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Kenny-Caffey Syndrome: A Radiology Case Report
e52
OOOOE April 2008
Abstracts
doses of common extra- and intraoral imaging techniques have increased as much as 186% from 1990 calculations. Discussion. Change in the assessment of risk may warrant a reassessment of examination frequency or the type of examination that is used. Dental radiographs are considerably more risky than previously thought.
APPLICATIONS OF CONE-BEAM COMPUTERIZED TOMOGRAPHY IN ENDODONTICS. J. Éthier, T. Maghsoodi, M. Noujeim, R. Langlais, and J. Preece, University of Texas Health and Science Center, San Antonio. Background. Cone-beam computerized tomography (CBCT) offers the prospect of more effective diagnosis and therefore improved treatment planning. Its role in periodontics, prosthodontics, oral surgery, and orthodontics has been acknowledged and is now being used on an everyday basis. These cases demonstrate the advantage of CBCT in the accurate diagnosis of complex endodontic cases. Objectives. Traditionally, an endodontic lesion is evaluated with conventional periapical radiographs. By changing the angulation of the beam, the dentist may evaluate the possibility of another root, an auxiliary canal, or a radicular fracture. With the advent of high-resolution CBCT, one can achieve greater accuracy, therefore both saving time and providing the highest standard of care. We examined 2 patients who presented to the dental clinic for an endodontic consultation. In both cases, radiolucencies were detected on periapical radiographs. The patients were referred to the radiology department for additional assessment with CBCT. Materials and methods. High-resolution 3-dimensional imaging technique was used in both cases. The initial treatment planning was done with periapical radiographs using a Gendex x-ray unit and a Schick sensor. In the first case, the CBCT was acquired using the Morita Accuitomo 3DX. In the second case, the volume was acquired with the Planmeca Promax 3D. Results. In the first case, the use of CBCT prevented the retreatment of a lingually fractured tooth. In the second case, the CBCT revealed a deviation from the norm, where an endodontic treatment could have been prevented. Discussion. In the first case, the initial diagnosis was determined as an endodontic failure necessitating retreatment. In the second case, the initial diagnosis was internal resorption, it was then altered to incisive canal cyst. Because of lingering uncertainty in these cases, the CBCT scans were acquired to further investigate the “lesions.” Conclusion. Dentists are very often faced with difficult cases to resolve and have to make their diagnosis with conventional techniques that can lead to misdiagnosis and result in unneeded root canal therapy. With the use of CBCT, misdiagnosis resulting in inappropriate treatment might be overcome.
KENNY-CAFFEY SYNDROME: A RADIOLOGY CASE REPORT. A. Hunter, T. Maghsoodi, S. Lavasani, M. Noujeim, and R. Langlais, Department of Dental Diagnostic Science, University of Texas Health Science Center, San Antonio (UTHSCSA). Background. Dr. John Caffey introduced the radiographic findings of Kenny-Caffey syndrome, publishing them in 1967. The findings were as follows: proportionate shortening of long bones with medullary stenosis in diaphyseal segments only, and the cortical walls surrounding the stenosed areas were thickened. This results clinically in a proportionately short/dwarfed person.
The calvarium was thin and sclerotic and lacked a diploic space and frontal sinus. The lack of diploic space was thought to be analogous to the stenosis of the medullary cavity in the long bones. The other paranasal sinuses developed normally. The patient reported closure of her anterior fontanel in her late teens. The maxillary and mandibular ridges were hypoplastic and sclerotic. The lower ribs showed a similar pattern of stenosis as in the long bones.1,2 Objectives. To present a case of Kenny-Caffey syndrome. Materials and methods. A Hispanic girl aged 15 years presented to our clinic with an esthetic concern about her missing teeth. On the initial radiographic exam a panoramic x-ray was taken using a Proline machine (Planmeca, Helsinki, Finland). A PA was also acquired during the initial exam on a Promax machine (Planmeca). Before surgery a cone-beam computerized tomogram was acquired (Accutomo 3-DX; J. Morita Co., Osaka, Japan): a simplant multiplanar and curvilinear reconstruction with segmentation 3-dimensional rendering. Results. Findings included multiple missing permanent teeth, hypotrophic frontal sinus, hypotrophic maxillary sinus, enlarged nasal cavity, and hypoplastic maxilla and mandible.
