Use of Lateral Cephalometric Analysis in Diagnosing Craniofacial Features in Papillon-Lefevre Syndrome

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1 Cse Report Print ISSN: Online ISSN: X DOI: /ijss/2017/508 Use of Lterl Cephlometric Anlysis in Dignosing Crniofcil Fetures in Ppillon-Lefevre Syndrome Ahishek Soni Senior Lecturer, Deprtment of Orl Medicine & Rdiology, Modern Dentl College nd Reserch Center, Indore, Mdhy Prdesh, Indi Astrct Knowledge of fcil structure is importnt s n orl physicin s our gol is to chieve idel fcil profile with esthetic hrmony. Therefore, the understnding of the hrd nd soft tissue structures is necessry in plnning proper mngement of the ptients. This clinicl cse report of Ppillon-Lefevre syndrome is n ttempt to illustrte the dvntge of specific cephlometric findings for ssessing the hrd nd soft tissue vriles in this group of ptients tht could e significnt for dignosis nd proper tretment plnning in estlishing the esthetic nd functionl orl rehilittion of ptients ffected with this syndrome. Key words: Bone, Cephlometric nlysis, Cephlometry, Crniofcil, Dentl, Ppillon-Lefevre disese, Rdiogrphy INTRODUCTION Ppillon-Lefevre syndrome (PLS) is n utosoml recessive disorder chrcterized y plmoplntr hyperkertosis nd rpidly progressive periodontitis, leding to premture loss of oth deciduous nd permnent teeth. 1,2 Literture often covers the etiologicl spect, clinicl mnifesttions, nd mngement options in these ptients, ut little ws found descriing specific crniofcil findings nd their role in the mngement of such ptients. This rticle reports clinicl cse of PLS lying emphsis on the use of specific cephlometric findings, oth for skeletl nd soft tissue vriles, in dignosing the significnt crniofcil fetures nd to justify their role in the evidence-sed mngement of the ptient with this syndrome. CASE REPORT A 20-yer-old mle ptient hd reported to the outptient deprtment, complining of esthetic prolems nd Access this rticle online Month of Sumission : Month of Peer Review : Month of Acceptnce : Month of Pulishing : difficulty in eting due to multiple missing teeth since childhood. Clinicl history reveled tht ptient hd n erly loss of primry teeth followed y sequentil loss of permnent teeth due to excessive moility y the ge of yers. The ptient lso gve history of thickening nd scling of the skin of plms nd soles since childhood, which ggrvtes during the monsoon seson. Pst medicl history ws noncontriutory. Prents were not of consnguineous mrrige, nd other fmily memers including silings were pprently norml. Extrorl exmintion reveled tht ptient hd n verge fcil height with competent lips. Lower lip ppers to e everted with deep mentolil sulcus. Upper lip ppers to e retruded. Cutneous mnifesttion showed well-demrcted, thickened, dry, nd scly kertotic plques on the dorsl surfce of plms, which undergo crusttions, crcking, nd deep fissuring. Similr kertotic plques hd lso een seen on the feet nd the nkle. Oculr exmintion reveled no normlity. Introrl exmintion reveled prtilly edentulous mxillry nd mndiulr rches with interrch distnce of 1.2 cm. The gingiv round the teeth ws inflmed nd swollen while the orl mucos covering the edentulous re ppered norml (Figure 1). Bsed on history nd clinicl exmintion, provisionl dignosis of PLS ws mde. A pnormic rdiogrph ws otined which reveled generlized loss of lveolr one nd vrile loss of Corresponding Author: Ahishek Soni, 263-Blji Vill, Shivom Estte, Sttion Rod, Dews , Mdhy Prdesh, Indi. Phone: / Tel.: E-mil: drhishek_soni@rediffmil.com 121 Interntionl Journl of Scientific Study Octoer 2017 Vol 5 Issue 7

2 Figure 1: A photogrph of the ptient dignosed with Ppillon-Lefevre syndrome: () Introrl picture showing prtilly edentulous mxillry nd mndiulr rches with decresed interincisl distnce; () cutneous fetures showing hyperkertotic plques on the plms nd feet one support round ll the present teeth. To ssess the crniofcil fetures in detil, lterl cephlometric nlysis ws dvised which showed reduced lower fcil height with low Frnkfort mndiulr plne ngle (FMA = 10 ). SNA ngle nd ANB ngle ppers to e reduced suggestive of the retrognthic mxill, leding to skeletl Clss III profile. NAPog (ngle of convexity) mesurement showed skeletl concvity reconfirming the Clss III skeletl reltionship. There ws compenstory increse in the soft tissue thickness noted. Altertion in the soft tissues ws evident for upper lip position; it ws more retrusive, nd nsolil ngle (NLA) ws found to e more cute (Figure 2). The lterl skull rdiogrph showed no evidence of intrcrnil clcifiction. Routine lortory investigtions were within norml limits. A microiologic exmintion y polymerse chin rection nlysis ws norml. Initilly, the ptient ws kept on moxicillin (500 mg TDS) nd metronidzole (400 mg TDS) for 3 weeks nd ws dvised chlorhexidine mouthwsh (0.2%) 2-3 times dily. This ws followed y orl prophylxis nd complete orl rehilittion. Complete orl rehilittion ws plnned with implnt-supported prosthesis. For implnt plcement nlysis, cone-em computed tomogrphy ws dvised which reveled generlized one loss in the mxill nd vrile mount of one loss in mndile with knifeedge lveolr ridge in the nterior region (Figure 3). Due to indequte one height in mxill nd mndile for implnt plcement nd unwilling of ptient to undergo for zygomtic implnt tretment, prosthetic rehilittion ws plnned y giving telescopic crown ttched with the denture se (Figure 4). Consulttion of dermtologist ws tken for the evlution of cutneous mnifesttions. On follow-up evlution, fter every 6 months for 3 yers, the plnned orl rehilittion tretment ws found to e c successful, nd the ptient fcil profile ws in good esthetic nd functionl hrmony (Figures 5 nd 6). DISCUSSION Figure 2: () Pre-tretment fcil view showing verge fcil height; () pre-tretment pnormic rdiogrph showing severe loss of lveolr one; (c) pre-tretment lterl profile view showing retruded upper lip, everted lower lip, nd deep mentolil sulcus; (d) pre-tretment lterl cephlogrms showing Clss III skeletl reltionship, reduced vlue for upper nterior fce height/lower nterior fce height, reduced Frnkfort mndiulr plne ngle, SNA nd ANB ngle, skeletl concvity s evident y NAPog mesurement, reduced nsolil ngle, nd deep mentolil sulcus Erly dignosis nd mngement of PLS re quite chllenging to the clinicins. The mjor determinnts for the successful rehilittion of the PLS ptients re n erly institution of well-plnned tretment nd complince with prevention progrm. A multidisciplinry pproch in mnging such ptient cn improve the prognosis nd qulity of life of the ffected individuls. d Interntionl Journl of Scientific Study Octoer 2017 Vol 5 Issue 7 122

3 c d Figure 3 () Cone-em computed tomogrphy imge showing generlized one loss in mxill; () vrile mount of one loss is evident in nd round the teeth in mndile; (c) 3D reconstruction imge of mndile showing knife-edge lveolr ridge in the nterior region; (d) 3D volumetric reconstruction imge of mxill nd mndile c d e f g Figure 4: Photogrph of the Ppillon-Lefevre syndrome ptient showing step-y-step complete orl rehilittion procedure: () Preprtion of teeth to receive metl copings; () primry metl copings; (c) order molding ws done with low fusing impression compound in the mxillry rch nd with ruer-sed putty mteril in the mndiulr rch nd finl impression ws mde with the elstomeric impression mteril; (d) mxillomndiulr record ws tken using fce-ow; (e) trnsfer of the fce-ow record on the rticultor; (f) finl prosthesis with the secondry copings plced in the denture; (g) introrl post-opertive view fter denture plcement with the right nd left posterior side occlusion Erly loss of the mxillry deciduous dentition is common in ptients with PLS. 3 Premture loss of either the deciduous or the permnent teeth will cuse loss of lveolr one in oth the verticl nd horizontl dimensions. 4 Bindyel et l., 4 in their study nlyzed lterl cephlogrms of eight PLS ptients for oth hrd- nd soft-tissue vriles nd reveled significntly ltered vlues for FMA, ANB, SNA, NAPog (ngle of convexity), nd nsolil (NLA) ngle. They suggested tht mny ptients with PLS develop Clss III reltionship. This is in greement with the study y Al-Khenizn, 5 which lso reported tht ptients with PLS hve the chrcteristics of skeletl Clss III mlocclusion. In the present cse lso, the ptient hs Clss III skeletl profile. Clss III skeletl reltionship in PLS ptient is minly ttriuted to retrognthic nd hypoplstic mxill rther thn prognthic mndile. This hs een reveled y the mesurement of SNA nd ANB ngle. 4 SNA ngle is the ngle formed y the intersection of S.N. Plne nd line joining nsion nd point A, which indictes the reltive nteroposterior positioning of the mxill in reltion to the crnil se. A lrger thn norml vlue indictes tht the mxill is prognthic (Clss II) while the smller vlue is suggestive of the retrognthic mxill (Clss III). ANB ngle is formed y the intersection of lines joining nsion to point A nd nsion to point B, which denotes the reltive position of the mxill nd mndile to ech other. An increse in this ngle is indictive of Clss II skeletl tendency while n ngle tht is less thn norml or negtive ngle is suggestive of skeletl Clss III reltionship. 6 In our cse, SNA nd ANB ngle is found to e reduced, suggestive of Clss III skeletl reltionship. PLS ptients lso showed decresed lower fcil height, minly ecuse of posterior (clockwise) inclintion of the mxill. This is evident y the rtio of upper nterior fce height (UAFH) to the lower nterior fce height (LAFH). 4 UAFH is the liner mesurement from nsion to nterior nsl spine, while LAFH is the liner mesurement from nterior nsl spine to menton. 6 The rtio of UAFH to LAFH is more significnt thn the individul mesurement of UAFH nd LAFH ecuse UAFH vries with the superior-inferior dimension of the size of n dult skull while the rtio of UAFH/LAFH indictes the lnce of fcil proportions. UAFH/LAFH rtio <0.8 indictes 123 Interntionl Journl of Scientific Study Octoer 2017 Vol 5 Issue 7

4 profile. It is formed y the intersection of line from nsion to point A nd line from point A to pogonion (NAPog). A positive ngle or n incresed ngle suggests prominent mxillry denture se reltive to the mndile. A decresed ngle of convexity or negtive ngle is indictive of prognthic profile. 7 In our cse, NAPog mesurement showed skeletl concvity reconfirming the Clss III skeletl reltionship. Due to this, the mndiulr incisors, if present, tend to e retroclined s dentl compenstion for mxillry retrognthism. 