- Email: [email protected]
Regional odontodysplasia
Regional odontodysplasia Case report
with etiologic
and treatment
considerations
Michael A. Kahn, DDSa and R. Lee Hinson, DDS,b Memphis, Little Rock, Ark.
Tenn.. and
UNIVERSITY OF TENNESSEE Regional odontodysplasia, a relatively rare developmental abnormality of the dental hard tissues, most often unilaterally and segmentally involves the anterior maxillary teeth of young females. Patients often have pulpal pathosis, periodontal manifestations, and irregularities of the eruption sequence of the affected teeth. The resultant reduced density of the enamel and dentin imparts a radiographic appearance of “ghosting” with correspondingly enlarged pulp chambers and canals. Etiology is uncertain; numerous local and systemic factors have been proposed. Treatment may sometimes be controversial, and timing of extractions and restorative procedures is critical. This case report illustrates the signs and symptoms of regional odontodysplasia, to aid clinicians in early recognition of the condition, to provide further evidence that a local circulatory disorder might be involved in its pathogenesis, and to illustrate treatment options that consider the patient’s development and welfare. (ORAL SURC ORAL MED ORAL PATHOL
1!291;72:46%7)
0
dontodysplasia is a relatively rare developmental anomaly of the dental hard tissues most often manifesting unilaterally in the maxillary anterior arch of young females. Enamel and dentin formation is defective in both the primary and permanent dentition, and the affected, often consecutive teeth have a unique radiographic appearance because of their markedly reduced radiodensity. In addition to the clinically abnormal color and texture of the enamel and subsequently exposed dentin, tooth eruption is delayed or does not occur. Furthermore, the patient can have localized pain and/or swelling adjacent to the affected teeth. Etiology remains uncertain and treatment may sometimes be controversial. Although first recognized in 1947 by McCall and Wald,’ Zegarelli et a1.2 in 1963 first termed the condition “odontodysplasia.” Various other terms have since been used (“localized arrested tooth development, ” “odontogenic dysplasia,” “odontogenesis imperfecta, ” “unilateral dental malformation,” “nonhereditary segmental amelogenesis imperfecta,” “ghost teeth,” and “regional odontodysplasia”).3 We investigated the clinical and radiographic signs and aAssistantProfessor,Oral Pathologyand Pathology,Department of Biologic and DiagnosticSciences,Collegesof Dentistry and Medicine,Universityof Tennessee. bin private practice,Little Rock. 7/M/28239 462
symptoms of regional odontodysplasia, present evidence for a vascular etiologic factor, and discuss the options for therapy. CASEREPORT
The patient, a Syear-old Oriental girl, came to a dental office in March 1987 with “pain when eating sweet things.” Intraoral examination revealed what appeared to be gross decay and yellow, discolored teeth in the lower left quadrant, especially the primary first and second molars. Because of lack of patient cooperation, an attempt to take bitewing radiographs was unsuccessful.Visual inspection of the excavated teeth suggested that pulpotomy procedures followed by stainless steel crowns would be needed in the future, and the parents were so informed. In August 1988 the patient returned for a general examination and prophylaxis, and examination of a blister on the gum. Bitewing radiographs were successfullytaken, and the patient was scheduled for pulpotomies and stainless steel crowns on the two primary molars. Subsequent review of the radiographs revealed that pulpotomy procedures had been performed during the interim (Fig. 1). Extraoral examination revealed no physical abnormalities, with symmetric bodily development. On closer examination of the head and neck area a faint red macule was seenon the skin of the left cheek near the inferior border (Fig. 2). The macule displayed fine varicosities, and the clinical impression was a hemangioma. The parents confirmed the presence of this lesion since the patient’s birth, although the lesion had dissipated gradually during the years. Detailed intraoral examination revealed a relatively car-
Regional odontodysplasia
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Number 4
Fig. 1. Intraoral posterior bitewing radiograph of left side. Previously treated primary mandibular molars and affected first permanent mandibular molar are evident.
3. Mandibular arch showing unilateral segmental nature of affected hypoplastic teeth.
Fig.
2. Cutaneous vascular malformation overlying affected teeth.
Fig.
ies-free mouth with normal occlusion, soft tissues, and developing dentition except for the lower left quadrant. The lower left primary central and lateral incisors, canine, and primary first and second molars showed rough, hypoplastic yellow enamel with pitting and a few chalky areas (Fig. 3). Draining fistulas were present between the first and second molars and between the canine and lateral incisor. Additional radiographs revealed a ghostlike appearance of the clinically affected teeth and the unerupted developing lower left permanent first molar and succedaneousteeth (Fig. 4). The pulp chambers were markedly enlarged. The patient was the product of an uncomplicated fullterm first pregnancy. She had no siblings and was in excellent health. She had not had any childhood diseasesor exposure to trauma or radiation. The patient was given penicillin V potassium (Pen-Vee K), 250 mg per 5 ml liquid, 1 teaspoon four times a day, in an attempt to resolve the infection Three weeks later intraoral examination revealed im-
Fig. 4. Occlusal radiograph depicting “ghosting” of affecting primary and permanent teeth of left mandibular arch.
provement in the draining fistulas. However, at a follow-up examination (November 1988) a fistula on the facialattached gingiva reappeared between the canine and lateral incisor. The lateral incisor was mobile and subsequently was extracted and sent to the University of Tennesseeoral pathology laboratory for histologic examination.