REFERENCES 1. Caffey J. Congenital stenosis of medullary spaces in tubular bones and calvaria in two proportionate dwarfs—mother and son; coupled with transitory hypocalcemic tetany. Am J Roentgenol Radium Ther Nucl Med 1967;100:1-11. 2. Kenny FM, Linarelli L. Dwarfism in cortical thickening of tubular bones. Transient hypocalcemia in a mother and son. Am J Dis Child 1966;111:201-7.
HYBRID BOTRYOID ODONTOGENIC CYST– GLANDULAR ODONTOGENIC CYST OF THE MANDIBLE. F. Jadu, S. Tremblay, M.J. Pharoah, G. Bradley, and D. Psutka, University of Toronto Faculty of Dentistry, Ontario, and Université Laval Faculté de médecine dentaire, Quebec City, Quebec, Canada. Case report. A 67-year-old woman was referred to the oral and maxillofacial radiology service at the Faculty of Dentistry, University of Toronto, with a chief complaint of pain related to the left mandible of 2 months’ duration. Intraoral examination revealed enlargement of the body of the mandible extending from the left retromolar area to approximately the right canine area. The overlying mucosa was normal. The imaging characteristics were of an extensive multilocular neoplasm with numerous curved coarse septa dividing the lesion into multiple cystic spaces. The appearance was most consistent with an ameloblastoma. Histopathologic examination revealed fibrous tissue with multiple cystic spaces lined by epithelium that varied in thickness and appearance. Most of the cystic spaces were lined by stratified squamous nonkeratinizing epithelium with plaque-like thickenings, characteristic of a botryoid odontogenic cyst (BOC). Other areas of lining epithelium showed columnar cells, mucous cells, and gland-like structures, characteristic of a glandular odontogenic cyst (GOC). The lesion was removed by curettage and has been followed closely for the past 2 years with no evidence of recurrence. Discussion. The BOC, which is considered the polycystic variant of the lateral periodontal cyst, was first described by Weathers and Waldron in 1973.1 It is a rare odontogenic cyst and believed to represent the intrabony counterpart of the gingival cyst of the adult. The GOC, on the other hand, was first described
OOOOE April 2008
Abstracts
doses of common extra- and intraoral imaging techniques have increased as much as 186% from 1990 calculations. Discussion. Change in the assessment of risk may warrant a reassessment of examination frequency or the type of examination that is used. Dental radiographs are considerably more risky than previously thought.
APPLICATIONS OF CONE-BEAM COMPUTERIZED TOMOGRAPHY IN ENDODONTICS. J. Éthier, T. Maghsoodi, M. Noujeim, R. Langlais, and J. Preece, University of Texas Health and Science Center, San Antonio. Background. Cone-beam computerized tomography (CBCT) offers the prospect of more effective diagnosis and therefore improved treatment planning. Its role in periodontics, prosthodontics, oral surgery, and orthodontics has been acknowledged and is now being used on an everyday basis. These cases demonstrate the advantage of CBCT in the accurate diagnosis of complex endodontic cases. Objectives. Traditionally, an endodontic lesion is evaluated with conventional periapical radiographs. By changing the angulation of the beam, the dentist may evaluate the possibility of another root, an auxiliary canal, or a radicular fracture. With the advent of high-resolution CBCT, one can achieve greater accuracy, therefore both saving time and providing the highest standard of care. We examined 2 patients who presented to the dental clinic for an endodontic consultation. In both cases, radiolucencies were detected on periapical radiographs. The patients were referred to the radiology department for additional assessment with CBCT. Materials and methods. High-resolution 3-dimensional imaging technique was used in both cases. The initial treatment planning was done with periapical radiographs using a Gendex x-ray unit and a Schick sensor. In the first case, the CBCT was acquired using the Morita Accuitomo 3DX. In the second case, the volume was acquired with the Planmeca Promax 3D. Results. In the first case, the use of CBCT prevented the retreatment of a lingually fractured tooth. In the second case, the CBCT revealed a deviation from the norm, where an endodontic treatment could have been prevented. Discussion. In the first case, the initial diagnosis was determined as an endodontic failure necessitating retreatment. In the second case, the initial diagnosis was internal resorption, it was then altered to incisive canal cyst. Because of lingering uncertainty in these cases, the CBCT scans were acquired to further investigate the “lesions.” Conclusion. Dentists are very often faced with difficult cases to resolve and have to make their diagnosis with conventional techniques that can lead to misdiagnosis and result in unneeded root canal therapy. With the use of CBCT, misdiagnosis resulting in inappropriate treatment might be overcome.
