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Freeman-Sheldon (Whistling face) syndrome

Year 1994, Volume: 28 Issue: 4, 265 - 268, 11.09.2006

Abstract

Freeman -Sheldon syndrome is a craniocarpotarsal dystrophy. This hereditary disorder which is also calIed the whistling-face syndrome include these major characteristics.1-An abnormal facies(microstomia,small nose. Long philtrum and enophtalmos) 2-Ulnar deviation and flexion contractures of the fingers 3-Resistant taIipes equinovarus 4-Small stature. About 1 00 cases have been reported in the Iiterature upto date. The major considerations in therapy are orthopaedic, We reviewed this syndrome throughout the Iiterature because of a pati.ent who have been hospitalized in the department of orthopedics and traumatology at the medical Faculty of Istanbul University. We noticed that hand and foot deformities are resistant to conservative treatment and they do not improve spontaneously with growth. Althongh this syndrome is rare we presented this case report and literature review to alert the orthopedic surgeons to the fact that better results are achieved by early diagnosis and early surgery.

Freeman-Sheldon Sendromu (Islık çalan yüz (Whistling-Face) sendromu)

Year 1994, Volume: 28 Issue: 4, 265 - 268, 11.09.2006

Abstract

Freeman Sheldon sendromu bir craniocarpotarsal distrofidir. Bir diğer adı ıslık çalan yüz sendromu olan bu herediter bozukluk başlıca şu özellikleri içerir. 1- Anormal yüz (mikrostomi, küçük burun, uzun filtrum ve enoftalmi), 2-El parmaklarında ulnar deviasyon ve fleksiyon kontraktürleri, 3- Rezistan talipes ekinovarus, 4-Kısa vücud yapısı. Bugüne kadar yaklaşık yüz vaka literatürde bildirilmiştir. Tedavide gözönüne alınacaklar özellikle ortopedik bozukluklardır. Biz de İstanbul Tıp Fakültesi Ortopedi ve Travmatoloji Anabilimdalın'da yatan bir hasta nedeni ile bu sendromu Iiteratürde gözden geçirdik. El ve ayaklardaki deformitelerin konservatif tedaviye rezistan olduğunu ve yaşla birlikte spontan gerilemenin olmadığını gördük. Bu sendromun az raslanmasına rağmen erken tam ve erken cerrahi girişimler ile iyi sonuçlar alındığı konusunda ortopedik cerrahların dikkatlerini çekmek için bu vakayı ve Iiteratür araştırmasını sunduk.

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Details

Primary Language English
Journal Section Case Report
Authors

Mehmet Demirhan This is me

Yusuf Ozturkmen This is me

Cengiz Sen This is me

Hulya Kayserili This is me

Yunus Sozen This is me

Publication Date September 11, 2006
Published in Issue Year 1994 Volume: 28 Issue: 4

Cite

APA Demirhan, M., Ozturkmen, Y., Sen, C., Kayserili, H., et al. (2006). Freeman-Sheldon (Whistling face) syndrome. Acta Orthopaedica Et Traumatologica Turcica, 28(4), 265-268. https://doi.org/10.3944/aott.v28i4.1502
AMA Demirhan M, Ozturkmen Y, Sen C, Kayserili H, Sozen Y. Freeman-Sheldon (Whistling face) syndrome. Acta Orthopaedica et Traumatologica Turcica. September 2006;28(4):265-268. doi:10.3944/aott.v28i4.1502
Chicago Demirhan, Mehmet, Yusuf Ozturkmen, Cengiz Sen, Hulya Kayserili, and Yunus Sozen. “Freeman-Sheldon (Whistling Face) Syndrome”. Acta Orthopaedica Et Traumatologica Turcica 28, no. 4 (September 2006): 265-68. https://doi.org/10.3944/aott.v28i4.1502.
EndNote Demirhan M, Ozturkmen Y, Sen C, Kayserili H, Sozen Y (September 1, 2006) Freeman-Sheldon (Whistling face) syndrome. Acta Orthopaedica et Traumatologica Turcica 28 4 265–268.
IEEE M. Demirhan, Y. Ozturkmen, C. Sen, H. Kayserili, and Y. Sozen, “Freeman-Sheldon (Whistling face) syndrome”, Acta Orthopaedica et Traumatologica Turcica, vol. 28, no. 4, pp. 265–268, 2006, doi: 10.3944/aott.v28i4.1502.
ISNAD Demirhan, Mehmet et al. “Freeman-Sheldon (Whistling Face) Syndrome”. Acta Orthopaedica et Traumatologica Turcica 28/4 (September 2006), 265-268. https://doi.org/10.3944/aott.v28i4.1502.
JAMA Demirhan M, Ozturkmen Y, Sen C, Kayserili H, Sozen Y. Freeman-Sheldon (Whistling face) syndrome. Acta Orthopaedica et Traumatologica Turcica. 2006;28:265–268.
MLA Demirhan, Mehmet et al. “Freeman-Sheldon (Whistling Face) Syndrome”. Acta Orthopaedica Et Traumatologica Turcica, vol. 28, no. 4, 2006, pp. 265-8, doi:10.3944/aott.v28i4.1502.
Vancouver Demirhan M, Ozturkmen Y, Sen C, Kayserili H, Sozen Y. Freeman-Sheldon (Whistling face) syndrome. Acta Orthopaedica et Traumatologica Turcica. 2006;28(4):265-8.