464
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October 199 1
Fig. 5. A, Low-power photomicrograph showing large cleft extending from extracted tooth’s incisal edge (I/ to its pulp chamber (PC). Note bacterial debris and necrotic pulp in cleft and pulp chamber, and interglobular dentin (ID) and smaller clefts (arrow) in surrounding dentin. (Hematoxylin-eosin stain; original magnification, X36.) B, Medium-power photomicrograph showing focus of polymorphonuclear leukocytes (arrow) in pulp, and irregular dentinal tubules with associatedinterglobular dentin. (Hematoxylin-eosin stain; original magnification, X 160.) PATHOLOGY
DISCUSSION
A lower left primary lateral incisor examined by the oral pathology laboratory was yellow-white and 1.1 cm long, and showed periapical external resorption. Although morphologically resembling a lateral incisor, the tooth had several small pits on its enamel facial surface. After decalcification the tooth was prepared for microscopic examination in the usual manner and stained with hematoxylin and eosin. Light microscopic inspection revealed an incisor tooth consisting of suppurative pulpitis and dental hard tissues. The amount of enamel matrix remaining after decalcification was insufficient for evaluation. A large cleft lined by bacteria and necrotic debris connected the incisal surface with the necrotic pulp (Fig. 5, A). The dentin contained a reduced number of dentinal tubules with an irregular course. Areas of clefts within the dentin, in addition to large amounts of interglobular dentin, were evident (Fig. 5). In focal areas the predentin zone appeared widened. The pulp chambers and pulp canals had a small amount of necrotic pulp with bacterial debris (Fig. 5, A) and a scattering of polymorphonuclear leukocytes (Fig. 5, B). No evidence of calcification was present within the pulp. The cementum appeared normal, yet external resorption was evident in the periapical region, The periodontal ligament content and structure also appeared normal with no evidence of calcification.
Odontodysplasia, a developmental defect in dental hard tissue morphogenesis, has been reported under various names, but because of the more common consecutive and asymmetric nature of the affected teeth, as in this case, we believe that re@*onal odontodysplasia is the most appropriate term. There have been reports in the literature of bilateral or multiquadrant cases, some of which crossed the midline.4-’ 1 These rarer instances should be referred to as generalized odontodysplasia. Regional odontodysplasia seems to be slightly more prevalent in females, has no racial predilection, and most commonly involves the maxillary arch (2: 1 over mandible).‘O Although no average age at diagnosis has been stated, the condition typically manifests during the time of primary tooth eruption and mixed dentition. Morphologically abnormal teeth with hypoplastic, discolored enamel and dentin are usual. Most often the central and lateral incisors and canine are involved, but any teeth, usually consecutive, may be affected. Often tooth eruption is delayed or fails. If a deciduous tooth is involved, the corresponding permanent tooth is invariably subsequently affected. Often the initial complaint is a periapical abscess and/or gingival swelling in the vicinity of the affected teeth. These symptoms have been attributed to bacterial access of the pulp through clefts in the defective
Volume 72 Number 4
enamel and dentin’ 2 or discontinuous odontogenic epithelium in the surrounding dental follicle.13 Besides clinical discoloration, initial discovery can result from crown fracture or routine radiographic survey. In this particular case the anomaly, having been first mistaken for gross caries and subsequent pulpal pathosis, was correctly diagnosed on further clinical examination. The asymmetric, segmental nature of affected teeth should alert clinicians that perhaps the caries are not routine. Significantly, tooth eruption does not yet seem to be delayed or failed in the affected quadrant. Previous reports of regional odontodysplasia have consistently described its unique characteristic radiographic features. 3-s*7-91’ l-20Because of defective, thin layers of enamel and dentin, subsequently reduced radiodensity results in a faint, fuzzy outline, creating a ghostlike appearance. The pulp chambers and canals are correspondingly enlarged, whereas the roots appear short and stubby with open apices. Additionally, true and false denticles are occasionally seen in the pulp. In the present case most of the radiographic features were evident. A suggestion of pulp calcification was present in the unerupted left permanent first molar. Unfortunately, previous dental treatment hindered evaluation of the primary molars’ pulp chambers and their contents, and the patient has not cooperated in attempts to acquire diagnostic periapical radiographs. The occlusal radiograph, however, clearly indicates the ghostly immature roots and enlarged pulp chambers of the affected teeth and the developing dentition (Fig. 4). The light microscope and ultrastructural features of odontodysplasia have been the subject of much investigation and at times debate.2’-25 Researchers agree that abnormalities exist in all derivatives of the tooth germ, and thus the name is most appropriate. In fact, although other conditions can be similar radiographically (e.g., amelogenesis imperfecta type III, or “shell teeth”), the histologic findings are distinctive. Because of abnormal differentiation of odontoblasts and ameloblasts, defective dentin and enamel are found. The enamel organ consists of irregular enamel with hypoplasia, hypomineralization, and degenerated globular calcifications. The dentin is hypoplastic and contains clefts, interglobular dentin, a widened predentin zone, and a decreased number of dentinal tubules. Osseous metaplasia of the dentin and a scalloped dentinoenamel junction have also been reported.16 Pulpal changes include fibrosis and calcifications. Round calcified bodies attached to the hypoplastic enamel or in the surrounding dental follicle have been reported.5 Sapp and Gardner24 conducted
