Maxillofacial Plastic and Reconstructive Surgery

Korean Association of Maxillofacial Plastic and Reconstructive Surgeons (대한악안면성형재건외과학회)
권호 표지

CASE REPORT OF TREATMENT OF MULTIPLE ODONTOGENIC KERATOCYSTS WITH BASAL CELL NEVUS SYNDROME USING PREOPERATIVE MARSUPIALIZATION AND ORTHODONTIC EXTRUSION

기저세포모반증후군과 연관된 다발성 치성각화방종의 치료시 술전 조대술과 술후 교정적 맹출의 사용 치험 1례

  • Gang, Tae-In (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Park, Young-Ju (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Nam, Jeong-Hun (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Ahn, Jang-Hoon (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Kang, Hae-Jin (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Song, Jun-Ho (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Chung, Jae-An (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University) ;
  • Shin, Jin-Eob (Department of Oral and Maxillofacial Surgery, College of Medicine, Kang-Nam Sacred Heart Hospital, Hallym University)
  • 강태인 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 박영주 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 남정훈 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 안장훈 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 강혜진 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 송준호 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 정재안 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실) ;
  • 신진업 (한림대학교 의과대학 강남성심병원 구강악안면외과학교실)
  • Published : 2009.09.30
Download PDF
⟨ Previous Next ⟩

Abstract

Basal cell nevus syndrome is a rare inherited disorder characterized by mulitple cutaneous basal cell carcinoma, pits of the palms and soles, cysts of the jaws, skeletal abnormalities and ectopic calcifications. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus early allowing an early diagnosis of these patients. We report a case of a 9-year-old boy with the various manifestation of basal cell nevus syndrome, which are multiple odontogenic keratocysts, pits of the soles, bifid ribs, ectopic calcification, macrocephaly, and hypertelorism, etc. Total five odontogenic keratocysts were found. For the reduction of the size of the odon-togenic keratocysts, following preoperative marsupialization, there were surgically enucleated. And the impacted upper right lateral incisor and canine are tracted orthodontically.

Keywords

References

  1. Shafer WG, Hine MK, Levy BM : A textbook of oral pathology, 4th ed. Saunders, 1983, p.273
  2. Gorlin RJ, Goltz RW : Multiple nevoid basal-cell epithelioma, Jaw cysts and bifid rib. A syndrome. N Engl J Med 262 : 908, 1960. Arch Dermatol 118 : 813, 1982
  3. Southwick GJ, Schwartz RA : The basal cell nevus syndrome: disasters occurring among a series of 36 patients. Cancer 44 : 2294, 1979 https://doi.org/10.1002/1097-0142(197912)44:6<2294::AID-CNCR2820440644>3.0.CO;2-G
  4. Lo Muzio L, Nocini P, Bucci P et al : Early diagnosis of nevoid basal cell carninoma syndrome. J Am Dent Assoc 130 : 669, 1999
  5. Donatsky O, Hjorting-Hansen E : Recurrence of the odontogenic keratocyst in 13 patients with the nevoid basal cell carcinoma syndrome. A 6-year follow-up. Int J Oral Surg 9 : 173, 1980 https://doi.org/10.1016/S0300-9785(80)80016-0
  6. Gorlin RJ : Nevoid basal cell carcinoma syndrome. Dermatol Clin 13 : 113, 1995
  7. Stoelinga PJ : Long-term follow-up on keratocysts treated according to a defined protocol. Int J Oral Maxillofac Surg 30 : 14, 2001 https://doi.org/10.1054/ijom.2000.0027
  8. Woolgar JA, Rippin JW, Browne RM : A comparative histological study of odontogenic keratocysts in basal cell naevus syndrome and control patients. J Oral Pathol Med 16 : 75, 1987 https://doi.org/10.1111/j.1600-0714.1987.tb00691.x
  9. Gorlin RJ : Nevoid basal cell carcinomab (Gorlin) syndrome : unanswered issues. J Lab Clin Med 134 : 551, 1999 https://doi.org/10.1016/S0022-2143(99)90092-6
  10. Kakarantza-Angelopoulou E, Nicolatou O : Odontogenic keratocysts: clinicopathologic study of 87 cases. J Oral Maxillofac Surg 48 : 593, 1990 https://doi.org/10.1016/S0278-2391(10)80472-0
  11. Howell JB, Anderson DE : "The basal-cell nevus"by Howell and Caro, January 1959. Commentary: The nevoid basal cell carcinoma syndrome
  12. Gorlin RJ : Nevoid basal cell carcinoma syndrome. Medicine 66 : 98, 1987
  13. Minami M, Urano Y, Ishigami T et al : Germline mutations of the PTCH gene in Japanese patients with nevoid basal cell carcinoma syndrome. J Dermatol Sci 27 : 21, 2001 https://doi.org/10.1016/S0923-1811(01)00104-9
  14. Wicking C, Bale AE : Molecular basis of the nevoid basal cell carcinoma syndrome. Curr Opini Pediatr 9 : 630, 1997 https://doi.org/10.1097/00008480-199712000-00013
  15. Lim YS , Kim DK , Kim SG et al : Basal cell nevus syndrome: review of 33 affected Korean individuals. J Kor Assoc Maxillofac Plast Reconstr Surg 25 : 337, 2003
  16. Pritchard LJ, Delfino JJ, Ivey DM et al : Variable expressivity of the multiple nevoid basal cell carcinoma syndrome. J Oral Maxillofac Surg 40 : 261, 1982 https://doi.org/10.1016/0278-2391(82)90215-4
  17. Freedberg IM, Eisen AZ, Wolff K et al : Dermatology in general medicine, 5th ed. New York, McGraw-Hill, 1999, p.1862
  18. Zerin JM, Ainge G : Facial deformity and rash. Invest Radiol 24 : 159, 1989 https://doi.org/10.1097/00004424-198902000-00015
  19. Myoung H , Hong SP , Hong SD et al : Odontogenic keratocyst: Review of 256 cases for recurrence and clinical copathologic parameters. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 91 : 328, 2001 https://doi.org/10.1067/moe.2001.113109
  20. Nakamura N, Mitsuyasu T, Mitsuyasu Y et al : Marsupialization for odontogenic keratocysts: long-term follow-up analysis of the effects and changes in growth characteristics. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 94 : 543, 2002 https://doi.org/10.1067/moe.2002.128022
  21. Zhao YF, Wei JX, Wang SP : Treatment of odontogenic keratocysts: a follow-up of 255 Chinese patients. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 94 : 151, 2002 https://doi.org/10.1067/moe.2001.125694
  22. Pogrel MA : Decompression and marsupialization as a treatment for the odontogenic keratocyst. Oral Maxillofac Surg Clin North Am 15 : 415, 2003 https://doi.org/10.1016/S1042-3699(03)00038-4
  23. Marker P, Brondum N, Clausen PP et al : Treatment of large odontogenic keratocysts by decompression and later cystectomy: a long-term follow-up and a histologic study of 23 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 82 : 122, 1996 https://doi.org/10.1016/S1079-2104(96)80214-9
  24. Ninomiya T, Kubota Y, Koji T et al : Marsupialization inhibits interleukin-1alpha expression and epithelial cell proliferation in odontogenic keratocysts. J Oral Pathol Med 31 : 526, 2002 https://doi.org/10.1034/j.1600-0714.2002.00029.x