Case report

 

Multiple Dentigerous Cysts: two case reports of nonsyndromic twins

Multiplos Quistos Dentigeros: relato de caso em gemeos nao sindromicos

 

Rita Catarina de Oliveira^a, Jonas Dantas Batista^b, Sergio Vitorino Cardoso^c, Flaviana Soares Rocha^b,*

^a School of Dentistry, University of Uberlândia, Minas Gerais, Brazil.

^b Oral & Maxillofacial Surgery and Implantology Department, School of Dentistry, University of Uberlândia, Minas Gerais, Brazil.

^c Pathology Department, School of Dentistry, University of Uberlândia, Minas Gerais, Brazil.

 

^*Correspondence to:

 

http://doi.org/10.24873/j.rpemd.2019.03.444

 

Abstract

Dentigerous cysts are odontogenic cysts associated with the crowns of permanent unerupted teeth. Multiple dentigerous cysts are very rare and mostly found in patients with syndromes.

This paper reports two homozygous twins with dentigerous cysts in both unerupted lower second molars. In one patient, a third dentigerous cyst was associated with an unerupted maxillary canine. Enucleation of smaller lesions and marsupialization of the larger lesions were performed to preserve the permanent teeth, with good final positioning.

Keywords: Dentigerous cyst, Impacted tooth, Twins

 

Resumo

Quistos dentígeros são quistos odontogénicos associados à coroa de dentes permanentes não erupcionados. Múltiplos quistos dentígeros são considerados raros e encontrados principalmente em pacientes com síndromes. Este trabalho apresenta um caso de gémeos homozigotos com quistos dentígeros em ambos os segundos molares inferiores não erupcionados.

Num dos pacientes, um terceiro quisto dentígero foi associado ao canino maxilar não erupcionado. Para o tratamento, foi realizada enucleação das lesões menores e marsupialização das lesões maiores para preservar os dentes permanentes, com bom posicionamento final.

Palavras-chave: Quisto dentígero, Dente não erupcionados, Gémeos

 

Introduction

The dentigerous cyst (DC) is the most common odontogenic cyst of developmental origin.¹ DCs are thought to result from the proliferation of the reduced enamel epithelium (REE) shortly after enamel formation during dental tissue development.^{2, 3} The DC may partially or completely involve the crown of an unerupted tooth, and be attached to the tooth at the cement‐enamel junction.^{2, 3} Degeneration of the stellate reticulum, accumulation of fluid between the layers of REE, diminished adherence of REE, and monoclonal proliferation in the REE through altered protein patched homolog (PTCH) signaling in the primary cilium have been suggested to explain the pathogenesis of DC.⁴

It is estimated that 1.4 DCs are found for every 100 unerupted teeth. This cyst is most common in males, has a peak incidence in the second decade of life, and affects mostly mandibular third molars.⁵ They are usually slow growing asymptomatic lesions that are not discovered until they affect surrounding organs, during orthodontic treatment planning or from the delayed eruption of a permanent tooth.^{5, 6} On radiographic examination, a DC commonly appears as a well-delimited unilocular radiolucency, encircling the crown of an unerupted tooth.⁶

Over 95% of DCs are single lesions.^{3, 5} Multiple occurrences of DC have been described in mucopolysaccharidosis (Maroteaux-Lamy and Hunter’s syndrome).^{7, 8} Moreover, the association of multiple DCs with other syndromes, such as cleidocranial dysplasia and basal cell nevus syndrome, has been poorly documented in the literature.⁵ However, strong evidence taken from a large series of cases has indicated that the multiplicity of DCs is not a syndromic event.⁵ Although some authors believe that multiple and bilateral DCs are rare in the absence of developmental syndromes,⁹ many cases of multiple DCs in nonsyndromic patients have previously been reported.¹⁰, ¹² We present the case of nonsyndromic twins with multiple DCs, a condition that, to our knowledge, has not been previously reported.

