Management of Amelogenesis Imperfecta in Childhood: Two Case Reports

Home , Amelogenesis imperfecta, Dentin dysplasia

International Journal of Environmental Research and Public Health

Case Report Management of Amelogenesis Imperfecta in Childhood: Two Case Reports

Mirja Möhn 1,*, Julia Camilla Bulski 1 , Norbert Krämer 1, Alexander Rahman 2 and Nelly Schulz-Weidner 1

1 Dental Clinic, Department of Pediatric Dentistry, Justus Liebig University, Schlangenzahl 14, 35392 Giessen, Germany; [email protected] (J.C.B.); [email protected] (N.K.); [email protected] (N.S.-W.) 2 Department of Conservative Dentistry, Periodontology and Preventive Dentistry, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany; [email protected] * Correspondence: [email protected]; Tel.: +49-641-9946241

Abstract: Amelogenesis imperfecta (AI) is deﬁned as an interruption of enamel formation due to genetic inheritance. To prevent malfunction of the masticatory system and an unaesthetic appearance, various treatment options are described. While restoration with a compomer in the anterior region and stainless steel crowns in the posterior region is recommended for deciduous dentition, the challenges when treating such structural defects in mixed or permanent dentition are changing teeth and growing jaw, allowing only temporary restoration. The purpose of this case report is to demonstrate oral rehabilitation from mixed to permanent dentition. The dentition of a 7-year-old patient with AI type I and a 12-year-old patient with AI type II was restored under general anesthesia to improve their poor aesthetics and increase vertical dimension, which are related to problems with Citation: Möhn, M.; Bulski, J.C.; self-conﬁdence and reduced oral health quality of life. These two cases show the complexity of dental Krämer, N.; Rahman, A.; care for structural anomalies of genetic origin and the challenges in rehabilitating the different phases Schulz-Weidner, N. Management of Amelogenesis Imperfecta in of dentition. Childhood: Two Case Reports. Int. J. Environ. Res. Public Health 2021, 18, Keywords: amelogenesis imperfecta; pediatric dentistry; dental care; therapy concept 7204. https://doi.org/10.3390/ ijerph18137204

Academic Editors: Lauren Bohner, 1. Introduction Marcel Hanisch and Fawad Javed Amelogenesis imperfecta (AI) is described as generalized defects in enamel formation in primary and permanent dentition because of a genetic disorder. The inherited malfor- Received: 22 May 2021 mation of teeth can be x-linked, autosomal dominant, autosomal recessive, or sporadic. In Accepted: 29 June 2021 particular, mutation or altered expression of the enamelin (ENAM), amelogenin (AMEL), Published: 5 July 2021 matrixmetalloproteinaise-20 (MMP20), kallikrein-4 (KLK4), and FAM83H genes is associated with the malfunction of enamel-forming proteins [1]. An association with a general or Publisher’s Note: MDPI stays neutral systematic disorder has not been reported. with regard to jurisdictional claims in The clinical manifestation includes four types of AI [2]. The most common phenotype published maps and institutional affil- is type I, characterized by a hypoplastic structure with a decreased quantity of enamel. The iations. teeth show reduced enamel thickness, rough surface, and various extensions of defects (Figure1)[ 3]. Type II, called hypomaturation, shows mottled and softer enamel due to defective protein maturation within the enamel matrix. Additionally, chipping of the enamel from the dentin can be found (Figure2)[ 1]. In AI type II, the enamel thickness is Copyright: © 2021 by the authors. normal. The secretion phase of the ameloblasts proceeds as usual, but in the maturation Licensee MDPI, Basel, Switzerland. phase, normal reabsorption of the secreted enamel matrix proteins does not take place. This article is an open access article Subsequently, a very high proportion of organic matter remains in the enamel [4]. distributed under the terms and Type III (hypocalcification) is associated with defects in calcification and appears in conditions of the Creative Commons enamel with normal thickness at the time of eruption. Because of the poor mineralization, Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a mixed 4.0/). appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5].

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Int. J. Environ. Res. Public Health 2021, 18, 7204 2 of 9 the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a mixed appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5]. mixed appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5].

(a) (b) (a) (b) FigureFigure 1.1. AA 55 1½-year-old-year-old patientpatient withwith AIAI typetype I:I: ((aa)) upperupper teeth;teeth; ((bb)) lowerlower teeth.teeth. ClinicalClinical exanimationexanimation revealedrevealed painpain andand Figure 1. A 5 ½2 -year-old patient with AI type I: (a) upper teeth; (b) lower teeth. Clinical exanimation revealed pain and hypersensitivityhypersensitivity inin yellowyellow teethteeth andand lossloss ofof dentaldental structurestructure (pits).(pits). hypersensitivity in yellow teeth and loss of dental structure (pits).

(a) (b) (a) (b) Figure 2. A 14-year-old patient with hypomaturation of AI: (a) upper teeth; (b) lower teeth. Clinical exanimation revealed Figureyellow 2.teeth A 14 14-year-old affecting-year-old oral patient health with related hypomaturation quality of life. of AI: ( a) upper teeth; ( b) lower teeth. Clinical Clinical exanimation revealed yellow teeth affecting oral health related quality of life. Due to the structure of enamel hypersensitivity, plaque accumulation and poor Due to the structure of enamel hypersensitivity, plaque accumulation and poor aestheticsDue toare the reported structure [6] of. To enamel prevent hypersensitivity, dental caries, gingival plaque accumulationinflammation, and open poor bite, aes- or aesthetics are reported [6]. To prevent dental caries, gingival inflammation, open bite, or theticsloss of arevertical reported dimension, [6]. To interdisciplinary prevent dental caries,patient gingival care is recommended. inflammation, openIn particular, bite, or loss of vertical dimension, interdisciplinary patient care is recommended. In particular, lossconservative of vertical or dimension, prosthetic interdisciplinary and orthodontic patient treatment care is are recommended. crucial for successful In particular, oral conservativeconservative or or prosthetic prosthetic and and orthodontic orthodontic treatment treatment are are crucial crucial for successful for successful oral reha- oral rehabilitation. Various treatment options have been described depending on the patient’s rehabibilitation.litation. Various Various treatment treatment options options have have been been described described depending depending on the on patient’sthe patient’s age age and socioeconomic conditions and the severity of malformation [7]. While stainless ageand and socioeconomic socioeconomic conditions conditions and and the severitythe severity of malformation of malformation [7]. While [7]. While stainless stainless steel steel crowns, strip crowns, and compomer restorations are common in primary dentition, steelcrowns, crowns, strip strip crowns, crowns, and compomerand compomer restorations restorations are commonare common in primary in primary dentition, dentition, the the challenge in mixed and permanent dentition in adolescents is care of the dentition thechallenge challenge in mixed in mixed and permanentand permanent dentition dentition in adolescents in adolescents is care is of care the dentitionof the dentition during during growth [8]. Whereas ceramic crowns and veneers are preferred for adults, growth [8]. Whereas ceramic crowns and veneers are preferred for adults, CAD/CAM duringCAD/CAM growth composites [8]. Whereas offer an ceramic opportunity crowns for and high veneers-quality arerestorations preferred in foradolescent adults, CAD/CAMcomposites offercomposites an opportunity offer an opportunity for high-quality for restorationshigh-quality inrestorations adolescent in children. adolescent The children. The advantages of this approach are less chair time and the possibility of children.advantages The of advantagesthis approach of are this less approach chair time are and less the chair possibility time and of intraoral the possibility repairs inof intraoral repairs in cases of material fractures. Besides the oral complications due to the intraoralcases of materialrepairs in fractures. cases of material Besides thefractures. oral complications Besides the oral due complications to the genetic due defects to the of genetic defects of the enamel, poor aesthetics can also be associated with problems with geneticthe enamel, defects poor of aestheticsthe enamel, can poor also aesthetics be associated can withalso problemsbe associated with with self-confidence problems with and reducedself-confidence oral health-related and reduced quality oral health of life-related [9,10]. quality of life [9,10]. self-confidenceThe aim of and this reduced paper oral was health to report-related the managementquality of life [9,10] of AI. patients from mixed TheThe aim of this paper was to report the management of AI patients from mixed dentition in childhood to permanent dentition in early adulthood by presenting two dentitiondentition in in childhood to permanenpermanentt dentition in early adulthood by presenting two patients, aged 7 and 12 years. For this purpose, the differences between direct filling patients, aged 7 and 12 years. For For this this purpose, the differences between direct fillingfilling therapies combined with prefabricated crowns in mixed dentition and indirect restoration therapiestherapies combined combined with with prefabricated prefabricated crowns crowns in mixed mixed dentition dentition and and indirect indirect restoration restoration in the permanent dentition were compared, and the patients were followed up at 3 and 6 months.

