PEDIATRIC DENTISTRY/Copyright © 1990 byThe American Academy of Pediatric Dentistry

Volume 12, Number 6

Infraorbital infection related to odontodysplasia: case reportFred S. Ferguson, DDS Curtis J. Creath, DMD, MS Beth Buono, DDS

Abstract

A case of a 3-year-old, white female with odontodysplasiais presented. Histological considerations and the rapid devel-opment of an infraorbital infection in this case encourage thepractitioner to pursue a more aggressive treatment plan thanmight otherwise be considered.

Introduction

Regional odontoclysplasia (also known asodontodysplasia, odontogenic dysplasia, odon-togenesis imperfecta, or "ghost teeth") is an unusuallocalized anomaly of the dentition. Thus far, approxi-mately 70 cases have been reported in the literature. Acase is presented to highlight the observation andtreatment concerns.

Literature Review

Odontodysplasia is a rare defect of tooth enamel anddentin. It can be discovered upon chief complaint, or asan incidental finding upon presentation for other dentalconcerns. These teeth usually are hypoplastic, oftenwith areas of enamel completely missing on the crown,and can be hypocalcified, yellow, or yellow-browncolored (Walton et al. 1978; Hovinga and Ingenhoes1979; Kerebel and Kerebel 1981; Dayal and Mani 1981;Kerebel and Kerebel 1982). It appears that both theprimary and the permanent dentition are involved, andthough the affected area usually is restricted to onequadrant, there are documented cases of both sides of anarch being involved, and one case of both maxillary andmandibular involvement (Lustmann et al. 1975). Theliterature suggests a 2:1 preference for the maxillaryarch over the mandibular, and a similar preference foranterior teeth over the posterior (Lustmann et al. 1975).Thus far, the majority of cases have been reported infemales (Lustmann et al. 1975).

Lustmann et al. (1975) reviewed 51 cases and founda significant number were associated to varying degreeswith abscesses or swollen gingival areas. Subsequentreports have verified this finding (Walton et al. 1978;Hovinga and Ingenhoes 1979; Anneroth and Ramstr6m

1980; Dayal and Mani 1981). Acute osteitis and facialswellings are not an uncommon finding (Anneroth andRamstr6m 1980; Dahll6f et al. 1987).

Radiographically, the enamel and dentin appear tohave similar radiodensity, and are difficult to distin-guish (Walton et al. 1978; Kerebel and Kerebel 1981).The thinness of the dentin and enamel, along with thesignificantly enlarged pulp chambers, give rise to thecharacteristic "ghost teeth" appearance of the affectedteeth. Sometimes defective or irregular mineralizationof teeth can be seen on radiographs, as can incompleteroot formation (Anneroth and Ramstr6m 1980; Schmid-Meier 1982). Dilated follicles around the crown alsohave been reported (Hovinga and Ingenhoes 1979).

The histological findings indicate significantly ab-normal enamel and dentin formation. Within theenamel layer are partially formed and irregularly pat-terned enamel calcifications, remnants of ameloblastsand significant pits (Hovinga and Ingenhoes 1979;Anneroth and Ramstr6m 1980; Kerebel and Kerebel1982). The DEJ has a unusual scalloped appearance(Sadeghi and Ashrafi 1981; Kerebel and Kerebel 1981).Within the dentin are amorphous areas ofhypermineralization, ground substance (collagen-freedentinal matrix), and additional areas ofhypomineralization and interglobular dentin (Gardnerand Sapp 1977; Anneroth and Ramstr6m 1980; Dayaland Mani 1981; Kerebel and Kerebe11982). The dentinaltubules have been described as larger and more ir-regular, and small canals within the dentin have alsobeen observed (Dayal and Mani 1981; Kerebel andKerebel 1982; Williams and High 1988). Calcifiednodules have been noted within the connective tissuesurrounding the tooth crown (Hovinga and Ingenhoes1979; Anneroth and Ramstr6m 1980). The enamel anddentin defects have not been correlated with chrono-logical insults to the tooth-forming tissues.

The pulp tissue has several abnormal characteristics.Wide pulp canals are a common, but not universal,finding (Hovinga and Ingenhoes 1979). A fibrous pulp

PEDIATRIC DENTISTRY: NOVEMBER/DECEMBER, 1990 - VOLUME 12, NUMBER 6 397

with numerous false and genuine denticles has beenreported (Hovinga and Ingenhoes 1979; Anneroth andRamstrom 1980; Dayal and Mani 1981). Atrophicallydegenerated odontoblasts with a mild inflammatoryreaction pericoronally were noted by Anneroth andRamstrom (1980). They and other investigators alsohave described necrotic pulp with periapical granula-tion tissue, and infiltration of the pulp with lympho-cytes, plasmocytes, and leukocytes (Hovinga andIngenhoes 1979; Anneroth and Ramstrom 1980; Sadeghiand Ashrafi 1981).

