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Orthodontic Treatment in a Patient With Papillon-LefèvreSyndromeChristopher J. Lux,* Birgit Kugel,* Gerda Komposch,* Sabine Pohl,† and Peter Eickholz‡

Case Report

Background: Report of a combined periodontal andorthodontic treatment in a patient with Papillon-LefèvreSyndrome (PLS).

Methods: A patient with PLS was treated ortho-dontically 26 months after the start of a combinedmechanical and antibiotic therapy. Clinical periodon-tal parameters were obtained 26 (t1), 60 (t2), and 79(t3) months after anti-infective therapy. The deepestsite of each tooth was sampled for microbiologicalanalysis at 26 and 60 months. Periodontal maintenancetherapy was provided every 6 weeks. After a stableperiodontal situation was achieved, orthodontic treat-ment, consisting of space opening for the upper canineswith a multibracket appliance and coil springs, wascarried out. In the lower jaw, crowding was resolvedby an orthodontic mesialization of the canines.

Results: Twenty-six months (t1) after the beginningof the combined mechanical and antibiotic therapy,6% of the sites exhibited 4 mm probing depth (PD) withbleeding on probing (BOP) or PD ≥5 mm. Sixty months(t2) after therapy the number of sites with 4 mm PDwith BOP or PD ≥5 mm had increased to 17%, and79 months after therapy (t3) 13% of all sites were sim-ilarly affected. From 26 to 60 months, a slight meanclinical attachment level (CAL) gain was observed,whereas the mean PD increased. From 60 to 79 months,there was a mean PD reduction. However, a significantmean attachment loss was also noted. After 26 months(t1), RNA probes failed to detect A. actinomycetem-comitans, P. gingivalis, or T. forsythensis from any site.Thirty-four months later (t2), subgingival recoloniza-tion was observed. A. actinomycetemcomitans wasdetected by RNA probes at three sites. At 26 and60 months (t1, t2), trypticase-soy with serum, baci-tracin, and vancomycin (TSBV) culture failed to detectA. actinomycetemcomitans at any of the sampled sites.Eighty-two months after the beginning of therapy (t4),none of the applied methods could detect A. actino-mycetemcomitans from the pooled samples from thedeepest pockets of each quadrant or the oral mucosa.In the present case, concomitant orthodontic treatment

with a fixed appliance could be performed without fur-ther pronounced periodontal deterioration. Space foreruption of the canines and premolars was created, inaddition to an alignment of the teeth.

Conclusion: After a successful combined mechan-ical and antibiotic periodontal therapy of the PLS perio-dontitis, moderate orthodontic tooth movements maybe possible within a complex interdisciplinary treat-ment regimen. J Periodontol 2005;76:642-650.

KEY WORDSFollow-up studies; orthodontics, corrective;Papillon-Lefèvre syndrome; periodontitis/drugtherapy; tooth movement.

Papillon-Lefèvre Syndrome (PLS) is a rare autosomal-recessive entity with palmo-plantar keratosis andan early onset periodontitis in the deciduous and

permanent dentitions.1,2 A number of publications havefocused on the periodontal management of this dis-ease3-11 as well as its genetic background.12-18 In sum-mary, literature shows that, in PLS, the success ofperiodontal treatment is highly variable and that PLSoften leads, even at early ages, to partial or completetooth loss. Until now, there has not been any informationavailable whether or not orthodontic tooth movements arerealizable in patients with periodontal destruction asso-ciated with PLS. Hence, the aim of this case report wasto describe, for the first time, the results of a combinedperiodontal-orthodontic approach in a patient sufferingfrom PLS.

CASE REPORTIn 1995, a male patient was referred to the Section ofPeriodontology, Department of Conservative Dentistry,Dental Hospital, University of Heidelberg at the age of 7years and 9 months. He was diagnosed with PLS due tohyperkeratosis of the palms and soles as well as advancedperiodontal disease affecting all erupted permanent teeth.All deciduous teeth had been lost prematurely.

Periodontal ExaminationsPeriodontal examinations were carried out at 26 (t1),60 (t2), 79 (t3), and 82 months (t4), and anti-infectivetherapy was begun at the age of 7 years 11 months

* Department of Orthodontics, University of Heidelberg, Heidelberg, Germany.† Department of Microbiology, University of Heidelberg.‡ Department of Conservative Dentistry, Section of Periodontology,

University of Heidelberg.

