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Научни трудове на Съюза на учените в България-Пловдив Серия Г. Медицина, фармация и дентална медицина т.ХХ1. ISSN 1311-9427 (Print), ISSN 2534-9392 (On-line). 2017. Scientific works of the Union of Scientists in Bulgaria-Plovdiv, series G. Medicine, Pharmacy and Dental medicine, VoLXXI. ISSN 1311-9427 (Print), ISSN 2534-9392 (On-line). 2017.
МНОЖЕСТВЕНИ ОДОНТОГЕННИ НЕСИНДРОМНИ КЕРАТОКИСТИ- ДОКЛАД НА КЛИНИЧЕН СЛУЧАЙ Таня Сбиркова1, Петя Печалова1, Галина Гавазова1, Елена Порязова2,
Димитър Господинов1 1Катедрапо Орални хирпргпх,ФДМ, МедицинскиУниверситет -
2 Катедра по Обща и Клиничнапатологип, МФ,И1едицински Университет -HLiobhhb
MULTIPLE, MULTIFOCAL O OOHiTOGENIC KERATOCYSTS INNON-SYnDROMEOATIENT: A CASE -REPORT Tanya Sbirkova1, Petia бдс°п1пуаGaHna Gavazova1, Elena Poriazova2, Dimitar Gospodinov1 1 Dcpertment of Oral Surgery, Faculty of Dental Medicine, Medical Unicersity- Plocdw 2 Department of Pathology, Faculty of Medicine, Medical University - Plovdiv
Abstract: Occurrence of multiple odontogenic keratocyst involving the jaws is rare. Odontogenic keratocysts (OKC) are common developmental odontogenic cysts affecting the maxillofacial region. Multiple OKCs are usually seen in association with Gorlin-Goltz syndrome which is also known as Nevoid Basal Cell Carcinoma Syndrome - a rare autosomal dominant disorder. In some cases OKC have multiple presentation without concomitant syndromic presentation. This is a report of a a case of multiple OKCs in a non-syndromic patient.
Key words: multiple odontogenic keratocyst, Gorlin-Goltz syndrome, Nevoid Basal Cell Carcinoma Syndrome
Introduction: Odontogenic keratocyst (OKC) is a developmental odontogenic cyst with specific histopathologic features and clinical behavior. In the latest WHO classification of odontogenic tumors in 2005, these lesions have been given the name "keratocystic odontogenic tumors". (1) Odontogenic keratocysts comprise 10 .11% of all cysts of the jaws. 4 . 27% of them are found in patients with basal cell nevus syndrome. (2) There is general agreement that the odontogenic keratocysts arise from cell rests of the dental lamina.(3) These cysts are associated with Nevoid basal cell carcinoma syndrome (NBCCS) involving the triad of multiple basal nevi, multiple odontogenic keratocysts and skeletal abnormalities. (4) In 1960, Gorlin and Goltz initially described the simultaneous existence of multiple basal cell carcinomas, multiple OKCs of the
mandible and the maxilla, bifid ribs, and other variable manifestations that comprise the basal cell nevus bifid rib syndrome. (3) This syndrome is called Gorlin- Goltz syndrome. The presence of OKC in children or adolescents before the age of 19 is seen in 75% of cases and can be the first sign of NBCCS.(5,6) R. B. Brannon made analysis of clinical features of 312 acceptable cases of odontogenic keratocysts and found that 5.8% were from patients with multiple keratocysts but with no other features of the syndrome. (7) In this article we present a rare case of young woman with multiple odontogenic keratocysts not associated with syndrome.