The etiology of odontodysplasia is relatively un-known. Possible causes have been described and dis-cussed in the literature, but no consensus has developedconcerning the ones that are most probable. The sug-gested etiologies of odontodysplasia include localizedvascular disorders, neural crest defects, toxic maternaldrug effects, genetic mutation, trauma, localized infec-tion, inherited genetic defect, somatic mutation, neuraldamage, localized viral infection that alters amelo- anddentinogenesis, and maternal hypoxia caused byfainting (Walton et al. 1978; Hovinga and Ingenhoes1979; Anneroth and Ramstrom 1980; Ferguson andGeary 1980; Dayal and Mani 1981; Mock et al. 1986;Dahllof et al. 1987; Williams and High 1988). The effecton both primary and permanent dentitions makes thediscussion of an isolated event more difficult.

Case ReportA 3-year-old, white female presented to the dental

emergency room for facial pain and swelling. Thepatient's medical history was unremarkable and thephysical examination, except for oral findings, waswithin normal limits. Upon clinical examination, anonfluctuant swelling that extended from the leftinfraorbital region to the left lateral border of the noseand upper left lip was noted. The parent said that theswelling had begun 2-3 hr before the child was broughtto the clinic. The child appeared tired and restless, andwas febrile at 100°F (oral). Intraorally, there was diffuseswelling of the left mucobuccal fold adjacent to severelydysplastic and mobile maxillary left primary centraland lateral incisors, and canine. No draining fistulaswere noted, nor were there any carious lesions on theaffected or adjacent teeth. There was no pharyngealswelling, nor any palpable nodes or masses. A maxil-lary occlusal radiograph showed three severely dys-plastic teeth with a periapical radiolucency associatedwith the lateral incisor.

The child's condition worsened while she was in theemergency room area. Within 2 hr, her temperature was103.6°F, and she had become more distraught. Herfacial swelling clearly had enlarged during this 2-hrtime span (Fig 1). After consultation with doctors in oralsurgery and pediatrics, the patient was admitted and

Fig1. Patient had developedsigni f icant swell ing of theavleolus, infraorbital and orbitalareas while receiving care inthe emergency room.

received intravenousantibiotics and hydra-tion. The patient re-mained febrile into thenext morning, at whichtime the swelling beganto localize. Her tem-perature was steady at101°F.

Because of the child'sdiscomfort, lack of coop-eration, and the need foradequate visualizationand removal of the of-fending teeth, she wasprepared for general an-esthesia. Informed con-sent was received fromthe parent for the antici-pated procedures, and

for general anesthesia. Under general anesthesia, ad-equate maxillary occlusal radiographs of the involvedarea (Figs 2a and 2b, see next page) were exposed. Themaxillary primary left central and lateral incisors andcanine were extracted. An incision and drainage of theleft maxillary mucobuccal vestibule also was per-formed. The patient was stable postoperatively, and hertemperature continued to decrease. Intravenous hy-dration and antibiotics were continued for one addi-tional day. The child was discharged one day postop-eratively with oral antibiotics. The patient returned tothe dental clinic for follow-up evaluation five dayspostoperatively. No swellings were present, and theextraction sites were healing well. The patient was seenone month postoperatively for evaluation, and then forregular dental recall visits.

DiscussionAlthough odontodysplasia can present as a rather

asymptomatic condition in either the primary and/orpermanent dentition, this case history shows why thecondition must be monitored carefully and continu-ously as long as the affected teeth remain in the oralcavity. Some of the frequently observed negative con-sequences of odontodysplasia suggest the need formore aggressive treatment.

The child presented had been examined when shewas 21 months old by a pediatric dentist, and the di-agnosis of odontodysplasia was determined at that timeby clinical and radiographic examination (Fig 3, see nextpage). The family was aware of the nature and possibleconsequences of the problem, including the prognosisof the primary teeth, and the probable involvement ofthe permanent teeth. The child was taken to the pedi-atric dentist every four months for follow-up exami-

398 ODONTODYSPLASIA-RELATED INFECTION: FERGUSON, CREATH, AND BUONO

Fig 2a. Occlusal radiograph at the time of general anesthesia(patient age - 3 1/2 years) showing the affected left primary andpermanent anterior teeth.