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(t0). The periodontal managementup to t1 has been described in aprevious publication.19 Briefly, anti-infective therapy comprised of oralhygiene instructions, supragingivalscaling, and subgingival instru-mentation with adjunctive systemic250 mg amoxicillin 3 times dailyand 250 mg metronidazole twice aday for 1 week was performed. Att1, t2, and t3, full mouth bleeding(gingival bleeding index: GBI)20 andplaque scores (plaque controlrecord: PCR)21 were recorded.Probing depths (PD) and verticalclinical attachment levels (CAL-V)were measured at six sites per tooth (mesio-buccal, mid-buccal, disto-buccal, disto-lingual, mid-lingual, andmesio-lingual) to the nearest 0.5 mm (t1) and to thenearest 1 mm (t2 and t3) using a straight periodontalprobe.§ The cemento-enamel junction (CEJ) was usedas a reference for the CAL-V measurements. Bleedingon probing (BOP) was recorded approximately 20 sec-onds after probing. The clinical horizontal probingattachment levels (CAL-H) were assessed to the near-est 0.5 mm at each furcation location using a color-coded, calibrated Nabers probe,� marked at 3 mmintervals.22,23

Complete microbiological sampling was performedat t1 and t2. At the sites with the deepest pockets ofeach tooth, samples of subgingival plaque were takenby inserting a sterile paper point into the pocket for20 seconds after isolating the area with cotton rolls.Immediately after sampling, the paper points weretransferred to vials containing 1 ml anaerobically ster-ilized half-strength Ringer’s solution. After sonicationfor 10 seconds, a sterile cotton swab was submergedinto the solution and spread on freshly preparedtrypticase-soy with serum, bacitracin, and vancomycin,(TSBV) agar plates for selective cultivation of A. actino-mycetemcomitans. Plates were incubated for 3 to5 days at 36°C in air supplemented with 5% CO2. Iden-tification of A. actinomycetemcomitans on TSBV agarwas essentially based on typical colony morphology(small circular colonies, adhering to the agar surface,frequently with star-like inner structure) and a posi-tive catalase test.24 An additional sample of the deep-est pocket of each tooth was taken with another sterilepaper point for 20 seconds. The sample from eachtooth was analyzed separately for A. actinomycetem-comitans, P. gingivalis, T. forsythensis, and T. denticolausing a commercially available RNA probe test.¶ Att4, three separate plaque samples were taken at thedeepest site of each quadrant with paper points. Threesets of four paper points each were pooled and ana-lyzed separately by the RNA probe test, by another

commercially available DNA probe test,# and by cul-ture as described above. Plaque from the left and theright cheek mucosa and the tongue was also sampledand cultured.

Orthodontic TreatmentThe clinical situation after successful periodontal treat-ment (Figs. 1 through 3) shows problems relating tothe future eruption of permanent canines and premo-lars. At the age of 10 years 1 month (t1), the lack ofspace for the upper canines (arrows; Fig. 2) was read-ily detected; i.e., the spaces between the lateral incisorsand the first premolars were only 1.7 mm (right side)

Figure 1.Intraoral view at 9 years 1 month.

Figure 2.Plaster casts at 10 years 1 month, 26 months (t1) after beginningactive periodontal treatment. Notice (arrows) the deficiency of spacefor the canines (upper jaw).

§ PCPUNC 15, Hu Friedy, Chicago, IL.� PQ2N, Hu Friedy.¶ IAI PadoTest 4.5, Institut für Angewandte Immunologie, Zuchwil,

Switzerland.# DMDx PathTek Test, ANAWA Laboratorien AG, Wangen-Zürich,

Switzerland.

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Figure 5.Starting point of orthodontic treatment in the lower jaw.The deficiencyof space for the canines and premolars is obvious.

Figure 3.Orthopantomogram at 9 years 9 months.

Figure 4.Intraoral view at 11 years 8 months, after space opening for the uppercanines.

and 0.8 mm (left side). In addition, the left upper sec-ond premolar was palatally displaced. In the lower arch,an overeruption of the central and lateral incisors(Fig. 1) was apparent, along with extreme horizontalbone loss seen on the panoramic radiograph (Fig. 3).Moreover, the anterior teeth exhibited slight crowdingwith a discrepancy of 1.5 mm. The space availabilityfor the lower canines and premolars was only 18.6mm on the left side and 20.7 mm on the right side,indicating that the total space for the respective suc-cessors was likely to be insufficient, particularly on theleft side.

Tooth movements upper arch. In the upper jaw, themain problems were the deficient space for the uppercanines and the palatal displacement of the upper leftsecond premolar. From the periodontal view, the cen-tral and lateral incisors as well as the first molarsalready exhibited considerable bone loss, whereas thecanines and premolars had a good prognosis due totheir later eruption with the concomitant periodontaltreatment. Hence, eruption and alignment of thecanines and premolars were regarded as major ortho-dontic treatment goals. The spaces for the uppercanines were initially opened with a multibracket appli-ance and coil springs. Orthodontic treatment wasbegun in only one jaw due to the uncertain tissue reac-tion of this syndrome. Figure 4 shows the results at theage of 11 years 8 months (45 months after the begin-ning of periodontal treatment, between t1 and t2) aftercreating space for the canines.

Tooth movements lower arch. In the lower jaw, themain problems were an overeruption of the lowerincisors and a moderate to severe crowding in thecanine and premolar region along with an impedederuption for the lower right second premolar (Fig. 5).The dental radiograph of the lower incisors (Fig. 6)evinced extreme bone loss for the lateral and evenmore pronounced bone loss for the central incisors.Consequently, the treatment plan for the lower jaw wasto extract the two central incisors, to slightly intrudethe two lateral incisors and, as a second step, tomesialize the two lateral incisors. The lower crowding

would therefore be resolved by the subsequent slightmesialization of the canines. At the age of 11 years9 months, after orthodontic space opening in the upperjaw, the two lower central incisors were extracted anda .016 × .022 TMA utility arch was inserted to slightlyintrude the lateral incisors. Intrusive forces were keptat very low levels (approximately 0.15 N for bothteeth). Subsequently, a multibracket appliance wasused to mesialize the lateral incisors and to mesialize,to a lesser extent, the canines. Figure 7 shows theresults at the age of 13 years 5 months after removalof the multibracket appliances in the upper and lowerjaws; i.e., 66 months after start of periodontal therapy,between t2 and t3. In the lower jaw, the two remain-ing incisors were stabilized by means of composite

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build-ups, such that a rigid block from canine to caninewas created, and the remaining spaces between theincisors and the canines were closed. Hawley retainerswere used for additional retention.

Periodontal Maintenance TherapyDuring the complete orthodontic treatment, the patientwas kept in a maintenance program, including oralhygiene instruction and professional tooth cleaning,once every 6 weeks to 2 months. Sites with PD of4 mm and BOP as well as sites with PD ≥5 mm under-went subgingival scaling as well as instillation of 1%

chlorhexidine gel. Each maintenance visit included theassessment of a full mouth bleeding (GBI)20 and plaquescore (PCR).21 At the age of 12 years 11 months(2 months after t2) 30% chlorhexidine chips** wereplaced subgingivally at teeth 3, 23, 26, and 30. At theage of 14 years 1 month (14 months after t2), thegerms of all third molars were removed under generalanesthesia at the Department of Oral and MaxillofacialSurgery, University Dental Hospital Heidelberg. Rein-strumentation of pathological pockets was performedin the course of this general anesthesia.

RESULTSPeriodontal Treatment

At t1, 6% of all sites exhibited PDof 4 mm with BOP or PD ≥5 mm.Eight percent of all sites bled onprobing (Tables 1, 2, and 3), anda CAL-V up to 6 mm was foundat the mandibular incisors andmaxillary molars. Furcation involve-ment was not found. The patient’scompliance with effective oral hy-giene was quite limited. Despitefrequent maintenance care, indi-vidual plaque control was unstable(PCR ranging between 1% and100%). At t2, the number of siteswith PD of 4 mm with BOP or PD

≥5 mm increased to 17%, and at t3, 13% of all siteswere similarly affected (Table 2). Fifty-three sites bledon probing at t2, and 24 sites exhibited BOP at t3(Table 1). At t2, furcation involvement was found forthe first time at the maxillary first molars (Table 2).From t1 to t2, a slight mean CAL gain was observed,whereas mean PD was increased markedly (Table 1).From t2 to t3, there was a mean PD reduction. How-ever, a significant mean attachment loss was alsonoted.

