Parry-Romberg 대한구순구개열학회지 Syndrome 환자에서악정형 15(1):1~10, 및교정 2012 치료 Parry-Romberg Syndrome 환자에서악정형및교정치료 유국호, 백형선 * 연세대학교치과대학병원치과교정학교실 ABSTRACT Orthopedic and Orthodontic Treatments of a Patient with Parry-Romberg Syndrome Kug-Ho You, Hyoung-Seon Baik* Department of Orthodontics, School of Dentistry, Yonsei University Parry-Romberg syndrome(prs) is a degenerative disease characterized by progressive hemifacial atrophy. A 10-year-old girl who had been treated for linear scleroderma at the dermatologic department visited the orthodontic department. The frontal facial photograph showed mild facial asymmetry. On the left side, mild atrophy of soft tissue, enophthalmos, cheek depression, and dry skin with dark pigmentation were observed. The radiograph showed the hypoplasia of both the maxilla and mandible on the left side. This case report describes the treatment of a patient with PRS for 7 years. To minimize the effect of progressive atrophy on the facial growth, a hybrid appliance was used. The facial photos and radiographic records were periodically taken to analyze the progression of PRS. Although it is impossible to prevent the progression of facial asymmetry, it appears to be possible to limit the atrophic effect. After the stabilization of PRS, the orthodontic treatment by the fixed appliance was performed. Additionally, autologous fat graft was performed three times at 6 month intervals. After the treatment, the patient had a confident smile and facial asymmetry was improved. Key words : Parry-Romberg syndrome, progressive hemifacial atrophy Ⅰ. 서론 Parry-Romberg syndrome(prs) 은 Parry 1) 와 Romberg 2) 에의해보고된드문퇴축성질환이다 3). 일반적으로 20세이전에발병되며, 남자보다여자에서발병률이높다 4). PRS 의원인은아직확실히밝혀지지는않았지만, 유전, 외상, 바이러스감염, 지방대사에서의중추적장애, 말초삼차신경염, 림프구성신경혈관염, 내분비장애, 자가면역력등이주요원인들로주장되어왔다 5,6). PRS 는여러임상적증후들을보인다. PRS 의가장특징적인임상적증후는이환측의반안면위축을동반한안면비대칭이다. 피부는점점얇아지고건조해지며, 피부에는색소들이침착되기도한다 7). - 1 -
유국호, 백형선 정상적인피부와비정상적인피부사이에경계선 (en coup de sabre) 이나타날수도있다 4). 안구함몰 (enophthalmos) 도관찰되며 6), 눈썹과머리카락이소실되거나탈색이발생할수도있다 8). 또한, 면역학적이상 9) 과삼차신경통, 간질, 편두통같은신경학적이상 10) 이나타나기도한다. 임상적으로골격과치아들의위치에중요한형태적변화들을일으키기위해서기능성장치 (functional appliance) 들이이용되어왔으며, PRS 를가지고있는두환자에서도, 기능성장치가하악골의성장을촉진시켜골격적기형을개선시킬수있음이보고되었다 11). 기능성장치중, hybrid appliance 는구성요소들에기반을둔개별적으로제작된장치로, 안면비대칭환자들에사용되어질 수있다 12). 이에, PRS 환자에서 hybrid appliance 의악정형효과와교정적인고려사항들에대하여보고하고자한다. Ⅱ. 증례보고 9세부터본원피부과에서선형경피증 (linear scleroderma) 으로치료를받아왔던 10세여환이안면비대칭과치열총생을주소로내원하였다. 구외임상검사상, 좌측안면부위에경미한연조직의위축, 볼부위의함몰, 안구함몰이있었으며, 검은색소침착을동반한건조한피부가관찰되었다. 상악정중선이좌측으로편위되었고, 교합평 Figure 1. The facial photograph showing mild facial asymmetry at the age of 10. Figure 2. The intraoral photograph showing the dental crowding at the age of 10. - 2 -
