An Unexpected Airway Complication in a Male Patient with Gol
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Abstract :
Goltz syndrome, also known as focal dermal hypoplasia, is a rare X-linked dominant multisystem syndrome presenting with cutaneous, skeletal, dental ocular, central nervous system and soft tissue abnormalities. This case report discusses an adult male patient with Goltz syndrome that was noted to have large, papillomatous, hypopharyngeal lesions upon induction of general anesthesia. We highlight challenges with airway management intraoperatively and postoperatively in patients with Goltz syndrome. Our aim is to increase awareness of the potential airway complications associated with this genetic disorder and to provide suggestions for optimal perioperative management for patients afflicted with Goltz syndrome.

Introduction :
Goltz-Gorlin syndrome (focal dermal hypoplasia or Goltz syndrome) not to be confused with Gorlin-Goltz syndrome (nevoid basal cell carcinoma), first described in 1962, is a highly variable condition that involves many organ systems of mesoectodermal origin [1]. The most common characteristic feature is thinning of the dermis resulting in subcutaneous fat herniation [2]. Areas that are frequently involved include skin (over 95% of reported cases), ocular, skeletal, craniofacial, and dental tissue [3]. Papillomas often appear in the skin, perioral, esophageal, perianal, genital, and ocular regions. There have been few reports of papillomas appearing in the upper airway (involving the tongue, tonsils, soft palate, pharynx, and larynx) [3–6]......

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5046003/
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