Delayed Tooth Emergence

Delayed Tooth Emergence Jeffrey M. Karp Pediatrics in Review 2011;32;e4 DOI: 10.1542/pir.
32-1-e4
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Article
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Delayed Tooth Emergence

Jeffrey M. Karp, DMD, MS*
Objectives
1. 2. 3. 4.
After completing this article, readers should be able to:
Author Disclosure Dr Karp has disclosed no nancial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/ investigative use of a commercial product/ device.
Recognize abnormalities in tooth emergence timing and order based on oral inspection. Discuss local and systemic causes of delayed tooth emergence. List treatment modalities available for management of delayed tooth emergence. Determine when timely referral to a dentist is necessary.
Introduction
Delayed tooth emergence (DTE) is a clinical term used when exposure of a tooth or multiple teeth through the oral mucosa is overdue, according to population norms based on chronologic age. DTE is common in childhood and adolescence, yet it is often overlooked or dismissed in pediatric primary care. Timely screening and recognition of DTE by clinicians can minimize medical, developmental, functional, and esthetic problems resulting from untreated underlying local and systemic causes. This article provides clinicians with an overview of conditions responsible for DTE in children. Multidisciplinary care for patients who experience DTE in medical, dental, and surgical settings also is discussed.
Odontogenesis
Human teeth develop through a series of complex, reciprocal interactions between the oral epithelium and migrating cranial neural crest ectomesenchymal cells of the rst branchial arch. This process is tightly regulated by more than 300 genes expressed temporospatially within the jaws. Dental patterning of the primary and permanent dentition is expressed in three dimensions, exerting morphogenetic controls over tooth number, position, size, and shape. In the end, the normal primary dentition consists of three tooth classes (four incisors, two canines, four molars) in each jaw, for a total of 20 teeth. Thirty-two teeth distributed among four tooth classes (8 incisors, 4 canines, 8 premolars, 12 molars) comprise the permanent dentition.
Tooth Eruption and Emergence

Tooth emergence, the clinical exposure of any part of a tooth through the oral mucosa, is the culmination of numerous developmental processes occurring within the jaws. Bony crypts house developing teeth during crown morphogenesis (size and shape) as well as hard tissue (eg, enamel, dentin) secretion and calcication. As root development begins, teeth initiate a physiologic process of vertical eruption through the overlying alveolar bone Abbreviations toward the oral mucosa. Bone remodeling in the area is necessary for progression of tooth eruption. Root developDTE: delayed tooth emergence ment exceeds two thirds of its nal length when the alveolar GE: gingival enlargement bone crest is reached. The primary dentition undergoes root HGF: hereditary gingival bromatosis resorption, followed by crown exfoliation, to permit emerKCOT: keratocytic odontogenic tumor gence of permanent incisors, canines, and premolars into the MPFM: maxillary permanent rst molar proper position within the dental arch. Permanent molars Mx.C.P1: maxillary canine/rst premolar do not replace primary teeth under normal circumstances. NBCCS: nevoid basal cell carcinoma syndrome Teeth make clinical emergence into the oral cavity when 75% PDC: palatally displaced canine of their roots length is achieved. SP: supernumerary premolar Numerous population studies conducted worldwide over
*Assistant Professor, Division of Pediatric Dentistry, Departments of Dentistry and Pediatrics, University of Rochester Medical Center, Rochester, NY. e4 Pediatrics in Review Vol.32 No.1 January 2011
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delayed tooth emergence
the past 100 years report marked variation in dental chronology based on race, ethnicity, and sex as well as environmental factors. Tooth development, eruption, and emergence in healthy mouths are genetically controlled, with high heritability scores reported in monozygotic twin studies. As seen in Table 1, tooth emergence and exfoliation times are usually presented as ranges of chronologic age to account for the previously mentioned factors. Clinicians should recognize that teeth that fail to emerge within 12 months of the normal range are considered delayed. In these cases, referral to a dentist is warranted for further clinical and radiographic assessment. Some cases require surgical treatment to permit tooth emergence.
Detection of DTE
DTE is a nonspecic clinical nding that can occur in a localized or generalized distribution. Oral inspection coupled with history can provide clinicians with substantial information to dene further the natural history and clinical manifestations of the underlying condition. Oral examination should consist of evaluation of the alveolar ridges as well as the alignment and morphology of the teeth that are present. The size and shape of the alveolar ridges can help determine whether DTE is due to abnormalities in tooth development, eruption, or emergence.
Table 1.
Tooth eruption through alveolar bone causes expansion and fullness of the alveolar ridge. On average, 2 months are required for a tooth to progress from causing palpable enlargement of the gingival tissues to overt clinical emergence. Palpation of the oral mucosa in the area of erupting teeth should cause localized tissue blanching if tooth emergence is imminent. In addition, redness of the mucosa or an eruption hematoma has been noted to precede tooth emergence in more than 30% of cases. Thin, knife-edge alveolar ridges suggest the absence of teeth in the area. The dentition should be inspected systematically for age-appropriate tooth counts (Figs. 1 and 2). Proper inspection requires a working knowledge of the differences in tooth morphology among tooth classes and between the two dentitions. Tooth counts should be assessed for appropriateness in timing and order. For the most part, the primary dentition adheres to the following emergence order in each jaw: central incisors, lateral incisors, rst molars, canines, and second molars. Although published emergence orders are available for the permanent dentition, clinicians observe countless variations in order as a result of numerous genetic, anatomic, and environmental inuences. Generalized timing delays in tooth emergence caused by systemic disease do not usually result in changes in the order
Tooth Emergence and Exfoliation

PRIMARY DENTITION Mandible Eruption (months) Exfoliation (years) 6 7 9 10 11 Mandible Eruption (years) 6 to 7 7 to 8 9 to 11 10 to 12 11 to 13 5.5 to 7 12 to 14 17 to 30 Root Complete (years) 9 to 10 10 12 to 15 12 to 13 12 to 14 9 to 10 14 to 16 18 Eruption (years) 7 to 8 8 to 9 11 to 12 10 to 11 10 to 12 5.5 to 7 12 to 14 17 to 30 to to to to to 7 8 11 12 13 Eruption (months) 6 8 16 11 20 to to to to to 10 12 20 18 30 Maxilla Root Complete (years) 9 to 10 11 12 to 15 12 to 13 12 to 14 9 to 10 14 to 16 18 Maxilla Exfoliation (years) 7 8 11 9 9 to to to to to 8 9 12 11 12
Central incisors Lateral incisors Canines First molars Second molars
5 7 16 11 20
to to to to to
8 10 20 18 30
PERMANENT DENTITION
Central incisors Lateral incisors Canines First premolars Second premolars First molars Second molars Third molars
Adapted from American Academy of Pediatric Dentistry, Guideline on management of the developing dentition and occlusion in pediatric dentistry. Reference Manual. 2009;32(6). Copyright American Dental Association. All rights reserved. Used with permission.