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inin the the permanent permanent dentition dentition wer weree compared, compared, and and the the patients patients were were followed followed up up at at 3 3 and and 6 6 months.months. Int. J. Environ. Res. Public Health 2021, 18, 7204 3 of 9 2.2. Case Case Reports Reports 2.1.2.1. Case Case Report Report 1: 1: Mixed Mixed Dentition Dentition 2. CaseAA 7 7 Reports-year-year-old-old girl girl was was referred referred to to our our pediatric pediatric polyclinic polyclinic due due to to aesthetic aesthetic problems problems and2.1.and Casesensitive sensitive Report teeth teeth 1: Mixed with with pain. Dentitionpain. The The mother mother felt felt extremely extremely affected affected in in her her social social life life by by her her daughter’s structural problem, as the child was being teased by other children at school daughter’sA 7-year-old structural girl problem, was referred as the to child our pediatric was being polyclinic teased by due other to aesthetic children problemsat school due to her teeth. The family history showed no abnormalities; neither parent had dueand sensitive to her teeth. teeth Thewith family pain. The history mother showed felt extremely no abnormalities; affected in her neither social parent life by had her phenotypic dental structure anomalies. Clinical examination revealed AI type I phenotypicdaughter’s structural dental structure problem, as anomalies. the child was Clinical being teased examination by other revealedchildren at AI school type due I hypomineralized teeth. Oral examination presented easily chipping enamel combined hypomineralizedto her teeth. The family teeth. history Oral examination showed no presented abnormalities; easily neither chipping parent enamel had phenotypic combined with reduced enamel thickness, rough surface, and various extensions of structural loss. withdental reduced structure enamel anomalies. thickness, Clinical roughexamination surface, and revealedvarious extensions AI type I of hypomineralized structural loss. Defectsteeth.Defects Oral in in enamel enamel examination matrix matrix presented formation formation easily showed showed chipping pitted pitted enamel and and grooved grooved combined enamel enamel with, ,especially reducedespecially enamel in in the the maxillarythickness,maxillary front roughfront and surface,and upper upper and and and various lower lower extensions first first permanent permanent of structural molars. molars. loss. In In Defectsaddition, addition, in enamelthe the maxilla maxilla matrixryry andformationand mandibular mandibular showed fronts pittedfronts andwere were grooved clearly clearly enamel, spacedspaced especially apart apart. . The The in the patient’s patient’s maxillary oral oral front hygiene hygiene and upper was was inadequateandinadequate lowerﬁrst due due permanent to to hypersensitivity hypersensitivity molars. In addition, and and the the the tooth tooth maxillary surface surface and structure. structure. mandibular A A fronts panoramic panoramic were radiographclearlyradiograph spaced revealed revealed apart. loss loss The of of patient’s enamel enamel (Figure oral(Figure hygiene 3). 3). Tooth Tooth was wear inadequatewear could could be be due detected detected to hypersensitivity (Figure (Figure 4). 4). A A carandcariesies the lesion lesion tooth in in surface region region structure. 64 64 could could Abe be panoramicdiagnosed diagnosed radiograph(Figure (Figure 5a 5a– revealed–d).d). loss of enamel (Figure3). ToothDueDue wear to to the couldthe extensive extensive be detected scope scope (Figure of of care, care,4). the the A cariespatient patient lesion underwent underwent in region comprehensive comprehensive 64 could be diagnosedrehabilita- rehabilita- tion,(Figuretion, which which5a–d). was was performed performed under under general general anesthesia anesthesia because because of of her her age age and and anxiety. anxiety. Ad- Ad- hesivehesive build build-ups-ups and and stainless stainless steel steel crowns crowns stabilized stabilized the the vertical vertical dimension. dimension.

