Volume 1, Issue 2 , Pages 100-106, June 2006
Multiple unilateral maxillary dentigerous cysts in a non-syndromic patient: A case report and review of the literature
Article Outline
Summary
Dentigerous cysts are the most common developmental odontogenic cysts of the jaw, arising from impacted, embedded or unerupted teeth. Although mostly solitary, multiple cysts occur in association with syndromes such as mucopolysaccharidosis type VI, cleidocranial dysplasia, basal cell nevus syndrome, and Gardner's syndrome. Multiple cysts in non-syndromic patients are extremely rare, occurring almost exclusively in the mandible. We report the case of a four-year-old girl with painless right-sided facial swelling, diagnosed with two dentigerous cysts in the right maxillary sinus, who successfully underwent enucleation. To our knowledge this is the only case of multiple unilateral maxillary dentigerous cysts in a healthy, non-syndromic patient.
Keywords: Dentigerous cyst, Odontogenic cyst, Odontogenic tumor, Odontoma, Facial swelling
1. Introduction
Dentigerous cysts are benign odontogenic lesions arising from the completed crown of impacted, embedded or unerupted teeth, and are most common in the first two decades of life [1]. They are the most common developmental odontogenic cyst, and second most common cystic lesion of the jaws next to radicular cysts. Seventy-five percent of dentigerous cysts arise in the mandible [2]. Patients are typically asymptomatic, and when not detected early enough on dental radiography, they may progress to a considerable size with the patient presenting with painless facial swelling.
Dentigerous cysts are usually solitary, but are known to be multiple in patients with certain syndromes, such as mucopolysaccharidosis type VI and basal cell nevus syndrome, Gardner's syndrome and Cleidocranial dysplasia [2], [3]. There are limited cases of multiple cysts occurring in non-syndromic patients. Dentigerous cysts are also known to occur in association with other pathologic odontogenic entities such as mucoepidermoid carcinoma, ameloblastoma and squamous cell carcinoma [4]. Alternatively, in rare instances, dentigerous cysts may themselves arise from supernumerary teeth, or even from odontomas [5].
This report describes a case of an otherwise healthy four-year-old non-syndromic girl who presented with two dentigerous cysts in the right maxillary sinus.
2. Case
A four-year-old girl was referred to the Department of Otolaryngology for a painless facial swelling involving the right cheek. The swelling had progressed in size over the course of 9 months. Three months prior to presentation, the patient developed right nasal obstructive symptoms accompanied by right ocular infections. There were no visual disturbances or paresthesias. There was no history of dental procedure or dental/gingival changes, nor was there any weight loss, fatigue, or generalized neurological symptoms. The patient maintained all of her cognitive and functional abilities and continued to thrive. She was in otherwise good health without any previous significant medical history or associated syndromes.
Physical examination revealed a swelling overlying the right maxillary sinus. There was no nasal deviation, nor was there any apparent ocular pathology. There were no skin changes and the area was non-tender (Fig. 1). Intranasal speculum examination revealed a bulge in the right lateral nasal wall causing obstruction. Oral examination revealed normal dentition without any gingival pathology or trismus. A full physical examination did not reveal any other findings.
Fig. 1.
Pre-operative evaluation of the patient reveals a right-sided facial swelling overlying the right maxillary sinus.
2.1. Imaging
Computed tomography revealed two large cystic structures in the right maxillary region each associated with an isolated secondary tooth. There was a smaller anteromedial cyst causing protrusion of the anterior maxillary wall, with a larger lateral cyst deforming, but not invading the right orbital floor (Fig. 2). Magnetic resonance imaging revealed abnormal fluid signal intensity increasing in T2 and low in T1 with subtle rim enhancement and expansion/thinning of the right maxillary sinus. The right osteomeatal complex was completely obliterated without extension into the orbit (Fig. 3). The pantomogram did not aid in the diagnostic or therapeutic processes.
Fig. 2.
Computed tomographic images. (A) Axial section demonstrating presence of a smaller anterosuperior cyst and a larger lateral cyst associated with a tooth anteriorly and posteriorly, respectively. (B) Coronal section demonstrating two cysts residing in the right maxillary sinus with distinct septation and obliteration of osteomeatal complex.
Fig. 3.