Figure 7.Intraoral view at 13 years 5 months, before and after composite build-ups.

Figure 6.Dental radiograph at 11 years 7 months. Note the severe bone lossaround the lower incisors.

** PerioChip, Dexxon Ltd., Hadera, Israel.

Table 1.

Full Mouth Bleeding and Plaque Scores(Mean ±± SD) at 26 (t1), 60 (t2), and 79 Months (t3)

Time N GBI PCR BOP PD CAL-V (months) Sites (%) (%) (%) (mm) (mm)

26 108 8 53 8 2.80 ± 1.09 1.76 ± 2.33

60 156 51 80 53 3.53 ± 1.31 1.49 ± 2.14

79 156 13 30 24 2.81 ± 1.18 2.11 ± 1.65

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Table 2.

Maxillary Probing Parameters

79 monthsFurcation 0 I 0 0 0 0 I 0CAL-V 2/2/2 6/4/9 2/1/1 2/1/1 1/1/1 1/1/1 2/2/3 3/1/1 2/1/1 2/2/1 1/1/2 1/1/1 5/2/5 3/2/2 PD 4*/3/4 5/2/8 3/2/3 3/1/3 3/2/3 3/1/3 4/2/4 4/2/2 3/2/2 4/2/3 3/1/3 3/2/3 5/2/5 5/2/4

60 monthsFurcation 0 I 0 0 0 0 0 0CAL-V 2/0/0 5/4/6 2/1/1 0/0/0 1/0/0 0/0/1 1/2/2 2/1/1 1/1/1 2/1/0 0/0/1 1/0/1 4/0/1 0/1/0PD 4/3/4 5/4/6 3/2/3 3/2/3 4/2/4 3/2/3 4/2/4 4/2/4 4/2/4 4/2/3 3/2/4 4/2/4 6/2/4 5/4/4

26 monthsFurcation 0 0 0 0 0 0CAL-V 3/3.5/5 0/0/0 0/0.5/1 1/0/1.5 1/0/1.5 3/0/1 1/0/0 0/0/0 0/0/0 0/0/0PD 3/4/5* 3/2/2 1.5/4.5/2.5 3/2/4.5 3/2/4.5 5/1/3 3/2/3 3/2/2 3/3/3 4/2/3

BuccalTooth 2 3 4 5 6 7 8 9 10 11 12 13 14 15

Lingual

26 monthsPD 4/4/6 3/2/3 3/3/4 3/2/2 2/2/5 2/2/2 2.5/2.5/2 4/2/3.5 4/2/2 5/5/4CAL-V 0/3/4 0/0/0 0/0/0 0/0/0 0/0/1 0/0/0 0/0/0 0/0/0.5 0/0/0 3/4/3Furcation 0 0 0 0

60 monthsPD 5/5/5 5/5/5 3/3/2 3/2/3 4/3/3 3/2/3 3/2/5 6/5/4 4/3/3 4/4/4 4/3/4 4/3/3 7/5/6 5/4/5CAL-V 0/0/0 5/6/5 1/0/1 0/0/0 0/0/0 0/0/1 0/0/3 3/3/1 1/1/1 0/0/0 1/0/1 1/0/1 6/6/5 0/0/0Furcation 0 0 I I I I 0 0

79 monthsPD 3/3/4 4/4/5 4/3/2 2/2/3 3/2/2 2/2/2 2/2/3 2/2/2 2/2/2 2/2/3 3/2/2 3/2/2 6/6/4 4/3/3CAL-V 1/2/3 4/4/5 3/2/1 1/1/1 1/1/1 1/1/1 1/1/1 1/1/1 1/2/1 1/1/1 2/1/1 1/1/1 6/7/4 2/1/1Furcation 0 0 I I I I 0 0

* Underline: sites that bled on probing.