Figure 3. FFigureigure 3. 3. Panoramic Panoramic X X X-ray-ray-ray of of 7 7 7-year-old-year-year-old-old patient. patient.

(a(a) ) (b(b) )

Figure 4. Plaster models of upper and lower jaws showing loss of tooth wear because of attrition and abrasion (physical tooth wear): (a) right side; (b) left side.

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Int. J. Environ. Res. Public Health 2021, 18, 7204 4 of 9 Figure 4. Plaster models of upper and lower jaws showing loss of tooth wear because of attrition and abrasion (physical tooth wear): (a) right side; (b) left side.

a b c

d e f

g h

i j

FigureFigure 5. 5. AA 7- 7-year-oldyear-old patient patient with with AI AI type type I. ( I.a– (af)– fPreoperative) Preoperative situation situation with with multiple multiple substance substance defects defects on all on teeth; all teeth; (c) after(c) after fluoride fluoride varnish varnish application. application. (g– (jg) –Postoperativej) Postoperative result. result. Oral Oral surgery surgery was was performed performed under under general general anesthesia, anesthesia, with with stainless steel crowns applied to second primary molars and adhesive filling materials in first primary molars, first molars stainless steel crowns applied to second primary molars and adhesive filling materials in first primary molars, first molars and anterior teeth. and anterior teeth.

™ StainlessDue to the steel extensive crowns scope (3M of, Neuss, care, the Germany) patient underwent were applied comprehensive to second primary rehabili- molars;tation, whichafter tangential was performed preparation, under adaptation, general anesthesia and control because of the of “snap her age effect”, and anxiety. metal ™ ™ ™ crownsAdhesive were build-ups cemented and with stainless glass steelionomer crowns cement stabilized (Ketac the Cem vertical Aplicap dimension., 3M , Neuss, Germany).Stainless First steel primary crowns molars (3M™ ,were Neuss, reconstructed Germany) were using applied an all to-in second-one adhesive primary system molars; ™ ™ ® (Scotchafter tangentialbond Universal, preparation, 3M adaptation,, Neuss, andGermany) control of and the compomer“snap effect”, (Dyract metal crowns, Dentsply were Sirona,cemented Bensheim, with glass Germany), ionomer except cement tooth (Ketac 64,™ whichCem was Aplicap severely™, 3M destroyed™, Neuss, and Germany). had to beFirst extracted. primary Permanent molars were molars reconstructed and anterior using teeth an all-in-one were covered adhesive with system direct (Scotchbond composite™ fillingUniversal, material 3M™ (Figure, Neuss, 5e Germany)–h). Without and removing compomer malformed (Dyract®, Dentsplyenamel, etching Sirona, Bensheim,with 34% phosphoricGermany), acid except (Scotchbond tooth 64, which™ Etchant, was 3M severely™, Neuss, destroyed Germany) and for had 60 to s bewas extracted. carried out Per-, followedmanent molars by rinsing and and anterior drying. teeth After were bonding covered (Scotchbond with direct™ composite Universal, ﬁlling 3M™,material Neuss, Germany),(Figure5e–h). a flow Without comp removingosite (Venus malformed® Diamond enamel, Flow A2, etching Kulzer, with Hanau, 34% phosphoric Germany) acidwas directly(Scotchbond applied,™ Etchant, and then 3M, ™composite, Neuss, Germany) (Venus® Diamond for 60 s was A2, carried Kulzer out,, Hanau, followed Germany) by rins- wasing andapplied. drying. After bonding (Scotchbond™ Universal, 3M™, Neuss, Germany), a ﬂow composite (Venus® Diamond Flow A2, Kulzer, Hanau, Germany) was directly applied, and then, composite (Venus® Diamond A2, Kulzer, Hanau, Germany) was applied.