Case report: A 14-year-old girl with complains of swelling of the chin and dislocation of the teeth of lower jaw was reffered to our clinic with two intraoral X-rays. (fig. 1)
Fig 1 Intraoral X-raies
The extraoral examination found facial asymmetry due to swelling in the region of the chin, painful on palpation, no change of skin color was observed. The mouth opening was normal, dislocation of the teeth of lower jaw and disocclusion were found. Intraoral clinical examination revealed oval swelling located over labial mucosa of mandibular anterior region with changed elasticity. To make a correct diagnosis we needed orthopantomogram (OPG). The OPG revealed the presence of two large, osteolytic formations in lower jaw with regular borders. The first formation involved the region from the right canine to the second left premolar, the second was located in the ramus of the mandible, involving the germ of the right mandibular third molar. (Fig. 2)
Fig 2 OPG
Prior to the treatment we checked the vitality of teeth involved in the lesion and established that left teeth were nonvital, while right teeth were with vital pulp (table 1):
_Table 1. Pulp vitality test of teeth, involving into the formation_
Tooth Value of pulp vitality test (microampers)
Left second premolar 132,4 |A
Left first premolar 161,2 |A
Left canine 157,4 |A
Left lateral incisor 149,3 |A
Left central incisors 159,4 |A
Right central incisor 024,8 |A
Right lateral incisor 020,6 |A
Right canine 014,1 |A
Because lesion in the frontal region was inflamed, we performed an intraoral incision for pus evacuation, put a rubber drainage and prescribed antibiotic (Amoxiclav 1,2 every 8h i.v.) and analgesic (Dialgin 1,0 every 12 h)
For the formation in the ramus of the lower jaw the surgical treatment was provide under general anesthesia and included extraoral incision around mandibular angle a modo Risdon, enucleation of the cyst, application of iodoform powder, filling the cystic cavity with a gauze drainage, and suturing. For the enucleation of the frontal formation we performed an intraoral incision situated from the first right molar to the first left molar, elevated mucoperiostal flap, removed the first left molar and performed the same steps like in the other formation. To prevent pathological fracture of the mandible and to stabilize the teeth we performed splinting with wire between the second premolars in both sides. Gauze drainages were removed into two consecutive visits in the third and fifth days, sutures were removed 10 days postoperatively. The surgical wounds healing by primary intention. No complications were noted during the observed 6 months
Fig. 3 Histopathologic view
Discussion: Multiple cysts (MC) involving the jaw are rare. Occurrence of MC without syndromic association is extremely rare. (8) When multiple, odontogenic keratocysts are usually associated with a syndrome so they should be evaluated thoroughly for the possibility of NBCCS as these lesions may be the first and only manifestation of this syndrome. (9) Multiple odontogenic keratocysts have higher recurrence rates (82%) compared with solitary keratocysts (61%). (9,10) In most cases multiple odontogenic keratocysts are associated with Gorlin- Goltz syndrome. OKC is one of the most aggressive odontogenic cysts of the oral cavity.(9) OKC is known for its rapid growth,(9) and its tendency to invade the adjacent tissues including bone.(9) It has a high recurrence rate.(9)
Odontogenic keratocysts may be found in any age with peak prevalence between 10 to 40 years old (1,7). Peak incidence is in second and third decade (3,8,11) and it is exceedingly rare
before 10 years of age. (8) Several studies indicate a male predilection (12,13), some studies do not correlate with this.(14,15)
The mandible is involved in 60 to 80% of cases with a marked tendency to occur in the posterior body and ascending ramus (1,14,16). Odontogenic keratocysts may occur in any part of the upper and lower jaw with the majority occurring in the mandible, most commonly in the angle of the mandible and ramus.(3,11,17)
Small odontogenic keratocysts are usually asymptomatic but larger ones may show clinical manifestations like pain, swelling or drainage into the oral cavity.(1) With the enlargement they can expand and perforate both buccal and lingual cortical plates and involve adjacent soft tissue. (8) In some cases of odontogenic keratocysts , teeth mobility could be noted.(3) Radiologically, these lesions demonstrate a well defined radiolucent area with smooth and often corticated margins and may be unilocular or multilocular.(1) In 25 to 40% of cases, an unerupted tooth is seen in association with the lesion (1). Some authors describe radiographic appearances of the lesions like variable from a unilocular or a multilocular radiolucency with scalloped and well-defined margins to soap-bubble or honeycomb radiolucency.(3,11)
Therapeutic interventions of KCOT include marsupialization and enucleation, combined with adjuvant cryotherapy with Carnoy's solution, and marginal or radical resection. (1,18) For unerupted permanent teeth in children, conservative treatment should be done first, because an aggressive operation can cause adverse effects on teeth development and its eruption. (1) Marsupialization followed by enucleation has the lowest recurrence rate among the conservative treatments.(19)
Histopathologically, odontogenic keratocysts with NBCCS show parakeratinized, intramural epithelial remnants, satellite cyst, lower thickness of epithelium, and fewer nuclei than the solitary cysts.(9,10,16)
Conclusion: Multiple odontogenic keratocysts are usually associated with NBCCS and can be the first sign of the syndrome. In case of presence of these lesions, patient should be evaluated about NBCCS and make him regular monitoring in time.