Case report

Two 14-year-old male twins were referred by their orthodontist to the Oral and Maxillofacial Surgery Service of the Federal University of Uberlândia, Brazil, for the evaluation of asymptomatic cystic lesions. Their past medical history showed no evidence of any significant alteration, and their routine laboratory tests were within normal limits. Extraoral examination revealed a symmetric appearance of the face, without swelling or tenderness.

Patient 1: The intraoral examination revealed a discrete swelling in the maxillary anterior labial vestibule extending from the left central incisor to the left lateral incisor. Teeth 23, 17, 27, 37 and 47 were absent of the oral cavity (Figure 1). The panoramic radiograph showed well-circumscribed, corticated and unilocular radiolucent lesions associated with the unerupted teeth 23, 37 and 47 (Figure 2). Based on clinical and radiographic findings, a working diagnosis of dentigerous cyst was made. The odontogenic keratocystic tumor was also considered in the differential diagnosis. Marsupialization of the larger lesion (tooth 47) and surgical enucleation of the smaller lesions (teeth 23 and 37) were performed under local anesthesia.

 

 

 

Care was taken to preserve the teeth involved. No complementary surgical procedures were necessary for these areas. In addition, an ulectomy was also performed in the region of teeth 17 and 27 to assist eruption. The postoperative period was uneventful.

Patient 2: The intraoral examination revealed the absence of teeth 17, 27, 37 and 47 (Figure 3). The panoramic radiograph showed well-circumscribed, corticated and unilocular radiolucent lesions associated with teeth 37 and 47 (Figure 4). A working diagnosis of dentigerous cyst was made, and the odontogenic keratocyst was considered in the differential diagnosis. Marsupialization of the larger lesion (tooth 37) and surgical enucleation of the smaller lesion (tooth 47) were performed under local anesthesia. Care was taken to preserve the tooth involved. No complementary surgical procedures were necessary for these areas. In addition, an ulectomy was performed in the region of teeth 17 and 27 to assist eruption. The postoperative period was uneventful.

 

 

 

Specimens were sent for histopathologic analysis, which confirmed the diagnosis of dentigerous cyst (Figure 5). After 12 months of follow-up, the teeth involved moved close to their ideal position (Figures 6, 7, 8 and 9). Both patients were referred to an orthodontist for orthodontic treatment.

 

 

 

 

 

 

Discussion and conclusions

DCs are benign odontogenic cysts surrounding the crowns of permanent teeth.¹ They may involve impacted, unerupted permanent teeth, supernumerary teeth and odontomas, and rarely involve deciduous teeth.^{3, 6, 11} The origin of DCs has been partially attributed to PTCH gene inactivation in a progenitor cell of the enamel organ.13 Some authors believe that PTCH alterations are necessary and may be the event that triggers the formation and growth of various cysts.^{14, 15} Other authors have reported a possible association between abnormal chromosome 1 and the occurrence of DCs.¹⁶ These assumptions suggest that genetic factors may be related to the pathogenesis of odontogenic cysts. In the present cases, the occurrence of such similar clinical conditions in homozygous twins was, at least, curious and rare, and genetic involvement cannot be ruled out.

Multiple DCs generally occur associated with syndromes such as mucopolysaccharidosis^{7, 8} and cleidocranial dysplasia.⁵ Both these diseases cause modifications in tooth development and may take part in the occurrence of multiple DCs^{17, 18} Furthermore, multiple mandibular cysts have previously been reported after prolonged use of cyclosporine A and calcium channel blockers;¹⁰ however, these are uncommon in the absence of a syndrome or systemic disease.⁹ The patients analyzed in this case had no clinical or laboratory findings that could point towards syndromes or other systemic changes.

Clinical and radiographic findings are almost conclusive in diagnosing DCs; however, the pathological analysis of the lesion is essential for a definitive diagnosis, especially in cases of multiple lesions, because other changes may share similar radiographic features. The odontogenic keratocyst tumor, for example, most commonly located in the mandibular body or ramus, as in the present case, can appear as multiple lesions in the basal cell nevus syndrome.¹⁷ That is an uncommon disorder characterized by numerous basal cell carcinomas, maxillary keratocysts and musculoskeletal malformations.^{17, 18} No microscopic and clinical features of this syndrome were found in the patients of the present case report.

