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Year : 2015  |  Volume : 5  |  Issue : 2  |  Page : 133-139

Multiple cysts of jaw and amelogenesis imperfecta - An unusual case report

Department of Oral Medicine and Radiology, KVG Dental College and Hospital, Sullia, Karnataka, India

Date of Web Publication10-Mar-2016

Correspondence Address:
Dr. Manaswita Tripathy
C/O Dr. Kailash Nath Tripathy, Plot No. 670/1187, Sum Bihar, Nuagaon, Post - Mallipada, Bhubaneswar - 751 003, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2231-6027.178502

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Radicular cyst is the most common odontogenic cyst occurring in the jaws. The cyst is commonly found in relation to the maxillary anterior teeth in the third and fifth decade of life. It arises from epithelial remnants and has been classified as inflammatory cyst, as a consequence of pulpal necrosis following caries, with an associated periapical inflammatory response. Hereditary defects like amelogenesis imperfecta can also lead to early loss of enamel, causing pulp exposure and necrosis, which ultimately may lead to radicular cyst. Radiographically, radicular cyst appears as a round or oval, well-circumscribed radiolucent image involving the apex of the tooth. This paper presents a case of multiple radicular cyst and amelogenesis imperfecta.

Keywords: Amelogenesis imperfect, computed tomography, radicular cyst

How to cite this article:
Tripathy M, Srivastava S, Chethan B R, Dsilva J. Multiple cysts of jaw and amelogenesis imperfecta - An unusual case report. Int J Oral Health Sci 2015;5:133-9

How to cite this URL:
Tripathy M, Srivastava S, Chethan B R, Dsilva J. Multiple cysts of jaw and amelogenesis imperfecta - An unusual case report. Int J Oral Health Sci [serial online] 2015 [cited 2023 Jun 1];5:133-9. Available from: https://www.ijohsjournal.org/text.asp?2015/5/2/133/178502

  Introduction Top

Radicular cysts are the most common inflammatory cysts arising from the epithelial remnants in the periodontal ligament following necrosis of the pulp, as a result of caries. But trauma in the form of blunt force or thermal injury during dental procedures can also be other possible causes. Most commonly found at the apices of the involved teeth, but may also be found on the lateral aspects of the roots in relation to lateral accessory root canals. Quite often a radicular cyst remains behind in the jaws after removal of the offending tooth and this is referred to as a residual cyst. Multiple radicular cysts can occur in an individual with multiple carious teeth, poor oral hygiene, genetic and dental hereditary defect.[1] Radiographically most of the radicular cyst appears as round and pear shaped, unilocular, radiolucent lesion in the periapical region.[2]

  Case Report Top

A 40 year old female reported to the outpatient department with a chief complaint of painless swelling in the right upper gum region since 2 years [Figure 1]. The history of present illness revealed that the swelling was insidious in onset, small in size and gradually increased over a period of 2 years. It was associated with pus discharge since 3 to 4 months. Patient had undergone removal of teeth in the right upper front teeth region 9 years back as they were decayed. Before patient had a habit of arecanut chewing with tobacco 5-6 times daily for 10 years. She had quit the habit since 6 years.
Figure 1: Swelling of the right side of face

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On extra oral examination the swelling was present on the right side of the face with obliteration of the right nasolabial fold [Figure 2]. Left submandibular lymph node was palpable and nontender. On inspection a solitary diffuse swelling of size 5 × 4.5 cm roughly oval in shape can be seen on the right side of the face. Overlying surface was apparently normal in appearance. Swelling was nontender, soft to firm in consistency.
Figure 2: Obliteration of right nasolabial fold

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On intra oral examination a solitary diffuse swelling of size 5 × 3.5 cm can be seen involving the edentulous ridge from 14 to root stump of 17 [Figure 3]. Overlying mucosa was slightly erythematous. Sinus opening can be seen with respect to edentulous ridge of 14. Another solitary diffuse swelling of size 3.5 × 1.5 cm can be seen [Figure 4] extending from 23 to 26. Sinus opening can be seen on the gingiva in relation to 23. Both the swellings were nontender, bony hard in consistency.
Figure 3: Swelling present on right edentulous ridge

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Figure 4: Swelling present on 23 region