References:
1. Kargahi N., Kalantari M. Non-syndromic multiple odontogenic keratocyst: a case report. J Dent Shiraz Univ Med Sci. 2013; 14(3): 151-154
2. Стоянов Хр. Случай на множествени несиндромни, одонтогенни кератокисти.Годишен сборник ИМАБ, том 10, кн 1, 2003:53-54
3. Maheshwari V., Patel N., Jadhav R., Engineer P. Non Syndromic Odontogenic Keratocyst: A Case Report. J of Dent and Med Scien. 2015;14(6): 64-67
4. Bartake AR, Shreekanth NG, Prabhu S, Gopalkrishnan K. Non-Syndromic Recurrent Multiple Odontogenic Keratocysts: A Case Report. Journal of Dentistry, Tehran University of Medical Sciences 2011;8(2)
5. Hammannavar R., Holikatti K., Bassappa S., Shinde N., Reddy M., Chidambaram YS. Multiple, Multifocal Odontogenic Keratocysts in Non-Syndrome Patient: A Case-report. OHDM 2014; 13(2)
6. Budnick S. Odontogenic cysts. In: Gale N, Luzar B (Editors) 19th European Congress of Pathology Pre-meeting. Head and neck pathology proceedings. Ljubljana. 2003; 122-132
7. Brannon RB. The odontogenic keratocyst. A clinicopathologic study of 312 cases. Part I. Clinical features. Oral Surgery, Oral Medicine, Oral Pathology. 1976; 42: 54-72.
8. Nirwan A.,Wanjari SP, Saikhedkar R., Karun V. Multiple non-syndromic odontogenic keratocysts in three siblings. BMJ Case Rep 2013. doi:10.1136/bcr-2012-007503
9. Kurdekar RS, Prakash J., Rana A.S., Kalra P. Non-syndromic odontogenic keratocysts: A rare case report. Natl J Maxillofac Surg. 2013 Jan-Jun; 4(1): 90-93.
10. Auluck A., Suhas S., Pai K.M. Multiple Odontogenic Keratocysts: Report of a Case. J Can Dent Assoc 2006 ;72:651 -6
11. Guruprasad Y., Chauhan D.S. Multiple odontogenic keratocyst in non syndromic patient.journal of cranio-maxillary disease/voll /issue 1/ Jan 2012
12. Mosqueda-Taylor A, Irigoyen-Camacho ME, Díaz-Franco MA, To-rres-Tejero MA.Odontogenic cysts. Analysis of 856 cases. Med Oral. 2002;7(2):89-96.
13. Sholapurkar AA, Reddy VM, Keerthilatha MP, Geetha V. NON-SYNDROMIC MULTIPLE ODONTOGENIC KERATOCYSTS: report of case. Rev Clín Pesq Odontol. 2008;4(3):193-199
14. Chirapathomsakul D, Sastravaha P, Jansisyanont P. A review of odontogenic keratocysts and the behavior of recurrences. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2006; 101: 5-9
15. Koseoglu BG, Atalay B, Erdem MA. Odontogenic cysts: a clinical study of 90 cases. J Oral Sci. 2004;46(4):253-7
16. Narsapur S.A., Choudhari S., Warad N.M., Manjunath S. Non-syndromic multiple odontogenic keratocysts associated with dental anomalies: A report of unusual case and its management.J of Indian Academy of Oral Medicine and Radiology. 2015;27(2)
17. Taiana Campos Leite; Valdir Meirelles Jr.Maria Elisa Rangel Janini: Odontogenic Keratocystic Tumor: A Clinical and Histopathologic Retrospective Study Based on the New WHO Classification: Int. J. Odontostomat.2011;5(3):227-234
18. Habibi A, Saghravanian N, Habibi M, Mellati E, Habibi M. Keratocystic odontogenic tumor: a 10-year retrospective study of 83 cases in an Iranian population. J Oral Sci 2007; 49: 229-235
19. Neelampari R Parikh. Nonsyndromic multiple odontogenickeratocysts: Report of case. J Advanc Dent Res 2010;1:71-74
Address for correspondence:
Tanya Sbirkova, D.D., Department of Oral Surgery, Faculty
of Dental Medicine, Medical University, 3, Hristo Botev Blvd., Bg - 4000 Plovdiv,
tel. +359 888493145, e-mail : tanya_sbirkova@abv.bg
