|Year : 2021 | Volume
| Issue : 3 | Page : 189-193
Conservative management of odontogenic keratocyst by marsupialization
Adel M Al-Juhni1, Ahmad F Al-Omar2, Hasan A Muaadi3, Anwar Minwer Al-Anazi4
1 Department of Dentistry, Security Forces Hospital, Riyadh, Saudi Arabia
2 Department of Oral and Maxillofacial Surgery, King Saud University, Riyadh, Saudi Arabia
3 Department of Oral and Maxillofacial Surgery, King Khalid University Hospital, Abha, Saudi Arabia
4 Department of Public Health, King Saud Bin Abdulaziz university for Health Sciences, Riyadh, Saudi Arabia
|Date of Submission||28-Aug-2021|
|Date of Decision||10-Sep-2021|
|Date of Acceptance||18-Oct-2021|
|Date of Web Publication||30-Dec-2021|
Dr. Adel M Al-Juhni
Department of Dentistry, Security Forces Hospital, Riyadh
Source of Support: None, Conflict of Interest: None
Odontogenic keratocyst (OKC) is a locally aggressive intraosseous tumor of odontogenic origin, associated with syndromes such as nevoid basal cell carcinoma syndrome. Also known as Gorlin syndrome, it is an autosomal dominant inherited condition. The disease is characterized by multiple basal cell carcinomas of the skin, multiple keratocystic odontogenic tumors, rib anomalies, and palmar and plantar pits. This article reviews a 3-year follow-up case that we have reported in the Dental University Hospital OMFS clinic at King Saud University, Riyadh, Saudi Arabia. The patient was a 13-year-old female that has been diagnosed with Gorlin–Goltz syndrome in 2018. She had multiple radiolucent lesions, two mandibular OKC, and maxillary dentigerous cyst. The extensive left OKC was treated with marsupialization followed by enucleation. Optimal results with low recurrence rate have been shown after using the marsupialization followed by enucleation and curettage, which agree with the present case results. Unfortunately, there is no gold standard or concrete recommendations that emphasize particular treatment modality. Nevertheless, this approach is considered effective and less invasive in treating OKCs, reducing the lesion size by drainage and decompression up to 47% of the initial size, allowing the preservation of critical anatomical structures. Furthermore, marsupialization prior to enucleation is an appropriate approach in young patients because it is the least treatment modality that interferes with the developmental process of jaws.
Keywords: Basal cell carcinoma, Carnoy's solution, Gorlin syndrome, case report, marsupialization, odontogenic keratocyst
|How to cite this article:|
Al-Juhni AM, Al-Omar AF, Muaadi HA, Al-Anazi AM. Conservative management of odontogenic keratocyst by marsupialization. Saudi J Oral Sci 2021;8:189-93
|How to cite this URL:|
Al-Juhni AM, Al-Omar AF, Muaadi HA, Al-Anazi AM. Conservative management of odontogenic keratocyst by marsupialization. Saudi J Oral Sci [serial online] 2021 [cited 2022 Aug 15];8:189-93. Available from: https://www.saudijos.org/text.asp?2021/8/3/189/334298
| Introduction|| |
Odontogenic keratocyst (OKC), formerly known as keratocystic odontogenic tumor (KCOT), is a locally aggressive multicystic or unicystic intraosseous cyst of odontogenic origin. It may appear as solitary nonsyndromic cyst or as multiple cysts, which is associated with syndromes such as Gorlin–Goltz syndrome. Nevoid basal cell carcinoma syndrome (NBCSS), also known as Gorlin syndrome, is an autosomal dominant inherited condition, caused by the mutation of PTCH1. The characteristic of this syndrome includes multiple OKC, multiple basal cell carcinomas of the skin, rib anomalies, and palmar and plantar pits., The cyst was reintroduced as KCOT by the World Health Organization (WHO) in 2005. The aggressive growth behavior, the high potential of recurrence, the chromosomal abnormalities, and the mutation of the PTCH gene in the etiology of KCOTs contributed to the reclassification of this lesion as a tumor. The WHO reclassified the lesion to OKC in 2018., The age of diagnosis occurs in a broad range. However, it peaks in the second and third decades and is more common in males.