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Figure 1. Development of the dentition from birth to 6 years
of age. Reprinted with permission from Logan WHG, Kronfeld R. Development of the human jaws and surrounding structures from birth to the age of fteen years. JADA. 1933;20(3):379 427. Copyright 1933 American Dental Association. All rights reserved. Adapted 2010 with permission of the American Dental Association. Schour L, Massler M. The development of human dentition. JADA. 1941;28(7):11531160. Copyright 1941 American Dental Association. All rights reserved. Adapted 2010 with permission of the American Dental Association.
Figure 2. Development of the dentition from age 7 to adulthood. Reprinted with permission from Logan WHG, Kronfeld R. Development of the human jaws and surrounding structures from birth to the age of fteen years. JADA. 1933;20(3):379 427. Copyright 1933 American Dental Association. All rights reserved. Adapted 2010 with permission of the American Dental Association. Schour L, Massler M. The development of human dentition. JADA. 1941;28(7):11531160. Copyright 1941 American Dental Association. All rights reserved. Adapted 2010 with permission of the American Dental Association.
antimeres (corresponding teeth on opposite side) usually occurs.
Causes of DTE
of tooth emergence or exfoliation. In contrast, localized disease should be investigated when the order of tooth emergence is altered. Three general rules exist for normal tooth development and emergence: 1) anterior teeth within a specic tooth class (eg, rst premolars) always precede posterior teeth within the same class (eg, second premolars), 2) mandibular teeth emerge earlier than their maxillary counterparts, and 3) symmetric emergence of tooth
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Anomalies in Tooth Number

Tooth agenesis, one of the most common developmental anomalies in humans, alters the order of tooth emergence. Although missing teeth are noted in only 1% of children in the primary dentition, approximately 30% of the general population fails to develop a full complement of primary and permanent teeth. Agenesis of one or more permanent third molars (wisdom teeth) affects about
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Figure 3. An 8-year-old white boy who has bilateral agenesis of the maxillary lateral incisors (3) causing a wide diastema between the maxillary central incisors. Photograph courtesy of Ryan Walker, DDS.
Figure 4. A 9-year-old white girl who has ectodermal dysplasia. Agenesis of the permanent maxillary lateral incisors and all mandibular incisors is seen, and a conical permanent maxillary central incisor (*) is present. Photograph courtesy of David Levy, DMD MS.
one in every ve people. A recent meta-analysis reported the prevalence of dental agenesis, excluding third molars, as 2.5% to 6.9%, depending on the race, sex, and country of study. (1) Tooth agenesis is slightly more common (1.3:1) in females versus males. Hypodontia is dened as the absence of up to six teeth. In more than 80% of patients, one or two teeth are missing. After the third molars, the mandibular second premolars, maxillary lateral incisors, and maxillary second premolars are affected most frequently, with a 1.5% to 3.1% prevalence rate. Unilateral tooth agenesis is seen more commonly, except for permanent maxillary lateral incisors (Fig. 3), which have a propensity toward bilateral agenesis. Only 0.14% of the general population has oligodontia, dened as the absence of six or more teeth. Oligodontia following autosomal dominant inheritance patterns can be indicative of PAX9, MSX1, or AXIN2 mutations. Ectodermal dysplasia should be considered when underdeveloped alveolar ridges are seen in the anterior jaws of predentate infants older than 7 months of age, when multiple primary teeth are absent, or when conical incisors are seen (Fig. 4). Recognition of missing teeth by number and location along with other physical ndings can aid in the diagnosis of numerous genetic diseases (Table 2). Clinicians should consider abnormal alignment and increased spacing of teeth as well as localized delays in primary tooth exfoliation as potential clinical manifestations of hypodontia (Fig. 5). Patients who manifest hypodontia may warrant consultation with a geneticist to rule out associated syndromes. Supernumerary teeth (hyperdontia) developing with the jaws often delay the eruption and emergence of permanent teeth. Hyperdontia is seen in 1.5% to 3.5% of
the general population. More than 80% of cases occur in the anterior maxilla, and supernumerary teeth presenting at this site can occur singly or in multiples, can have normal incisor anatomy, can be conical (Fig. 6), or can appear to have cuspal morphology. The teeth can emerge into the mouth or be inverted within the maxilla. A single supernumerary tooth that develops in the primary palate directly behind the maxillary central incisors is called a mesiodens. These teeth account for more than 50% of all supernumerary teeth reported in epidemiologic studies. Altered fusion between the medial nasal process and the maxillary facial process during embryogenesis produces the presence of two maxillary lateral incisors on the affected side, as is seen occasionally in the general population and more commonly in children born with isolated cleft lip and cleft lip and palate. Maxillary permanent fourth molars or rudimentary paramolars constitute approximately 18% of all supernumerary teeth. Supernumerary premolars (SPs), on the other hand, develop in 0.64% of the general population. A 3:1 male-to-female distribution is seen. SPs are the most common type of hyperdontia occurring in the mandible. Their development appears to be genetically controlled, although the pattern of inheritance remains unclear. These teeth usually have normal premolar anatomy. Five out of every six SPs fail to emerge clinically, and they can cause impaction of adjacent teeth (Fig. 7). They are often incidental ndings on panoramic radiographs in adolescence. Many can develop after the emergence of age-appropriate premolars. Surgical removal of a supernumerary tooth becomes necessary when it impedes or deects age-appropriate tooth eruption and emergence. One in four patients who has a history of extra teeth in the anterior maxilla later
Table 2.
Genetic Diseases With Anomalies in Tooth Number

Inheritance AD AD AD Trisomy 21 AD AD AD AD Sporadic Primary and permanent tooth agenesis, conical incisors, anodontia Cleft lip and palate, mandibular lip pits Dental Findings Other Findings
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Condition
Gene
Autosomal dominant oligodontia None documented Colon polyps and cancer, cleft lip and palate Cleft lip and palate Nail hypoplasia (especially toenails)
Witkop syndrome
PAX9 AXIN2 MSX1 MSX1
Van der Woude
IRF6
Down syndrome
Numerous
Ellis-van Creveld
EVC
Dysmorphic facies, congenital heart disease, intellectual disability, leukemia, thyroid dysfunction, hearing loss, maxillary hypoplasia Chondrodysplasia, polydactyly, congenital heart defects
Apert syndrome
FGFR2
Agenesis of permanent molars Permanent tooth agenesis across tooth types Agenesis of second premolars and third molars Agenesis of permanent mandibular incisors and second molars, maxillary permanent canines Permanent tooth agenesis, second premolars, maxillary lateral incisors Agenesis of incisors and second premolars, pegshaped lateral incisors, maxillary canine-rst premolar transposition Tooth agenesis, enamel hypoplasia, multiple oral frenula, premature exfoliation of primary teeth Permanent tooth agenesis Craniosynostosis, maxillary hypoplasia, hand anomalies Blue sclera, multiple fractures None documented
Osteogenesis imperfecta Incisor-premolar hypodontia Hypodontia, dentinogenesis imperfecta Agenesis of lateral incisors and second premolars, taurodontism, ectopic maxillary canines Permanent tooth agenesis, ectopic eruption of permanent incisors
COL1A1/2 Unknown
Hutchinson-Guilford progeria syndromes
LMNA
Hypohidrotic ectodermal dysplasia Xd AD, AR AD, AR Xd
Precocious senility, early death, coronary artery disease, beaked nose, baldness, lipodystrophy, short stature Defective hair, nails, skin; hypohidrosis; poor hearing; respiratory infections Defective hair, nails, eyes; intellectual disability; autochthonous tattooing Hypohidrosis, immunodeciency Glaucoma, redundant periumbilical skin Cleft palate, hand anomalies, intellectual disability
Incontinentia pigmenti
EDA EDAR EDARR IKK
NEMO
AD Xd AD AD
Axenfeld-Rieger syndrome
PITX2
Orofacial-digital syndrome type 1 Holoprosencephaly
CXORF5
Permanent tooth agenesis, conical teeth, delayed exfoliation of primary dentition Agenesis, conical teeth, delayed tooth emergence Agenesis of incisors and canines, enamel hypoplasia, conical teeth Agenesis of incisors and canines Solitary maxillary central incisor Multiple supernumerary teeth, retained primary teeth, impacted permanent teeth Supernumerary teeth, tooth agenesis, jaw osteomas, impacted teeth
Numerous
Cleidocranial dysplasia
RUNX2
Gardner syndrome AD
APC
Seizures, syndromic facies, premaxillary agenesis, cleft lip and palate, hypotelorism Hypoplastic calvaria, absent clavicles, midface hypoplasia, delayed fontanelle closure, short stature, scoliosis, sinus/respiratory infections, hearing loss Gastrointestinal polyps, multiple osteomas, skin and soft-tissue tumors, cancer in 50% by age 30
ADautosomal dominant, ARautosomal recessive, XdX-linked
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Figure 5. A 14-year-old African American boy who has hypodontia. The mandibular right second premolar (**) did not develop. Clinically, the mandibular right second primary molar (3) shows delayed exfoliation. The permanent third molars continue to develop in the jaws (*). Photograph courtesy of Aliakbar Bahreman, DDS, MS.
Figure 7. A 15-year-old Hispanic boy who has delayed exfo-
liation of the mandibular right primary molars (3) as well as delayed emergence of the mandibular left premolars (*). An age-appropriate set of permanent teeth is present in the maxillary arch. Four supernumerary mandibular premolars, two on each side, are the cause for the delayed emergence of the mandibular premolars. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
develops SPs. Moreover, SPs, unlike other supernumerary teeth, recur in 8% of patients. Of note, natal and neonatal teeth should be maintained when possible because they not supernumerary in more than 90% of cases. Clinicians who suspect the presence of a supernumerary tooth should refer the child to a dentist for radiographic examination.
