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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

Intraoral features.
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fig2: Intraoral features.

Mentions: On clinical examination, the patient demonstrated with short stature with the standing height of 138 cm and weight of 58 kg. The facial form was euryprosopic with deficient midface and absence of upper incisors showing on rest and smile. The hands were broad with short, spoon shaped phalanges and dystrophic nails as shown in Figure 1. The eyes were brown-black in color with normal appearing sclera. On intraoral examination, all permanent teeth were present except for second permanent molars in both arches. There was Class III incisor relationship with reverse overjet of 2 mm, bilateral lateral open bite, narrow and grooved palate, irregular and malpositioned teeth, and v-shaped maxillary and mandibular dental arches (Figure 2). The dental hygiene was satisfactory with mild plaque and calculus deposits on upper anterior lingual surface and lower anterior labial and lingual surfaces. There was no periodontal disease; however, mild edema and color changes were observed. There were Class I carious lesions present on mandibular first permanent molars which were restored with composite fillings.


Pycnodysostosis with Special Emphasis on Dentofacial Characteristics.

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

Intraoral features.
© Copyright Policy
Related In: Results  -  Collection

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

fig2: Intraoral features.
Mentions: On clinical examination, the patient demonstrated with short stature with the standing height of 138 cm and weight of 58 kg. The facial form was euryprosopic with deficient midface and absence of upper incisors showing on rest and smile. The hands were broad with short, spoon shaped phalanges and dystrophic nails as shown in Figure 1. The eyes were brown-black in color with normal appearing sclera. On intraoral examination, all permanent teeth were present except for second permanent molars in both arches. There was Class III incisor relationship with reverse overjet of 2 mm, bilateral lateral open bite, narrow and grooved palate, irregular and malpositioned teeth, and v-shaped maxillary and mandibular dental arches (Figure 2). The dental hygiene was satisfactory with mild plaque and calculus deposits on upper anterior lingual surface and lower anterior labial and lingual surfaces. There was no periodontal disease; however, mild edema and color changes were observed. There were Class I carious lesions present on mandibular first permanent molars which were restored with composite fillings.

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