At t1, RNA probes failed to detect A. actinomyce-temcomitans, P. gingivalis, or T. forsythensis at anysite. Only three sites were found to harbor T. denticola(Table 4). At t2, subgingival recolonization was evi-denced by detection of at least one of the periodontalpathogens at each sampled site. However, A. actino-mycetemcomitans was detected by RNA probes onlyat three sites (Table 4). TSBV culture failed to detectA. actinomycetemcomitans at any of the sampled sitesboth at t1 and t2. At t4, none of the methods usedcould detect A. actinomycetemcomitans in the pooledsamples from the deepest pockets of each quadrant ororal mucosa. The RNA probe test detected only T. den-ticola (7 × 104); the DNA probe detected P. gingivalis (1× 103), Prevotella intermedia (3 × 103), T. forsythensis (2× 103), and T. denticola (1 × 103) in the pooledsamples.

Orthodontic TreatmentFigure 8 shows the results of the orthodontic treatmentat the age of 14 years 10 months (close to t4) after a

retention period of 17 months. In the upper jaw, thecanines were aligned almost correctly, and in the lowerjaw, the crowding was resolved by mesialization of thecanines. The panoramic radiograph (Fig. 9) showsmoderate horizontal bone loss in both jaws, and ver-tical bone loss at all first molars and incisors. In addi-tion to endodontic treatment of the upper left firstmolar, restorative treatment of several teeth was nec-essary.

DISCUSSIONThe objective of this case report was to show an ortho-dontic treatment in a case of PLS after a successfulcombined mechanical and antibiotic periodontal treat-ment of PLS periodontitis.19 Papillon-Lefèvre syndromeis an inherited, rare autosomal recessive disorder witha prevalence of 1 to 4 per million.19 PLS patients showpalmo-plantar hyperkeratosis in combination withsevere periodontitis which affects both primary andpermanent dentitions.1,2 Several different mutations inthe CTSC gene that encodes for cathepsin C have been

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Table 3.

Mandibular Probing Parameters

79 monthsFurcation 0 0 0 0CAL-V 2/1/2 6/2/4 1/2/1 1/1/1 1/1/1 4/5/5 6/5/5 2/0/1 1/1/1 2/2/1 4/6/7 4/1/1PD 4/3*/3 5/2/5 3/2/2 3/2/2 2/2/2 2/1/2 3/1/2 2/1/2 2/2/2 4/3/2 4/4/4 5/2/2

60 monthsFurcation 0 0 0 0CAL-V 0/0/0 8/4/3 0/0/1 0/0/0 0/0/0 6/4/5 7/4/5 0/0/0 0/0/0 0/0/1 5/6/7 2/0/0PD 4/4/4 6/3/6 3/2/4 3/3/3 2/2/2 4/1/4 6/1/5 2/2/3 4/3/3 4/2/4 7/5/5 4/3/4

26 monthsFurcation 0 0CAL-V 2/0/1 0/0/0 4.5/5/5.5 6.5/6/6.5 8/7/7.5 5.5/2.5/5 0/0/0 2/0/3PD 4/2/3 3/2/2 3/1/2 2/1/2 2/1.5/3 3/1/4 3/2/3 5/2/5

LingualTooth 31 30 29 28 27 26 25 24 23 22 21 20 19 18

Buccal

26 monthsPD 4/4/3 3/3/2.5 2/1/2.5 2/1/2 2/1.5/3 2/2/3 2/2.5/3 5/2/2CAL-V 3/4/0 0/0/0 5/5/5 5.5/6/5.5 5.5/5/5 4.5/4.5/4.5 0/0/0 3/0/0Furcation 0 0

60 monthsPD 3/3/5 5/4/8 3/3/2 2/2/3 3/2/2 6/1/3 4/1/5 2/2/3 3/1/3 3/2/3 4/3/5 5/5/4CAL-V 0/0/0 5/4/6 0/1/0 0/0/0 0/0/0 8/4/5 6/5/7 0/0/0 1/0/0 1/0/1 3/3/4 0/0/0Furcation 0 0 0 0

79 monthsPD 3/3/3 4/3/5 3/2/2 2/1/2 3/2/1 5/1/3 2/1/5 2/1/3 4/2/3 3/2/3 4/2/5 3/2/3CAL-V 1/1/3 4/3/4 2/1/1 1/1/1 2/1/0 6/3/4 4/3/5 1/1/1 2/1/1 1/1/2 3/2/5 2/1/2Furcation 0 0 0 0

