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دکتر اصلان احمدی / مقالات تخصصی / Fraser syndrome with laryngeal webs
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Fraser syndrome with laryngeal webs

 Introduction

Fraser syndrome, first described by George Fraser in 1962, is a
rare autosomal recessive disorder characterized by cryptophthalmos
(failure of eyelid formation) and syndactyly (webbing of fingers and
toes) [1]. Fewer than 140 cases of this syndrome have been reported
so far in the literature [2–4]. This genetic disorder may be
also associated with urinary tract, ear, nose or laryngeal abnormalities
[5]. ENT manifestations of Fraser syndrome include abnormalities
of ears (meatal stenosis, dysplastic pinna), nose (hypoplastic
notched nares, choanal stenosis or atresia), and larynx (glottic web,
subglottic stenosis) [6]. Although laryngeal webs are rather
uncommon, involving one fifth of the patients, they may cause
serious respiratory distress [7,8]. Meanwhile, laryngeal stenosis
and consequential respiratory distress are the most common cause of
death in affected patients who died in the first week of life.

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