Nearly 65%–100% of patients with Gorlin–Goltz syndrome have been diagnosed with OKC. OKC may occur in both maxilla and mandible, yet it has a high tendency to mandible particularly in the third molar area and ramus. Patients with OKC exhibit predominant symptoms of swelling and drainage, along with other accompanying symptoms such as pain, trismus, and paresthesia in fewer cases.,, The duration of symptoms varies from 1 month to 6 years, and a majority of patients exhibit symptoms for <3 months. A considerable number of cases, however, are incidentally diagnosed during routine dental examination, and numerous articles have reported the frequency of such cases to be ranging from 5.5% to 42.5% with respect to different sample sizes.,,
| Case Report|| |
We reported this case in the Dental University Hospital OMFS clinic at King Saud University, Riyadh, Saudi Arabia. The patient was a 13-year-old female that has been diagnosed with Gorlin–Goltz syndrome (GGS) in 2018. The patient came seeking orthodontic treatment, with a history of cleft lip and palate. Orthopantomogram interpretation revealed that she had multiple radiolucent lesions, congenitally missing lower second premolars and retained primary maxillary 2nd molars and mandibular left primary 2nd molar [Figure 1]. There was a 38 mm × 30 mm unilocular, well-defined, left mandibular OKC extending to the ramus of the mandible, involving the partially erupted mandibular left second molar [Figure 1]a. There was also a 10 mm × 11 mm small, round, well-defined OKC, located distal to the unerupted mandibular right second molar [Figure 1]b. A pericoronal radiolucent dentigerous cyst was associated with horizontally impacted maxillary right second molar [Figure 1]c. Cone-beam tomography showed the left OKC expanding the buccal plate laterally from the distal root of the lower first molar to the ascending ramus of the mandible posteriorly [Figure 2]a and [Figure 2]b. The small right OKC slightly expanded the ramus of the mandible buccolingually [Figure 2]b. Maxillary dentigerous cyst obliterated the right sinus with the horizontally impacted second molar [Figure 2]c.
|Figure 2: (a-c) Cone beam computed tomography showing the expanded lesion|
Click here to view
Under general anesthesia, the right small right OKC and maxillary dentigeous cyst were enucleated with removal of the involved upper right and lower second molars, and marsupialization was performed for the left extensive OKC. Biopsy and histopathological investigations confirmed the diagnosis [Figure 3]. Multiple follow-up visits were scheduled in the 1st year for evaluation; during these visits, the cyst cavity was irrigated with saline solution, and oral hygiene instructions were emphasized. The partially enucleated OKC showed explicit size reduction and significant bone remodeling [Figure 4] and [Figure 5].
|Figure 3: Light microscopy photograph showing odontogenic keratocyst with focal ulceration (H and E, ×100)|
Click here to view
The patient underwent the second surgery 11 months after marsupialization, enucleation, and peripheral ostectomy had been carried out for the lower left OKC under general anesthesia. [Figure 6] illustrates bone deposition and evident size reduction. [Figure 7] shows complete remission.
| Discussion|| |
GGS is a heritable disease with various manifestations, and it can occur from 1 in 57,000 to 1 in 256,000 of the population. According to research, the cause of GGS is a mutation of a tumor suppressor gene called patched (PTCH), which is mapped to 9q22.3 chromosomes.[3.9] Several surgical modalities treat OKC, such as enucleation with peripheral ostectomy, using a Carnoy's solution, marsupialization prior to enucleation, and resection.,
Unfortunately, there is no gold standard or concrete recommendations that emphasize particular treatment modality; nevertheless, numerous studies have attempted to recognize the correlation between the type of treatment and the recurrence of OKC. A large systematic review analysis has been conducted to evaluate what treatment modalities yield a low recurrence rate (RR). With a total 2287 OKCs in 35 studies, it was revealed as follows: enucleation with ostectomy (17.4%), enucleation and application of Carnoy's solution (11.5%), marsupialization followed by cystectomy (14.6%), and complete resection (8.4%).
