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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

(A) Hypertrophy of maxillary ridge (B) Hypertrophy of mandibular alveolar ridge and (C) Intraoral view
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Figure 2: (A) Hypertrophy of maxillary ridge (B) Hypertrophy of mandibular alveolar ridge and (C) Intraoral view

Mentions: Intraoral examination (Figs 2A to C) revealed high arched palate, narrowed and V-shaped mandibular arches, maxillary and mandibular alveolar ridge hypertrophy and short lingual frenulum depicting partial ankyloglossia. Teeth present were 11, 52, 53, 54, 55, 16, 21, 62, 63, 64, 26, 31, 32, 72, 73, 74, 75, 41, 82, 83, 84, 85. Palatally erupting 12, over retained 52, 62, 72, 82, lingually erupting 32, 42, mesiolingually rotated 31, 41.


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

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

(A) Hypertrophy of maxillary ridge (B) Hypertrophy of mandibular alveolar ridge and (C) Intraoral view
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: (A) Hypertrophy of maxillary ridge (B) Hypertrophy of mandibular alveolar ridge and (C) Intraoral view
Mentions: Intraoral examination (Figs 2A to C) revealed high arched palate, narrowed and V-shaped mandibular arches, maxillary and mandibular alveolar ridge hypertrophy and short lingual frenulum depicting partial ankyloglossia. Teeth present were 11, 52, 53, 54, 55, 16, 21, 62, 63, 64, 26, 31, 32, 72, 73, 74, 75, 41, 82, 83, 84, 85. Palatally erupting 12, over retained 52, 62, 72, 82, lingually erupting 32, 42, mesiolingually rotated 31, 41.

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