The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): a case report

Maroteaux-Lamy syndrome is one of the genetic disorders involving disturbances in mucopolysaccharide metabolism resulting in increased storage of acid mucopolysaccharide in various tissues. The basic defect in Maroteaux-Lamy syndrome is a deficiency of arylsulfatase B, which leads to accumulation of dermatan sulfate in tissues and their urinary excretion. The deposition of mucopolysaccharides leads to a progressive disorder involving multiple organs that often results in death in the second decade of life. This disease, which has several oral and dental manifestations, is first diagnosed on the basis of clinical findings. A large head, short neck, corneal opacity, open mouth associated with an enlarged tongue, enlargement of skull, and a long antero-posterior dimension are the main characteristic features. Dental complications can be severe and include unerupted dentition, dentigerous cystlike follicles, malocclusions, condylar defects, and gingival hyperplasia. An 11-year-old boy with Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is described in this article, with special emphasis on the oral manifestations.     

A pigmented dentigerous cyst in a patient with multiple dentigerous cysts of the jaws: a case report

AIM: The aim of this article is to report an unusual and interesting case of non-syndromic multiple dentigerous cysts of the maxilla and mandible associated with melanin pigment and melanocytes in the epithelial lining in one of the cysts. In addition, embryologic aspect and origin of melanocytes are briefly discussed. BACKGROUND: Dentigerous cysts are the second most common odontogenic cysts after radicular cysts. They are usually solitary with multiple cysts reported on occasion in association with syndromes. While melanocytes and melanin-pigment are widely distributed in the skin, the nervous system, certain types of mucosa, uveal tract, and other areas, they are not normally present within bone in mammals. REPORT: A 37-year-old Iranian male was referred to an oral surgeon by his dentist for evaluation and treatment of multiple cyst-like radiolucent lesions around the crowns of some unerupted permanent teeth in both maxillary and mandibular anterior areas. The biopsy revealed numerous granules of melanin-pigment distributed throughout the epithelial lining of one of these cysts in addition to the characteristic histopathologic features of adentigerous cyst. Histochemical analysis confirmed the pigment was melanin, and many of the cells containing melanin were immunohistochemically positive for s-100 protein. SUMMARY: Although the specific origin and pathologic significance of the melanocytes described here cannot be explained, it may be of interest for future investigations.     

Two dentigerous cysts in the mandible of one patient. Case report

A bstract — This paper reports a case of two separate dentigerous cysts in the mandible of one patient. An unusual feature was the relationship of one of the cysts to an unerupted lateral incisor. The prudence of accounting for any missing teeth in patients and the value of panoramic radiographic screening is illustrated.     

细胞角蛋白(CK)13在含牙囊肿衬里上皮的表达

目的 研究纤毛柱状上皮向鳞状上皮化生时细胞角蛋白13的表达，探讨其表达在上皮化生方面的意义。方法 取54例含牙囊肿和6例鼻腭管囊肿，分别采用细胞角蛋白13单克隆抗体进行免疫组织化学染色、HE染色和RT-PCR方法研究细胞角蛋白13基因的表达。结果 54例含牙囊肿中，46例为复屡鳞状上皮细胞衬里，CK13均呈阳性表达；8例混合性上皮衬里，其中的鳞状上皮细胞部分CK13也呈阳性表达；纤毛柱状上皮部分和6例鼻腭管囊肿的纤毛柱状上皮细胞均呈阴性表达。RT-PCR结果显示细胞角蛋白13基因在复屡鳞状上皮中表达最强，在混合性上皮中表达减弱，在纤毛柱状上皮中表达极弱。结论 细胞角蛋白13基因（CK13-mRNA）在纤毛柱状上皮、混和性上皮和复屡鳞状上皮三种不同上皮衬里中均有表达，但表达强度不同。呈由弱到强的变化。在鳞状上皮细胞中细胞角蛋白13呈强阳性表达；在纤毛柱状上皮细胞中细胞角蛋白13呈阴性；可是，当纤毛柱状上皮中出现鳞状上皮细胞时，可见细胞角蛋白13呈阳性表达，因而，细胞角蛋白13是纤毛柱状上皮细胞向鳞状上皮细胞化生过程中的标志性产物，但是有待进一步验证。     

Experimental dentigerous cysts and enamel hypoplasia: their possible significance in explaining the pathogenesis of human dentigerous cysts

Abstract Cysts lined by epithelium were often found in association with tooth-germ isografts in hamster cheek pouch. They developed from odontogenic epithelium and were in close relation to the crowns of involved teeth. Cysts associated with tooth-germ isografts from 5-day-old animals (17 out of 36) commenced their formation shortly after transplantation as a result of enamel organ degeneration. Teeth associated with these cysts often showed enamel hypoplasia. Accordingly, 86 teeth involved in human dentigerous cysts were examined and 43 were found to possess areas of enamel hypoplasia on their occlusal surfaces or incisal edges. It is suggested that the pathological process initiating cystic degeneration in the enamel organ was also accompanied by degeneration of ameloblasts. When tooth-germs from 2-day-old hamsters were transplanted, cystic spaces developed only after completion of enamel formation, 6 weeks following transplantation (in six out of 11 transplants), as a result of separation between the cells of the reduced enamel epithelium. Enamel hypoplasia was not a conspicuous feature. These experimental and clinical observations suggest that there may be at least two types of dentigerous cyst, perhaps with different causes, arising at different stages of tooth development.     

Multiple odontogenic keratocysts in a patient with type II (mitis) Ehlers-Danlos syndrome

Ehlers-Danlos syndrome (EDS) is now considered to be a heterogeneous collection of fundamentally distinct disorders. The many and varied sub-types of EDS have been distinguished using clinical, genetic and in some cases biochemical criteria and this has important implications as regards prognosis and the prediction of inheritance patterns. The major clinical features of EDS are reviewed, with particular emphasis placed on the possible complications encountered in the practice of oral surgery. A case of Type II mitis EDS is described, that presented with multiple odontogenic keratocysts.     

Evaluation of mast cells in periapical cysts, dentigerous cysts, and keratocystic odontogenic tumors

J Oral Pathol Med (2012) 41 : 630–636 Background: Several cell types are associated with the development of cystic and tumoral odontogenic lesions. Among inflammatory cells, mast cells can be associated with their pathogenesis. The aim of this study was to analyze mast cells in periapical cysts, dentigerous cysts, and keratocystic odontogenic tumors. Methods: Tissue sections were submitted to toluidine blue staining and immunohistochemistry with antibody anti‐tryptase (clone G3). Mast cells were quantitated using Image‐Pro Plus software to obtain the mean number of mast cells in three regions: epithelial, superficial portion of the fibrous wall and deep portion of the fibrous wall from 20 periapical cysts, 20 dentigerous cysts (six non‐inflamed and 14 inflamed) and 20 keratocystic odontogenic tumors (four non‐inflamed and 16 inflamed). Results: The mean number of mast cells detected per lesion by immunohistochemistry (4.1) was higher than by histochemistry (1.5) (P < 0.0001). Inflamed dentigerous cysts and keratocystic odontogenic tumors showed a higher mean number of mast cells than non‐inflamed lesions in all regions. The deep region from all cysts showed the highest mean number of degranulated mast cells, except for non‐inflamed keratocystic odontogenic tumors analyzed by immunohistochemistry. Conclusions: Immunohistochemical staining detected higher number of mast cells than histochemistry. The higher number of mast cells observed in inflamed lesions could indicate the participation of these cells in the inflammatory response in odontogenic lesions. The prevalence of degranulated mast cells in the deep region suggests intense activity of these cells, possibly related to growth of cystic lesions.     