Parry-Romberg Syndrome 환자에서악정형및교정치료 Figure 3. The panoramic view at the age of 10. Figure 5. 3-dimensional computed tomography at the age of 13. Figure 4. The hybrid appliance which was constituded of buccal shields on the left side and posterior bite plane on the right side. 면의기울기가관찰되었다 (Figure 1). 악관절에특이한이상소견은없었으며, 안면근육의기능도정상적이었다. 구내임상검사상, 혼합치열기상태였으며, 치열의총생이관찰되었고, 영구치가맹출할공간이부족하였다 (Figure2). 정모두부방사선사진상, 좌측상악골과하악골의열성장이관찰되었으며, 측모두부방사선사진상, ANB 5.1 로경미 Table 1. Data showing the inclinations of maxillary, occlusal, and mandibular planes related to Z-line and the distances of J and Ag from midsagittal reference line(msr) according to a frontal asymmetry analysis 13) The length of treatment Z^J Z^U6 Z^Ag MSR-J diff. MSR-Ag diff. Initial 0.9 1.5 3.0 0.5 0.5 21 months 0.9 1.5 2.5 1.9 2.5 49 months 1.5 2.0 3.4 2.4 3.0 Z, zygomatico-frontal suture point; J, the intersecting point between the zygomatic process and maxillary tuberosity; U6, the palatal cusp tip of the maxillary first molar; Ag, the superior point of antegonial notch; Diff., the difference between the left and right sides(right left). - 3 -
유국호, 백형선 Table 2. Data showing the calculated symmetry of ramus and condylar heights expressed in percentages according to the formula of Habets et al. 14) : [(right-left)/(right+left)] x 100 The length of treatment Condylar plus ramus height Ramus height Condylar height Initial 10.1 9.0 17.7 33 months 8.6 8.0 11.1 49 months 9.0 8.3 12.8 한골격성 II급부정교합을보였다. 파노라마방사선사진상, 좌측악관절의저성장이관찰되었으며, 치아의상실은없었으나, 좌측상악중절치와측절치의치근이짧았으며, 우측소구치들에비하여좌측하악소구치들의치근발육이미약하였다 (Figure 3). 먼저영구치열의맹출공간을확보하기위하여, 상악에는 straight-pull head gear 를, 하악에는 lip bumper 를 7개월동안사용하였다. 그후, 좌측안면부위의성장을도모하기위해서, 구성교합 (construction bite) 을채득한뒤, 우측에 bite block, 좌측에 buccal shield 를장착한 hybrid appliance 를제작하였다 (Figure 4). 4주동안의적 응기간을가진후, 악정형효과를얻기위하여하루에적어도 14시간은장착하도록하였다. Hybrid appliance 를 21개월동안장착한후, 안면비대칭을정확히평가하기위해 3차원컴퓨터단층촬영을시행하였으며 (Figure 5), 정모두부방사선사진상에서좌우비대칭을비교해본결과 13), 하악골에서수직적으로비대칭의개선이있었다 (Table 1). Hybrid appliance 를 33개월동안장착한후, 파노라마방사선사진상에서하악골의좌우비대칭을비교해본결과 14), 하악과두와하악지부위의좌우비대칭이개선되었다 (Table 2). Hybrid appliance 를총 49개월동안계속장착하였으며, 사춘기최대성장기를거치면서반안면 Figure 6. The facial photograph showing the atrophy of soft tissue, enophthalmos, and dry skin with dark pigmentation on the left side at the age of 15. - 4 -
Parry-Romberg Syndrome 환자에서악정형및교정치료 Figure 7. The intraoral photograph showing the dental crowding and posterior open bite on the left side at the age of 15. Figure 8. The panoramic view at the age of 15. 위축 (hemifacial atrophy) 이다시심하게진행되었다 (Table 1,2). 15세때, 구외임상검사상, 좌측 부위에심한연조직의위축, 안구함몰이관찰되었다. 건조한피부에는검은색소가침착되었으며, 정상피부와비정상피부조직사이에선형적인상처 (en coup de sabre) 가관찰되었다. 상하순은후퇴되었으며, 좌측입술선이상방으로올라가서좌측앞니들이노출되었다 (Figure 6). 구내임상검사상, 상하악에서치열의총생이관찰되었고, 상하악치열의정중선이일치하지않았다. 혀의좌측부분이위축되었고, 상악좌측구개부는협착되었으며, 좌측구치부에서는개방교합이관찰되었다 (Figure 7). 정모두부방사선사진상, 우측에비하여좌측상하악골의열성장이관찰되었으며, 파노 Figure 9. The facial photograph after autologous fat graft and the orthodontic treatment by the fixed appliance at the age of 17. - 5 -
유국호, 백형선 Figure 10. The panoramic view at the age of 17. 라마방사선사진상, 좌측상악중절치와측절치, 하악소구치부위에서짧은치근이관찰되었다 (Figure 8). 주기적인방사선사진및임상검사결과상, 반안면위축이다소안정화되었다고판단되어, 고정식교정장치를이용하여교정치료를시작하였다. 짧은치근을고려하여, 치열의총생은치간삭제를 이용하여해소하였고, 약한힘으로좌측소구치와대구치를정출시켜교합을개선하기로하였다. 또한, 교정치료동안좌측안면부위의심미적인개선을위해자가지방이식 (autologous fat graft) 을시행하였다. 지방은자신의둔부에서채취하였고, 지방이식후남은지방은냉동보관하여자가지방이식을 6개월간격으로 3번시행하였다. 교정치료와자가지방이식후, 치아배열, 교합, 안면비대칭이개선되었다 (Figure 9). 파노라마방사선사진상, 치근이더짧아지지않았으며, 치근의위치도개선되었다 (Figure 10). 17세때, 반안면위축이더이상진행되지않는다고판단되어, 고정식교정장치를제거하였으며, 재발을방지하기위하여상악좌측에는 buccal shield, 하악전치부에는 lip pad 를장착한 circumferential retainer 를장착하였다 (Figure 11). 교정치료후 6 개월뒤, 안모와교합은안정적으로유지되고있었다 (Figure 12). Figure 11. The maxillary circumferential retainer with buccal shield and mandibular circumferential retainer with lip shield. Figure 12. The intraoral and facial photographs after retention for 6 months. - 6 -
Parry-Romberg Syndrome 환자에서악정형및교정치료 Ⅲ. 고찰 Parry-Romberg syndrome(prs) 은많은임상적인특징들을나타내지만, 발생유무와심한정도는개개인별로다양하다. 본환자가처음내원했을당시에는경미한안면비대칭과선형경피증 (linear scleroderma) 을보였다. PRS 와선형경피증과의연관성에대해서는여러의견들이있다. PRS 와선형경피증은서로별개의질환이라는의견도있고 15,16), 같은발병원리를갖는다는의견도있다 17). 피부의경화가없는 PRS 두환자에서, 이환측의조직검사를시행한결과, 경피증과같은조직검사결과가보고되기는하였지만, 피부경화증의존재유무로 PRS 를진단하기에는과학적인증거가부족하다고사료된다 4). 주기적인방사선사진과임상적검사를통하여, PRS 의여러임상적특징들을파악하는것이중요하다. 구강악안면환경들이변하면이에따라악골, 치아, 근신경들의적응이발생하므로 18), 악정형치료의주요목적은기능성장치들을이용하여안면부위의환경을변화시켜안면성장을조절하는것이다. PRS 환자의치료에서도진행되는반안면위축을최소화하는것이중요하다. 본환자는최대성장기를거치면서안면비대칭이다시심해지기는하였지만, hybrid appliance 를장착함으로써안면비대칭의개선이있었으며, 특히하악골에서좌우비대칭의개선이있었다. 그러므로, PRS 환자에서도안면비대칭을개선하는악정형효과를얻기위하여 hybrid appliance 가이용될수있다고사료된다. 그러나, 협측판이협측연조직을차단하고정중구개봉합에서의성장을도모하여좌측상악골의위축을개선하기를기대하였지만, 좌측상악골의성장은부족하였으며, 결국구치부는절단교합이되었다. 마찬가지로, PRS 환자에서편측성구치부반대교합이보고되기도하였다 19). 본환자 에서는협측연조직과혀의위축때문에, 기능성장치를이용하여상악골의횡적성장을개선하기에는한계가있었던것으로사료된다. 악정형치료의기간은 PRS 의안정과연관되어있다. PRS 는활성적인기간을지난다음에안정기를가지는자가제한적인질환으로생각되어진다 20). PRS 는다양한나이에안정화되기때문에 21), 안면위축의진행과정은연속적인방사선사진과구외사진촬영으로관찰하는것이필요하다. 반안면위축을심미적으로향상시키기위해서는연조직재건이무엇보다도중요하며, 이를위해서는자가지방이식 22), 자가조직이식 23), 그리고동종이식물질의이식 24) 등의방법들이있다. 그중지방이식은가장많이사용되는방법중하나로 10), 경미하거나중증도의안면위축에서만족스러운결과들을보여준다 25). 지방이식후의장기간결과들을보여주는사례는거의없지만 26), 아직까지지방이식은반안면위축을가지고있는환자에게좋은치료방법이다 27). 본환자의반안면위축정도를고려해보았을때, 지방이식을포함한합성치료가적절하다고판단되었으나 25), 환자와의상의후, 오직자가지방이식만을시행하기로결정하였고, 세번의지방이식을통해심미적인개선을얻을수있었다. PRS 환자에서는치근의형성이미약하거나지연된치아맹출이일어날수도있으며 19), 국소특정신경영양적이론 (regional specific neurotrophic theory) 이가능한원인으로설명되어질수있을지도모른다 23). Hybrid appliance 는반안면위축부위의성장을도모하기위해하악골의위치를변화시키고, 이환측구치부의수직적맹출을유도하게되므로, 치아맹출기전에이상이있을경우에는이환측에구치부개방교합이발생할수있다. 지연된치아맹출은여러생리학적기전들의결과이므로 28), 치주인대가비정상적인지아닌지를판단하는것이중요하 - 7 -
