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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

Orthopantomography revealing erupting 11, 22, 36, 46
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Figure 3: Orthopantomography revealing erupting 11, 22, 36, 46

Mentions: Orthopantomography (Fig. 3) revealed erupting 11, 22, 36, 46. Lateral cephalograph (Fig. 4A) depicts midfacial hypoplasia and mandibular micrognathism. Normal skeletal maturity was assessed through hand wrist graphs (Figs 4B and C) which exhibited clinodactyly of fifth finger. The patient was referred to the department of Pediatrics for further evaluation where she was identified as a case of Robinow syndrome. Patient underwent full body survey. Ultrasonography of abdomen and ECG was taken which were normal. Full skeletal survey revealed vertebral deformities which were characteristic of recessive form of the syndrome. A final diagnosis of an autosomal recessive Robinow syndrome was given. Anesthetic interventions were alerted to be preceded with precautions.


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

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

Orthopantomography revealing erupting 11, 22, 36, 46
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Orthopantomography revealing erupting 11, 22, 36, 46
Mentions: Orthopantomography (Fig. 3) revealed erupting 11, 22, 36, 46. Lateral cephalograph (Fig. 4A) depicts midfacial hypoplasia and mandibular micrognathism. Normal skeletal maturity was assessed through hand wrist graphs (Figs 4B and C) which exhibited clinodactyly of fifth finger. The patient was referred to the department of Pediatrics for further evaluation where she was identified as a case of Robinow syndrome. Patient underwent full body survey. Ultrasonography of abdomen and ECG was taken which were normal. Full skeletal survey revealed vertebral deformities which were characteristic of recessive form of the syndrome. A final diagnosis of an autosomal recessive Robinow syndrome was given. Anesthetic interventions were alerted to be preceded with precautions.

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