Clinical Photos
Hypopharyngeal Synechiae in Lyell Syndrome (Toxic Epidermal Necrolysis) Zacharias Vourexakis, MD1, and Fre´de´ric Heymans, MD1
Otolaryngology– Head and Neck Surgery 2015, Vol. 153(1) 152–153 Ó American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599815580981 http://otojournal.org
No sponsorships or competing interests have been disclosed for this article.
Keywords Lyell syndrome, complications, hypopharynx, dysphagia Received February 22, 2015; revised March 16, 2015; accepted March 18, 2015.
L
yell syndrome (LS), or toxic epidermal necrolysis, is a condition characterized by diffuse erythema, acute extensive necrosis, bullous detachment, and exfoliation of large epidermal (.30%) and mucosal surfaces. Its estimated annual incidence is 0.4 to 1.9/106, with etiologies most commonly reported being drug reactions followed by infectious sequelae.1 Mortality is reported as high as 25% to 30%,2 and at least half the survivors may experience longterm sequelae such as cutaneous, ocular, and genitourinary tract complications, as well as dysphagia.3 We report the case of persisting dysphagia in a patient recovering from LS. Case studies involving fewer than 6 individuals are not subject to review by ethical committees in Switzerland; informed consent was obtained from the patient.
Clinical Case A 28-year-old woman was diagnosed with LS following acetaminophen ingestion. The disease was complicated with pneumonia and severe rhabdomyolysis, resulting in 2 months of inpatient treatment. Apart from widespread scarring and skin discoloration at discharge (Figure 1), the main complaint in the follow-up period was dysphagia to solid food and, to a lesser degree, dysphagia to liquids and occasional episodes of choking. Physical examination of the oral cavity and the pharynx was unremarkable, and only a nonspecific image of linear stenosis in the hypopharynx was apparent during videofluoroscopy. Direct pharyngoscopy under general anesthesia revealed multiple longitudinal synechiae between the anterior and posterior hypopharyngeal wall mucosa (Figure 2, arrows), leaving narrow passages from the oropharynx to the esophagus (see video at www.otojournal.org. Pharyngeal suspension and endoscopic examination under general anesthesia; the video starts in the esophagus with a 0° telescope slipping upwards, between two mucosal synechiae, to the pharynx).
Figure 1. Diffuse skin scarring on the back of the patient, 5 months after initial diagnosis of Lyell syndrome.
Suspension pharyngoscopy allowed endoscopic lysis and resection of the synechiae with a CO2 laser. Oral steroids, proton-pump inhibitors, and antibiotics were prescribed postoperatively. Swallowing improved greatly, and there were no dysphagia-related complaints at the 6-month follow-up visit.
Discussion Hypopharyngeal dysfunction is rarely reported but should be taken into consideration in patients with LS complaining of persistent dysphagia.4 To our knowledge, our pictures represent a rare, if not unique, documentation of hypopharyngeal synechiae in patients recovering from LS. Since 1 Department of Otolaryngology/Head and Neck Surgery, University Hospital of Geneva, Geneva, Switzerland
Corresponding Author: Zacharias Vourexakis, MD, Department of Otolaryngology/Head and Neck Surgery, University Hospital of Geneva, Rue Gabrielle-Perret-Gentil 4, 1205 Geneva, Switzerland. Email: [email protected]
Downloaded from oto.sagepub.com at University of Sussex Library on August 10, 2015
Vourexakis and Heymans
153 appropriately investigated and resolved; Fre´de´ric Heymans, contribution to the acquisition/analysis/interpretation of data for the work, revising the work critically for important intellectual content, final approval of the version to be published, agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Disclosures Competing interests: None. Sponsorships: None. Funding source: None.
Supplemental Material Additional supporting information may be found at http://otojournal .org/supplemental. Figure 2. Suspension of the larynx and direct pharyngoscopy under general anesthesia revealed multiple adhesions (arrows) between opposing surfaces of the hypopharyngeal mucosa.
clinical examination and swallowing studies may be inadequate for proper assessment, endoscopy under general anesthesia may be crucial in accurate diagnosis and treatment. Author Contributions Zacharias Vourexakis, conception/design of the work, contribution to the acquisition/analysis/interpretation of data, drafting the work and revising it critically for important intellectual content, final approval of the version to be published, agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are
References 1. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69:173. 2. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013;69:187. 3. Harr T, French LE. Toxic epidermal necrolysis and StevensJohnson syndrome. Orphanet J Rare Dis. 2010;5:39. 4. Barrera JE, Meyers AD, Hartford EC. Hypopharyngeal stenosis and dysphagia complicating toxic epidermal necrolysis. Arch Otolaryngol Head Neck Surg. 1998;124:1375-1376.