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After rehabilitation, the patient was reevaluated after 3 months. Follow Follow-up-up 6 months laterlaterAfter showed rehabilitation, a satisfactory satisfactory the patient aesthetic was reevaluated and functional functional after result. result. 3 months. Since Since Follow there there- up was 6 months no space laternarrowing showed in a region satisfactory 74, we aesthetic did not need and to functional fabricate aresult. space Since maintainer there (Figure was no6 6). ). space narrowing in region 74, we did not need to fabricate a space maintainer (Figure 6). a a

b b

b c b c Figure 6. Follow-up 6 months after restoration under general anesthesia: (a) upper teeth; (b) lower teeth; (c) front teeth. Figure 6. Follow-up 6 months after restoration under general anesthesia: (a) upper teeth; (b) lower teeth; (c) front teeth. All FigureAll restorations 6. Follow-up in 6situ, months no abnormalities. after restoration under general anesthesia: (a) upper teeth; (b) lower teeth; (c) front teeth. Allrestorations restorations in in situ, situ, no no abnormalities. abnormalities. 2.2. Case Report: Permanent Dentition 2.2.2.2. Case Case Report Report:: Permanent Permanent Dentition Dentition A 12-year-old patient presented to our polyclinic because of the unattractive aesthet- icsA of 12A his- 12-year-oldyear teeth-old due patient patientto AI presented type presented II. In toaddition to our our polyclinic polyclinic to the change because because in of color of the the unattractiveof unattractive all permanent aesthet- aesthetics teeth, icstheof of his hisloss teethteeth of verticaldue dueto toAI dimensionAI type type II. II. In Inand addition addition the gaps to to the thein changethe change dentition in in color color were of of all allimpressive permanent permanent (Figure teeth, teeth, the7). loss of vertical dimension and the gaps in the dentition were impressive (Figure7). The theThe loss 12 of-year vertical molars dimension had not andyet (completely)the gaps in the erupted dentition into werethe oral impressive cavity. In (Figure addition 7). to 12-year molars had not yet (completely) erupted into the oral cavity. In addition to AI, the TheAI, 12 the-year patient molars presen had tednot with yet (completely) cardiac disease erupted and immunodeficiency. into the oral cavity. As In he addition was a foster to patient presented with cardiac disease and immunodeﬁciency. As he was a foster child, his AI,child, the patient his family presen historyted with was cardiacnot available. disease and immunodeficiency. As he was a foster child,family his historyfamily history was not was available. not available.

(a) (b) (a) (b)

Figure 7. Cont.

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(c)( c)

FigureFigureFigure 7. A7. 7.12A A-12year 12-year-old-year-old-old patient patient patient showing showing showing clinical clinical clinical signs signs signs of AIof of AItype AI type typeII: II:(a II:,b(a);, (b aupper,);b upper); upper teeth teeth teeth (c) (frontc) ( cfront) front teeth. teeth. teeth. BesidesBesidesBesides the the yellow the yellow yellow color, color, color, gaps gaps gaps between between between teeth teeth teeth are are predominant. are predominant. predominant.

