E c to d e rm a l d y s p la s ia s pose a c h a lle n g e in h a b it a t io n of fu n c tio n and a p p e a ra n c e o f th e d e n titio n . T h e la tte r is im p o rta n t in th e d e v e lo p in g p e rs o n a lity o f th e c h ild . A m a x illa ry o v e rla y d e n tu re and m a n d ib u la r c a s t d e n tu re w ith a B a k e r b a r and c a s t g o ld th im b le c ro w n s w e re c o n s tru c te d fo r a 61/ 2-y e a r-o ld b o y w ith th is c o n d itio n . T h e p a re n ts re p o rte d im p ro v e m e n t in th e c h ild ’s a ttitu d e a fte r d e n ta l tre a tm e n t.

Considerations in dental treatm ent of children with ectoderm al dysplasia

I ■Hj s

K e n n e th D. S n a w d e r, DMD, Je ffe rso n ville , Ind

Ectoderm al dysplasia, a syndrom e reported to be a triad o f hypodontia, hypotrichosis, and hypohidrosis, is com m only associated with other com ponents o f defective developm ent of struc­ tures o f ectoderm al origin.1 Several types of ectoderm al dysplasia are known. T he condition may not becom e apparent until the second year o f life and m ay be diagnosed only after repeated episodes o f fever o f unknow n origin.2 A lthough originally it was thought to be trans­ m itted as an X-linked recessive trait, with the gene being carried by the female and the syn­ drom e m anifested in the male, som e females have been reported to m anifest this syndrom e.2 Several unorthodox patterns o f inheritance have been re p o rte d .1 T he main intraoral feature is anodontia or oli­ godontia. T h ere is usually a decrease in the ver­ tical dim ension as a result of the congenital ab­ sence of teeth and the accom panying alveolar bone. T his gives rise to the pouting, everted lips and an indistinct vermilion border. Pseudorhagades also may be present. D evelopm ent of jaw s and face is otherw ise essentially norm al. The anterior teeth, when present, are conical in sh ap e .1,2 Since children with ectoderm al dysplasia usually have normal m entality and life expec­ tancy, dental care is extrem ely im portant for them . T h eir facial appearance w arrants a pro­ fessional concern for their em otional well being and social progress. Functional needs also must be considered since the difficulty these children

experience in m asticating m ay cause nutritional problem s. T w o and 3 year olds can w ear partial dentures successfully. T heir ability to m asticate food in­ creases, and their nutritional status definitely im proves. A partial denture may be adjusted or rem ade at intervals to allow for the eruption o f perm anent teeth. D enture construction at an early age is desirable to ensure m asticatory efficiency and to reduce the psychologic prob­ lem that may cause the child to feel different.3 T he report presented here directs attention to the special considerations that may be involved in treating patients with ectoderm al dysplasia and to the dram atic change in personality seen by the parents of a patient after com pletion of his dental treatm ent.

Report of case A 6 ^-year-old white boy with ectoderm al dys­ plasia (Fig 1) was seen at the Jam es W hitcom b Riley C hildren’s H ospital D ental Clinic on Sept 13, 1967. H e said that “ the children at my school m ake fun of me because o f my teeth— they call me ‘rat te e th .’ ” T hese com m ents upset him so much that he convinced his parents to seek dental care for him. This was his first visit to the dentist. T he medical history revealed tw o X-linked inherited problem s, ectoderm al dysplasia (anhiJADA, Vol. 93, Decem ber 1976 ■ 1177

Fig 1 ■ Left,

6

-year-old patient with ecto ­

dermal dysplasia. Notice blond, sparse, stiff scalp hair and scant eyebrow s and eyelashes. Frontal em inences are prominent and nose is d epressed and broad. Lips are thick and dry.

Right,

intraoral

view

before

dental

treatm ent show ing oligodontia and conically shaped anterior teeth.

drotic type) and agammaglobulinemia (Bruton’s type). The child was afflicted with recurrent in­ fections and had spent considerable time in the hospital. In fact, he had been hospitalized five times between 2 and 8 months of age for recurrent infections, failure to thrive, and hyperthermia. According to the medical record, diagnosis of ectodermal dysplasia at 8 months of age had been determined on the basis of the history (a sibling who had ectodermal dysplasia died at age 2 years and two other siblings who died shortly after birth) and recurrent high fevers that responded to antibiotic therapy. Other features also were thought to be compatible with those of ectoder­ mal dysplasia.