Magnetic resonance imaging. (A) Axial T2-weighted image demonstrating two distinct hyperintense cystic lesions in the right maxillary sinus. (B) Coronal T2 weighted image demonstrating two hyperintense cystic lesions residing in the right maxillary sinus.
2.2. Management
Under general anesthesia, the patient underwent enucleation of the two right intramaxillary cysts and their respective teeth via a Caldwell-Luc approach and endoscopic sinus surgery (Fig. 4). Intraoperative findings revealed a third tooth in the maxillary sinus not associated with any cyst and was left in situ. The patient had an uneventful post-operative course and was discharged home the next day. Follow-up at 10 days showed good healing at the surgical sites without any paresthesias.
Fig. 4.
(A) Intra-operative image of a Caldwell-Luc approach with fenestration into the right maxillary sinus. (B) Impacted adult tooth associated with the larger lateral dentigerous cyst being removed.
2.3. Pathology
Histologic analysis revealed the presence of two dentigerous cysts each arising from a formed secondary tooth. Presence of tissue consistent with compound odontoma was also present, but not deriving from or giving rise to these cysts (Fig. 5).
Fig. 5.
Tissue microscopy. (A) Lining of dentigerous cyst epithelium. (B) Presence of enamel and dentin from compound odontoma. (C) Demonstration of cyst epithelium, enamel, and dentin.
3. Discussion
Dentigerous, or follicular cysts, are the second most common odontogenic cysts of the jaws after the periapical or radicular cyst, and make up an estimated 14–20% of all jaw cysts [6]. They are mostly associated with impacted, embedded or unerupted teeth, and most commonly arise from mandibular third molars, followed by maxillary third molars and maxillary canines [4]. In 5% of cases, they arise from supernumerary teeth, and in limited cases can arise from odontomas [5]. They are more prevalent in males and are most common in the first two decades of life, although they still are known to occur well into late adulthood [6].
The pathogenesis of these cysts is unknown. They are benign developmental epithelial-lined lesions that arise from fluid accumulation in the space between the reduced enamel epithelium and the completed tooth crown [1]. This accumulation may be secondary to obstructed venous return secondary to tooth impaction leading to transudation of fluid across capillary beds [7].
In most cases, dentigerous cysts are discovered incidentally on dental radiography, presenting as small radiolucent lesions. In these cases, the cysts are easily treated via surgery. Alternatively, were the impacted tooth to erupt, the cyst bursts and ceases to exist. Due to their large growth potential however, they can grow quite large when not detected at an early enough stage, potentially disrupting the surrounding dentition leading to root resorption, or even invasion and remodelling of the surrounding bone [3]. Despite this potential invasiveness, patients present with a progressive swelling that is almost invariably painless, unless the cyst becomes inflamed or infected.
3.1. Differential diagnosis
A broad range of conditions can lead to a clinical presentation of facial swelling, with categories ranging from neoplastic, inflammatory, cutaneous/infectious, allergic, or traumatic. Lesions falling in the category of odontogenic cyst or tumor as elements of the differential diagnosis will be discussed later.
Non-odontogenic developmental cysts such as nasolabial and nasopalatine duct cysts can present with a facial swelling, as can nonepithelial cysts such as aneurysmal bone cyst, and idiopathic bone cavity [1]. Other considerations should include soft-tissue sarcomas such as rhabdomyosarcoma and angiosarcoma, and hard-tissue sarcomas such as osteosarcoma. Still other possibilities are fibromyxoid chondroma, fibro-osseous lesions, chondrosarcoma, lymphoma and central giant cell granuloma. On imaging, and by virtue of the clinical history, many of these lesions can be ruled out.
3.2. Imaging
Radiographically, the dentigerous cyst is a unilocular radiolucent lesion with well-circumscribed sclerotic borders that are often radiopaque. The lesion is associated with the crown of an unerupted or impacted tooth and a diameter of at least 5
mm is postulated to be necessary to differentiate it from that of a normal follicular space [8]. Other odontogenic cysts such as radicular cysts and odontogenic keratocysts share similar radiographic characteristics in that they are also radiolucent and relatively common, although both are associated with an older demographic.
Certain odontogenic tumors are also radiolucent and must be included in the differential diagnosis. Ameloblastoma is the most common radiolucent tumor in this category, that can behave similarly to the dentigerous cyst, and whose unicystic variant, that is most prevalent in adolescence [4] and occurs as early as age 5, can be confused with the dentigerous cyst on microscopy [9]. The follicular subtype of adenomatoid odontogenic tumor is easily confused with dentigerous cysts before tissue diagnosis [10]. Odontoma, odontogenic fibroma, and cementomas all share radiologic features with the dentigerous cyst [2], as does ameloblastic fibroma, which occurs mostly in the pediatric age group [7]. Pindborg tumors, although rare and occurring mostly in middle-age, are radiolucent with well-circumscribed borders [2]. Given the similarity in radiographic presentations, some advocate the use of incisional biopsy to rule out the possibility of more aggressive lesions such as odontogenic keratocyst or cystic ameloblastoma that would necessitate a different surgical approach [6].
When cysts are especially large, with maxillary sinus involvement as in our patient, the pantomogram is often not of great aid. Computed tomographic scans provide superior bony detail, allowing for the visualization of the size and extent of the lesion with determination of orbital or nasal invasion or involvement. Again, with larger lesions, it also aids in planning of a surgical approach [1]. Mucoceles, retention cysts, and pseudocysts are also included in the differential diagnosis when a maxillary sinus cyst is visualized involving maxillary expansion; this is in addition to the array of radiolucent lesions mentioned above that can also be visualized on CT [2].
Magnetic resonance imaging is of lesser importance than CT scan with respect to bony detail, but can provide additional soft tissue information with respect to the nature of the lesion itself. On T1-weighted images, cystic lesions appear homogeneously hypointense, with T2-weighted images appearing hyperintense [2]. In patients such as ours, where the maxilla is involved, one can expect the associated teeth of the dentigerous cysts to appear hypointense on both T1 and T2 and lie eccentrically in the wall of the cyst [2]. Other hypointense lesions often found in the maxillary sinus include chronic secretions, air, acute hemorrhage and mycetoma but are found in the center of the sinus instead of the periphery [2].
3.3. Multiple cysts in the syndromic patient
Dentigerous cysts occur mostly as solitary lesions. Multiple cysts are rare, and when present, are commonly associated with an underlying syndrome. These include Maroteaux-Lamy syndrome (Mucopolysaccharidosis, type VI), cleidocranial dysplasia, basal cell nevus syndrome (Gorlin's syndrome), and Gardner's syndrome [2], [8].
The mucopolysaccharidoses are a group of diseases characterized by the inability to degrade mucopolysaccarides secondary to corresponding genetic defects. Type VI, or Maroteaux-Lamy syndrome is characterized by unerupted dentition, dentigerous cysts, malocclusions, condylar defects, and gingival hyperplasia secondary to impaired degradation of dermatan sulphate [8]. Although appearing healthy at birth, this condition is diagnosed in early childhood with short stature, corneal opacification, hepatosplenomegaly, facial dysmorphism, joint stiffness, including claw-hand deformity, and valvulopathy.
Cleidocranial dysplasia displays an autosomal dominant pattern of inheritance and leads to prolonged retention of deciduous teeth, delayed eruption of secondary dentition, unerupted supernumerary teeth and dentigerous cysts. Other features include maxillary micrognathia, frontal and parietal bossing, partial or complete absence of clavicles and short stature [8].
Gardner's syndrome is associated with the presence of multiple supernumerary teeth and multiple dentigerous cysts. Although its onset is in puberty with gastrointestinal polyps appearing later in life, it follows an autosomal dominant pattern of inheritance. Other features include epidermal cysts or osteomas.
Basal cell nevus syndrome (Gorlin-Goltz syndrome) is named for a constellation of multiple basal cell carcinomas, bifid ribs plantar/palmar pits, ectopic calcifications, and odontogenic keratocysts, although dentigerous cysts have been associated with this syndrome as well [11]. The presence of multiple odontogenic cysts, calcified falx cerebri, and multiple cutaneous nevi establishes a diagnosis of basal cell nevus syndrome [11].