The standard treatment for a DC is enucleation and removal of the cyst-associated impacted or unerupted tooth. However, larger lesions can be surgically drained and marsupialized to relieve the pressure within the cyst.¹⁹ In the present cases, enucleation was performed to treat the smaller lesions, and complete removal of the cyst was possible, without damaging the teeth. The larger lesions were marsupialized, which decreased the intracystic pressure, promoting the reduction of cysts and favorable bone formation. This minimally invasive procedure reduced the risk of large bone defects and paresthesia.

In addition, it had the advantage of preserving the tooth, allowing its spontaneous eruption. The possible disadvantage would be the presence of a cavity that needed care and irrigation.

In the present case, the good oral hygiene and cooperation of the patients contributed to the successful treatment. It is important to remember that, when marsupialization is chosen as the therapeutic option, a second surgical procedure may be necessary to remove remnants of the lesion, which did not happen in this case.

Although DCs are ranked as the second most common odontogenic cysts, their development as multiple lesions is unusual. The involvement of homozygous twins has not been previously reported. Radiographic examination for the investigation of unerupted teeth is essential to evaluate the presence of possible odontogenic cysts and tumors and aim for an early diagnosis of the lesion to preserve anatomical structures. Enucleation and marsupialization can be most successfully used as treatment.

 

References

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². Imada TS, Neto VT, Bernini GF, Silva Santos PS, Rubira-Bullen IR, Bravo-Calderòn D, et al. Unusual bilateral dentigerous cysts in a nonsyndromic patient assessed by cone beam computed tomography. Contemp Clin Dent. 2014;5:240-2.

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⁵. Zhang LL, Yang R, Zhang L, Li W, MacDonald-Jankowski D, Poh CF. Dentigerous cyst: a retrospective clinicopathological analysis of 2082 dentigerous cysts in British Columbia, Canada. Int J Oral Maxillofac Surg. 2010;39:878-82.

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¹³. Gondim JO , Neto JJ , Nogueira RL , Giro EM . Conservative management of a dentigerous cyst secondary to primary tooth trauma. Dent Traumatol. 2008;24:676-9.

¹⁴. Levanat S, Pavelic´ B, Crnic´ I, Oreskovic´ S, Manojlovic´ S. Involvement of PTCH gene in various inflammatory cysts. J Mol Med (Berl). 2000;78:140-6.

¹⁵. Pavelic´ B, Levanat S, Crnic´ I, Kobler P, Anic´ I, Manojlovic´ S, et al. PTCH gene altered in dentigerous cysts. J Oral Pathol Med. 2001;30:569-76.

¹⁶. Batra P, Roychoudhury A, Balakrishan P, Parkash H. Bilateral dentigerous cyst associated with polymorphism in chromosome 1qh+. J Clin Pediatr Dent. 2004;28:177-81.

¹⁷. Deboni MC, Brozoski MA, Traina AA, Acay RR, Naclério- Homem Mda G. Surgical management of dentigerous cyst and keratocystic odontogenic tumor in children: a conservative approach and 7-year follow-up. J Appl Oral Sci, 2012;20:268-71.

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^*Corresponding author:

Flaviana Soares Rocha

E-mail address: flavianasoares.rocha@gmail.com

 

Ethical disclosures

Protection of human and animal subjects. The authors declare that no experiments were performed on humans or animals for this study.

Confidentiality of data. The authors declare that they have followed the protocols of their work center on the publication of patient data.

Right to privacy and informed consent. The authors have obtained the written informed consent of the patients or subjects mentioned in the article. The corresponding author is in possession of this document.

 

Conflict of interest

The authors have no conflicts of interest to declare.

 

Article history:

Received 19 February 2019

Accepted 12May 2019

Available online 29 May 2019