Regional odontodysplasia
465
an ultrastructural study of the soft tissue follicular calcifications. They described two types of calcifications, A and B, which were composed of aberrant afibrillar cementum and fibrillar cementum, respectively. Schmid-Meie@ reported that the root cementurn was hypoplastic. The affected exfoliated tooth showed some of the previously mentioned microscopic features. Most notable and diagnostic were areas of clefts, interglobular dentin, and irregular and decreased number of dentinal tubules. It is probable that the initial bacterial access to the pulp was through the large existing cleft of the incisal area (Fig. 5, A). The predentin zone appeared widened focally. Loss of the enamel matrix in the decalcification procedure precluded evaluation of the enamel and hindered examining the dentinoenamel junction. The lack of viable pulp and large numbers of bacteria in the pulp chamber and canal reflected the overwhelming bacterial infection of the tooth, which eventuated in irreversible pulpitis with fistula formation. No denticles were visible in the pulp, nor were soft tissue follicular calcifications evident. Other published case reports did not cite follicular calcifications, but as in this case the possibility exists that their absence reflects sampling error rather than a unique subset of the condition. The lack of pulpal denticles is unique but could also reflect sampling error. The cementum appears normal, and the periodontal ligament attachment apparatus shows no pathologic signs. The etiology of odontodysplasia has remained obscure, although numerous theories have been proposed and considered. 4, 15,I8 Suggested factors include local trauma, infection, local ischemia caused by a vascular defect or disorder, irradiation, metabolic and nutritional disturbances or vitamin deficiency, hyperpyrexia, genetic (hereditary) factors, local somatic mutations, neural crest migration disorder, Rh incompatibility, activation of a latent virus in odontogenic epithelium during tooth germ development, and systemic disease. Many of the preceding genetic or local factors have been eliminated because of their inability to explain specific observations (i.e., the distribution and temporal aspects of the affected teeth). It seems logical that, whatever the specific pathogenesis, the causative factor must be active in both deciduous and permanent dentitions. Several reports in the last few years have linked regional odontodysplasia with other conditions, namely hemangiomas,4, 9* ’ ‘326 unilateral hypoplasia of the zygomaticomaxillary complex,26 and the epidermal nevus syndrome. ‘* We believe it seems most plausible that either a local neural disorder (e.g., failure of
466
Kahn and Hinson
neural crest cells to migrate) or a local vascular defect resulting in local ischemia could account for the asymmetric, segmental nature of regional odontodysplasia and cases associated with syndromes.‘s Walton et al.4 reported three cases of regional odontodysplasia with a birthmark occurring on the skin overlying the affected teeth. Other authors9*’ ‘. 26 reported similar findings and speculated a vascular factor. Interestingly, our patient also had a congenital cutaneous hemangioma overlying the affected teeth. We present this case as further possible evidence that vascular pathogenesis should be strongly considered. Ligation27 or resection28 of various head and neck arteries in animal experiments have resulted in similar changes of the dental hard tissues. Conceivably a blood vessel defect or other local disturbance of vascular supply creates a localized ischemia that affects odontogenesis. This factor could cause a continuous ischemia as proposed by Walton et a1.,4 accounting for similar involvement of the primary and permanent dentition. Admittedly, a purely neural disorder cannot be ruled out, and conceivably a combined neurovascular condition could be involved. The possibility also exists that the affected teeth and the hemangioma are independent, concomitant events of genetic or polygenic origin with no cause-and-effect relationship between the two. Treatment of odontodysplasia has been somewhat controversial. Most clinicians elect to extract the involved teeth as soon as possible and insert a prosthetic replacement (e.g., acrylic partial denture). Other clinicians have emphasized restorative procedures, if possible, to protect the erupted affected teeth.5, I5 Timing is the critical factor. In young children unrestorable abscessed teeth should be extracted, but ideally as many affected teeth as possible should be preserved to retain normal jaw development and to lessen the psychological effects. In older children abscessed permanent teeth should be extracted, with others retained until a permanent prosthesis can be constructed. Because of the young age of our patient, the shortterm treatment philosophy was to maintain the present dentition so that normal jaw development may proceed for as long as possible. The deciduous lower left first and second molars have been retreated and will be followed closely because of their guarded prognosis. As long as clinical signs and radiographic features indicate no reinfection, these teeth will be retained in the short run because it is our view that the psychological and developmental benefits outweigh immediate extraction. We anticipate long-term treatment to include extracting the deciduous molars and fabricating an acrylic partial denture. If the lower left
ORAL SURG ORAL. MED ORAL PATHOL October 199 I
permanent molar (or other succedaneous teeth) fails to erupt, further prostheticconstruction will be needed to avoid drifting and supraeruption of the opposing dentition. Besides the acrylic partial denture, the patient likely will later need stainless steel crowns to protect the erupted, affected teeth. Thus the treatment goals include aiding mastication and eruption of uncovered teeth, increasing function, improving esthetics, decreasing psychological effects, and protecting erupted affected teeth.
