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Chondroectodermal dysplasia: a rare syndrome.

Tahririan D, Eshghi A, Givehchian P, Tahririan MA - J Dent (Tehran) (2014)

Bottom Line: This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common.It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures.The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers.

View Article: PubMed Central - PubMed

Affiliation: Resident, Department of Pediatric Dentistry, Faculty of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran.

ABSTRACT
Chondroectodermal dysplasia (Ellis-Van Creveld syndrome) is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

No MeSH data available.


Related in: MedlinePlus

Oligodontia
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f2-jod-11-361: Oligodontia

Mentions: All the primary incisors, canines, and first molars, as confirmed by radiographs, were congenitally missing (Figure 2). She did not exhibit gingival hypertrophy, and had missing teeth in the gingival area. The rest of her physical examination was significant for polydactyly (hexadactyly) of both hands, and dystrophic nails (Figure 3). Her head morphology and facial appearance were normal, except for hidrotic and fine hair.


Chondroectodermal dysplasia: a rare syndrome.

Tahririan D, Eshghi A, Givehchian P, Tahririan MA - J Dent (Tehran) (2014)

Oligodontia
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4290765&req=5

f2-jod-11-361: Oligodontia
Mentions: All the primary incisors, canines, and first molars, as confirmed by radiographs, were congenitally missing (Figure 2). She did not exhibit gingival hypertrophy, and had missing teeth in the gingival area. The rest of her physical examination was significant for polydactyly (hexadactyly) of both hands, and dystrophic nails (Figure 3). Her head morphology and facial appearance were normal, except for hidrotic and fine hair.

Bottom Line: This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common.It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures.The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers.

View Article: PubMed Central - PubMed

Affiliation: Resident, Department of Pediatric Dentistry, Faculty of Dentistry, Isfahan University of Medical Sciences, Isfahan, Iran.

ABSTRACT
Chondroectodermal dysplasia (Ellis-Van Creveld syndrome) is a rare autosomal recessive congenital abnormality. This syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. It is imperative to not overlook the cardiac complications in patients with this syndrome during dental procedures. The case presented here, although quite rare, was detected under normal conditions and can be alarming for dental care providers. Clinical reports outline the classical and unusual oral and dental manifestations, which help health care providers diagnose chondroectodermal dysplasia, and refer patients with this syndrome to appropriate health care professionals to receive treatment to prevent further cardiac complications and bone deformities.

No MeSH data available.


Related in: MedlinePlus