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Pycnodysostosis: A bone dysplasia with unusual oral manifestation.

Balaji MS, Sundaram RK, Karthik P, Asokan K - Indian J Dent (2014)

Bottom Line: Patients with pycnodyostosis usually have normal intelligence, sexual development and life span.This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply.This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, SRM Dental College, Ramapuram, Chennai, India.

ABSTRACT
Pycnodysostosis, a sclerosing bone dysplasia, is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million. Patients with pycnodyostosis usually have normal intelligence, sexual development and life span. This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply. This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis.

No MeSH data available.


Related in: MedlinePlus

A 47-year-old man, 127 cm in height
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Figure 1: A 47-year-old man, 127 cm in height

Mentions: A 47-year-old man reported to the department with a complaint of deformed lower jaw for the past 10 years. History revealed that the patient had undergone extraction of his teeth that was uneventful, following which there was fracture of jaw at the extraction site. Subsequently, there was frequent exfoliation of teeth with fracture at different sites in the lower jaw. The patient was asymptomatic and therefore negligent about it. He reported to the department for management of his deficient jaw. His medical history revealed that he had multiple fractures of the upper and lower limbs and a history of diabetes mellitus and hypertension. Family history revealed that there was consanguinity in his parents. General examination revealed that the patient's height was 127 cm and weight was around 49 kg, with proportionate dwarfism [Figure 1]. The hand and feet had short digits with overlying cutaneous wrinkles that tapered off with large overriding nails [Figure 2].


Pycnodysostosis: A bone dysplasia with unusual oral manifestation.

Balaji MS, Sundaram RK, Karthik P, Asokan K - Indian J Dent (2014)

A 47-year-old man, 127 cm in height
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4260389&req=5

Figure 1: A 47-year-old man, 127 cm in height
Mentions: A 47-year-old man reported to the department with a complaint of deformed lower jaw for the past 10 years. History revealed that the patient had undergone extraction of his teeth that was uneventful, following which there was fracture of jaw at the extraction site. Subsequently, there was frequent exfoliation of teeth with fracture at different sites in the lower jaw. The patient was asymptomatic and therefore negligent about it. He reported to the department for management of his deficient jaw. His medical history revealed that he had multiple fractures of the upper and lower limbs and a history of diabetes mellitus and hypertension. Family history revealed that there was consanguinity in his parents. General examination revealed that the patient's height was 127 cm and weight was around 49 kg, with proportionate dwarfism [Figure 1]. The hand and feet had short digits with overlying cutaneous wrinkles that tapered off with large overriding nails [Figure 2].

Bottom Line: Patients with pycnodyostosis usually have normal intelligence, sexual development and life span.This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply.This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, SRM Dental College, Ramapuram, Chennai, India.

ABSTRACT
Pycnodysostosis, a sclerosing bone dysplasia, is a rare autosomal recessive disorder with an estimated prevalence rate of one in one million. Patients with pycnodyostosis usually have normal intelligence, sexual development and life span. This condition is characterized by increased bone density and fragility along with oral manifestations like malposition teeth, hypoplastic maxilla, receded chin and delayed eruption of permanent teeth with discharging sinuses in the jaws because of poor blood supply. This is one such rare case report of a 47-year-old patient presenting with a complaint of fractured jaw and reviewing the clinical and radiographic characteristics of pycnodysostosis.

No MeSH data available.


Related in: MedlinePlus