Delayed Dental Age

Biologic delays in dental development generally retard emergence of the primary and permanent dentitions. Delayed dental age has been studied using tooth counts from clinical inspection as well as the stage of tooth formation on panoramic radiography. As mentioned, DTE using clinical tooth counts is an inexact measure of dental age due to a host of local factors. Dental age scores
Figure 6. A conical mesiodens (3) has emerged into the anterior maxilla, causing the permanent maxillary right central incisor (*) to emerge late and out of position. Surgical removal of the mesiodens is recommended. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
are best determined using radiographic stages of tooth formation. Few studies have focused on the primary dentition because radiography is limited by patient cooperation. However, numerous methods have been proposed to score dental age using a variety of statistical methods based on scores of crown and root formation for the permanent teeth. The Demirjian method, originally studied in a French Canadian pediatric population, is used most commonly. (2) This method scores the mandibular left permanent teeth, excluding the third molars, according to eight developmental stages. More than 100 studies have used the Demirjian method and modications of it to compare dental age to the chronologic age of a population. This method, although validated through epidemiologic studies, gives varied results by sex, race, and ethnicity of the population of study. Dental age scoring using these methods is used commonly in forensics and immigration proceedings for unaccompanied minors as a means of age estimation when additional information is not available. Dental age does not consistently correlate with skeletal age and the timing of puberty. However, the mandibular canine has been shown to be the best indicator of pubertal onset using tooth formation stages. In general, skeletal age delayed by systemic disease or malnutrition is often two to six times more severe than the delay noted in dental age. Using the Demirjian method and others in conjunction with clinical tooth counts, patients who have a host of systemic diseases have been found to have delayed dental age. Most studies, however, involve a limited
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number of affected individuals, lending poor statistical power. In addition, numerous genetic syndromes have DTE (also described as delayed tooth eruption) listed as a clinical nding. Case reports and studies involving these patients do not usually assess dental age based on radiographic parameters. Nonetheless, oral inspection of children who have Down syndrome, hypothyroidism, growth hormone deciency, hypopituitarism, and chronic malnutrition often results in a nding of DTE. In small case-control studies, patients who have hypodontia and those who have palatally displaced canines (PDCs) are also noted to have delayed dental age. DTE resulting from delayed dental age in children who have Down syndrome remains untreatable. In contrast, growth hormone therapy has been shown in preliminary studies to accelerate dental maturation and improve the timing of tooth emergence. (3) Although preterm birth has been associated with delayed dental age according to chronologic age, dental age normalizes when the childs term age is used. (4) Similarly, children who have enamel and dentin anomalies due to X-linked hypophosphatemic rickets do not present initially with delayed dental age. They do, however, develop spontaneous dental abscesses due to microscopic abnormalities in the mineralized dental tissues that allow ingress of microorganisms and pulpal necrosis. Early primary tooth loss due to infection can slow the dental development of the permanent successors and lead to DTE.
Dental Crowding
Insufcient space in the jaws for eruption and emergence of teeth constitutes the most benign, yet common,
source of DTE in children. A tooth-to-jaw size discrepancy is often responsible for dental crowding. This disharmony occurs as a result of: 1) normal-size teeth in small jaws, 2) larger-size teeth in normal-size jaws, or 3) a combination of both. Children who have constricted, V-shaped alignment of the teeth are more likely have tooth crowding than those in whom the dental arch is U-shaped. Dental crowding among primary incisors predicts moderate-to-severe crowding in the permanent dentition. Early tooth loss due to dental caries raises a childs risk for dental crowding and delayed emergence of permanent teeth. Primary teeth serve as placeholders for their successors. Premature extraction of primary canines or molars results in migration of adjacent teeth (Fig. 8), loss of dental arch length and circumference, and shift of dental midlines toward the side of early tooth loss. Pediatric dentists and orthodontists attach appliances to teeth adjacent to tooth extraction sites to maintain space for later permanent tooth emergence. The presence of supernumerary teeth as well as fused teeth (Fig. 9) can exacerbate dental crowding. Later developing teeth can remain unerupted in the jaws or be forced to emerge ectopically when adjacent teeth are impediments to the normal eruption path. Odontogenic pathology and jaw bone disorders also worsen dental crowding through displacement of unerupted and emerged teeth into compact areas of the jaws. Dental crowding can be alleviated by transverse expansion of the jaws. Posterior retraction of medially positioned molars also increases the amount of space for future tooth emergence. In some cases, dental crowding necessitates the removal (serial extraction) of healthy primary canines and molars as well as permanent rst premolars sequentially to allow proper alignment of the permanent dentition in adolescence and adulthood. Dentists, orthodontists, and oral maxillofacial surgeons
Figure 8. Space loss in the maxillary left quadrant is seen when compared with the contralateral side due to early extraction of the maxillary left primary molars because of dental caries. The maxillary permanent rst molar (bottom right) has migrated into the space previously occupied by the primary molars due to the lack of a space maintenance appliance.
Figure 9. A 6-year-old African American girl who has a maxillary left primary incisor fused (**) to a supernumerary tooth. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
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work collaboratively on these cases to obtain optimal treatment outcomes.
Ectopic Tooth Eruption

Abnormalities in the path of tooth eruption also can cause delayed tooth emergence in the permanent dentition. The literature suggests that 2% to 6% of children demonstrate ectopic tooth eruption. The maxillary permanent rst molars and canines are affected most commonly. The prevalence of ectopic eruption is substantially higher (20%) in children born with cleft lip and palate, likely due to genetic and anatomic differences. Under normal circumstances, the maxillary permanent rst molar (MPFM) follows an eruption path posterior to the maxillary second primary molar. It emerges through the gingival tissues and uses the posterior surface of the primary molar to guide its eruption into functional occlusion with teeth in the opposing jaw. Ectopic MPFMs take a medial eruption course, leading them under the crown of the second primary molar (Fig. 10). This eruption disturbance, often detected on dental radiographs between 5 and 7 years of age, delays MPFM emergence and often causes root resorption of the primary second molar, with some cases persisting until the primary tooth is exfoliated prematurely. Two thirds of ectopic MPFMs self-correct, usually by 7 years of age. For the remaining cases, orthodontic management is necessary to prevent anterior migration of the ectopic MPFM and future impaction of the ipsilateral maxillary second premolar. Clinically, this anomaly can be detected through premature mobility of the primary second molar or mesial angulation of the MPFM, with emergence of the distal (away from midline) cusps only. PDCs in the maxilla should be suspected in children
Figure 11. Asymmetric expansion of the anterior palate (3).