REFERENCES
McCall JO, Wald SS. Clinical dental roentgenology. 2nd ed. Philadelphia: WB Saunders, 1947:150. Zegarelli EV, Kutscher AH, Applebaum E, et al. Odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1963;16: 187-93. Dayal PK, Mani NJ. Odontodysplasia, report of a case. J Oral Med 1981;36:79-81. Walton JL, Witkop CJ Jr, Walker PO. Odontodysplasia: report of three cases with vascular nevi overlying the adjacent skin of the face. ORAL SURG ORAL MED ORAL PATHOL 1978;46:676-84. 5. Lowe 0, Duperon DF. Generalized odontodvsplasia. J Pedod _ . 1985;9:232-43. 6. I. 8. 9.
10. 11. 12. 13.
14. 15.
16.
17. 18. 19. 20.
Galeone RJ, Philips JF, Pincock DG. Odontodysplasia. ORAL SURC ORAL MED ORAL PATHOL 1970:29:879-80. Herman NG, Moss SJ. Odontodysplasia: report of case. J Dent Child 1977;44:52-4. Hovinga J, Jngenhoes R. Regional odontodysplasia. Int J Oral Surg 1979;8:474-7. Fearne J, Williams DM, Brook AH. Regional odontodysplasia: a clinical and histological evaluation. J Int Assoc. Dent Child 1986;17:21-5. Lustmann J, Klein H, Ulmansky M. Odontodysplasia: report of two cases and review of the literature. ORAL SURG ORAL MED ORAL PATHOL 1975;39:781-93. Guzman R, Elliott MA, Rossie KM. Odontodysplasia in a pediatric patient: literature review and case report. Pediatr Dent 1990;12:45-8. Gardner DG, Sapp JP. Regional odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1973;35:351-64. Dahlijf G, Lindskog S, Theorell K, et al. Concomitant regional odontodysplasia and hydrocephalus. ORAL SURG ORAL MED ORAL PATHOL 1987;63:354-7. Pinkham JR, Burkes EJ. Odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1973;36:841-9. Sadeghi EM, Ashrafi MH. Regional odontodysplasia: clinical, pathologic and therapeutic considerations. J Am Dent Assoc 1981;102:336-9. Hintz CS, Peters RA.^...Odontodysplasia: - report - of an - unusual case and a revtew ot the literature. URAL SURG URAL MED ORAL PATHOL 1972;34:744-50. Alexander WN, Lilly GE, Irby WB. Odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1966;22:814-20. Slootweg PJ, Meuwissen PRM. Regional odontodysplasia in epidermal nevus syndrome. J Oral Pathol 1985;14:256-62. Pruhs RJ, Simonsen CR. Sharma PS, et al. Odontodysplasia. J Am Dent Assoc 1975;91:1057-66. Herold RCB, Lally ET, Gold L. Abnormal tooth tissue in human odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1976;42:357-65.
21.
Lustmann J, Ulmansky M. Structural changes in odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1976;41:193202.
22. Kerebel B, Kerebel L-M. Enamel in odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 198 1;52:404- IO.
Regional odontodysplasia
Volume 72 Number 4 23. Gardner DG, Sapp JP. Ultrastructural, electron-probe, and microhardness studies of the controversial amorphous areas in the dentin of regional odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1977;44:549-59. 24. Sapp JP, Gardner DG. Regional odontodysplasia: an ultrastructural and histochemical study of the soft-tissue calcifications. ORAL SURC ORAL MED ORAL PA~HOL 1973;36:383-92. 25. Gardner DG. The dentinal changes in regional odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1974;38:887-97. 26. Schmid-Meier E. Unilateral odontodysplasia with ipsilateral hypoplasia of the midface: a case report. J Maxillofac Surg 1982;10:119-22. 27. Kraus BS, Myers RE, Clark GR. Teratogenic effects of carotid
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ligation on the developing dentition of the rhesus monkey. Teratology 1969;2:163-72. 28. Lunin M, Devore D. The etiology of regional odontodysplasia [Abstract]. J Dent Res 1976;55:B109. Reprint
requests:
Michael A. Kahn, DDS University of Tennessee College of Dentistry Department BIDX 875 Union Ave. Memphis, TN 38163
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with etiologic
and treatment
considerations
Michael A. Kahn, DDSa and R. Lee Hinson, DDS,b Memphis, Little Rock, Ark.