4 Figure 5: () Post-tretment fcil view; () post-tretment pnormic rdiogrph; (c) post-tretment lterl profile view; (d) post-tretment lterl cephlogrms. All showing improvement in the fcil esthetics nd functionl rehilittion of ptient c Figure 6: Trcing of relevnt cephlometric lndmrks: () Pre-tretment cephlometric trcing; () post-tretment cephlometric trcing. Lndmrks depicted on cephlometric trcing N: Nsion; Point A; Point B; Pog: Pogonion; Me: Menton; NLA: Nsolil ngle; SNA ngle: Intersection of S-N plne nd line joining nsion nd point A; ANB ngle: Intersection of lines joining nsion to point A nd nsion to point B; upper nterior fce height (UAFH) - liner mesurement from nsion to nterior nsl spine (N-ANS); lower nterior fce height (LAFH) - liner mesurement from nterior nsl spine to menton (ANS-Me); ngle of convexity (NAPog) - intersection of line from nsion to point A nd line from point A to pogonion; nsolil ngle (NLA) - formed etween the lower order of the nose nd line connecting the intersection of nose nd upper lip with the tip of the lip (lrle superius) greter LAFH, or longer LAFH, while UAFH/LAFH rtio >0.8 indictes smller LAFH or shorter LAFH. Due to decresed lower fcil height, the FMA ngle is lso reduced. Another prmeter to e ssessed is the ngle of convexity, which revels the convexity or concvity of the skeletl d Soft tissue evlution should e tken into considertion in such ptients during tretment plnning. 8,9 A frequently used soft tissue prmeter is the NLA. 10 The NLA is representtive of soft tissue profile nd remins n excellent clinicl nd cephlometric prmeter to revel the nteroposterior position of the mxill nd skeletl mlocclusions. 11 NLA is the ngle formed etween the lower order of the nose nd line connecting the intersection of the nose nd upper lip with the tip of the lip (lrle superius). Incresed NLA reflects mxillry retrusion or retroclined mxillry nterior nd decresed NLA reflects prognthic mxill or proclined upper nteriors. 12 It hs een suggested tht reltively smll NLA dds to the Clss III chrcteristics of ffected ptients. 4 Another soft tissue prmeter is the evlution of mentolil sulcus, which represents the concvity elow the lower lip. 12 In our cse, NLA ws found to e more cute indicting mxillry retrusion with deep mentolil sulcus. Erly dignosis nd well-plnned tretment protocol of Clss III mlocclusion is recommended for PLS ptients to chieve norml mxillry growth, to prevent trumtic occlusion of the nterior teeth, nd to improve the ptient s fcil profile. 13 Orthodontic correction is documented pproch in the literture for erly correction of mild skeletl Clss III discrepncy. 14,15 Moreover, this typiclly requires stle nd helthy dentl nd periodontl tissues. For those with PLS, rpid periodontl rekdown could result in loss of some of the dentition. However, the literture shows tht tht orthodontic tretment comined with n ntiiotic regimen cn successfully control the periodontl signs of PLS nd result in the mintennce of helthy dentition In our cse lso, the ptient ws initilly kept on prophylctic ntiiotics followed y complete orl prophylxis. Furthermore, implnt therpy hs proved to e successful in these ptients. 20 Dentl implnts offered etter stility nd retention of prosthesis, improved comfort nd mstictory efficiency, nd lso the improved esthetics. According to Dhnrjni, 1 the use of implnts in ptients with severe periodontitis hs een reported, nd the results indicte tht periodontlly compromised ptients cn e Interntionl Journl of Scientific Study Octoer 2017 Vol 5 Issue 7 124