The maxillary primary canine on the ipsilateral side (*) has been exfoliated. This clinical presentation is indicative of an untreated palatally displaced canine. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
Figure 10. The maxillary permanent rst molars (*) are erupting in an ectopic position under the crowns of the maxillary primary second molars. Root resorption of the primary second molars is also occurring. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
older than 9 years of age when alveolar ridge palpation adjacent to the buccal vestibule lacks a canine bulge, a clinical nding suggestive of normal canine eruption. The early manifestations of PDCs can be detected on panoramic radiography because ectopic maxillary canines often appear more horizontal on the lm and tend to overlap the root of the mature ipsilateral lateral incisor. Early extraction of the adjacent maxillary primary canine corrects the eruption path and spatial orientation of PDCs in almost 70% of cases. PDCs are associated with other dental anomalies (small-size maxillary permanent lateral incisors, infraocclusion of primary molars, and enamel hypoplasia) that can be detected by clinicians through oral inspection. Delayed exfoliation of the ipsilateral maxillary primary canine or asymmetric anterior palatal enlargement with or without primary canine loss (Fig. 11) are late clinical manifestations of PDCs. If left untreated, ectopic eruption of the maxillary canine leads to tooth impaction in the hard palate. Surgical tooth exposure, forced orthodontic traction, and space regaining in the maxillary anterior segment through xed orthodontic appliances (braces) becomes necessary. Tooth transposition also results in delayed tooth emergence in many cases. This abnormality of dental position occurs more frequently in the maxilla than the mandible. Maxillary canine/rst premolar (Mx.C.P1) transposition cases (Fig. 12) occur most commonly, with a prevalence of 0.25%. Based on a review of 143 cases, Mx.C.P1 transposition appears to be polygenic, with a propensity for occurrence in females. (5) A higher prevalence of Mx.C.P1 transposition is seen among children who have Down syndrome. Clinically, agenesis of the ipsilateral lateral incisor is common. Twenty-seven percent of published Mx.C.P1 cases occur bilaterally.
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Figure 12. Maxillary permanent left canine with left rst
Figure 13. The maxillary primary central incisors are delayed
premolar transposition. In this case, reshaping of the teeth with dental composite restorations can permit normal function and satisfactory esthetics. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
in exfoliation. They are forcing the maxillary permanent central incisors to erupt in the anterior palate. When the child occludes his teeth, the maxillary permanent central incisors are behind (crossbite) the mandibular incisors (3). Orthodontic correction of this condition becomes necessary. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
Early permanent tooth loss due to dental caries or trauma as well as traumatic displacement of developing, unerupted teeth within the jaws accounts for most other cases of transposition in the maxilla, including canine/ lateral incisor, canine/rst molar, lateral incisor/central incisor, and canine/central incisor patterns. Mandibular canine/lateral incisor transposition, identied in 0.03% of dental patients, often is seen in conjunction with permanent third molar agenesis, suggestive of genetic inuences. Transposition cases, if recognized early enough, usually can be managed effectively with interceptive orthodontics without surgery.
Delayed Exfoliation of Primary Teeth

Delayed exfoliation of primary teeth is intimately associated with delayed root development and eruption of their permanent successors. As a result, permanent tooth agenesis or delayed dental maturity typically results in delayed exfoliation of primary teeth according to chronologic age. In these cases, the timing of primary tooth root resorption is appropriate from a biologic standpoint. In contrast, primary tooth exfoliation is considered biologically delayed when the primary tooth remains in place despite permanent tooth root length greater than 75% of its expected nal length. Primary teeth that appear biologically ready for exfoliation are common in primary care. These teeth are usually retained in soft tissue or interlocked between adjacent teeth, limiting their ability to be removed at home. Children also tend to delay tooth removal if they
feel that pain is likely. Timely extraction of over-retained primary teeth is indicated if maxillary permanent incisors will be deected palatally and malocclusion such as anterior crossbite (Fig. 13) is likely to occur. Soft-tissue infection is another indication for tooth extraction when food becomes impacted under the exfoliating primary tooth. Lingual emergence of mandibular permanent incisors is common but rarely a cause for concern. In these cases, further emergence of the permanent teeth ultimately causes exfoliation of their predecessors, followed by anterior repositioning of the permanent incisors within the dental arch by tongue pressure. Extraction of overretained mandibular primary incisors is needed more frequently in cases of severe dental crowding. Infraoccluded primary molars (teeth that fail to reach the normal occlusal plane) are reported to occur in 5% of the general population. These teeth often appear to be ankylosed on clinical examination because they are immobile to palpation and tend to be submerged in the gingival tissues compared with continually erupting adjacent teeth (Fig. 14). Nonetheless, infraoccluded primary molars usually exfoliate within 1 year of the normal range as long as the permanent tooth successor is present with adequate root formation. Infraoccluded primary molars can become surgical problems if the crowns of the adjacent teeth are allowed to migrate over top of them. In addition, alveolar bone levels surrounding the adjacent teeth can approximate the crown of the infraoccluded molar, leading to im-
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Figure 14. The mandibular left primary rst molar is infra-
Figure 16. A 6-year-old boy born in Uganda presents with
occluded. The adjacent teeth continue to erupt while it remains stationary, creating the clinical appearance of a tooth submerging into the gingiva. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
malformed mandibular primary canines (*) and missing maxillary primary canines (). His history corroborates that canine extirpation was completed before emigration from Uganda. Photograph courtesy of Terry Farquhar, RN, DDS.
paired exfoliation and delayed premolar emergence (Fig. 15). Close monitoring of infraoccluded primary molars by dentists is recommended to avoid these complications.