유국호, 백형선 다. 유착이나일차성맹출이상 (primary failure of eruption) 과같이치주인대에이상이있다면 29), 치아는교정력에반응하지않고교합은보철물이나다른외과적치료에의해회복되어야하므로, 이에대한고려가필요하다. 그러나, 아직 PRS 환자에서치아가교정력에의해움직이지않는다는보고는없었으며, 법랑질, 상아질및치아의생활력도임상적으로정상이다 30). 그러므로, PRS 환자에서치아이동에는문제가없는것으로보이지만, 짧은치근의가능성과그정도를예측하기힘들기때문에, 방사선사진으로주기적인관찰을하는것이필요하며, 교정치료계획수립시에도이러한특징들을반드시고려해야한다. Ⅳ. 결론 Parry-Romberg syndrome 환자에서 hybrid appliance 는반안면위축을개선하기위해사용되어질수있으며, 반드시환자의악골과치아의특징들을고려하여치료계획을수립해야한다. 참고문헌 1. Parry CH. Collections from the Unpublished Medical Writings of the Late Caleb Hillier Parry. 1825;London: Underwood:478. 2. Romberg HM. Klinische. 1846;Berlin: Forrtner:75-81. 3. Rogers BO. Progressive Facial Hemiatrophy (Romberg's Disease): A Review of 772 Cases. Proceedings of the 3rd International Conference on Plastic Surgery. Excerpta Medica. ICS 1964;66:681-689. 4. Tollefson MM, Witman PM. En coup de sabre morphea and Parry-Romberg syndrome: a retrospective review of 54 patients. J Am Acad Dermatol 2007;56:257-263. 5. Pensler JM, Murphy GF, Mulliken JB. Clinical and ultrastructural studies of Romberg's hemifacial atrophy. Plast Reconstr Surg 1990;85:669-674; discussion 675-666. 6. Miller MT, Sloane H, Goldberg MF, Grisolano J, Frenkel M, Mafee MF. Progressive hemifacial atrophy(parry- Romberg disease). J Pediatr Ophthalmol Strabismus 1987;24:27-36. 7. Dervis E. Progressive hemifacial atrophy with linear scleroderma. Pediatr Dermatol 2005;22:436-439. 8.Chapman MS, Peraza JE, Spencer SK. Parry-Romberg syndrome with contralateral and ipsilateral extremity involvement. J Cutan Med Surg 1999;3: 260-262. 9. Garcia-de la Torre I, Castello-Sendra J, Esgleyes-Ribot T, Martinez-Bonilla G, Guerrerosantos J, Fritzler MJ. Autoantibodies in Parry-Romberg syndrome: a serologic study of 14 patients. J Rheumatol 1995; 22:73-77. 10. Stone J. Parry-Romberg syndrome: a global survey of 205 patients using the Internet. Neurology 2003;61:674-676. 11. Grippaudo C, Deli R, Grippaudo FR, Di Cuia T, Paradisi M. Management of craniofacial development in the Parry-Romberg syndrome: report of two patients. Cleft - 8 -
Parry-Romberg Syndrome 환자에서악정형및교정치료 Palate Craniofac J 2004;41:95-104. 12.Vig PS, Vig KW. Hybrid appliances: a component approach to dentofacial orthopedics. Am J Orthod Dentofacial Orthop 1986;90:273-285. 13. Grummons DC, Kappeyne van de Coppello MA. A frontal asymmetry analysis. J Clin Orthod 1987;21:448-465. 14. Habets LL, Bezuur JN, Naeiji M, Hansson TL. The Orthopantomogram, an aid in diagnosis of temporomandibular joint problems. II. The vertical symmetry. J Oral Rehabil 1988;15:465-471. 15.Duymaz A, Karabekmez FE, Keskin M, Tosun Z. Parry-Romberg syndrome: facial atrophy and its relationship with other regions of the body. Ann Plast Surg 2009;63:457-461. 