* Underline: sites that bled on probing.

found,14,15,18 a fact which can also explain the periodon-tal condition.12,13,17 Cathepsin C is the physiologicalactivator of groups of serine proteases from immuneand inflammatory cells vital for defense of an organ-ism.16 In some PLS cases, neutrophil abnormalities ordysfunctions leading to recurrent infections or com-plications such as pyogenic liver abscesses have beendescribed.3,10,25 In the subgingival plaque, Gram-neg-ative anaerobic bacteria and A. actinomycetemcomi-tans could be detected.6,8,19 However, no particularperiodontal pathogen is invariably associated withPLS.11

The therapy of the dermal affections is often per-formed with oral retinoids. With respect to periodonti-tis, several therapy options with variable success rateshave been described. Affected patients often lose nearlyall of their teeth as teenagers, hence extraction of allerupted teeth followed by an edentulous period wasproposed to minimize the subsequent infection of theunerupted teeth.4,5,9 Nevertheless, this is a rather rad-ical treatment with loss of a number of teeth. Although

systemic administration of various antibiotics in com-bination with debridement often failed,4,6 some patientswere successfully treated without tooth extractions. Inthose cases, A. actinomycetemcomitans could not bedetected or was eliminated by therapy.6,7,26, Hence, A.actinomycetemcomitans seems to play an importantrole in PLS periodontitis.11 In aggressive periodontitis,A. actinomycetemcomitans can be reliably suppressedbelow detection levels by a combination of systemicamoxicillin and metronidazole in addition to mechani-cal debridement.26 In the present patient, from baselineto 26 months after anti-infective therapy, the perio-dontal conditions improved significantly.19 Sixty monthsafter start, however, there was a mean increase in PD,and 79 months after the start a mean attachment losswas found when compared to 26 months. The overallresults may be regarded as successful, in particularwhen the baseline conditions are considered19 and withrespect to the quite unstable individual oral hygiene ofthe patient. Sixty months after anti-infective therapy, A.actinomycetemcomitans could be detected at three

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sites. However, the number of bacteria detected at thedifferent sites did not exceed the threshold for increasedrisk of further attachment loss.27 Another antibiotictreatment was therefore not considered. Eighty-twomonths after the beginning and 16 months after theend of active orthodontic treatment, A. actino-mycetemcomitans could no longer be detected.Mechanical instrumentation and instillation may haveagain suppressed A. actinomycetemcomitans below thedetection level. A combined mechanical and antibioticretreatment may become necessary in the future forthis patient to prevent further periodontal destruction.If the patient continues to comply with periodontalmaintenence treatment, as he does at the moment(once every 6 weeks to 2 months), fairly stable perio-dontal conditions may be maintained. However, itshould be noted that the patient’s mother is a drivingforce behind the child’s compliance. Periodontal stabilityis likely to suffer when the patient grows out of hismother’s influence and possibly decides to drop outmaintenance therapy. His unstable individual oralhygiene might point to this direction. However, whatteeth are primarily at risk if the patient fails to continuemaintenance therapy? Residual pockets (≥5 mm) andmost pronounced attachment loss are found at the firstmolars and second mandibular incisors. Thus, perio-

dontal deterioration may be expected there first. If, asa consequence of periodontal destruction these teethare lost, the patient will be old enough for bridgeworkin the mandibular anterior region and will be able tohave his first molars replaced. Alternatively, the firstmolars may not be replaced if esthetics and functionare not impaired. Even if progression of periodontaldisease occurs in the future, periodontal treatment sofar has saved periodontal attachment at most teeth forthe patient up to 15 years of age. Possibly, as the patientmatures, he will gain insight into the significance ofmaintenance therapy while he still has enough teeth tomaintain oral function. It will be of outmost importancefor the patient to receive frequent maintenance therapy.

Information on orthodontic treatment in patients withaggressive periodontitis is still limited. Few authorshave described orthodontic movements of periodon-tally affected teeth in children28,29 and others reportpreprosthetic space management after extractions dueto periodontal reasons.30 So far, no information is avail-able concerning orthodontic tooth movements in PLS.In our patient, a combination of periodontal treatment(author PE), orthodontic therapy (author CJL), andrestorative treatment (author BK) resulted in well-aligned arches with an acceptable occlusion and withno or only minimal bone loss. The panoramic radio-

Table 4.