Trauma
Tooth development, eruption, and emergence can be altered by dental or maxillofacial trauma in infancy or childhood. Trauma to developing primary teeth is relatively rare. Extreme root curvature (aka dilaceration) and eruption failure of maxillary incisors have been reported
Figure 15. A 12-year-old white boy who has infraoccluded maxillary second primary molars (*). The adjacent teeth have erupted over the top of the infraoccluded teeth, causing them to become impacted in the jaw. This condition impedes the maxillary second premolars from erupting into the mouth. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
as sequelae of traumatic laryngoscopy and prolonged endotracheal intubation in infancy. Clinicians also may encounter children who have emigrated from Eastern Africa and appear to have DTE of the primary canines or other adjacent teeth on clinical inspection (Fig. 16). This nding is consistent with the practice of ebinyo, in which tribal healers remove these teeth in infancy to prevent or treat high fevers, vomiting, or diarrhea in the child. Damage, displacement, or extraction of adjacent primary and permanent teeth also can be seen. Mandibular fractures due to falls, motor vehicle crashes, or child abuse can disturb teeth developing along the line of fracture. Infection and inadvertent placement of plates and screws during jaw xation also jeopardizes adjacent developing teeth. Similarly, children born with micrognathia (eg, Pierre Robin sequence, Goldenhar syndrome) who require mandibular distraction osteogenesis to prevent long-term tracheostomy can have permanent molar tooth germs displaced or destroyed during mandibular osteotomy and placement of the internal distraction device. Prophylactic enucleation of tooth germs in planned sites of distractor pins is advocated by some surgeons to improve bone volume and treatment outcomes. Intrusion of primary incisors into the dental alveolus commonly results in developmental changes to their permanent successors. The amount of internal displacement and direction of primary tooth displacement coupled with the age of the child aid clinicians in determining whether enamel hypoplasia, root dilaceration, or tooth germ displacement are possible sequelae. ReimPediatrics in Review Vol.32 No.1 January 2011 e13
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Figure 17. A 9-year-old African American girl who has delayed exfoliation of the maxillary right primary central incisor (#). This tooth is discolored due to dental trauma. Her permanent incisor emergence order is affected. Extraction of the over-retained primary incisor is indicated. Photograph courtesy of Aliakbar Bahreman, DDS, MS.
plantation of avulsed primary incisors after trauma predisposes them to delayed tooth exfoliation because destruction of the periodontal ligament apparatus and ankylosis between the alveolar bone and the tooths root often occur. In these cases, ectopic permanent incisor eruption occurs along with rotation of adjacent teeth (Fig. 17). This problem is seen infrequently because dentists and rst responders at accident sites are educated to avoid replantation of avulsed primary teeth.
Jaw Bone Pathology

Tooth development and emergence often are affected by jaw pathology. In some cases, dental abnormalities occur as a result of inadequate bone remodeling, and in other disorders, displacement of developing teeth is caused by expanding jaw lesions. Children who have infantile osteopetrosis experience marked delays in tooth emergence as well as tooth agenesis and enamel hypoplasia. These clinical manifestations are directly related to osteoclast dysfunction. Stem cell rescue of those who have osteopetrosis can restitute normal tooth eruption and emergence of the permanent dentition. Various types of osteogenesis imperfecta present with dental developmental anomalies. Delayed tooth emergence is seen in 20% of patients who have osteogenesis imperfecta type III. Ectopic tooth eruption is another common nding in affected individuals. Bisphosphonate therapy used in the management of osteogenesis imperfecta can cause delayed tooth emergence of 1.6 years relative to matched controls.
To date, bisphosphonate therapy has not been associated with osteonecrosis of the jaws, as is reported in adult patients using these medications. McCune-Albright syndrome is a sporadic multisystem disease characterized by polyostotic brous dysplasia, cafe au lait hyperpigmentation, and precocious puberty. Craniofacial forms of brous dysplasia result in progressive facial, palatal, and jaw asymmetries. The maxilla is affected more commonly than the mandible, with a ground-glass appearance of the lesion noted through panoramic radiography or computed tomography scans. Oligodontia as well as tooth impaction, displacement, and rotations are common in affected patients. Jaw osteomas and supernumerary teeth often are the rst manifestations of Gardner syndrome in puberty. Early recognition is necessary to permit monitoring of gastrointestinal polyps because malignant transformation occurs in 50% of patients by age 30. Permanent teeth often fail to erupt in patients born with cleidocranial dysplasia because the teeth lack secondary cementum. Extraction of primary teeth that have failed to exfoliate normally does not promote eruption of their permanent successors. In addition, supernumerary teeth can impede tooth emergence. Surgical exposure of unerupted teeth followed by orthodontic traction has limited success. Oral rehabilitation for these patients often centers on jaw reconstruction and the use of dental prostheses. Cherubism is a rare autosomal dominant disease that affects the jaws. The condition is characterized by bilateral expansion of the posterior mandible and, in some cases, the maxilla and facial bones. Bony expansion of the jaws causes the individual to have a chubby cheeked, cherubic appearance. The osseous lesions are usually multilocular radiolucencies affecting the angles and ascending rami of the mandible. They are histologically dened by multinucleated giant cells in a loose brous stroma. The lesions tend to increase in size until puberty, after which lesion stabilization or even regression is noted. Bilateral expansion of these lesions causes marked displacement of developing and emerged teeth. Failure of tooth eruption due to severe dental crowding and malocclusion is common. Watchful monitoring is the usual course of action unless expansion progresses rapidly.