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Patient also had multiple clinically missing teeth. Generalized yellowish discolouration of teeth, pulpal exposure and severe abrasion with loss of vertical dimension was also noticed [Figure 5]. Based on the clinical findings a provisional diagnosis of infected residual cyst in relation to missing 14, infected radicular cyst with respect to 23 and amelogenesis imperfecta was given. Differential diagnosis of Radicular cyst and Dentigerious cyst was considered for the swelling present on the right side.
Figure 5: Yellowish discolouration of teeth with multiple pulpal exposure and loss of vertical dimension

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Fine needle aspiration from the 14, 15 edentulous ridge region was carried out which showed glistening straw coloured fluid [Figure 6]. The smear showed predominantly neutrophils admixed with lymphocytes, clusters and sheets of hemosiderin laden macrophages in a background of few RBCs, cholesterol crystals and proteinaceous material. Biochemical analysis revealed Total protein: 359 mg/dl, Sugar: 34 mg/dl, Cholesterol: 238 mg/dl.
Figure 6: Fine needle aspiration showing glistening straw coloured fluid

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Panoramic radiograph [Figure 7] revealed multiple root stumps with obliteration of pulp chambers, impacted 18, 27, 36, 46. A large well defined radiolucency of size approximately 3 × 4.5 cm in size, with well corticated border was noted filling the entire right maxillary sinus, the origin of which was not clear, but seems to arise from edentulous alveolar ridge wrt missing 14. Multiple, small, well defined radiolucencies with well corticated border were noticed at the apex of 15, 18, 23, 24, 25, 36.
Figure 7: Panoramic radiographs showing multiple cysts

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Intra oral periapical radiograph (IOPAR) in relation to right anterior edentulous alveolar ridge [Figure 8], which revealed a large well-defined radiolucency of size approximately 3 × 4 cm with partial sclerotic border, origin of which couldn't be appreciated in the radiograph. IOPAR taken with respect to 17 showed root stump and impacted 18. Ill defined radiolucency with loss of lamina dura was seen at the periapical region of 17 and 16 with furcation involvement which was suggestive of periapical abscess. A well defined radiolucency of size approximately 2 × 2.5 cm on the mesial aspect of 18 extending from the cervical part till the apex with a well corticated border, suggestive of periapical cyst in relation to 18 [Figure 9]. Intra oral periapical radiograph taken wrt 22 showed well defined radiolucency of size approximately 3 × 2.5 cm, with well corticated border, suggestive of periapical cyst [Figure 10].
Figure 8: IOPAR of right anterior edentulous ridge

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Figure 9: IOPAR wrt 18

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Figure 10: IOPAR wrt 22

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Maxillary anterior cross sectional occlusal radiograph [Figure 11] revealed expansion of the buccal cortex, on the right side maxilla, with elevation of the floor of the right maxillary sinus.
Figure 11: Maxillary anterior cross sectional occlusal radiograph

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Computed tomography (CT) revealed multiple well defined peripherally enhancing, expansile, unilocular lytic lesions (?Radicular cyst) arising from the maxillary alveolar ridge [Figure 12] and [Figure 13]. The largest lesion with bony wall was noted, of size 32 × 30 × 40 mm extending superiorly into the right maxillary antral cavity. A thin bony plate was well delineated between the lesion and the maxillary sinus, suggesting extra antral origin of the lesion. The root of the right central incisor was noted within the lytic lesion. The lesion was displacing the adjacent teeth and causing bony remodeling of the maxilla (with cortical erosions) and walls of the maxillary sinus. [Figure 14] and [Figure 15] shows three dimensional reconstructions of CT images.
Figure 12: Axial CT scan showing unilocular cystic lesions on the maxillary ridge

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Figure 13: Coronal CT showing large unilocular cyst arising from 11 and 23

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Figure 14: Reconstructions showing perforation of the buccal cortex

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Figure 15: Reconstructions showing perforation of the buccal cortex

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Extraction of 11 and 22 was carried out along with surgical curettage, and the specimen was subjected to histopathological examination. Histopathological examination revealed [Figure 16] mainly connective tissue and few areas of epithelium. Connective tissue was densely collageneous with spindle shaped fibroblasts, inflammatory infiltrate comprising of lymphocytes and plasma cells. Few blood vessels were also noticed. Based on the radiological findings and histological report final diagnosis of infected radicular cyst wrt 11 and 22 were given. Enucleation of all the cysts was advised.
Figure 16: Histopathological examination showing mainly connective tissue and few areas of epithelium