Tenn.. and
UNIVERSITY OF TENNESSEE Regional odontodysplasia, a relatively rare developmental abnormality of the dental hard tissues, most often unilaterally and segmentally involves the anterior maxillary teeth of young females. Patients often have pulpal pathosis, periodontal manifestations, and irregularities of the eruption sequence of the affected teeth. The resultant reduced density of the enamel and dentin imparts a radiographic appearance of “ghosting” with correspondingly enlarged pulp chambers and canals. Etiology is uncertain; numerous local and systemic factors have been proposed. Treatment may sometimes be controversial, and timing of extractions and restorative procedures is critical. This case report illustrates the signs and symptoms of regional odontodysplasia, to aid clinicians in early recognition of the condition, to provide further evidence that a local circulatory disorder might be involved in its pathogenesis, and to illustrate treatment options that consider the patient’s development and welfare. (ORAL SURC ORAL MED ORAL PATHOL
1!291;72:46%7)
0
dontodysplasia is a relatively rare developmental anomaly of the dental hard tissues most often manifesting unilaterally in the maxillary anterior arch of young females. Enamel and dentin formation is defective in both the primary and permanent dentition, and the affected, often consecutive teeth have a unique radiographic appearance because of their markedly reduced radiodensity. In addition to the clinically abnormal color and texture of the enamel and subsequently exposed dentin, tooth eruption is delayed or does not occur. Furthermore, the patient can have localized pain and/or swelling adjacent to the affected teeth. Etiology remains uncertain and treatment may sometimes be controversial. Although first recognized in 1947 by McCall and Wald,’ Zegarelli et a1.2 in 1963 first termed the condition “odontodysplasia.” Various other terms have since been used (“localized arrested tooth development, ” “odontogenic dysplasia,” “odontogenesis imperfecta, ” “unilateral dental malformation,” “nonhereditary segmental amelogenesis imperfecta,” “ghost teeth,” and “regional odontodysplasia”).3 We investigated the clinical and radiographic signs and aAssistantProfessor,Oral Pathologyand Pathology,Department of Biologic and DiagnosticSciences,Collegesof Dentistry and Medicine,Universityof Tennessee. bin private practice,Little Rock. 7/M/28239 462
symptoms of regional odontodysplasia, present evidence for a vascular etiologic factor, and discuss the options for therapy. CASEREPORT
The patient, a Syear-old Oriental girl, came to a dental office in March 1987 with “pain when eating sweet things.” Intraoral examination revealed what appeared to be gross decay and yellow, discolored teeth in the lower left quadrant, especially the primary first and second molars. Because of lack of patient cooperation, an attempt to take bitewing radiographs was unsuccessful.Visual inspection of the excavated teeth suggested that pulpotomy procedures followed by stainless steel crowns would be needed in the future, and the parents were so informed. In August 1988 the patient returned for a general examination and prophylaxis, and examination of a blister on the gum. Bitewing radiographs were successfullytaken, and the patient was scheduled for pulpotomies and stainless steel crowns on the two primary molars. Subsequent review of the radiographs revealed that pulpotomy procedures had been performed during the interim (Fig. 1). Extraoral examination revealed no physical abnormalities, with symmetric bodily development. On closer examination of the head and neck area a faint red macule was seenon the skin of the left cheek near the inferior border (Fig. 2). The macule displayed fine varicosities, and the clinical impression was a hemangioma. The parents confirmed the presence of this lesion since the patient’s birth, although the lesion had dissipated gradually during the years. Detailed intraoral examination revealed a relatively car-
Regional odontodysplasia
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463
Number 4
Fig. 1. Intraoral posterior bitewing radiograph of left side. Previously treated primary mandibular molars and affected first permanent mandibular molar are evident.
3. Mandibular arch showing unilateral segmental nature of affected hypoplastic teeth.
Fig.
2. Cutaneous vascular malformation overlying affected teeth.
Fig.
ies-free mouth with normal occlusion, soft tissues, and developing dentition except for the lower left quadrant. The lower left primary central and lateral incisors, canine, and primary first and second molars showed rough, hypoplastic yellow enamel with pitting and a few chalky areas (Fig. 3). Draining fistulas were present between the first and second molars and between the canine and lateral incisor. Additional radiographs revealed a ghostlike appearance of the clinically affected teeth and the unerupted developing lower left permanent first molar and succedaneousteeth (Fig. 4). The pulp chambers were markedly enlarged. The patient was the product of an uncomplicated fullterm first pregnancy. She had no siblings and was in excellent health. She had not had any childhood diseasesor exposure to trauma or radiation. The patient was given penicillin V potassium (Pen-Vee K), 250 mg per 5 ml liquid, 1 teaspoon four times a day, in an attempt to resolve the infection Three weeks later intraoral examination revealed im-
Fig. 4. Occlusal radiograph depicting “ghosting” of affecting primary and permanent teeth of left mandibular arch.
provement in the draining fistulas. However, at a follow-up examination (November 1988) a fistula on the facialattached gingiva reappeared between the canine and lateral incisor. The lateral incisor was mobile and subsequently was extracted and sent to the University of Tennesseeoral pathology laboratory for histologic examination.