DueDueDue to tothe to the strong the strong strong need need need for for aesthetic for aesthetic aesthetic improvement improvement improvement and and andthe the associated the associated associated general general general disease, disease, disease, it it was it was was decided decided decided to to perform to perform perform all all-composite all-composite-composite rehabilitation. rehabilitation. rehabilitation. In In this In this this direct direct direct approach, approach, approach, resin resin resin compositecompositecomposite restoration restoration restoration was was wasused used used for for the for the transitional the transitional transitional treatment treatment treatment of ofhypomature of hypomature hypomature AI. AI. AI. First,First,First, an an impression an impression impression of ofthe of the themaxilla maxilla maxilla and and and mandible mandible mandible with with with A -Asilicone A-silicone-silicone material material material (Panasil (Panasil (Panasil® ® ® PuttyPuttyPutty Fast, Fast, Fast, Kettenbach, Kettenbach, Kettenbach, Germany) Germany) Germany) was was was taken taken taken through through through advance advance advancedd dchairside. chairside. chairside. Due Due Due to to the to the the sufficientsufficientsufficient space space space of ofthe of the theupper upper upper and and and lower lower lower jaws, jaws, jaws, there there there was was was no no noneed need need for for for preparation. preparation. preparation. Full Full Full compositecompositecomposite crowns crowns crowns were were were built built built up up on up on plaster on plaster plaster models models models after after after articulation articulation articulation according according according to toaverage to average average valuesvaluesvalues by by the by the dental the dental dental technician. technician. technician. A jawA Ajaw jawrelation relation relation determination determination determination could could could not not notbe beperformed be performed performed due due due to totheto the patient the patient patient’s’s insufficient’s insufficient insufficient compliance. compliance. compliance. Due Due Due to tothis to this thisparticular particular particular feature, feature, feature, it shouldit itshould should be bepointed be pointed pointed outout,out, in, inthis in this thiscase case case,, that, that thatadaptation adaptation adaptation to tothe to the thenew new new mandibular mandibular mandibular posture posture posture would would would be berequired be required required after after after insertion.insertion.insertion. The The Theparents parents parents were were were informed informed informed about about about this this thisin indetail. in detail. detail. UnderUnderUnder general general general anesthesia, anesthesia, anesthesia, the the restorations the restorations restorations were were were tried tried tried on on and on and andcemente cemente cementedd withd with with dual dual dual-- - ® ™ ™ curingcuringcuring resin resin resin cement cement cement (Variolink (Variolink (Variolink® Esthetic,® Esthetic,Esthetic, Ivoclar Ivoclar Ivoclar™ Vivadent™ VivadentVivadent™, ™Ellwangen,, Ellwangen,, Ellwangen, Germany) Germany) Germany) using using using ® ® ® ™ ™ MonobondMonobondMonobond® Plus® PlusPlus and and and Adhese Adhese Adhese® Universal® UniversalUniversal VivaPen VivaPen VivaPen® (Ivoclar® (Ivoclar(Ivoclar™ ™Vivadent VivadentVivadent™, ™Ellwangen,, Ellwangen,, Ellwangen, Germany).Germany).Germany). The The Theocclusion occlusion occlusion was was wasslightly slightly slightly raised raised raised to toprovide to provide provide sufficient sufficient sufficient space space space for for th for isth thisrestorativeis restorative restorative ® ™ ™ reconstruction.reconstruction.reconstruction. Fissure Fissure Fissure sealing sealing sealing (Helioseal (Helioseal (Helioseal®, ® Ivoclar, Ivoclar, Ivoclar™ ™Vivadent VivadentVivadent™, ™Ellwangen,, Ellwangen,, Ellwangen, Germany) Germany) Germany) ™ waswaswas performed performed performed on on the on the second the second second permanent permanent permanent molars, molars, molars, and and andstainless stainless stainless steel steel steel crowns crowns crowns (3M (3M (3M™, ™Neuss,, Neuss,, Neuss, Germany)Germany)Germany) were were were placed placed placed on on all on all first all first first permanent permanent permanent molars molars molars and and andcemented cemented cemented with with with glass glass glass ionomer ionomer ionomer ™ ™ ™ cementcementcement (Ketac (Ketac (Ketac™ Cem™ CemCem Aplicap Aplicap Aplicap™, ™3M, 3M™,, 3M ™Neuss,, Neuss,, Neuss, Germany) Germany) Germany) (Figure (Figure (Figure 8) .8 ).8 ).

(a)( a) Figure 8. Cont.

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(b)

