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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

Skull radiograph with patent anterior fontanelle.
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fig3: Skull radiograph with patent anterior fontanelle.

Mentions: The skull radiograph demonstrated frontal bossing and significant suture diastasis with patent anterior fontanelle as well as wormian bodies (Figure 3). On dental panoramic radiograph, all permanent teeth were present amongst which all second permanent molars were impacted. There were vertical and horizontal impactions of maxillary and mandibular second permanent molars, respectively. The right mandibular second molar exhibited abnormal shape and morphology. The root apices of mandibular premolars and molars showed abnormal curvature. The mandible showed a striking feature of thin and narrow body with an obtuse gonial angle and elongated condylar neck (Figure 4). The cephalometric radiographic examination showed skeletal Class III sagittal relationship of jaws (ANB = −4°), retropositioned maxilla and mandible with respect to anterior cranial base (SNA = 71°, SNB = 75°), mild tendency towards hyperdivergent pattern of growth (Go-Gn-SN = 38°), decreased anterior and posterior facial heights (N-Me = 96 mm, S-Go = 58 mm), but normal Jarabak's ratio (PFH/AFH = 60.4%). The COGS (cephalometrics for orthognathic surgery) analysis showed deficiency of mandibular body (SN = 64 mm, Go-Pog = 55 mm). The upper incisors inclination with respect to anterior cranial base appeared normal (UI-SN = 98°) whereas, lower incisors in relation with mandibular plane were proclined (IMPA = 102°). The upper lip relation with respect to Ricketts E-line appeared slightly recumbent whereas, the lower lip was normal (UL-E-line = −6 mm, LL-E-line = −1 mm). The Steiner Holdaway analysis demonstrated a retropositioned chin in relation with lower incisors (Holdaway ratio = 9 : −3). All the cephalometric measurements were compared with the standard Caucasian norms (Figure 5) [8]. On Skeletal age assessment, the patient belonged to CS-6 of cervical vertebral maturation stages as proposed by Baccetti et al. [9] with visible concavity and curvature at the lower border of second to sixth cervical vertebrae.


Pycnodysostosis with Special Emphasis on Dentofacial Characteristics.

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

Skull radiograph with patent anterior fontanelle.
© Copyright Policy
Related In: Results  -  Collection

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

fig3: Skull radiograph with patent anterior fontanelle.
Mentions: The skull radiograph demonstrated frontal bossing and significant suture diastasis with patent anterior fontanelle as well as wormian bodies (Figure 3). On dental panoramic radiograph, all permanent teeth were present amongst which all second permanent molars were impacted. There were vertical and horizontal impactions of maxillary and mandibular second permanent molars, respectively. The right mandibular second molar exhibited abnormal shape and morphology. The root apices of mandibular premolars and molars showed abnormal curvature. The mandible showed a striking feature of thin and narrow body with an obtuse gonial angle and elongated condylar neck (Figure 4). The cephalometric radiographic examination showed skeletal Class III sagittal relationship of jaws (ANB = −4°), retropositioned maxilla and mandible with respect to anterior cranial base (SNA = 71°, SNB = 75°), mild tendency towards hyperdivergent pattern of growth (Go-Gn-SN = 38°), decreased anterior and posterior facial heights (N-Me = 96 mm, S-Go = 58 mm), but normal Jarabak's ratio (PFH/AFH = 60.4%). The COGS (cephalometrics for orthognathic surgery) analysis showed deficiency of mandibular body (SN = 64 mm, Go-Pog = 55 mm). The upper incisors inclination with respect to anterior cranial base appeared normal (UI-SN = 98°) whereas, lower incisors in relation with mandibular plane were proclined (IMPA = 102°). The upper lip relation with respect to Ricketts E-line appeared slightly recumbent whereas, the lower lip was normal (UL-E-line = −6 mm, LL-E-line = −1 mm). The Steiner Holdaway analysis demonstrated a retropositioned chin in relation with lower incisors (Holdaway ratio = 9 : −3). All the cephalometric measurements were compared with the standard Caucasian norms (Figure 5) [8]. On Skeletal age assessment, the patient belonged to CS-6 of cervical vertebral maturation stages as proposed by Baccetti et al. [9] with visible concavity and curvature at the lower border of second to sixth cervical vertebrae.

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