16. Orozco-Covarrubias L, Guzman-Meza A, Ridaura-Sanz C, Carrasco Daza D, Sosa-de-Martinez C, Ruiz-Maldonado R. Scleroderma 'en coup de sabre' and progressive facial hemiatrophy. Is it possible to differentiate them? J Eur Acad Dermatol Venereol 2002;16:361-366. 17. Blaszczyk M, Janniger CK, Jablonska S. Childhood scleroderma and its peculiarities. Cutis 1996;58:141-144, 148-152. 18. McNamara JA, Jr. Neuromuscular and skeletal adaptations to altered function in the orofacial region. Am J Orthod 1973;64:578-606. 19. Foster TD. The effects of hemifacial atrophy on dental growth. Br Dent J 1979;146:148-150. 20. Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphea(localized scleroderma) in Olmsted County 1960-1993. J Rheumatol 1997;24:73-80. 21. Lakhani PK, David TJ. Progressive hemifacial atrophy with scleroderma and ipsilateral limb wasting(parry-romberg syndrome). J R Soc Med 1984;77:138-139. 22. Sterodimas A, Huanquipaco JC, de Souza Filho S, Bornia FA, Pitanguy I. Autologous fat transplantation for the treatment of Parry-Romberg syndrome. J Plast Reconstr Aesthet Surg 2009;62: e424-426. 23. Mazzeo N, Fisher JG, Mayer MH, Mathieu GP. Progressive hemifacial atrophy (Parry-Romberg syndrome).case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:30-35. 24. Bagal A, Dahiya R, Tsai V, Adamson PA. Clinical experience with polymethylmethacrylate microspheres(artecoll) for soft-tissue augmentation: a retrospective review. Arch Facial Plast Surg 2007;9:275-280. 25. Guerrerosantos J, Guerrerosantos F, Orozco J. Classification and treatment of facial tissue atrophy in Parry-Romberg disease. Aesthetic Plast Surg 2007;31: 424-434. 26. Eremia S, Newman N. Long-term follow-up after autologous fat grafting: analysis of results from 116 patients followed at least 12 months after receiving the last of a minimum of two treatments. - 9 -
유국호, 백형선 Dermatol Surg 2000;26:1150-1158. 27.Xie Y, Li Q, Zheng D, Lei H, Pu LL. Correction of hemifacial atrophy with autologous fat transplantation. Ann Plast Surg 2007;59:645-653. 28.Suri L, Gagari E, Vastardis H. Delayed tooth eruption: pathogenesis, diagnosis, and treatment. A literature review. Am J Orthod Dentofacial Orthop 2004;126: 432-445. 29. Frazier-Bowers SA, Koehler KE, Ackerman JL, Proffit WR. Primary failure of eruption: further characterization of a rare eruption disorder. Am J Orthod Dentofacial Orthop 2007;131:578 e571-511. 30.Glass D. Hemifacial atrophy. British Journal of Oral Surgery 1963-1964; 1:194-199. 교신저자 Hyoung-Seon Baik Department of Orthodontics, School of Dentistry, Yonsei University, Seoul, Korea Tel : +02-2228-8600 / Fax : +02-393-9858 / E-mail : baik@yuhs.ac - 10 -