PEDIATRICDENTISTRY/Copyright © 1990 by The AmericanAcademy of Pediat~-ic Dentistry Volume 12, Number2 Complete anodontia of the permanent dentition: case report Paul E. Schneider, DDS, MSD Abstract A disturbanceduring the embryologyof tooth formation of addition, permanent teeth are absent, but the primary the seven-year-oldgirl presented in this case resulted in her teeth endure. Symptomsof mesoectodermal dysgenesis having the unusual combinationof nearly a completecomple- include a wide face, eye deformity, muscle dystrophy, ment of primaryteeth but anodontiaof the permanentdenti- underdeveloped premaxilla, and sometimes complete tion. It could not be determinedif this child had any of the hypodontia (Gorlin and Sedano 1971). many syndromes or conditions characterized by missing Ectodermal dysplasia is suspected when a patient teeth. Clinical characteristics and managementof this case are has dystrophic fingernails and toenails, a maladyof the described. eccrine and sebaceous glands, and oligodontia. Types of ectodermal dysplasia include: hypohidrotic or Introduction anhidrotic, hydrotic or Clouston, ectrodactyly, Rapp- About four weeks after fertilization, the cells of the Hodgkin, and Robinson (Behrman and Vaughn 1983). future toothbearing area proliferate and form the ep- Hypohydrotic ectodermal dysplasia usually is sex- ithelial band called the dental lamina. Ten swellings linked recessive with full expression in males. In some appear along the dental lamina and develop into the families (particularly if there is consanguinity), trans- primary teeth. The dental lamina continues to grow and mission is autosomal recessive with expression in both by the end of the fourth monthin utero, the tooth germs sexes. Symptoms include hypohidrosis, anomalous for the permanent first molars have formed. This proc- dentition, hypotrichosis, episodes of fever, frontal boss- ess continues until the third molars are initiated in four- ing, malar hypoplasia, a flattened nasal bridge, everted year-old children. Agenesis of the teeth is the result of a lips, hyperpigmentedperiorbital skin, and low-set ears. disturbance in this process, and the ectodermal tissues The skin over the body is thin and hypopigmented, the do not proliferate and differentiate into highly special- hair is sparse, multiple teeth are congenitally absent, ized cells which make up the tooth buds (Ooe 1981; and the anterior teeth are conical and spaced. There are Provenza 1988). The reason for this deficiency is un- other less common symptoms. Gorlin and Sedano known. Such an interference is relatively common,and (1971) described a 4 1/2 year old girl with hypohidrotic the prevalence of congenitally absent teeth is reported to ectodermal dysplasia and the complete absence of pri- be as high as 8%(Pito 1987). The permanent teeth most mary and permanent teeth. frequently missing congenitally are the third molars, The hydrotic form of ectodermal dysplasia is the second premolars, and the maxillary lateral incisors (Tal autosomal dominant form. Aplasia of the sweat glands, 1981). Many syndromes have hypodontia as a feature, absence of sebaceous glands, dry skin, blond, fine, stiff, and therefore congenitally missing teeth maybe a sig- scant hair, as well as missing eyelashes and eyebrows nificant diagnostic feature of a syndromeor an associ- are common. Few and conical teeth are typical. The ated finding. Fifty-six syndromes have hypodontia as a electrodactyly, Rapp-Hodgkin and Robinson types of clinical feature (Hall 1983). Anodontia, the complete ectodermal dysplasia are autosomal dominant. The absence of the primary and permanent teeth, is a rare ectrodactyly type is characterized by cleft lip and palate and ectodermal anomalies such as thin, dry skin with condition usually associated with a systemic disorder. There is no single etiology for anodontia. It has been glandular deficiency, and anomalies of the hands, feet, proposed that partial or complete anodontia is an evo- eyes, and urinary tract. The dentition also is defective. lutionary stage which will result in mankind not having The Rapp-Hodgkin type of ectodermal dysplasia has teeth (Herbst and Apfelstaedt 1930; Sperber 1963). symptoms which include hypohidrosis, sparse fine hair, oral clefts, and retarded growth. Finally, the Robin- Three syndromes characterized by partial or complete anodontia are oculomandibulodyscephaly, mesoec- son type has symptoms which include deafness, nail todermal dysplasia, and ectodermal dysplasia (Gorlin involvement, and partial anodontia (Behrman 1983). and Pindborg 1964). In a review of the literature, Beierle and Jorgenson Oculomandibulodyscephaly is characterized by (1978) found 14 cases of complete anodontia of the microphthalmia, blue schleras, and microcephaly. In