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Robinow Syndrome: A Rare Case Report and Review of Literature.

Soman C, Lingappa A - Int J Clin Pediatr Dent (2015)

Bottom Line: Covesdem syndrome was the name entitled for the recessive form previously.How to cite this article: Soman C, Lingappa A.Int J Clin Pediatr Dent 2015;8(2):149-152.

View Article: PubMed Central - PubMed

Affiliation: Assistant Professor, Department of Oral Medicine and Radiology, Amrita School of Dentistry, Kochi, Kerala, India.

ABSTRACT
Robinow syndrome is an extremely rare genetic disorder. Short-limbed dwarfism, abnormalities in the head, face, and external genitalia, as well as vertebral defects comprise its distinct features. This disorder exists in dominant and recessive patterns. Patients with the dominant pattern exhibit moderate symptoms. More physical characteristics and skeletal abnormalities characterize the recessive group. The syndrome is also known as Robinow-Silverman-Smith syndrome, Robinow dwarfism, fetal face, fetal face syndrome, fetal facies syndrome, acral dysostosis with facial and genital abnormalities, or mesomelic dwarfism-small genitalia syndrome. Covesdem syndrome was the name entitled for the recessive form previously. Here, we report a case of 8-year-old female with a autosomal recessive Robinow syndrome having skeletal and vertebral defects. How to cite this article: Soman C, Lingappa A. Robinow Syndrome: A Rare Case Report and Review of Literature. Int J Clin Pediatr Dent 2015;8(2):149-152.

No MeSH data available.


Related in: MedlinePlus

Extraoral view exhibiting typical orofacial features
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Figure 1: Extraoral view exhibiting typical orofacial features

Mentions: Extraoral examination (Fig. 1) revealed macrocephaly, frontal bossing, hypertelorism, prominent eyes, down-slanting palpebral fissures, broad nasal bridge, upturned nose, long philtrum, deficient malar prominence depicting midfacial hypoplasia and mandibular retrognathism.


Robinow Syndrome: A Rare Case Report and Review of Literature.

Soman C, Lingappa A - Int J Clin Pediatr Dent (2015)

Extraoral view exhibiting typical orofacial features
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4562051&req=5

Figure 1: Extraoral view exhibiting typical orofacial features
Mentions: Extraoral examination (Fig. 1) revealed macrocephaly, frontal bossing, hypertelorism, prominent eyes, down-slanting palpebral fissures, broad nasal bridge, upturned nose, long philtrum, deficient malar prominence depicting midfacial hypoplasia and mandibular retrognathism.

Bottom Line: Covesdem syndrome was the name entitled for the recessive form previously.How to cite this article: Soman C, Lingappa A.Int J Clin Pediatr Dent 2015;8(2):149-152.

View Article: PubMed Central - PubMed

Affiliation: Assistant Professor, Department of Oral Medicine and Radiology, Amrita School of Dentistry, Kochi, Kerala, India.

ABSTRACT
Robinow syndrome is an extremely rare genetic disorder. Short-limbed dwarfism, abnormalities in the head, face, and external genitalia, as well as vertebral defects comprise its distinct features. This disorder exists in dominant and recessive patterns. Patients with the dominant pattern exhibit moderate symptoms. More physical characteristics and skeletal abnormalities characterize the recessive group. The syndrome is also known as Robinow-Silverman-Smith syndrome, Robinow dwarfism, fetal face, fetal face syndrome, fetal facies syndrome, acral dysostosis with facial and genital abnormalities, or mesomelic dwarfism-small genitalia syndrome. Covesdem syndrome was the name entitled for the recessive form previously. Here, we report a case of 8-year-old female with a autosomal recessive Robinow syndrome having skeletal and vertebral defects. How to cite this article: Soman C, Lingappa A. Robinow Syndrome: A Rare Case Report and Review of Literature. Int J Clin Pediatr Dent 2015;8(2):149-152.

No MeSH data available.


Related in: MedlinePlus