Clinicopathologic features of dentigerous cysts in the maxillary sinus

Dentigerous cyst (DC) in the maxillary sinus is extremely rare. This study evaluated the clinical features, etiologic factors, imaging, treatment, and signs and symptoms of this type of DC. Clinical symptoms, radiographic findings, surgical methods, and histopathologic findings of 4 patients with DCs in the maxillary sinus were evaluated. In addition, we reviewed the literature reports of DCs in the maxillary sinus over the past 40 years. Our 4 patients with DC in the maxillary sinus showed the following results: (1) all the patients were males at first 3 decades of age, (2) swelling and/or yellow-green pus discharges from pharyngeal cavity were common symptoms, (3) the involved teeth in the DC were the maxillary third molar teeth and supernumerary teeth, and (4) Caldwell-Luc approach was performed in these 4 patients. The patients with DC in the maxillary sinus should be evaluated thoroughly by extraoral and intraoral examinations, proper diagnostic imaging procedures, and pathologic examination to avoid misdiagnosis of maxillary sinusitis. The standard treatment for DC in the maxillary sinus is often removed by Caldwell-Luc approach. Furthermore, marsupialization or functional endoscopic sinus surgery may be performed because of the size, location of the cysts, or the age of the patients.     

Comparison of angiogenesis in keratocystic odontogenic tumours, dentigerous cysts and ameloblastomas

Objective:  The aim of the present study was to evaluate and compare angiogenesis in keratocystic odontogenic tumours, dentigerous cysts (DCs) and ameloblasomas using monoclonal antibody against CD34.
Materials and methods:  Microvessel density was assessed in a total of 53 cases including 20 keratocystic odontogenic tumours, 13 DCs and 20 ameloblastomas (14 solid and six unicystic variants). Microvessel density was expressed as the mean number of microvessels per high-power-field.
Results:  Statistically significant differences in mean microvessel density were observed between keratocystic odontogenic tumours, DCs and solid ameloblastomas ( P  < 0.001). Mean microvessel density was significantly higher in solid ameloblastomas compared with both keratocystic odontogenic tumours and DCs; and was also significantly higher in keratocystic odontogenic tumours than in DCs.
Conclusion:  Within the limitations of the present study, it can be suggested that angiogenesis may be one of the mechanisms possibly contributing to the different biological behaviours of keratocystic odontogenic tumours, DCs and solid ameloblastomas.     

Dental findings in mucopolysaccharidosis type IV A (Morquio's disease type A)

Dental examinations on nine patients with mucopolysaccharidosis type IV A (MPS IV A, Morquio's disease type A) were carried out. Detailed medical, radiologic, and biochemical studies of each case were also performed independently. Dental changes were present in all cases, although the severity varied. The severity of the dental changes did not correlate with the clinical or biochemical findings in all cases. These dental changes are seen only in MPS IV A (N-acetylgalactosamine-6-sulphate sulphatase deficiency) and are not found in MPS IV B (beta-galactosidase deficiency) or the recently delineated MPS IV C (enzyme defect unknown). The dental changes can aid in the diagnosis of patients affected by MPS IV A and are especially useful in mild atypical cases.     

Metallothionein in the radicular,dentigerous, orthokeratinized odontogenic cysts and in keratocystic odontogenic tumor

J Oral Pathol Med (2011) 40 : 270–276 Background: Metallothionein (MT) is a protein correlated with cellular differentiation and proliferation, as well as with the inhibition of apoptosis. The aims were to report and to compare the MT expression in odontogenic cysts and keratocystic odontogenic tumor (KOT); to correlate the MT with cellular proliferation; and to evaluate the influence of the inflammation in MT. Methods: Nine cases of radicular cyst (RC), nine dentigerous cyst (DC), four orthokeratinized odontogenic cyst (OOC), and eight KOT were submitted to immunohistochemistry using anti‐MT and anti‐Ki‐67. Indexes of MT (IMT) and Ki‐67 (IK) were obtained. Lesions were grouped according to inflammation: mild‐to‐moderate (group A) and intense (group B). Results: IMT proved to be highest in RC (91%), followed by DC (89%), KOT (78%), and OOC (63%). IMT was inversely correlated with IK in KOT, and OCC, but was positively correlated with RC and DC. No differences in IMT and in IK could be observed between groups A and B. Conclusions: The higher IMT found in RC and DC compared to OCC and KOT, as well as the differences between the last ones, is possibly correlated with their different histopathological features and clinical behavior. In RC and DC, MT may play a role in cellular proliferation. However, it seems that MT is either less or is not related to proliferation in OOC and in KOT. Moreover, inflammation does not seem to alter IMT and IK.