Bilateral or multiple dentigerous cysts in non-syndromic patients are exceedingly rare. Only 15 cases have been reported in the literature thus far, with 13 involving the mandible exclusively, one involving the maxilla exclusively, and one involving both bones [2], [8], [11], [13]. The range of ages reported has been from 3 to 57, with only 2 of the 15 presenting with pain. One of these patients was thought to have developed these cysts secondary to cyclosporine A and calcium channel blocker use [12], and another patient was found to have chicken pox concurrently [13].
Our patient had been born at term without any perinatal complications or pathologic diagnoses. She had been followed from birth on a regular basis by a pediatrician, and had cognitive and functional abilities at par or superior to girls her age. Based on a lack of patient history, family history, or physical findings relating to systemic or syndromic pathology, we were able to rule out the aforementioned syndromes, which would have otherwise been present.
Thus far, there has not been a case reported of unilateral maxillary multiple dentigerous cysts in an otherwise healthy non-syndromic patient. Our case is, to the best of our knowledge, the first to be reported.
3.4. Dentigerous cysts associated with other lesions
Dentigerous cysts are lined by a layer of non-keratinized stratified squamous epithelium with a surrounding wall of thin connective tissue containing odontogenic epithelial rests. There are a number of lesions that can develop within the cyst or from components of its wall. Odontogenic tumors such as ameloblastoma or epidermoid carcinoma have been known to be derived from the epithelial lining of the cyst1, with 17% of ameloblastomas arising from dentigerous cysts [4]. Squamous cell carcinoma may also occur [4]. Mucoepidermoid carcinoma occasionally develops from mucous cells sometimes present in the epithelial wall [1], [4].
In contrast to the above lesions that can arise from a dentigerous cyst, there are different structures or lesions that can give rise to the dentigerous cyst. Impacted, or unerupted secondary teeth are invariably the most common sources. In rare instances, dentigerous cysts can arise from supernumerary teeth, or even from odontomas of the complex type [5]. In our case both dentigerous cysts arose from formed secondary teeth. It is important to note that the associated tissue diagnosed as being that of compound odontoma did not give rise to the dentigerous cysts but was an incidental finding.
Odontoma is a hamartoma that arises from ameloblasts and odontoblasts, which in turn forms enamel and dentin, respectively in an abnormal pattern. They can be classified as compound, which is more common, and complex. In compound odontoma, the enamel and dentin form tooth-like structures. In complex odontoma, the dental tissues are more disorganized. In one study, compound odontomas were reported to be associated with dentigerous cysts, in approximately 9% of cases [14]. Despite this apparent association, there are few cases in the literature relating these lesions.
3.5. Treatment
Enucleation has been the standard treatment for dentigerous cysts along with extraction of the associated tooth. This is necessitated by the cyst's potential to displace or block eruption of surrounding teeth, destroy or cause fracture of bone, or invade nervous structures [6]. In the child however, the loss of permanent dentition can lead to functional, cosmetic and psychological consequences. Some authors advocate a more conservative approach, evaluating individual treatment on a case by case basis, based on cyst size, patient age, extent of cyst invasion of surrounding structures, along with functional and cosmetic significance of the impacted tooth itself [6]. Treatment alternatives would include marsupializing the cyst before enucleation, or leaving the cyst-associated tooth in place followed by orthodontic traction and eventual eruption [6].
Whatever the surgical approach used, a tissue analysis is essential in making a final diagnosis. Enucleation allows for histologic examination of the entire epithelial lining which more conservative approaches do not. This is important given that other neoplasms such as squamous cell carcinoma may arise from the dentigerous cyst, that may be missed if only a limited area of cystic tissue is examined. Dentigerous cysts recur in very rare instances.
3.6. Conclusion
In this case report, we describe a four-year-old non-syndromic girl in otherwise good health, diagnosed as having two dentigerous cysts residing in the right maxillary sinus leading to painless facial swelling. Tissue microscopy confirmed the diagnosis of dentigerous cysts arising from formed secondary dentition. In addition to this, was a microscopic diagnosis of compound odontoma. To our knowledge this is the first case of multiple unilateral maxillary dentigerous cysts to be reported in a healthy non-syndromic patient. It is still unclear as to what will come of the impacted tooth not associated with cystic material that was left in situ.
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PII: S1871-4048(05)00018-3
doi:10.1016/j.pedex.2005.12.005
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 2 , Pages 100-106, June 2006
