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Achondroplasia with oligodontia: Report of a rare case.

Kale L, Khambete N, Sodhi S, Kumar R - J Oral Maxillofac Pathol (2013)

Bottom Line: It has been considered as the most common short-limbed dwarfism syndrome.Very few authors have reported the presence of oligodontia in achondroplastic patients.The present paper reports a rare case of oligodontia in a young, female, achondroplastic patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, Chhatrapati Shahu Maharaj Shikshan Sanstha Dental College and Hospital, Aurangabad, Maharashtra, India.

ABSTRACT
Achondroplasia is considered as a form of skeletal dysplasia/dwarfism that manifests with stunted stature and disproportionate limb shortening. Achondroplasia is of special interest in the field of dentistry because of its characteristic craniofacial features. It has been considered as the most common short-limbed dwarfism syndrome. Very few authors have reported the presence of oligodontia in achondroplastic patients. The present paper reports a rare case of oligodontia in a young, female, achondroplastic patient.

No MeSH data available.


Related in: MedlinePlus

Clinical picture of patient showing short stature and rhizomelic shortening of arms and legs
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Figure 1: Clinical picture of patient showing short stature and rhizomelic shortening of arms and legs

Mentions: The patient appeared to be well-adjusted, healthy and intelligent. General physical examination showed short stature, with normal trunk length and rhizomelic shortening of the arms and legs [Figure 1]. Lumbar lordosis; prominent buttocks; and short, stubby fingers with trident hand configuration were also evident. Anthropometry (at age 16 years) revealed a height of 124 cm (midparental target height 143.6 cm by Tanner's formula), weight of 42 kg, mesomelic dwarfism with upper limb to lower limb ratio of >1 and an arm span of 100 cm (expected to be 124 cm). The head circumference was 58 cm. Her vital signs were within the normal limits. Systemic examination did not reveal any other abnormality. Extraoral examination revealed facial features such as brachycephaly, midfacial hypoplasia, flat nasal bridge, frontal bossing and competent lips. A concave facial profile was also noticed; however, the mandible appeared normal and the chin was not prominent [Figure 2].


Achondroplasia with oligodontia: Report of a rare case.

Kale L, Khambete N, Sodhi S, Kumar R - J Oral Maxillofac Pathol (2013)

Clinical picture of patient showing short stature and rhizomelic shortening of arms and legs
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3927355&req=5

Figure 1: Clinical picture of patient showing short stature and rhizomelic shortening of arms and legs
Mentions: The patient appeared to be well-adjusted, healthy and intelligent. General physical examination showed short stature, with normal trunk length and rhizomelic shortening of the arms and legs [Figure 1]. Lumbar lordosis; prominent buttocks; and short, stubby fingers with trident hand configuration were also evident. Anthropometry (at age 16 years) revealed a height of 124 cm (midparental target height 143.6 cm by Tanner's formula), weight of 42 kg, mesomelic dwarfism with upper limb to lower limb ratio of >1 and an arm span of 100 cm (expected to be 124 cm). The head circumference was 58 cm. Her vital signs were within the normal limits. Systemic examination did not reveal any other abnormality. Extraoral examination revealed facial features such as brachycephaly, midfacial hypoplasia, flat nasal bridge, frontal bossing and competent lips. A concave facial profile was also noticed; however, the mandible appeared normal and the chin was not prominent [Figure 2].

Bottom Line: It has been considered as the most common short-limbed dwarfism syndrome.Very few authors have reported the presence of oligodontia in achondroplastic patients.The present paper reports a rare case of oligodontia in a young, female, achondroplastic patient.

View Article: PubMed Central - PubMed

Affiliation: Department of Oral Medicine and Radiology, Chhatrapati Shahu Maharaj Shikshan Sanstha Dental College and Hospital, Aurangabad, Maharashtra, India.

ABSTRACT
Achondroplasia is considered as a form of skeletal dysplasia/dwarfism that manifests with stunted stature and disproportionate limb shortening. Achondroplasia is of special interest in the field of dentistry because of its characteristic craniofacial features. It has been considered as the most common short-limbed dwarfism syndrome. Very few authors have reported the presence of oligodontia in achondroplastic patients. The present paper reports a rare case of oligodontia in a young, female, achondroplastic patient.

No MeSH data available.


Related in: MedlinePlus