Fig 2b. Occlusal radiograph giving a clear picture of theaffected permanent successors. (Primary teeth F through J areshown.)

nations. There had been no problems until the acuteepisode requiring hospital admission occurred.

In the review of Lustmann et al. (1975), almost half ofthe cases which presented symptomatically forodontodyplasia did so because of gingival swelling.Though the reason for these frequent infections is notclear, the nearness of the pulp to the oral fluids and thereported large dentinal tubules probably encouragebacterial contamination of the pulpal tissue. Inflam-matory cells frequently have been reported in the pulpof odontodysplastic teeth, even those not associatedwith gingival swelling. Leaving affected teethuntreated in the oral cavity appears to render themsignificantly susceptible to pulpal infection. Extractionof the affected teeth or immediate restoration with fullcrowns would seem warranted for preventive reasons.However, since these teeth are often only partiallyerupted, as in the case presented, restoration may not bea viable choice, and allowing the teeth to remain maymake the patient susceptible to infection. The speedwith which infection proceeded in the case presentedheightens this concern. In addition, pulpal therapyunder restorations probably is contraindicated, giventhe large size and irregular shape of the pulp (and roots).Also, preventing pulp exposure during preparation forfull crown coverage restoration may be difficult.

The radiograph (Fig 2b) shows that the permanentsuccessors also are likely to be affected. Given whathappened with the primary teeth, aggressive treatmentof the permanent teeth when they erupt probably iswarranted.

Although the case presented seems to represent anextreme outcome among reports of odontodysplasia,there have been enough other reports of gingival and

Fig 3. Occlusal radiograph taken at 21 months showedmalformations of the partially erupted primary left central andlateral incisors.

facial swelling to suggest the need for aggressive pre-ventive treatment of this anomaly (Lustmann et al. 1975;Dahllof et al. 1987). The histological literature suggeststhat this developmental defect is predisposed to pulpalinfection, and other associated sequelae. Therefore,"preventive" full-crown restoration, or extraction ofaffected teeth, seems to be the appropriate treatmentoptions for patients with odontodysplasia. Decisions towait and observe the condition before committing toany treatment (as decided by parents and/or the den-tist) require continuous and frequent monitoring. Itshould be pointed out that frequent observation did notprevent the detrimental outcome in this case.

PEDIATRIC DENTISTRY: NOVEMBER/DECEMBER, 1990 ~ VOLUME 12, NUMBER 6 399

Dr. Ferguson is associate professor and Dr. Creath is assistant profes-sor, Department of Children’s Dentistry, School of Dental Medicine,State University of New York at Stony Brook. Dr. Buono is resident,University Hospital, State University of New York at Stony Brook.

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Dahll6f G, Lindskog S, Theorell K, Ussisoo R: Concomitant regionalodontodysplasia and hydrocephalus. Oral Surg 63:354-57, 1987.

Dayal PK, Mani NJ: Odontodysplasia, report of a case. J Oral Med36:79-81, 1981.

Ferguson JW, Geary CPM: Regional odontodysplasia. Aust Dent J25:148-51, 1980.

Gardner DG, Sapp JP: Ultrastructural, electron-probe, andmicrohardness studies of the controversial amorphous areas inthe dentin of regional odontodysplasia. Oral Surg 44:549-59,1977.

Hovinga J, Ingenhoes R: Regional odontodysplasia. Int J Oral Surg8:474-77, 1979.

Kerebel B, Kerebel LM: Enamel in odontodysplasia. Oral Surg 52:404-10, 1981.

Kerebel B, Kerebel LM: Structural, ultrastructural,microradiographic, and electron-probe studies of an unusual caseof regional odontodysplasia. J Dent Res 61:1056-62, 1982.

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Mock D, Aidelbaum MR, Ch~pnick P: Familial amelodentinal dys-plasia. Oral Surg 61:485-91, 1986.

Sadeghi EM, Ashrafi MH: Regional odontodysplasia: clinical,pathologic, and therapeutic considerations. J Am Dent Assoc102:336-39, 1981.

Schmid-Meier E: Unilateral odontodysplasia with ipsilateral hyp-oplasia of the mid-face: a case report. J Maxillofac Surg 1 O: 119-22,1982.

Walton JL, Witkop CJ, Walker PO: Odontodysplasia: report of threecases with vascular nevi overlying the adjacent skin of the face.Oral Surg 46:676-84, 1978.

Williams SA, High AS: Odontodysplasia associated with orbitalcoloboma. Br DentJ 164:390-94, 1988.

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400 ODONTODYSPLASIA-RELATED INFECTION: FERGUSON, CREATH, AND BUONO