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.
Please wait while flipbook is loading. For more related info, FAQs and issues please refer to DearFlip WordPress Flipbook Plugin Help documentation.DearFlip: Loading PDF 94% ...