title: Surgery of the head and neck in patient with Kniest dysplasia: Is wound healing an issue?
abstract: Kniest dysplasia is a type II collagen disorder that arises from a genetic mutation of the COL2A1 gene that results in short stature, midface anomalies, tracheomalacia, and hearing loss. Disruption of the normal collagen pathway can lead to many changes given its critical role in the body, and can cause complications with respect to wound healing. We present a case in which a patient with Kniest dysplasia successfully underwent multiple procedures in the head and neck region including cochlear implantation, mandibular distraction, palatoplasty, and laryngotracheal reconstruction. All procedures did not have any associated complications with respect to wound healing, indicating that surgery in this population can take place as indicated and surgery should not be contraindicated or delayed.
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, Weill Cornell Medical College, New York, NY, USA.
authors.forename: Qasim
authors.initials: Q
authors.lastname: Husain
authors.affiliation: Department of Oral, Maxillofacial Surgery, Weill Cornell Medical College, New York, NY, USA.
authors.forename: Jungsuk
authors.initials: J
authors.lastname: Cho
authors.affiliation: Department of Oral, Maxillofacial Surgery, Weill Cornell Medical College, New York, NY, USA.
authors.forename: Jay
authors.initials: J
authors.lastname: Neugarten
authors.affiliation: Department of Otolaryngology - Head & Neck Surgery, Weill Cornell Medical College, New York, NY, USA. Electronic address: vkm2001@med.cornell.edu.
authors.forename: Vikash K
authors.initials: VK
authors.lastname: Modi
mesh_terms: D002972:Cleft Palate / Q000601:surgery*; D019929:Cochlear Implantation; D003095:Collagen Diseases / Q000601:surgery*; D004392:Dwarfism / Q000601:surgery*; D005145:Face / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D006819:Hyaline Membrane Disease / Q000601:surgery*; D007223:Infant; D008297:Male; D019186:Mandibular Advancement / Q000379:methods; D056948:Orthognathic Surgical Procedures; D010009:Osteochondrodysplasias / Q000601:surgery*; D019857:Osteogenesis, Distraction; D013517:Otorhinolaryngologic Surgical Procedures; D014945:Wound Healing
keywords: COL2A1 mutation; Cleft palate; Cochlear implantation; Kniest dysplasia; Laryngotracheal reconstruction; Mandibular distraction; Micrognathia; Tracheotomy; Type II collagen; Wound healing