Microbiological Parameters (RNA probes)

60 monthsAa n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. 1 × 103 n.d. n.d.Pg 4 × 104 1.4 × 105 n.d. 5 × 104 1 × 104 n.d. 5 × 104 3 × 104 7 × 104 1.4 × 105 n.d. n.d. 2 × 104 n.d.Tf 4 × 104 n.d. 4 × 104 2 × 104 n.d. n.d. 1 × 104 n.d. n.d. 2 × 104 2 × 104 n.d. n.d. 3 × 104

Td 3 × 104 3 × 104 n.d. n.d. 4 × 104 3 × 104 1 × 104 2 × 104 2 × 104 9 × 104 5 × 104 6 × 104 4 × 104 n.d.

26 monthsAa n.d.* n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Pg n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Tf n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Td n.d. 2 × 104 n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.

Maxilla Tooth 2 3 4 5 6 7 8 9 10 11 12 13 14 15

Mandible Tooth 31 30 29 28 27 26 25 24 23 22 21 20 19 18

26 monthsAa n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Pg n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Tf n.d. n.d. n.d. n.d. n.d. n.d. n.d. n.d.Td n.d. n.d. n.d. 2 × 104 n.d. 1 × 104 n.d. n.d.

60 monthsAa n.d. n.d. n.d. 4 × 103 n.d. n.d. 3.7 × 104 n.d. n.d. n.d. n.d. n.d.Pg n.d. n.d. 1 × 104 8 × 104 n.d. n.d. 5 × 104 3 × 104 1 × 104 1 × 104 2 × 104 7 × 104

Tf 4 × 104 1 × 105 1 × 104 n.d. 3 × 104 6 × 104 n.d. n.d. 1 × 104 2 × 104 1 × 104 8 × 104

Td 3 × 104 3 × 104 2.8 × 105 1.1 × 105 n.d. 3 × 104 6 × 104 5 × 104 5 × 104 1.1 × 105 2 × 104 1 × 104

* nd = not detectable.

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graph at the age of 14 years 10 months shows animproved periodontal situation for the mandibular lat-eral incisors.

The patient had lost only two permanent teeth by age15; nevertheless, long-term prognosis of the lower lat-eral incisors, as well as the first molars in both jaws, stillremains critical. In general, the patient showed poor com-

pliance during the entire treatment,and in spite of frequent periodontalmaintenance treatment appoint-ments, several restorative measureswere necessary due to unstable oralhygiene. The patient also declineda proper vertical orthodontic align-ment of the lower second molarsdue to the required treatment time.Finally, it should be mentioned thatin the upper jaw the extraction ofthe first premolars probably wouldresult in the best long-term stabilityof the orthodontic tooth alignment.However, in the present case, erup-tion and alignment of the caninesand premolars was regarded as amajor orthodontic treatment goalfor the upper jaw, and extraction ofpremolars was declined with regardto the limited long-term prognosisof the incisors and molars. In addi-tion, with respect to the poor oralhygiene, only removable appliances(Hawley retainers) instead of fixedretainers were used for retention.

Only moderate orthodontic toothmovements of up to 3 mm werecarried out, and this limitationmust be considered. In addition, itwas decided not to fully uprightthe roots of the lower lateralincisors due to their pronouncedbone loss. Hence, this case report

still leaves open whether pronounced orthodontic rootmovements are conformable with PLS, and furtherinvestigations are necessary for clarification. Neverthe-less, the present case report shows that moderateorthodontic tooth movements may be possible inPapillon-Lefèvre-Syndrome within a complex interdis-ciplinary treatment regimen.

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Figure 9.Orthopantomogram at 14 years 10 months.

Figure 8.Intraoral views at 14 years 10 months, after a retention period of 17 months (83 months afterbeginning of periodontal treatment).

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Correspondence: Priv.-Doz. Dr. Christopher J. Lux, Departmentof Orthodontics, University of Heidelberg, Im NeuenheimerFeld 400, 69120 Heidelberg, Germany. Fax: 49-6221-565753;e-mail: christopher_lux@med.uni-heidelberg.de.

Accepted for publication July 23, 2004.

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