Agammaglobulinemia was diagnosed through electrophoretic studies of serum proteins and chromotography for urinary amino acids. This disease produces deficiencies of certain mem­ bers of the plasma cell series, causing an inade­ quate synthesis of immunoglobulins and forma­ tion of antibodies. The syndrome is character­ ized by frequent, severe, recurrent infections caused primarily by the common pyogenic bac­ teria. If lymphocyte function is normal, as it is in the majority of instances, the patients tend to respond in a relatively normal manner to viral, fungal, and mycobacterial infections . 4 A note in the medical records by the medical genetics department at Indiana University Medical Cen-

Fig 2 ■ Left, intraoral view of maxilla. Two anterior teeth

w ere removed

b e cau se of

their labial inclination and interference with path of insertion of overlay denture. Right, maxillary overlay denture.

Fig 3 ■ Left, intraoral view of Baker bar that w as used to aid in retention of mandibular cast denture. Right, mandibular cast denture with clasps to attach to Baker bar.

1178 ■ JADA, Vol. 93, December 1976

Fig 4 ■ Left, patient one month after inser­ tion of dentures. His feeling of psychological security is apparent in picture. Above, intra­ oral view of maxillary and mandibular den ­ tures in place and in occlusion.

ter, on Dec 22, 1967, indicated that the occur­ rence of the two genetic diseases was thought to be coincidental. ■ Dental treatment: The oral clinical and radiographic examination revealed oligodontia, ab­ sence of alveolar bone except in the areas where teeth were present, pegged-shaped incisors, and a deep overbite (Fig 1). A maxillary overlay denture and a mandibular cast denture were the preferred restorations. Because of the labial inclination of the maxillary central incisors, these teeth had to be removed to permit insertion of the denture (Fig 2). The severe lack of alveolar bone in the mandibular arch made it necessary to gain retention in an­ other fashion. Cast gold thimble crowns were constructed for the mandibular canines, and a Baker bar was soldered to the crowns (Fig 3). Gold overlay crowns were made to fit over the thimble crowns and incorporated into the cast framework of the lower denture. Clasps to retain the denture to the Baker bar also were included, as recommended by Marvin O. Barnett, DM D, a maxillofacial prosthodontist. After the treatment (Fig 4), the child’s parents reported positive changes in his personality and behavior patterns, evidenced by his wanting to go to school and his more outgoing attitude. They said that he was also experiencing better accep­ tance by his peer group.

Sum m ary

Characteristics of ectodermal dysplasia and agammaglobulinemia have been presented and a case in a 61/i-year-old boy having both genetic diseases is reported. The child had been mocked by his peers at school. A maxillary overlay den­ ture and mandibular cast denture were construct­ ed using a Baker bar and cast gold thimble crowns. The parents reported that the patient’s personality changed favorably after dental treat­ ment was completed. He is now 13 years of age, has had his dentures replaced once, and enjoys a rather active life with the aid of antibiotics and supplemental globulins.

Dr. Snaw der, formerly associate professor and chairman of the departm ent of pedodontics, is consultant to the departm ent and director of pedodontic hospital program s, University of Louisville S ch ool of Dentistry. His practice add ress is Suite 204, 207 Sparks Ave, Jeffersonville, Ind 47130. 1. Gorlin, R.J., and Pindborg, J.J. Syndrom es of the head and neck. New York, McGraw-Hill Book Co., 1964. 2. Finn, S.B. Clinical pedodontics, ed 3. Philadelphia, W. B. Saunders Co., 1967. 3. McDonald, R.E. Dentistry for the child and adolescent. St. Louis, C. V. Mosby Co., 1969. 4. Nelson, W.E.; Vaughan, V.C.; and McKay, R.J. Textbook of pediatrics, ed 9. Philadelphia, W. B. Saunders Co., 1969.

Snawder: ECTODERMAL DYSPLASIA ■ 1179

Considerations in dental treatment of children with ectodermal dysplasia.

Characteristics of ectodermal dysplasia and agammaglobulinemia have been presented and a case in a 6 1/2-year-old boy having both genetic diseases is ...
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