Skip to main content
Top
Published in: Journal of Anesthesia 3/2015

01-06-2015 | Clinical Report

Anesthetic management of pediatric patients with Emanuel syndrome

Authors: Reiko Nishinarita, Takahiro Mihara, Nobuhito Nakamura, Yoshihisa Miyamoto, Koui Ka

Published in: Journal of Anesthesia | Issue 3/2015

Login to get access

Abstract

Emanuel syndrome is a rare anomaly associated with multiple systemic malformations. We present two cases involving pediatric patients with Emanuel syndrome. The first patient presented with micrognathia and had patent ductus arteriosus and a single kidney. The patient was difficult to intubate with McGRATH® but was successfully intubated with an Airtraq® device. The second patient did not present with micrognathia and was not difficult to intubate. A thorough examination of the heart, kidney, and spinal cord is important when planning the anesthetic management of patients with Emanuel syndrome. Moreover, adequate preparation for a difficult airway is essential, and the Airtraq® device may be useful for intubating patients with Emanuel syndrome with micrognathia.
Literature
1.
go back to reference Drum ET, Herlich A, Levine B, Mayhew JF. Anesthesia in a patient with chromosome 11;22 translocation: a case report and literature review. Paediatr Anaesth. 2005;15:985–7.PubMedCrossRef Drum ET, Herlich A, Levine B, Mayhew JF. Anesthesia in a patient with chromosome 11;22 translocation: a case report and literature review. Paediatr Anaesth. 2005;15:985–7.PubMedCrossRef
2.
go back to reference Carter MT, St Pierre SA, Zackai EH, Emanuel BS, Boycott KM. Phenotypic delineation of Emanuel syndrome (supernumerary derivative 22 syndrome): clinical features of 63 individuals. Am J Med Genet A. 2009;149A:1712–21.PubMedCentralPubMedCrossRef Carter MT, St Pierre SA, Zackai EH, Emanuel BS, Boycott KM. Phenotypic delineation of Emanuel syndrome (supernumerary derivative 22 syndrome): clinical features of 63 individuals. Am J Med Genet A. 2009;149A:1712–21.PubMedCentralPubMedCrossRef
3.
go back to reference Hirabayashi Y, Shimada N, Nagashima S. Tracheal intubation using pediatric Airtraq optical laryngoscope in a patient with Treacher Collins syndrome. Paediatr Anaesth. 2009;19:915–6.PubMedCrossRef Hirabayashi Y, Shimada N, Nagashima S. Tracheal intubation using pediatric Airtraq optical laryngoscope in a patient with Treacher Collins syndrome. Paediatr Anaesth. 2009;19:915–6.PubMedCrossRef
4.
go back to reference Péan D, Desdoits A, Asehnoune K, Lejus C. Airtraq laryngoscope for intubation in Treacher Collins syndrome. Paediatr Anaesth. 2009;19:698–9.PubMedCrossRef Péan D, Desdoits A, Asehnoune K, Lejus C. Airtraq laryngoscope for intubation in Treacher Collins syndrome. Paediatr Anaesth. 2009;19:698–9.PubMedCrossRef
5.
go back to reference Vlatten A, Soder C. Airtraq optical laryngoscope intubation in a 5-month-old infant with a difficult airway because of Robin sequence. Paediatr Anaesth. 2009;19:699–700.PubMedCrossRef Vlatten A, Soder C. Airtraq optical laryngoscope intubation in a 5-month-old infant with a difficult airway because of Robin sequence. Paediatr Anaesth. 2009;19:699–700.PubMedCrossRef
6.
go back to reference Iwai H, Kanai R, Takaku Y, Hirabayashi Y, Seo N. Successful tracheal intubation using the pediatric Airtraq optical laryngoscope in a pediatric patient with Robin sequence. Masui. 2011;60:189–91.PubMed Iwai H, Kanai R, Takaku Y, Hirabayashi Y, Seo N. Successful tracheal intubation using the pediatric Airtraq optical laryngoscope in a pediatric patient with Robin sequence. Masui. 2011;60:189–91.PubMed
7.
go back to reference Raymondos K, Seidel T, Sander B, Gerdes A, Goetz F, Helmstädter V, et al. The intubation scoop (i-scoop)—a new type of laryngoscope for difficult and normal airways. Anaesthesia. 2014;69:990–1001.PubMedCrossRef Raymondos K, Seidel T, Sander B, Gerdes A, Goetz F, Helmstädter V, et al. The intubation scoop (i-scoop)—a new type of laryngoscope for difficult and normal airways. Anaesthesia. 2014;69:990–1001.PubMedCrossRef
8.
go back to reference Apfelbaum JL, Hagberg CA, Caplan RA, