Literature DB >> 24225909

Improving oral function and cosmesis in a case of Freeman-Sheldon syndrome.

Vishal Vyas1, Riaz Agha, Tariq Ahmad.   

Abstract

Children born with the rare congenital condition Freeman-Sheldon syndrome (FSS) have a characteristic facial appearance: microstomia, a long philtrum, 'H-shaped' chin abnormality and 'pinched lips' in addition to extra-facial features such as kyphoscoliosis and hand deformities. Such children often have problems with oral continence and difficulties with speech leading to both nutritional and psychosocial concerns. Prompt correction through surgery is therefore important in effectively managing the condition. We report the case of a 7-year-old girl who presented with the appearance of scarred lips with difficulties in closing her mouth, a speech impediment and oral incontinence. Using a muscle-sparing technique to reconstruct the upper and lower lips, better oral continence was achieved alongside markedly improved speech and an aesthetically pleasing result. Taken together, this case aptly illustrates the benefits of lip reconstruction in improving function and cosmesis in patients with FSS.

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Mesh:

Year:  2013        PMID: 24225909      PMCID: PMC3830411          DOI: 10.1136/bcr-2013-009191

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  4 in total

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Authors:  F BURIAN
Journal:  Br J Plast Surg       Date:  1963-04

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Authors:  E A Freeman; J H Sheldon
Journal:  Arch Dis Child       Date:  1938-09       Impact factor: 3.791

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Authors:  L M Ferreira; E Minami; J de M Andrews
Journal:  Br J Plast Surg       Date:  1994-04

4.  Clinical characteristics and natural history of Freeman-Sheldon syndrome.

Authors:  David A Stevenson; John C Carey; Janice Palumbos; Ann Rutherford; Joyce Dolcourt; Michael J Bamshad
Journal:  Pediatrics       Date:  2006-03       Impact factor: 7.124

  4 in total

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