral Incisors were restored to give
appropriate shape with Composite Resin
JIOH, December 2010, Volume 2 (Issue 4) www.ispcd.org
76
[Figure-4]. Final impressions were made with
elastomeric impression material in upper arch
and zinc-oxide eugenol in lower arch. Master
casts were made with dental stone. Face bow
transfer of maxillary arch was done. Vertical
jaw relations were established. Centric relation
was recorded. Then casts were mounted on semi
adjustable (Hanau) articulator and teeth were set
in balanced occlusion [Figure-5].
Fig-6: Denture inserted in to patient mouth
Fig-5: Maxillo mandibular relation mounted on semi
adjustable articulator
Final trial was taken in the patient’s mouth to
verify vertical relations, centric relations etc.
Then acrylisation was carried out to fabricate Fig-7: Postoperative patient photo
upper acrylic removable partial and lower
complete dentures were fabricated. Laboratory month intervals to make necessary adjustments
remounting was done to remove interference in and monitor the oral hygiene.
centric and protrusive movements. Dentures The patient adapted well to the dentures,
were then inserted in to patient’s mouth and and the treatment improved his masticatory and
checked for high points [Figure-6]. speech functions, aesthetics, and established a
The dentures were planned to remake at more favorable plane of occlusion. The patient’s
intervals to allow for the eruption of the social confidence also improved significantly as
permanent teeth. The patient and his mother a result of treatment. The patient will be
were instructed to handle the dentures carefully evaluated regularly and a more definitive
and to have the patient wear them at all times treatment will be performed once the growth of
except while tooth brushing and sleeping. The child is complete.
dentures were adjusted carefully and regular
recall appointments were scheduled at two- Discussion
ED is usually a difficult condition to treat
with prosthodontic restorations because of the
typical oral deficiencies and the young age
when they are evaluated for treatment.
Therefore, when treating a child with ED, it is
important to motivate both the child as well as
JIOH, December 2010, Volume 2 (Issue 4) www.ispcd.org
77
his parents prior to the treatment and to work bone and also the growing age might cause
with them to ensure their compliance (15). problems in placing endosseous implants in
Literature shows children rejected by their growing children is not recommended as a
peer groups are more likely to become routine practice (18, 19). The short-term
aggressive, delinquent, and may experience survival data reported by Guckes et al (20)
mental health problems in adulthood (16). suggests that it is possible to successfully place
Therefore, successful treatment of the present dental implants in male and female patients of
case can be expected to assist the patient both different ages with ED and congenitally missing
physically and psychologically. The boy’s teeth. However, they stated that a careful
attitude, self confidence, and peer group evaluation of each patient is necessary to
interaction showed signs of significant determine the bone volume available for implant
improvement during treatment. Clinical reports placement (20).
have stated the importance of prosthetic dental Due to the boy’s young age, on-going
treatment in patients with anodontia or development of the jaws and insufficient
hypodontia for physiological and psychosocial quantity of alveolar bone, endosseous implant
reasons (10, 13). placements were not possible. The bone height
The successful use of any prosthesis is and width was not sufficient for implant
dependent on the cooperation and insertion. Application of removable dentures
communication between the dental team and the may be the only restorative option for this
patient and his parents. For example, great care patient. Implant retained prosthesis can be
will need to be taken to maintain the boy’s oral planned after the completion of growth.
hygiene in order to benefit from the long-term
treatment plan. In addition, the boy and his Summary:
parents must be educated and motivated about Young patients with ED need to be
the dental problems related to his genetic and evaluated early by a dental professional to
psychological conditions (15, 16). determine the oral ramifications of the
Treatment of the ED patient generally condition. When indicated, appropriate care
includes a removable and/or fixed partial needs to be rendered throughout the child’s
denture, a complete denture prosthesis growth cycle to maintain oral functions as well
(overlaying affected teeth if the vertical as to address the aesthetic needs of the patient.
dimension of occlusion permits), and an implant This clinical report demonstrates that removable
retained prosthesis when indicated (17). These partial dentures associated with direct composite
treatment modalities can be used individually or restorations can be a reversible and economical
in combination to provide an optimal result. The method of treatment for young ED patients.
proper sequencing of treatment is important to
achieve the desired function and aesthetic References:
results. Because of early age intervention and
the need to frequently modify the intraoral 1. Pigno MA, Blackman RB, Cronin RJ,
prosthesis during rapid growth periods, a Cavazos E. Prosthodontic management of
removable partial denture or complete denture ectodermal dysplasia: A review of
prosthesis is indicated initially (17). Hypodontia literature. J Prosthet Dent1996; 76: 541-5.
is associated with lack of development of the 2. NaBadalung DP. Prosthodontic rehabilitation
alveolar ridge and results in less volume of bone of an anhidrotic ectodermal dysplasia
for support of a conventional prosthesis. patient: A clinical report. J Prosthet Dent
Therefore, implant supported prosthesis can be 1999; 81: 499-502.
recommended for these patients. But the lack of
JIOH, December 2010, Volume 2 (Issue 4) www.ispcd.org
78
3. Tarjan I, Gabris K, Rozsa N. Early prosthetic 11. Hobkirk JA, Brook AH. The management of
treatment of patients with ectodermal patients with severe hypodontia. J Oral
dysplasia: a clinical report. J Prosthet Dent Rehabil 1980;7:289-98.
2005; 93:419-24. 12. Winstanley RB. Prosthodontic treatment of
4. Guckes AD, McCarthy GR, Brahim J. Use of patients with hypodontia. J Prosthet Dent
endosseous implants in a 3-year-old child 1984;52:687-91.
with ectodermal dysplasia: case report and 13. Snawder KD. Considerations in dental
5-year follow-up. Pediatr Dent 1997; treatment of children with ectodermal
19:282-5. dysplasia. J Am Dent Assoc 1976;93:1177-
5. Ramos V, Giebink DL, Fisher JG, 9.
Christensen LC. Complete dentures for a 14. Tanner BA. Psychological aspects of
child with hypohidrotic ectodermal hypohidrotic ectodermal dysplasia. Birth
dysplasia: a clinical report. J Prosthet Dent Defects Orig Artic Ser 1988;24:263-75.
1995;74:329-31. 15. Itthagarun A, King NM. Oral rehabilitation
6. Bonilla ED, Guerra L, Luna O. Overdenture of hypohidrotic ectodermal dysplasia
prosthesis for oral rehabilitation of patient: a 6-year follow-up. Quintessence
hypohidrotic ectodermal dysplasia: a case Int 2000; 312:642-8.
report. Quintessence Int 1997;28:657-65. 16. Itthagarun A, King NM. Ectodermal
7. Suri S, Carmichael RP, Tompson BD. dysplasia: a review and case
Simultaneous functional and fixed applied report.Quintessence Int 1997;28:595-602.
therapy for growth modification and dental 17. Hickey AJ, Vergo JT. Prosthetic treatments
alignment prior to prosthetic habilitation in for patients with ectodermal dysplasia. J
hypohidrotic ectodermal dysplasia: a Prosthet Dent 2001;86:364-8.
clinical report. J Prosthet Dent 18. Guckes AD, Brahim JS, McCarthy GR,
2004;92:428-33. Rudy SF, Cooper LF. Using endosseous
8. Yenisey M, Guler A, Unal U. Orthodontic dental implants for patients with ectodermal
and prosthodontic treatment of ectodermal dysplasia. J AmDent Assoc 1991;122:59-
dysplasia-a case report. Br Dent J 62.
2004;196:677-9. 19. Cronin RJ, Oesterle LJ. Implant used in
9. Levin LS. Dental and oral abnormalities in growing patients. Treatment planning
selected ectodermal dysplasia. Birth concerns. Dent Clin North Am1998; 42:1-
Defects 1988;24:205-27. 34.
10. Pavarina AC, Machado AL, Vergani CE, 20. Guckes AD, Scurria MS, King TS,
Giampaolo ET. Overlay removable partial McCarthy GR, Brahim JS. Prospective
dentures for a patient with ectodermal clinical trial of dental implants in persons
dysplasia: a clinical report. J Prosthet Dent with ectodermal dysplasia. J Prosthet Dent
2001;86:574-7. 2002;88:21-5.
Source of Support: Nil
Conflict of Interest: Not Declared
JIOH, December 2010, Volume 2 (Issue 4) www.ispcd.org