464
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October 199 1
Fig. 5. A, Low-power photomicrograph showing large cleft extending from extracted tooth’s incisal edge (I/ to its pulp chamber (PC). Note bacterial debris and necrotic pulp in cleft and pulp chamber, and interglobular dentin (ID) and smaller clefts (arrow) in surrounding dentin. (Hematoxylin-eosin stain; original magnification, X36.) B, Medium-power photomicrograph showing focus of polymorphonuclear leukocytes (arrow) in pulp, and irregular dentinal tubules with associatedinterglobular dentin. (Hematoxylin-eosin stain; original magnification, X 160.) PATHOLOGY
DISCUSSION
A lower left primary lateral incisor examined by the oral pathology laboratory was yellow-white and 1.1 cm long, and showed periapical external resorption. Although morphologically resembling a lateral incisor, the tooth had several small pits on its enamel facial surface. After decalcification the tooth was prepared for microscopic examination in the usual manner and stained with hematoxylin and eosin. Light microscopic inspection revealed an incisor tooth consisting of suppurative pulpitis and dental hard tissues. The amount of enamel matrix remaining after decalcification was insufficient for evaluation. A large cleft lined by bacteria and necrotic debris connected the incisal surface with the necrotic pulp (Fig. 5, A). The dentin contained a reduced number of dentinal tubules with an irregular course. Areas of clefts within the dentin, in addition to large amounts of interglobular dentin, were evident (Fig. 5). In focal areas the predentin zone appeared widened. The pulp chambers and pulp canals had a small amount of necrotic pulp with bacterial debris (Fig. 5, A) and a scattering of polymorphonuclear leukocytes (Fig. 5, B). No evidence of calcification was present within the pulp. The cementum appeared normal, yet external resorption was evident in the periapical region, The periodontal ligament content and structure also appeared normal with no evidence of calcification.
Odontodysplasia, a developmental defect in dental hard tissue morphogenesis, has been reported under various names, but because of the more common consecutive and asymmetric nature of the affected teeth, as in this case, we believe that re@*onal odontodysplasia is the most appropriate term. There have been reports in the literature of bilateral or multiquadrant cases, some of which crossed the midline.4-’ 1 These rarer instances should be referred to as generalized odontodysplasia. Regional odontodysplasia seems to be slightly more prevalent in females, has no racial predilection, and most commonly involves the maxillary arch (2: 1 over mandible).‘O Although no average age at diagnosis has been stated, the condition typically manifests during the time of primary tooth eruption and mixed dentition. Morphologically abnormal teeth with hypoplastic, discolored enamel and dentin are usual. Most often the central and lateral incisors and canine are involved, but any teeth, usually consecutive, may be affected. Often tooth eruption is delayed or fails. If a deciduous tooth is involved, the corresponding permanent tooth is invariably subsequently affected. Often the initial complaint is a periapical abscess and/or gingival swelling in the vicinity of the affected teeth. These symptoms have been attributed to bacterial access of the pulp through clefts in the defective
Volume 72 Number 4
enamel and dentin’ 2 or discontinuous odontogenic epithelium in the surrounding dental follicle.13 Besides clinical discoloration, initial discovery can result from crown fracture or routine radiographic survey. In this particular case the anomaly, having been first mistaken for gross caries and subsequent pulpal pathosis, was correctly diagnosed on further clinical examination. The asymmetric, segmental nature of affected teeth should alert clinicians that perhaps the caries are not routine. Significantly, tooth eruption does not yet seem to be delayed or failed in the affected quadrant. Previous reports of regional odontodysplasia have consistently described its unique characteristic radiographic features. 3-s*7-91’ l-20Because of defective, thin layers of enamel and dentin, subsequently reduced radiodensity results in a faint, fuzzy outline, creating a ghostlike appearance. The pulp chambers and canals are correspondingly enlarged, whereas the roots appear short and stubby with open apices. Additionally, true and false denticles are occasionally seen in the pulp. In the present case most of the radiographic features were evident. A suggestion of pulp calcification was present in the unerupted left permanent first molar. Unfortunately, previous dental treatment hindered evaluation of the primary molars’ pulp chambers and their contents, and the patient has not cooperated in attempts to acquire diagnostic periapical radiographs. The occlusal radiograph, however, clearly indicates the ghostly immature roots and enlarged pulp chambers of the affected teeth and the developing dentition (Fig. 4). The light microscope and ultrastructural features of odontodysplasia have been the subject of much investigation and at times debate.2’-25 Researchers agree that abnormalities exist in all derivatives of the tooth germ, and thus the name is most appropriate. In fact, although other conditions can be similar radiographically (e.g., amelogenesis imperfecta type III, or “shell teeth”), the histologic findings are distinctive. Because of abnormal differentiation of odontoblasts and ameloblasts, defective dentin and enamel are found. The enamel organ consists of irregular enamel with hypoplasia, hypomineralization, and degenerated globular calcifications. The dentin is hypoplastic and contains clefts, interglobular dentin, a widened predentin zone, and a decreased number of dentinal tubules. Osseous metaplasia of the dentin and a scalloped dentinoenamel junction have also been reported.16 Pulpal changes include fibrosis and calcifications. Round calcified bodies attached to the hypoplastic enamel or in the surrounding dental follicle have been reported.5 Sapp and Gardner24 conducted