A study conducted by Pogrel and Jordan supported marsupialization as an effective treatment modality in some patients with OKC; the authors performed a marsupialization for ten nonsyndromic patients, and the result was complete remission and bone formation for the ten patients in an average interval between 7 and 19 months. According to many authors, the acceptable period to assess the RR is 5 years. Resection is an aggressive treatment that causes facial disfigurement with other possible morbidities; however, it is suitable for large lesions and exhibits optimal cure; a study conducted by Zecha et al. revealed zero RR in three patients treated with resection.
Enucleation alone is a debatable approach that demonstrates unpredictable results. In this study, recurrence has occurred in 26% of 102 patients, which can be considered an acceptable percentage. Conversely, another study showed that 60% of OKC recurred from the treatment of enucleation alone. The high RR could be resulted from two causes as stated in the study by:  one reason is histological which caused by the presence of dental lamina, and the other is surgically related, that is uncompleted removal of epithelium lining due to anatomical difficulty.
Combining these treatment modalities, enucleation with ostectomy and applying Carnoy's solution were highly effective and exhibited 0% RR. Carnoy's solution is a hemostatic chemical agent that penetrates and cauterizes the bone, and subsequently enhances necrosis to the lesion to provide a safe margin. 10 mL of the solution constitutes a mixture of 3 mL of chloroform, 6 mL of ethanol, 1 mL of glacial arctic acid, and 1 g of ferric chloride. It is used as a complementary technique after OKC enucleation, which is usually applied for 5 min to denature the cystic bone cavity., The solution was first discovered in 1973; even though the understanding of its effectiveness came late, a study showed the significance of Carnoy's solution in lowering the RR between patients who received it and patients who did not (2.5% and 13.5% RR, respectively). Despite its efficacy, Carnoy's solution has several complications including dehiscence (22.7%), recurrence (4.5%), paresthesia (18.2%), and infection (4.5%). Dehiscence is associated with lesions treated by marsupialization, while paresthesia is influenced by direct manipulation of an exposed nerve and the time of solution application.,
In another approach advocated by Pogrel et al., there is 1–2 mm bone ostectomy beyond postenucleation cystic margin with the assistance of methylene blue staining. Although this technique may increase the cure rate, there are no recent studies that support it. Topical 5-fluorouracil is commonly used in the treatment of OKC. A cohort study conducted from 2006 to 2014 which aimed to compare the efficacy between 5-fluorouracil and Carnoy's solution after enucleation revealed insignificant difference; however, paresthesia of inferior alveolar nerve was lower in 5-fluorouracil and evident in Carnoy's solution.
Marsupialization followed by enucleation has proven to yield optimal results with low RR, and this fact is highly aligned with the present case result. Therefore, enucleation and marsupialization appear to be the most common and conservative treatment, and they have a minimum RR before resection., In addition, marsupialization prior to enucleation reduces the lesion size up to 47% of the original size. Consequently, it is considered an appropriate approach in young patients and the minor treatment modality that interferes with the developmental process of the jaws.,
In our case, the left mandibular OKC was expanded in all directions (anteroposterior, buccolingual, and superoinferior) and extended to the mandible ramus. One year after marsupialization of the lesion, enucleation and peripheral osteotomy was done. After almost 2 years, the result was evident bone deposition with no recurrence. Besides, there was no influence on esthetic and function; moreover, no sign of paresthesia took place. This patient will continue to be monitored in future at a regular follow-up every year.
| Conclusions|| |
Treatment options of the presented OKC case have been discussed, and there is no gold standard of OKC treatment. However, a conservative approach with the least RR is the optimal treatment of choice. Marsupialization, followed by enucleation, has shown remarkable results in our case.
Statement of informed consent
Written informed consent was obtained from the patient (s) for their anonymized information to be published in this article.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their name and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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;49:229-35.