KENNY-CAFFEY SYNDROME: A RADIOLOGY CASE REPORT. A. Hunter, T. Maghsoodi, S. Lavasani, M. Noujeim, and R. Langlais, Department of Dental Diagnostic Science, University of Texas Health Science Center, San Antonio (UTHSCSA). Background. Dr. John Caffey introduced the radiographic findings of Kenny-Caffey syndrome, publishing them in 1967. The findings were as follows: proportionate shortening of long bones with medullary stenosis in diaphyseal segments only, and the cortical walls surrounding the stenosed areas were thickened. This results clinically in a proportionately short/dwarfed person.
The calvarium was thin and sclerotic and lacked a diploic space and frontal sinus. The lack of diploic space was thought to be analogous to the stenosis of the medullary cavity in the long bones. The other paranasal sinuses developed normally. The patient reported closure of her anterior fontanel in her late teens. The maxillary and mandibular ridges were hypoplastic and sclerotic. The lower ribs showed a similar pattern of stenosis as in the long bones.1,2 Objectives. To present a case of Kenny-Caffey syndrome. Materials and methods. A Hispanic girl aged 15 years presented to our clinic with an esthetic concern about her missing teeth. On the initial radiographic exam a panoramic x-ray was taken using a Proline machine (Planmeca, Helsinki, Finland). A PA was also acquired during the initial exam on a Promax machine (Planmeca). Before surgery a cone-beam computerized tomogram was acquired (Accutomo 3-DX; J. Morita Co., Osaka, Japan): a simplant multiplanar and curvilinear reconstruction with segmentation 3-dimensional rendering. Results. Findings included multiple missing permanent teeth, hypotrophic frontal sinus, hypotrophic maxillary sinus, enlarged nasal cavity, and hypoplastic maxilla and mandible.
REFERENCES 1. Caffey J. Congenital stenosis of medullary spaces in tubular bones and calvaria in two proportionate dwarfs—mother and son; coupled with transitory hypocalcemic tetany. Am J Roentgenol Radium Ther Nucl Med 1967;100:1-11. 2. Kenny FM, Linarelli L. Dwarfism in cortical thickening of tubular bones. Transient hypocalcemia in a mother and son. Am J Dis Child 1966;111:201-7.
HYBRID BOTRYOID ODONTOGENIC CYST– GLANDULAR ODONTOGENIC CYST OF THE MANDIBLE. F. Jadu, S. Tremblay, M.J. Pharoah, G. Bradley, and D. Psutka, University of Toronto Faculty of Dentistry, Ontario, and Université Laval Faculté de médecine dentaire, Quebec City, Quebec, Canada. Case report. A 67-year-old woman was referred to the oral and maxillofacial radiology service at the Faculty of Dentistry, University of Toronto, with a chief complaint of pain related to the left mandible of 2 months’ duration. Intraoral examination revealed enlargement of the body of the mandible extending from the left retromolar area to approximately the right canine area. The overlying mucosa was normal. The imaging characteristics were of an extensive multilocular neoplasm with numerous curved coarse septa dividing the lesion into multiple cystic spaces. The appearance was most consistent with an ameloblastoma. Histopathologic examination revealed fibrous tissue with multiple cystic spaces lined by epithelium that varied in thickness and appearance. Most of the cystic spaces were lined by stratified squamous nonkeratinizing epithelium with plaque-like thickenings, characteristic of a botryoid odontogenic cyst (BOC). Other areas of lining epithelium showed columnar cells, mucous cells, and gland-like structures, characteristic of a glandular odontogenic cyst (GOC). The lesion was removed by curettage and has been followed closely for the past 2 years with no evidence of recurrence. Discussion. The BOC, which is considered the polycystic variant of the lateral periodontal cyst, was first described by Weathers and Waldron in 1973.1 It is a rare odontogenic cyst and believed to represent the intrabony counterpart of the gingival cyst of the adult. The GOC, on the other hand, was first described