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Pycnodysostosis with Special Emphasis on Dentofacial Characteristics.

Khoja A, Fida M, Shaikh A - Case Rep Dent (2015)

Bottom Line: Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients.This will guide them in planning realistic treatment goals.The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.

View Article: PubMed Central - PubMed

Affiliation: Section of Dentistry, Department of Surgery, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi 74800, Pakistan.

ABSTRACT
Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.

No MeSH data available.


Related in: MedlinePlus

Soft palate and airway dimensions.
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fig6: Soft palate and airway dimensions.

Mentions: On airway analysis, the soft palate dimensions were 35 mm for soft palate length as measured from posterior nasal spine to tip of the soft palate (PNS-P) and 9.6 mm for soft palate thickness (maximum thickness of soft palate measured on line perpendicular to PNS-P) [10]. The upper airway space (width of airway behind middle of soft palate along parallel line to Go-B) was 8.4 mm, middle airway space (width of airway along parallel line to Go-B line through tip of the soft palate) was 7.5 mm, and inferior airway space (width of airway space along Go-B line) was 11 mm [10] (Figure 6). Despite the narrow upper airway space, there were not any problems associated with breathing during wakefulness or sleep.


Pycnodysostosis with Special Emphasis on Dentofacial Characteristics.

Khoja A, Fida M, Shaikh A - Case Rep Dent (2015)

Soft palate and airway dimensions.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4663328&req=5

fig6: Soft palate and airway dimensions.
Mentions: On airway analysis, the soft palate dimensions were 35 mm for soft palate length as measured from posterior nasal spine to tip of the soft palate (PNS-P) and 9.6 mm for soft palate thickness (maximum thickness of soft palate measured on line perpendicular to PNS-P) [10]. The upper airway space (width of airway behind middle of soft palate along parallel line to Go-B) was 8.4 mm, middle airway space (width of airway along parallel line to Go-B line through tip of the soft palate) was 7.5 mm, and inferior airway space (width of airway space along Go-B line) was 11 mm [10] (Figure 6). Despite the narrow upper airway space, there were not any problems associated with breathing during wakefulness or sleep.

Bottom Line: Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients.This will guide them in planning realistic treatment goals.The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.

View Article: PubMed Central - PubMed

Affiliation: Section of Dentistry, Department of Surgery, The Aga Khan University Hospital, Stadium Road, P.O. Box 3500, Karachi 74800, Pakistan.

ABSTRACT
Pycnodysostosis is an autosomal recessive disorder that manifests as osteosclerosis of the skeleton due to the defective osteoclasts mediated bone turnover. The diagnosis of this disorder is established on the basis of its characteristic features and must be differentially diagnosed with other bone disorders. Dental surgeons should be aware of the limitations and possible adverse oral complications such as osteomyelitis of bone in these patients. This will guide them in planning realistic treatment goals. This paper reports the clinical and radiographic features of pycnodysostosis with the great emphasis on its dentofacial characteristics. The aim of this case report is to give an insight into the etiology, pathogenesis, and differential diagnosis of this disorder and to prepare the dentists and maxillofacial surgeons to overcome the challenges in treating these patients.

No MeSH data available.


Related in: MedlinePlus