FigureFigure 8. 8.AA 12 12-year-old-year-old patient patient with with AI AI type type II: II: (a ()a )upper upper teeth; teeth; (b (b) )lower lower teeth. teeth. Oral Oral surgery surgery was was performedperformed under under general anesthesia anesthesia using using indirect indirect composite composite restorations restorations and stainless and stainless steel crowns. steel crowns. Three-month follow-ups were recommended to identify and repair possible defects at an earlyThree stage.-month In follow our case,-ups the were patient recommended adapted to to full identify crown treatmentand repair very possible well defects and had atno an symptoms early stage. in In the our temporomandibular case, the patient adapted joint after to follow-upfull crown within treatment 6 months. very well and had no symptoms in the temporomandibular joint after follow-up within 6 months. 3. Discussion 3. DiscussionRehabilitating a patient with AI is challenging from both the functional and aesthetic pointRehabilitating of view. The a patient complexity with ofAI theis challenging disease requires from both an interdisciplinary the functional and approach aesthetic to pointachieve of view. optimal The treatment complexity results. of the Several disease treatment requires options an interdisciplinary have been proposed. approach Recently, to achievethe use optimalof bonded treatment restorations results. has gained Several popularity treatment due options to the many have advantages been proposed. of these Recently,materials, the including use of excellent bonded aesthetics, restorations conservative has gained approach, popularity and improved due to the wear many make. advantagesDental of rehabilitation these materials, is one including important excellent part of aesthetics, improving conservative oral health-related approach, quality and of improvedlife for children wear make. with generalized structure defects. The main objective of dental treatment of patientsDental rehabilitation with hereditary is structuralone important anomalies part of is improving to prevent nearbyoral health caries-related damage quality [6]. In ofaddition, life for the children dentist with should generalized counteract structure the abrasion defects. of the The clinical main crown objective by performing of dental treatmentearly treatment of patients in order with to prevent hereditary dimension structural loss anomalies and tooth lossis to [ 3 prevent]. In every nearby case, thecaries age damageof the patient [6]. In mustaddition, be considered the dentist in should treatment counteract planning. the In ourabrasio case,n aestheticof the clinical rehabilitation crown bywas performing very important early treatment for both patients. in order to prevent dimension loss and tooth loss [3]. In every case,According the age to Toupenayof the patient et al., must there be is considered no agreement in treatment regarding theplanning. protocol In forour therapy case, except the timing: treatment should begin as early as possible to prevent tooth sensitivity aesthetic rehabilitation was very important for both patients. and enamel loss [3]. According to Toupenay et al., there is no agreement regarding the protocol for While stainless steel crowns, strip crowns, and compomer restorations are common in therapy except the timing: treatment should begin as early as possible to prevent tooth primary dentition, the growing jaw and the changing of teeth present challenges in terms sensitivity and enamel loss [3]. of treatment options in mixed and permanent dentition. Therefore, the full spectrum of While stainless steel crowns, strip crowns, and compomer restorations are common dental materials should be exhausted during the development of a young adolescents. In in primary dentition, the growing jaw and the changing of teeth present challenges in particular, primary and mixed dentition only allow temporary therapy: conventional/resin- terms of treatment options in mixed and permanent dentition. Therefore, the full based glass ionomer cements, compomers/composites, strip crowns, preformed metal, or spectrum of dental materials should be exhausted during the development of a young tooth-colored crowns [11]. adolescents. In particular, primary and mixed dentition only allow temporary therapy: In our first case, we mainly chose direct adhesive filling materials for tooth build-up conventional/resin-based glass ionomer cements, compomers/composites, strip crowns, because of the small loss of substance. It is typical for AI type I that the existing malformed preformedenamel has metal, similar or ortooth identical-colored characteristics crowns [11] to. enamel that is formed regularly [1]. For ad- hesiveIn our therapy, first case, this meanswe mainly that achose normal direct etching adhesive pattern filling can materials be expected for and tooth the build adhesive-up becausesystem of used the small will act loss identically of substance. to physiologically It is typical for AI formed type I enamel. that the Therefore,existing malformed it was not enamelnecessary has tosimilar remove or theidentical enamel characteristics partially or completely to enamel inthat this is case.formed Especially regularly in [1] younger. For adhesivepatients therapy,with teeth this that means have just that erupted, a normal it can etching be beneficial pattern for can the be practitioner expected and and the the adhesivepatient tosystem be able used to restore will act them identically noninvasively to phy butsiologically still functionally formed enamel. and aesthetically. Therefore, Con- it wasventional not necessary or resin-based to remove glass the ionomer enamel cementspartially would or completely not have in been this suitablecase. Especially due to their in youngerlower flexural patients strength with teeth and that wear have resistance. just erupted, These it properties can be beneficial allow the for material the practitioner to be used andtemporarily the patient chairside, to be butable it to should restore not them be applied noninvasively under optimal but still conditions functionally when using and aesthetically.general anesthesia Conventional [12]. The or development resin-based ofglass compomers ionomer (polyacrylic/polycarboxyliccements would not have been acid