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  Discussion Top

Kramer (1974) has defined a cyst as 'a pathological cavity having fluid, semifluid or gaseous contents and which is not created by the accumulation of pus'. Most cysts, but not all, are lined by epithelium.[3] Radicular cysts are the most common inflammatory cysts and arise from the cell rest of malassez in the periodontal ligament following death and necrosis of the pulp as a result of caries or trauma. The other sources of epithelium, such as crevicular epithelium, sinus lining, or epithelium lining of fistulous tracts, also have been implicated in development of radicular cyst.[4] Radicular cyst is also known as periapical cyst, periodontal cyst, root end cyst or dental cyst. The incidence of radicular cysts is 0.5-3.3% of the total number in both primary and permanent dentition.[5] Very few cases are seen in the first decade, after which there is a fairly steep rise, with a peak frequency in the third decade. There are large numbers of cases in the fourth and fifth decades, after which there is a gradual decline. It is more commonly found in males than in females.[6] Radicular cysts are painless unless infected. They occur in all tooth-bearing areas of the jaws (60% are found in the maxilla and 40% in the mandible). There is a particularly high frequency in the maxillary anterior region.[3]

Pathogenesis of radicular cysts has been described as comprising of three distinct phases: The phase of initiation, the phase of formation and the phase of enlargement.[7]

Phase of initiation

The epithelial linings of radicular cysts are derived from the epithelial cell rests of Malassez in the periodontal ligament. These cells come to lie in periapical granulomas associated with teeth with necrotic, often infected, pulps. The dormant cell rests of Malassez begin to proliferateas a direct effect of inflammation probably under the influence of bacterial antigens. A key factor, which may initiate the inflammation and immune response, is now thought to be Bacterial Endotoxins released from the necrotic pulp. Endotoxins result in production of cytokines with pro-inflammatory and bone-resorbing activities.

Phase of cyst formation

This is the process by which a cavity comes to be lined by the proliferating odontogenic epithelium. There are two main theories regarding the formation of the cyst cavity:Firstly, 'The nutritional deficiency theory'. It is based on the assumption that the central cells of the epithelial strands become removed from their source of nutrition and undergo necrosis and liquefactive degeneration. The accumulating products in turn attract neutrophilic granulocytes into the necrotic area. The microcavities containing degenerating epithelial cells, infiltrating mobile cells and tissue fluid coalesce to form the cyst cavity lined by stratified epithelium.[8]'The abscess theory' - It postulates that the proliferating epithelium lines an abscess cavity formed by tissue necrosis and lysis because of the innate nature of the epithelial cells to cover exposed connective tissue surfaces.[9]

Phase of enlargement

The presence of necrotic tissue in the cyst lumen attract neutrophilic granulocytes, which extravasate and transmigrate through the epithelial lining into the cyst cavity. The lytic products of the dying cells in the cyst lumen release a greater number of molecules. As a result, the osmotic pressure of the cyst fluid rises to a level higher than that of the tissue fluid. The tissue fluid diffuses into the cyst cavity so as to raise the intra luminal hydrostatic pressure well above the capillary pressure. The increased intra cyst pressure may lead to bone resorption and expansion of the cyst.

Radicular cysts are usually asymptomatic and are left unnoticed, until detected by routine radiographic examination. Long standing cases radicular cyst may undergo an acute exacerbation of the cystic lesion and develops signs and symptoms such as swelling, tooth mobility and displacement of unerupted tooth.[10] Associated teeth are always non-vital and may show discoloration.[11] In the present case the swelling was long standing i.e., of two years, which became infected over a period of time, all the teeth were nonvital and showed yellow discolouration with loss of crown structure.

Possible reasons for development of radicular cyst

In the past, dental caries were more frequent in maxillary incisors than other teeth, had silicate restorations, with consequent high risk to their pulps. Second, there is the high prevalence of palatal invaginations in the maxillary lateral incisors. Third, maxillary anterior teeth are probably more prone than others to traumatic injuries which may lead to pulp death.[3] Radicular cysts are probably the most common cause of swelling of the jaws. These are slowly enlarging swellings. In the maxilla there may be buccal or palatal enlargement, whereas in the mandible it is usually labial or buccal and only rarely lingual. Radicular cyst is the related presence of a tooth with a non-vital pulp, occasionally; a sinus may lead from the cyst cavity to the oral mucosa.