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an ultrastructural study of the soft tissue follicular calcifications. They described two types of calcifications, A and B, which were composed of aberrant afibrillar cementum and fibrillar cementum, respectively. Schmid-Meie@ reported that the root cementurn was hypoplastic. The affected exfoliated tooth showed some of the previously mentioned microscopic features. Most notable and diagnostic were areas of clefts, interglobular dentin, and irregular and decreased number of dentinal tubules. It is probable that the initial bacterial access to the pulp was through the large existing cleft of the incisal area (Fig. 5, A). The predentin zone appeared widened focally. Loss of the enamel matrix in the decalcification procedure precluded evaluation of the enamel and hindered examining the dentinoenamel junction. The lack of viable pulp and large numbers of bacteria in the pulp chamber and canal reflected the overwhelming bacterial infection of the tooth, which eventuated in irreversible pulpitis with fistula formation. No denticles were visible in the pulp, nor were soft tissue follicular calcifications evident. Other published case reports did not cite follicular calcifications, but as in this case the possibility exists that their absence reflects sampling error rather than a unique subset of the condition. The lack of pulpal denticles is unique but could also reflect sampling error. The cementum appears normal, and the periodontal ligament attachment apparatus shows no pathologic signs. The etiology of odontodysplasia has remained obscure, although numerous theories have been proposed and considered. 4, 15,I8 Suggested factors include local trauma, infection, local ischemia caused by a vascular defect or disorder, irradiation, metabolic and nutritional disturbances or vitamin deficiency, hyperpyrexia, genetic (hereditary) factors, local somatic mutations, neural crest migration disorder, Rh incompatibility, activation of a latent virus in odontogenic epithelium during tooth germ development, and systemic disease. Many of the preceding genetic or local factors have been eliminated because of their inability to explain specific observations (i.e., the distribution and temporal aspects of the affected teeth). It seems logical that, whatever the specific pathogenesis, the causative factor must be active in both deciduous and permanent dentitions. Several reports in the last few years have linked regional odontodysplasia with other conditions, namely hemangiomas,4, 9* ’ ‘326 unilateral hypoplasia of the zygomaticomaxillary complex,26 and the epidermal nevus syndrome. ‘* We believe it seems most plausible that either a local neural disorder (e.g., failure of
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neural crest cells to migrate) or a local vascular defect resulting in local ischemia could account for the asymmetric, segmental nature of regional odontodysplasia and cases associated with syndromes.‘s Walton et al.4 reported three cases of regional odontodysplasia with a birthmark occurring on the skin overlying the affected teeth. Other authors9*’ ‘. 26 reported similar findings and speculated a vascular factor. Interestingly, our patient also had a congenital cutaneous hemangioma overlying the affected teeth. We present this case as further possible evidence that vascular pathogenesis should be strongly considered. Ligation27 or resection28 of various head and neck arteries in animal experiments have resulted in similar changes of the dental hard tissues. Conceivably a blood vessel defect or other local disturbance of vascular supply creates a localized ischemia that affects odontogenesis. This factor could cause a continuous ischemia as proposed by Walton et a1.,4 accounting for similar involvement of the primary and permanent dentition. Admittedly, a purely neural disorder cannot be ruled out, and conceivably a combined neurovascular condition could be involved. The possibility also exists that the affected teeth and the hemangioma are independent, concomitant events of genetic or polygenic origin with no cause-and-effect relationship between the two. Treatment of odontodysplasia has been somewhat controversial. Most clinicians elect to extract the involved teeth as soon as possible and insert a prosthetic replacement (e.g., acrylic partial denture). Other clinicians have emphasized restorative procedures, if possible, to protect the erupted affected teeth.5, I5 Timing is the critical factor. In young children unrestorable abscessed teeth should be extracted, but ideally as many affected teeth as possible should be preserved to retain normal jaw development and to lessen the psychological effects. In older children abscessed permanent teeth should be extracted, with others retained until a permanent prosthesis can be constructed. Because of the young age of our patient, the shortterm treatment philosophy was to maintain the present dentition so that normal jaw development may proceed for as long as possible. The deciduous lower left first and second molars have been retreated and will be followed closely because of their guarded prognosis. As long as clinical signs and radiographic features indicate no reinfection, these teeth will be retained in the short run because it is our view that the psychological and developmental benefits outweigh immediate extraction. We anticipate long-term treatment to include extracting the deciduous molars and fabricating an acrylic partial denture. If the lower left
ORAL SURG ORAL. MED ORAL PATHOL October 199 I
permanent molar (or other succedaneous teeth) fails to erupt, further prostheticconstruction will be needed to avoid drifting and supraeruption of the opposing dentition. Besides the acrylic partial denture, the patient likely will later need stainless steel crowns to protect the erupted, affected teeth. Thus the treatment goals include aiding mastication and eruption of uncovered teeth, increasing function, improving esthetics, decreasing psychological effects, and protecting erupted affected teeth.