5 successfully treted with implnts. However, in the present cse, ecuse of the severity of the skeletl discrepncies nd the unvilility of dequte one height nd lso, the strict regimen required to mintin helthy dentition, orthognthic surgery followed y zygomtic implnt might e tretment lterntive for this ptient. However, s ptient ws not willing for such surgicl intervention, prosthetic orl rehilittion with telescopic crown ttched with the denture se ws plnned. Continuous monitoring nd frequent recll ppointments hve shown to minimize the further periodontl deteriortion. CONCLUSION In summry, it ppers resonle to conclude tht stepwise mngement protocol should e followed in ptient with PLS to prevent further one loss nd to mintin the structurl integrity of orofcil musculture. Cephlometric nlysis is proven to e vlid dignostic option with significnt clinicl enefits in tretment plnning of ptient with PLS; this not only improves the esthetics ut lso help in functionl orl rehilittion of the ptient. We hope tht this clinicl cse study my serve s guide for further future cse studies with lrger representtive smple to confirm our findings nd to justify n evidence-sed mngement protocol. REFERENCES 1. Dhnrjni PJ. Ppillon-Lefevre syndrome: Clinicl presenttion nd rief review. Orl Surg Orl Med Orl Pthol Orl Rdiol Endod 2009;108:e Shh J, Goel S. Ppillon-Lefevre syndrome: Two cse reports. Indin J Dent Res 2007;18: Hrt TC, Shpir L. Ppillon-lefèvre syndrome. Periodontol 2000;6: Bindyel NA, Ullro C, Suri L, Al-Frr E. Cephlometric findings in ptients with Ppillon-Lefèvre syndrome. Am J Orthod Dentofcil Orthop 2008;134: Al-Khenizn S. Ppillon-lefévre syndrome: The response to citretin. Int J Dermtol 2002;41: Rossi M, Stuni MB, Silv LA. Cephlometric evlution of verticl nd nteroposterior chnges ssocited with the use of onded rpid mxillry expnsion pplince. Dent Press J Orthod 2010;15: Bhljhi SI. Cephlometrics. In: Bhljhi SI, editor. Orthodontics-The Art nd Science. 4 th ed. New Delhi: Ary Pulishers; p Tweed CH. Indictions for the extrction of teeth in orthodontic procedure. Am J Orthod Orl Surg 1944;42: Umle VV, Singh K, Azm A, Bhrdwj M, Kulshresth R. Evlution of nsl proportions in dults with clss I nd clss II skeletl ptterns: A cephlometric study. J Orthod Sci 2017;6: Holdwy RA. A soft-tissue cephlometric nlysis nd its use in orthodontic tretment plnning. Prt I. Am J Orthod 1983;84: Elis AC. The importnce of the nsolil ngle in the dignosis nd tretment of mlocclusions. Int J Orthod 1980;18: Bhljhi SI. Orthodontic dignosis. In: Bhljhi SI, editor. Orthodontics: The Art nd Science. 4 th ed. New Delhi: Ary Pulishers; p AlSrheed MA, Al-Sehiny FS. Comined orthodontic nd periodontic tretment in child with ppillon lefèvre syndrome. Sudi Med J 2015;36: Ngn PW, Hgg U, Yiu C, Wei SH. Tretment response nd long-term dent fcil dpttions to mxillry expnsion nd protrction. Semin Orthod 1997;3: Frnchi L, Bccetti T, McNmr JA. Postpuertl ssessment of tretment timing for mxillry expnsion nd protrction therpy followed y fixed pplinces. Am J Orthod Dentofcil Orthop 2004;126: Wiee CB, Häkkinen L, Putnins EE, Wlsh P, Lrjv HS. Successful periodontl mintennce of cse with ppillon-lefèvre syndrome: 12-yer follow-up nd review of the literture. J Periodontol 2001;72: Pcheco JJ, Coelho C, Slzr F, Contrers A, Slots J, Velzco CH. Tretment of ppillon-lefèvre syndrome periodontitis. J Clin Periodontol 2002;29: Toygr HU, Kircelli C, Firt E, Guzeldemir E. Comined therpy in ptient with ppillon-lefèvre syndrome: A 13-yer follow-up. J Periodontol 2007;78: Lux CJ, Kugel B, Komposch G, Pohl S, Eickholz P. Orthodontic tretment in ptient with ppillon-lefèvre syndrome. J Periodontol 2005;76: Ullro C, Crossner CG, Lundgren T, Stålld PA, Renvert S. Osseointegrted implnts in ptient with ppillon-lefèvre syndrome. A 4 1/2-yer follow up. J Clin Periodontol 2000;27: How to cite this rticle: Soni A. Use of Lterl Cephlometric Anlysis in Dignosing Crniofcil Fetures in Ppillon-Lefevre Syndrome. Int J Sci Stud 2017;5(7): Source of Support: Nil, Conflict of Interest: None declred. 125 Interntionl Journl of Scientific Study Octoer 2017 Vol 5 Issue 7