Odontogenic Cysts and Tumors

Epithelial-lined jaw cysts derived from odontogenic epithelium commonly impair eruption of developing teeth, producing alterations in tooth emergence timing or order. Dentigerous cysts, originating from a separation of the follicle around the crown of an unerupted tooth, comprise approximately 20% of all odontogenic cysts.
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Figure 19. A 17-year-old white girl who presents with painless
swelling of the mandibular left posterior jaw has age-appropriate dentition on clinical examination. On panoramic radiography, a large unilocular radiolucency is seen along with marked displacement of the unerupted third molar. Histopathologic examination conrmed the lesion to be a dentigerous cyst.
Figure 18. A 13-year-old white girl presents with delayed
exfoliation of the maxillary right primary canine (*). On dental radiography, a large unilocular cyst is present around the crown of the unerupted permanent canine. Histopathologic examination reveals a dentigerous cyst.
Mandibular third molars, followed by maxillary permanent canines (Fig. 18), are affected most commonly. Dentigerous cysts around supernumerary teeth and odontomas also are seen frequently. Usually, the lesion should measure at least 3 to 4 mm in diameter on radiograph to be called a dentigerous cyst rather than a variation in normal follicular anatomy. These lesions are found more often in the second decade, with the highest prevalence noted in white patients. Dentigerous cysts can grow very large and have a tendency to displace the involved tooth within the jaw (Fig. 19). Treatment of these lesions involves either marsupialization or enucleation of the cyst with or without removal of the unerupted tooth. Recurrence is rare after complete removal of the cyst. Keratocytic odontogenic tumors (KCOTs), previously known as odontogenic keratocysts, have been reported to account for 2% of all oral biopsies performed in children younger than 16 years of age, according to retrospective review of a United States dental school biopsy service. (6)
KCOTs are aggressive tumors that have a marked tendency for development in the posterior body and ascending ramus of the mandible. An unerupted tooth is involved in 25% to 40% of cases, mimicking a dentigerous cyst. KCOTs have thin, friable walls that make complete enucleation and thorough curettage difcult. As a result, recurrence is common. In locally aggressive cases, jaw resection followed by bone grafting may be necessary. The presence of multiple KCOTs warrants further testing for nevoid basal cell carcinoma syndrome (NBCCS). Gorlin syndrome, as it also is called, is characterized by multiple KCOTs as well as multiple basal cell carcinomas, hyperkeratosis of the palms and soles, skeletal abnormalities, intracranial ectopic calcications, and facial dysmorphia. NBCCS is caused by mutations in the PTCH1 gene. It is transmitted as an autosomal dominant trait and is reported in fewer than 1 in 57,000 individuals, with a 1:1 male-to-female ratio. Multidisciplinary care by dental professionals, pediatricians, dermatologists, and neurologists is recommended. Ameloblastomas have been described as the most clinically signicant odontogenic tumor. They arise from cells of odontogenic epithelial origin. Multicystic lesions are seen most commonly across the lifespan. However, only 8.7% to 15% of all ameloblastomas in Western countries develop in the pediatric population. Fifty percent of unicystic intraosseous ameloblastomas are diagnosed in the second decade of life. Most of these tumors develop as asymptomatic lesions in the posterior mandiPediatrics in Review Vol.32 No.1 January 2011 e15
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ble. An unerupted third molar as well as teeth adjacent to it often can become involved. These tumors resemble cysts on surgical exposure. As such, they usually are treated by enucleation with curettage. Recurrence rates ranging from 10% to 20% are seen. Block resection can become necessary in select cases. On very rare occasions, ameloblastomas act as malignant tumors, with hematogenous spread of metastatic disease. Odontomas are the most common odontogenic tumors, accounting for approximately 30% of lesions. They develop in both jaws, with greater prevalence in the maxilla. They are equally distributed between both sexes. Two types, compound and complex, are seen. Compound odontomas are well-circumscribed masses of tiny teeth of various numbers. The teeth are usually coneshaped and have normal delineation of tooth layers. Complex odontomas are similar but do not have organized dental structures. They are easily removed by enucleation and do not recur. Fifty-ve percent of them are diagnosed when delayed permanent tooth emergence or delayed exfoliation of a primary tooth is seen. More than 20 other types of odontogenic cysts and tumors can develop in the jaws. Histopathologic examination is necessary to discriminate these lesions, including identication of specic odontogenic elements and mineralized tissue. If left untreated, odontogenic disease can cause displacement and mobility of teeth, delayed tooth emergence, root resorption, pain, jaw swelling, and paresthesia. Large cystic lesions in the posterior mandible can lead to pathologic jaw fractures.
Figure 20. An 8-year-old white boy who has a history of trauma to the maxillary anterior teeth presents with delayed emergence of the maxillary permanent right central and lateral incisors. The contralateral permanent incisors are already present. The outline of the unerupted teeth can be seen within the gingiva. Surgical exposure was necessary to permit tooth emergence.