REFERENCES
McCall JO, Wald SS. Clinical dental roentgenology. 2nd ed. Philadelphia: WB Saunders, 1947:150. Zegarelli EV, Kutscher AH, Applebaum E, et al. Odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1963;16: 187-93. Dayal PK, Mani NJ. Odontodysplasia, report of a case. J Oral Med 1981;36:79-81. Walton JL, Witkop CJ Jr, Walker PO. Odontodysplasia: report of three cases with vascular nevi overlying the adjacent skin of the face. ORAL SURG ORAL MED ORAL PATHOL 1978;46:676-84. 5. Lowe 0, Duperon DF. Generalized odontodvsplasia. J Pedod _ . 1985;9:232-43. 6. I. 8. 9.
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Galeone RJ, Philips JF, Pincock DG. Odontodysplasia. ORAL SURC ORAL MED ORAL PATHOL 1970:29:879-80. Herman NG, Moss SJ. Odontodysplasia: report of case. J Dent Child 1977;44:52-4. Hovinga J, Jngenhoes R. Regional odontodysplasia. Int J Oral Surg 1979;8:474-7. Fearne J, Williams DM, Brook AH. Regional odontodysplasia: a clinical and histological evaluation. J Int Assoc. Dent Child 1986;17:21-5. Lustmann J, Klein H, Ulmansky M. Odontodysplasia: report of two cases and review of the literature. ORAL SURG ORAL MED ORAL PATHOL 1975;39:781-93. Guzman R, Elliott MA, Rossie KM. Odontodysplasia in a pediatric patient: literature review and case report. Pediatr Dent 1990;12:45-8. Gardner DG, Sapp JP. Regional odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1973;35:351-64. Dahlijf G, Lindskog S, Theorell K, et al. Concomitant regional odontodysplasia and hydrocephalus. ORAL SURG ORAL MED ORAL PATHOL 1987;63:354-7. Pinkham JR, Burkes EJ. Odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1973;36:841-9. Sadeghi EM, Ashrafi MH. Regional odontodysplasia: clinical, pathologic and therapeutic considerations. J Am Dent Assoc 1981;102:336-9. Hintz CS, Peters RA.^...Odontodysplasia: - report - of an - unusual case and a revtew ot the literature. URAL SURG URAL MED ORAL PATHOL 1972;34:744-50. Alexander WN, Lilly GE, Irby WB. Odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1966;22:814-20. Slootweg PJ, Meuwissen PRM. Regional odontodysplasia in epidermal nevus syndrome. J Oral Pathol 1985;14:256-62. Pruhs RJ, Simonsen CR. Sharma PS, et al. Odontodysplasia. J Am Dent Assoc 1975;91:1057-66. Herold RCB, Lally ET, Gold L. Abnormal tooth tissue in human odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1976;42:357-65.
21.
Lustmann J, Ulmansky M. Structural changes in odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1976;41:193202.
22. Kerebel B, Kerebel L-M. Enamel in odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 198 1;52:404- IO.
Regional odontodysplasia
Volume 72 Number 4 23. Gardner DG, Sapp JP. Ultrastructural, electron-probe, and microhardness studies of the controversial amorphous areas in the dentin of regional odontodysplasia. ORAL SURGORAL MED ORAL PATHOL 1977;44:549-59. 24. Sapp JP, Gardner DG. Regional odontodysplasia: an ultrastructural and histochemical study of the soft-tissue calcifications. ORAL SURC ORAL MED ORAL PA~HOL 1973;36:383-92. 25. Gardner DG. The dentinal changes in regional odontodysplasia. ORAL SURG ORAL MED ORAL PATHOL 1974;38:887-97. 26. Schmid-Meier E. Unilateral odontodysplasia with ipsilateral hypoplasia of the midface: a case report. J Maxillofac Surg 1982;10:119-22. 27. Kraus BS, Myers RE, Clark GR. Teratogenic effects of carotid
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ligation on the developing dentition of the rhesus monkey. Teratology 1969;2:163-72. 28. Lunin M, Devore D. The etiology of regional odontodysplasia [Abstract]. J Dent Res 1976;55:B109. Reprint
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Michael A. Kahn, DDS University of Tennessee College of Dentistry Department BIDX 875 Union Ave. Memphis, TN 38163
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