Sometimes more than one radicular cyst may be found in a patient. It is believed that there are cyst prone individuals who show a particular susceptibility to develop radicular cysts. It is possible that an immune mechanism may inhibit cyst formation in most individuals and that cyst-prone subjects have a defective immunological surveillance and suppression mechanism. It is also possible that some individuals have a genetic tendency to develop radicular cysts. Multiple radicular cysts may also be seen in patients with hereditary dental defects (e.g., multiple dens-in-dente or dentinogenesis imperfecta), but in these cases this is because of morphological defects resulting in early exposure and death of the pulp.

Amelogenesis imperfect can also lead to multiple radicular cysts as seen in the present case. Amelogenesis imperfecta (AI) is a hereditary disorder that expresses a group of conditions that cause developmental alterations in the structure of enamel.[12] In general, it affects all or nearly all of the teeth in both the primary and permanent dentitions.[13]

The most widely accepted classification 13 of AI includes four types: Hypoplastic, hypomaturation, hypo-calcified, and hypomaturation-hypoplastic with taurodontism.[14] Clinical features of AI may include low caries susceptibility, rapid attrition, excessive calculus deposition, and gingival hyperplasia. Other pathologies associated with AI are enlarged follicles, impacted permanent teeth; ectopic eruption, congenitally missing teeth, crown and/or root resorption, and pulp calcification.[15] The present case had gross detoriation of tooth structure due to rapid generalized abrasion. In the present case 18, 27, 36, 46 were impacted, along with that root resortion with obliteration of pulp chambers were noticed in multiple teeth.

Radiographic features of radicular cyst

In most cases the epicenter of a radicular cyst is located approximately at the apex of a nonvital tooth. Occasionally it appears on the mesial or distal surface of a tooth root, at the opening of an accessory canal. They are round or ovoid radiolucencies. The periphery usually has a well defined cortical border. In infected or rapidly enlarging cysts, the radiopaque margin may not be present. The outline of a radicular cyst usually is curved or circular unless it is influenced by surrounding structures such as cortical boundaries. Most of the radicular cysts are radiolucent. Occasionally, dystrophic calcification may develop in long-standing cysts, appearing as sparsely distributed, small particulate radiopacities. A large radicular cyst can cause displacement and resorption of the roots of adjacent teeth. In rare cases the cyst may resorb the roots of the related non-vital tooth. The cyst may invaginate the antrum, but there should be evidence of a cortical boundary between the contents of the cyst and the internal structure of the antrum. Cysts may displace the mandibular alveolar nerve canal in an inferior direction. The outer cortical plates of the maxilla or mandible may expand in a curved or circular shape.[16]

Histologically it is lined by stratified squamous epithelium. In the majority of cases the epithelium is from 6 to 20 cell layers thick, but may be up to 50 cell layers thick in some areas. The lumen is filled with fluid and cellular debris. On occasion, the wall of the cyst consists of dense fibrous connective tissue. Often with an inflammatory infiltrate containing lymphocytes variably intermixed with neutrophils, plasma cells, histiocytes, and (rarely) mast cells and eosinophils are seen. The epithelial lining may demonstrate linear or arc-shaped calcifications known as Rushton bodies. Dystrophic calcification, cholesterol clefts with multinucleated giant cells, red blood cells, and areas of hemosiderin pigmentation may be present in the lumen, wall or both.[17]

The choice of treatment may be determined by some factors such as extension of the lesion, relation with anatomic structures, origin, clinical characteristics of the lesion, cooperation and systemic condition of the patient.[18] Treatment options for radicular cysts can be conventional nonsurgical RCT when lesion is localized or surgical treatment like enucleation, marsupialization or decompression when the lesion is large.[19] In the present case report surgical enucleation of multiple radicular cysts under general aneasthesia was carried out.

  Conclusion Top

To conclude, a radicular cyst is a common condition found in the oral cavity. Multiple radicular cysts can occur in an individual with genetic tendency, defective immunological surveillance and suppression mechanism, hereditary dental defects. Amelogenesis imperfecta especially hypomaturation type, in advance cases, causes grossly worn tooth structure, severe abrasion and obliteration of pulp chamber as seen in this case. Necrosis of exposed pulp tissue can ultimately lead to radicular cyst formation. All cases of amelogenesis imperfecta, especially hypoclcified type should undergo proper radiological evaluation to rule out presence of any radicular cyst.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16]

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