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[The Freeman-Sheldon Syndrome].
Z Orthop Ihre Grenzgeb. 1983 Sep-Oct; 121(5):630-3.ZO

Abstract

Freeman-Sheldon syndrome (FSS) or craniocarpotarsal dysplasia is characterized by a triad of symptoms: 1. Masklike, whistling facial expression (whistling face syndrome), 2. Ulnar deviation of digits II to V and adduction contracture of the thumb and 3. Foot deformities. The pathogenesis of this malformation is unknown. Heredity is autosomal dominant and not sex-linked. Genetic counseling of affected individuals is imperative. The differential diagnosis should exclude the possibility of arthrogryposis multiplex congenita and particularly congenital windmill deformity of the fingers, which can also be accompanied by foot deformities. The foot and hand deformities associated with FSS are resistant to treatment and require consistent conservative and operative measures. Multiple, extensive operative interventions were unavoidable in both cases described.

Authors

No affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
English Abstract
Journal Article

Language

ger

PubMed ID

6649811

Citation

Aldinger, G, and J Eulert. "[The Freeman-Sheldon Syndrome]." Zeitschrift Fur Orthopadie Und Ihre Grenzgebiete, vol. 121, no. 5, 1983, pp. 630-3.
Aldinger G, Eulert J. [The Freeman-Sheldon Syndrome]. Z Orthop Ihre Grenzgeb. 1983;121(5):630-3.
Aldinger, G., & Eulert, J. (1983). [The Freeman-Sheldon Syndrome]. Zeitschrift Fur Orthopadie Und Ihre Grenzgebiete, 121(5), 630-3.
Aldinger G, Eulert J. [The Freeman-Sheldon Syndrome]. Z Orthop Ihre Grenzgeb. 1983 Sep-Oct;121(5):630-3. PubMed PMID: 6649811.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [The Freeman-Sheldon Syndrome]. AU - Aldinger,G, AU - Eulert,J, PY - 1983/9/1/pubmed PY - 1983/9/1/medline PY - 1983/9/1/entrez SP - 630 EP - 3 JF - Zeitschrift fur Orthopadie und ihre Grenzgebiete JO - Z Orthop Ihre Grenzgeb VL - 121 IS - 5 N2 - Freeman-Sheldon syndrome (FSS) or craniocarpotarsal dysplasia is characterized by a triad of symptoms: 1. Masklike, whistling facial expression (whistling face syndrome), 2. Ulnar deviation of digits II to V and adduction contracture of the thumb and 3. Foot deformities. The pathogenesis of this malformation is unknown. Heredity is autosomal dominant and not sex-linked. Genetic counseling of affected individuals is imperative. The differential diagnosis should exclude the possibility of arthrogryposis multiplex congenita and particularly congenital windmill deformity of the fingers, which can also be accompanied by foot deformities. The foot and hand deformities associated with FSS are resistant to treatment and require consistent conservative and operative measures. Multiple, extensive operative interventions were unavoidable in both cases described. SN - 0044-3220 UR - https://www.unboundmedicine.com/medline/citation/6649811/[The_Freeman_Sheldon_Syndrome]_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2008-1053289 DB - PRIME DP - Unbound Medicine ER -