primary and permanent teeth. They also described three cases in which primary teeth were present but the 1 1 2 ANODONTIA -- CASE REPORT: SCHNEIDER permanent teeth were missing. Such cases that have been radiographically documented are summarized in the Table. Case Report A seven-year, seven-month-old Caucasian female reported to the Pediatric Dentistry Clinic at LSU School of Dentistry with a chief complaint of carious primary teeth and missing permanent teeth. Her medical history revealed a period of jaundice shortly after birth but no heart, respiratory, bleeding problems, or allergies. She took no medications. Her height was in the 25th percentile and weight in the 50th percentile. The child was evaluated by an endocrinolo- gist who concluded that even though her symptoms included onychodysplasia, hyperkeratosis of the palms Fig 1. Facial profile shows Fig 2. Full face shows normal depressed nasal bridge and hair, eyes, and facial of the hands and soles of the feet, frontal bossing, and everted lower lip. appearance. saddle nose, the diagnosis of ectodermal dysplasia could not be confirmed. The hereditary background correctly. The primary maxillary lateral incisors were included no known consanguinity in the family or his- congenitally absent, and both the mandibular primary tory of anodontia or ectodermal dysplasia in either the lateral and the right central incisors had exfoliated (Figs maternal or paternal lineages. The grandfather had 3 and 4, see next page). In centric occlusion, the terminal conical mandibular central and lateral incisors. The plane was flush bilaterally, the midline was coincident, mother and father had complete primary and perma- the incisors had no overjet or overbite, and the posterior nent dentitions. The one sibling, a sister, nine years old, teeth were in bilateral crossbite. There were six carious had a normal complement of primary and permanent teeth including the maxillary right and mandibular teeth. right second primary molars. The primary teeth were Extraoral examination revealed a well-developed, slightly delayed in eruption. Neither the parent nor the well-nourished child who was cooperative for examina- child were aware of a bruxism habit, and the loss of the tion. The face was symmetrical, the profile convex, the enamel on the incisal edges of the incisors was probably lower lip everted, and the chin prominent (Figs 1 and 2). the result of attrition due to the end-on occlusion. Facial proportions were nearly normal, although the Confirmation of complete anodontia of the perma- lower facial height was almost equal to the upper facial nent teeth was made by panoramic radiograph (Fig 5, height because the vertical dimension was reduced. The see next page), maxillary, and mandibular anterior soft tissue drape appeared normal in fullness, was occlusal, and bite-wing radiographs of the eight-year- nonwrinkled, and had normal pigmentation. Lacrima- old child. Although the potential for the formation of tion was observed, and the child had normal visual, permanent teeth still may be present at age eight years, olfactory, and gustatory sensations. there was no radiographic evidence of follicular sacs, Oral examination revealed normal mucosal, palatal, calcification of cusps, or other indications of tooth for- and periodontal tissues. The tongue was normal in size, mation. The mandibular primary molars were tauro- and no tongue thrust was exhibited. Linguoalveolar, donts and the incisors; canines and primary molars bilabial and sibilant speech sounds were articulated were conical. TABLE. Summary of Radiographically Confirmed Cases of Children with The first phase of dental treatment in- Primary Teeth and Anodontia of the Permanent Dentition. cluded restoring the anterior carious teeth with resin restorations and the posterior teeth Number of with amalgam or stainless steel crowns. The Author Age Sex Primary Teeth Treatment enamel over the functioning occlusal surfaces Clark 1922 5 Female 5 overdentures of the noncarious posterior teeth showed no Ware 1938 12 Male 18 not described evidence of attrition, and protective restora- Hutchinson 8 Male 20 not described tions were not indicated. To improve esthet- 1953 ics, a modified lingual arch was fabricated to Swallow 11 Male 20 maxillary replace the three missing mandibular incisors. overdenture The child demonstrated excellent home-care Sperber 1963 5 Male 5 overdentures habits, meticulously brushed with a soft Herman 1977 14 Female 17 overdentures PEDIATRIC DENTISTRY: APRIL/MAY, 1990 ~ VOLUME 12, NUMBER 2 113 Fig 3. Mandibular intraoral occlusal view demonstrating arch Fig 4. Maxillary intraoral occlusal view of the congenially form and exfoliated primary incisors. absent lateral and conical central incisors. toothbrush, and consumed