Arshad F. Syndromic odontogenic keratocyst: A case report and review of literature. J Int Soc Prev Community Dent 2016;6:84-8.
Fujii K, Miyashita T. Gorlin syndrome (nevoid basal cell carcinoma syndrome): Update and literature review. Pediatr Int 2014;56:667-74.
Speight PM, Takata T. New tumour entities in the 4th
edition of the World Health Organization Classification of Head and Neck tumours: Odontogenic and maxillofacial bone tumours. Virchows Arch 2018;472:331-9.
Li TJ. The odontogenic keratocyst: A cyst, or a cystic neoplasm? J Dent Res 2011;90:133-42.
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.
Al-Jarboua MN, Al-Husayni AH, Al-Mgran M, Al-Omar AF. Gorlin-Goltz syndrome: A case report and literature review. Cureus 2019;11:e3849.
Ortega García de Amezaga A, García Arregui O, Zepeda Nuño S, Acha Sagredo A, Aguirre Urizar JM. Gorlin-Goltz syndrome: Clinicopathologic aspects. Med Oral Patol Oral Cir Bucal 2008;13:E338-43.
Gorlin RJ. Nevoid basal cell carcinoma (Gorlin) syndrome: Unanswered issues. J Lab Clin Med 1999;134:551-2.
Khan AA, Qahtani SA, Dawasaz AA, Saquib SA, Asif SM, Ishfaq M, et al.
Management of an extensive odontogenic keratocyst: A rare case report with 10-year follow-up. Medicine (Baltimore) 2019;98:e17987.
Al-Moraissi EA, Dahan AA, Alwadeai MS, Oginni FO, Al-Jamali JM, Alkhutari AS, et al.
What surgical treatment has the lowest recurrence rate following the management of keratocystic odontogenic tumor? A large systematic review and meta-analysis. J Craniomaxillofac Surg 2017;45:131-44.
Pogrel MA, Jordan RC. Marsupialization as a definitive treatment for the odontogenic keratocyst. J Oral Maxillofac Surg 2004;62:651-5.
Kaczmarzyk T, Mojsa I, Stypulkowska J. A systematic review of the recurrence rate for keratocystic odontogenic tumour in relation to treatment modalities. Int J Oral Maxillofac Surg 2012;41:756-67.
Pitak-Arnnop P, Chaine A, Oprean N, Dhanuthai K, Bertrand JC, Bertolus C. Management of odontogenic keratocysts of the jaws: A ten-year experience with 120 consecutive lesions. J Craniomaxillofac Surg 2010;38:358-64.
Finkelstein MW, Hellstein JW, Lake KS, Vincent SD. Keratocystic odontogenic tumor: A retrospective analysis of genetic, immunohistochemical and therapeutic features. Proposal of a multicenter clinical survey tool. Oral Surg Oral Med Oral Pathol Oral Radiol 2013;116:75-83.
Ribeiro Junior O, Borba AM, Alves CA, de Gouveia MM, Coracin FL, Guimarães Jünior J. Keratocystic odontogenic tumors and Carnoy's solution: Results and complications assessment. Oral Dis 2012;18:548-57.
Pogrel MA. The keratocystic odontogenic tumor. Oral Maxillofac Surg Clin 2013;25:21-30.
Akbari M, Chen H, Guo G, Legan Z, Ghali G. Basal cell nevus syndrome (Gorlin syndrome): Genetic insights, diagnostic challenges, and unmet milestones. Pathophysiology 2018;25:77-82.
Antonoglou GN, Sándor GK, Koidou VP, Papageorgiou SN. Non-syndromic and syndromic keratocystic odontogenic tumors: Systematic review and meta-analysis of recurrences. J Craniomaxillofac Surg 2014;42:e364-71.
Lench NJ, Telford EA, High AS, Markham AF, Wicking C, Wainwright BJ. Characterisation of human patched germ line mutations in naevoid basal cell carcinoma syndrome. Hum Genet 1997;100:497-502.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]