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modified composites) combines the advantages of glass ionomer cements (easy application) and composites (aesthetics). Nowadays, compomers (e.g., Dyract®) are the first choice for restoring primary teeth. In contrast, composites should be used as a long-term filling material in permanent dentition because of their higher wear resistance and compressive, flexural, and tensile strength [13]. Only the second primary molars were restored with stainless steel crowns to repair circular defects in this case. The advantages of metal crowns are easy adaptation, gentle preparation, and time-saving handling [14]. Preformed ceramic crowns for molars and incisors require strict preparation and involve high abrasion of antagonists [15]. In follow-up, we could not find any loss of filling, which was also to be expected regarding the etching pattern, which did not differ from the healthy enamel. For affected permanent teeth, various full crowns are indicated. Depending on the patient’s age, metal, composite, and ceramic crowns are common. While individual ceramic crowns are contraindicated in adolescents due to jaw growth, composites can provide a temporary restoration. In particular, newly introduced high-performance CAD/CAM composites enable aesthetic restoration of malformed permanent teeth in young patients [16]. The patient in our second case showed severe loss of enamel in permanent dentition with excess space in the upper and lower jaws. Therefore, indirect composite crowns were used to rebuild the vertical dimension. Previous impressions and cooperation with the dental technician made rehabilitation of the chewing system easier. The interdental spaces could be used to avoid grinding of the teeth. The process of preparing the teeth could protect the hard dental tissue. Other authors have described direct composite restoration in combination with a wax-up to restore complex cases [17]. However, this treatment is very time-consuming and demanding. In the case of adhesive restorations, it should be noted that normal conditioning (etching pattern, effectiveness of the adhesive system) is not possible in affected enamel, so very early failure and loss of restorations are often recorded [18]. In addition, the remaining enamel can repeatedly flake off, so the corresponding tooth will need a new restoration or the existing one will need to be expanded [4]. Generally, a successful therapy concept is based on a close recall program with oral hygiene instruction, remotivation, and fluoride application. In this way, carious lesions or restorative defects and gingivitis can be prevented. A limitation of our study is that up to now there has only been a follow-up of 6 months after intervention. However, since the parents’ compliance with the follow-up appointments is considered reliable, we expect a good prognosis with regard to the aspects mentioned above. In addition, the use of electric toothbrushes should also be considered regarding the practical implementation of home oral hygiene. A study by Preda et al. showed that electric toothbrushes were superior to manual toothbrushes in plaque removal. Therefore, rotating–oscillating or sonic-action heads should be recommended for patients with difficult hygienic conditions to avoid bacterial infiltration [19]. Due to the extensive treatment needs, complex treatment measures, and often age- related insufficient cooperation, comprehensive rehabilitation under general anesthesia cannot be avoided. Overall, the patients report less sensitivity, better oral hygiene ability, and better quality of life.

4. Conclusions The cases described in this paper show the complexity of the dental care of structural anomalies of genetic origin. Patients with hereditary structural anomalies require close lifelong dental care to maintain the therapeutic results.

Author Contributions: Conceptualization, M.M. and N.S.-W.; methodology, M.M.; software, J.C.B.; validation, M.M., N.S.-W. and N.K.; formal analysis, M.M.; investigation, M.M., N.S.-W. and J.C.B.; resources, N.K.; data curation, A.R.; writing—original draft preparation, M.M. and N.S.-W.; writing— review and editing, M.M., J.C.B., N.K., A.R. and N.S.-W.; visualization, J.C.B.; supervision, N.K.; project administration, N.K. All authors have read and agreed to the published version of the manuscript. Int. J. Environ. Res. Public Health 2021, 18, 7204 9 of 9

Funding: This research received no external funding. Institutional Review Board Statement: Ethical review and approval were waived for this study, due to individual parents’ consent. Informed Consent Statement: Informed consent was obtained from all subjects involved in the study. Written informed consent was obtained from the patients’ parents to publish this paper. Data Availability Statement: Not applicable. Conﬂicts of Interest: The authors declare no conﬂict of interest.

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