terized by proliferation of connective tissue extracellular matrix in response to gingival drug metabolism. Phenytoin, nifedipine, and cyclosporine are the most common catalysts of the condition. Poor oral hygiene exacerbates GE through inammatory mechanisms. The anterior gingival tissues are involved more frequently. Males tend to be more severely affected for poorly understood reasons. The use of multiple anticonvulsant medications in addition to phenytoin increases the severity of phenytoin-induced GE. Addi-
Gingival Enlargements
The gingiva and oral mucosa provide the last barrier to tooth emergence when sufcient space is present in the dental arch. Under normal circumstances, reduced enamel epithelium of erupting teeth fuses with the oral mucosa, permitting emergence of the dentition. Gingival remodeling also is necessary for emergence and continued tooth eruption over time. A variety of genetic and environmental conditions active in the gingival tissues can preclude either localized or generalized tooth emergence. Tooth emergence can be delayed when the gingival tissue becomes scarred as a result of oral trauma (Fig. 20). Eruption cysts can form over emerging teeth when uid extravasation occurs between the tooth crown and the overlying gingival tissues. These conditions are usually selflimiting with time and optimal oral hygiene. If persistence of the lesions affects normal emergence and alignment of adjacent teeth, surgical excision may become necessary. Drug-induced gingival enlargement (GE) in severe cases can impair tooth emergence. Drug-induced GE is charace16 Pediatrics in Review Vol.32 No.1 January 2011
Figure 21. An 8-year-old African American boy who has hereditary gingival bromatosis. Marked gingival enlargement with delayed tooth emergence can be seen. Surgical resection of the gingiva is necessary to permit tooth emergence. Photograph courtesy of Paul Romano, DDS, MS.
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tive effects are seen when nifedipine and cyclosporine are used in organ transplant patients. Cyclosporine also has been implicated in the development of oral eruption cysts in select cases. Tacrolimus, another immunosuppressive agent, has not been found to cause GE after organ and hematopoietic stem cell transplantation. In fact, some clinicians believe that substitution of cyclosporine with tacrolimus can reverse GE in these patients. Surgical management of GE through gingival resection may become necessary if mastication, speech, and esthetics become problematic. Hereditary gingival bromatosis (HGF) is a rare condition affecting 1 in 350,000 individuals that has no sex predilection. Clinically, HGF affects the emergence of the permanent teeth. The clinical manifestations of HGF vary, with malpositioned teeth, delayed exfoliation of primary teeth, delayed emergence of permanent teeth, malocclusion, and open lip posture seen (Fig. 21). HGF is usually managed through optimal oral hygiene practices and surgical resection if esthetics and function are compromised.
References
1. Polder BJ, Vant Hof MA, Van der Linden FPGM, KuijpersJagtman AM. A meta-analysis of the prevalence of dental agenesis in permanent teeth. Community Dent Oral Epidemiol. 2004;32: 217226 2. Demirjian A, Goldstein H, Tanner JM. A new system of dental age assessment. Hum Biol. 1973;45:211227 3. Krekmanova L, Carlstedt-Duke J, Dahllo f MC. Dental maturity in children of short staturea two-year longitudinal study of growth hormone substitution. Acta Odontol Scand. 1999;57: 9396 4. Paulsson L, Bondemark L, So derfeldt B. A systematic review of the consequences of premature birth on palatal morphology, dental occlusion, tooth-crown dimensions, and tooth maturity and eruption. Angle Orthod. 2004;74:269 279 5. Peck S, Peck L. Classication of maxillary tooth transpositions. Am J Orthod Dentofac Orthop. 1995;107:505517 6. Shah SK, Le MC, Carpenter WM. Retrospective review of pediatric oral lesions from a dental school biopsy service. Pediatr Dent. 2009;31:14 19
Suggested Reading
Summary
The presence of DTE, a commonly overlooked nding in primary care, signals abnormalities in tooth formation, eruption, or emergence. DTE often occurs through benign acquired processes such as tooth loss due to dental caries or tooth-jaw size discrepancy. However, the detection of DTE is important because early identication can minimize the comorbidity associated with systemic disease, genetic syndromes, or odontogenic pathology. Pediatricians can reduce the burden of care related to DTE through appropriate history taking and oral inspection during health supervision visits. Children awaiting emergence of teeth for more than 12 months beyond normal chronologic ranges or those who experience localized alterations in the normal emergence order should be referred to a dentist for further evaluation.
American Academy of Pediatric Dentistry. Guideline on management of the developing dentition and occlusion in pediatric dentistry. Reference Manual. 2009;32(6). Accessed August 2009 at: http://www.aapd.org/media/Policies_Guidelines/ G_DevelopDentition.pdf Bailleul-Forestier I, Berdal A, Vinckier F, et al. The genetic basis of inherited anomalies of the teeth. Part 2: syndromes with signicant dental involvement. Eur J Med Genet. 2008;51: 383 408 Bailleul-Forestier I, Molla M, Verloes A, Berdal A. The genetic basis of inherited anomalies of the teeth: Part 1: clinical and molecular aspects of non-syndromic dental disorders. Eur J Med Genet. 2008;51:273291 Frank CA. Treatment options for impacted teeth. JADA. 2000; 131:623 632 Huber KL, Suri L, Taneja P. Eruption disturbances of the maxillary incisors: a literature review. J Clin Pediatr Dent. 2008;32: 221230 Slootweg PJ. Lesions of the jaws. Histopathology. 2009;54: 401 418
Delayed Tooth Emergence Jeffrey M. Karp Pediatrics in Review 2011;32;e4 DOI: 10.1542/pir.32-1-e4
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including high resolution figures, can be found at: http://pedsinreview.aappublications.org/content/32/1/e4 This article cites 11 articles, 1 of which you can access for free at: http://pedsinreview.aappublications.org/content/32/1/e4#BIBL This article, along with others on similar topics, appears in the following collection(s): Genetics/Dysmorphology http://pedsinreview.aappublications.org/cgi/collection/genetics_ dysmorphology Ear, Nose and Throat Disorders http://pedsinreview.aappublications.org/cgi/collection/ear_nose_ throat_disorders Emergency Care http://pedsinreview.aappublications.org/cgi/collection/emergenc y_care Information about reproducing this article in parts (figures, tables) or in its entirety can be found online at: /site/misc/Permissions.xhtml Information about ordering reprints can be found online: /site/misc/reprints.xhtml
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