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Functional upper airway obstruction in a child with Freeman-Sheldon syndrome.
ORL J Otorhinolaryngol Relat Spec. 2002 Jan-Feb; 64(1):53-6.OJ

Abstract

Freeman-Sheldon syndrome is defined as a combination of microstomia, deep set eyes, small palpebral fissures, arthrogryposis with ulnar deviation of the hand, talipes equinovarus and generalized muscular hypertension. Respiratory and swallowing problems are frequently encountered in these patients due to small orifices of mouth and nose. Obstruction of the upper airway tract resulting in tracheostomy has only been described twice. The described child manifested the typical dysmorphic features of Freeman-Sheldon syndrome and suffered from serious respiratory distress and swallowing difficulties from birth. The boy died at the age of 7 months after accidental decannulation of the tracheostoma during sleep. He did not show anatomical or histopathological abnormalities in the pharyngeal, laryngeal or tracheal regions. We assume that the only explanation of the repeated obstructive episodes is a functional muscular obstruction.

Authors+Show Affiliations

Neonatologische Intensivmedizin, Kinderklinik, Aachen, Deutschland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

11891401

Citation

Schefels, Joerg, et al. "Functional Upper Airway Obstruction in a Child With Freeman-Sheldon Syndrome." ORL; Journal for Oto-rhino-laryngology and Its Related Specialties, vol. 64, no. 1, 2002, pp. 53-6.
Schefels J, Wenzl TG, Merz U, et al. Functional upper airway obstruction in a child with Freeman-Sheldon syndrome. ORL J Otorhinolaryngol Relat Spec. 2002;64(1):53-6.
Schefels, J., Wenzl, T. G., Merz, U., Ramaekers, V., Holzki, J., Rudnik-Schoeneborn, S., Hermanns, B., & Hörnchen, H. (2002). Functional upper airway obstruction in a child with Freeman-Sheldon syndrome. ORL; Journal for Oto-rhino-laryngology and Its Related Specialties, 64(1), 53-6.
Schefels J, et al. Functional Upper Airway Obstruction in a Child With Freeman-Sheldon Syndrome. ORL J Otorhinolaryngol Relat Spec. 2002 Jan-Feb;64(1):53-6. PubMed PMID: 11891401.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Functional upper airway obstruction in a child with Freeman-Sheldon syndrome. AU - Schefels,Joerg, AU - Wenzl,Tobias G, AU - Merz,Ulrich, AU - Ramaekers,Vincent, AU - Holzki,Josef, AU - Rudnik-Schoeneborn,Sabine, AU - Hermanns,Benita, AU - Hörnchen,Helmut, PY - 2002/3/14/pubmed PY - 2002/8/29/medline PY - 2002/3/14/entrez SP - 53 EP - 6 JF - ORL; journal for oto-rhino-laryngology and its related specialties JO - ORL J Otorhinolaryngol Relat Spec VL - 64 IS - 1 N2 - Freeman-Sheldon syndrome is defined as a combination of microstomia, deep set eyes, small palpebral fissures, arthrogryposis with ulnar deviation of the hand, talipes equinovarus and generalized muscular hypertension. Respiratory and swallowing problems are frequently encountered in these patients due to small orifices of mouth and nose. Obstruction of the upper airway tract resulting in tracheostomy has only been described twice. The described child manifested the typical dysmorphic features of Freeman-Sheldon syndrome and suffered from serious respiratory distress and swallowing difficulties from birth. The boy died at the age of 7 months after accidental decannulation of the tracheostoma during sleep. He did not show anatomical or histopathological abnormalities in the pharyngeal, laryngeal or tracheal regions. We assume that the only explanation of the repeated obstructive episodes is a functional muscular obstruction. SN - 0301-1569 UR - https://www.unboundmedicine.com/medline/citation/11891401/Functional_upper_airway_obstruction_in_a_child_with_Freeman_Sheldon_syndrome_ L2 - https://www.karger.com?DOI=10.1159/000049271 DB - PRIME DP - Unbound Medicine ER -