Downloaded from oto.sagepub.com at University of Sussex Library on August 10, 2015
Hypopharyngeal Synechiae in Lyell Syndrome (Toxic Epidermal Necrolysis) Zacharias Vourexakis, MD1, and Fre´de´ric Heymans, MD1
Otolaryngology– Head and Neck Surgery 2015, Vol. 153(1) 152–153 Ó American Academy of Otolaryngology—Head and Neck Surgery Foundation 2015 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0194599815580981 http://otojournal.org
No sponsorships or competing interests have been disclosed for this article.
Keywords Lyell syndrome, complications, hypopharynx, dysphagia Received February 22, 2015; revised March 16, 2015; accepted March 18, 2015.
L
yell syndrome (LS), or toxic epidermal necrolysis, is a condition characterized by diffuse erythema, acute extensive necrosis, bullous detachment, and exfoliation of large epidermal (.30%) and mucosal surfaces. Its estimated annual incidence is 0.4 to 1.9/106, with etiologies most commonly reported being drug reactions followed by infectious sequelae.1 Mortality is reported as high as 25% to 30%,2 and at least half the survivors may experience longterm sequelae such as cutaneous, ocular, and genitourinary tract complications, as well as dysphagia.3 We report the case of persisting dysphagia in a patient recovering from LS. Case studies involving fewer than 6 individuals are not subject to review by ethical committees in Switzerland; informed consent was obtained from the patient.
Clinical Case A 28-year-old woman was diagnosed with LS following acetaminophen ingestion. The disease was complicated with pneumonia and severe rhabdomyolysis, resulting in 2 months of inpatient treatment. Apart from widespread scarring and skin discoloration at discharge (Figure 1), the main complaint in the follow-up period was dysphagia to solid food and, to a lesser degree, dysphagia to liquids and occasional episodes of choking. Physical examination of the oral cavity and the pharynx was unremarkable, and only a nonspecific image of linear stenosis in the hypopharynx was apparent during videofluoroscopy. Direct pharyngoscopy under general anesthesia revealed multiple longitudinal synechiae between the anterior and posterior hypopharyngeal wall mucosa (Figure 2, arrows), leaving narrow passages from the oropharynx to the esophagus (see video at www.otojournal.org. Pharyngeal suspension and endoscopic examination under general anesthesia; the video starts in the esophagus with a 0° telescope slipping upwards, between two mucosal synechiae, to the pharynx).
Figure 1. Diffuse skin scarring on the back of the patient, 5 months after initial diagnosis of Lyell syndrome.
Suspension pharyngoscopy allowed endoscopic lysis and resection of the synechiae with a CO2 laser. Oral steroids, proton-pump inhibitors, and antibiotics were prescribed postoperatively. Swallowing improved greatly, and there were no dysphagia-related complaints at the 6-month follow-up visit.
Discussion Hypopharyngeal dysfunction is rarely reported but should be taken into consideration in patients with LS complaining of persistent dysphagia.4 To our knowledge, our pictures represent a rare, if not unique, documentation of hypopharyngeal synechiae in patients recovering from LS. Since 1 Department of Otolaryngology/Head and Neck Surgery, University Hospital of Geneva, Geneva, Switzerland
Corresponding Author: Zacharias Vourexakis, MD, Department of Otolaryngology/Head and Neck Surgery, University Hospital of Geneva, Rue Gabrielle-Perret-Gentil 4, 1205 Geneva, Switzerland. Email: [email protected]
Downloaded from oto.sagepub.com at University of Sussex Library on August 10, 2015
Vourexakis and Heymans
153 appropriately investigated and resolved; Fre´de´ric Heymans, contribution to the acquisition/analysis/interpretation of data for the work, revising the work critically for important intellectual content, final approval of the version to be published, agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Disclosures Competing interests: None. Sponsorships: None. Funding source: None.
Supplemental Material Additional supporting information may be found at http://otojournal .org/supplemental. Figure 2. Suspension of the larynx and direct pharyngoscopy under general anesthesia revealed multiple adhesions (arrows) between opposing surfaces of the hypopharyngeal mucosa.
clinical examination and swallowing studies may be inadequate for proper assessment, endoscopy under general anesthesia may be crucial in accurate diagnosis and treatment. Author Contributions Zacharias Vourexakis, conception/design of the work, contribution to the acquisition/analysis/interpretation of data, drafting the work and revising it critically for important intellectual content, final approval of the version to be published, agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are
References 1. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part I: introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. J Am Acad Dermatol. 2013;69:173. 2. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis, part II: prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013;69:187. 3. Harr T, French LE. Toxic epidermal necrolysis and StevensJohnson syndrome. Orphanet J Rare Dis. 2010;5:39. 4. Barrera JE, Meyers AD, Hartford EC. Hypopharyngeal stenosis and dysphagia complicating toxic epidermal necrolysis. Arch Otolaryngol Head Neck Surg. 1998;124:1375-1376.
Downloaded from oto.sagepub.com at University of Sussex Library on August 10, 2015
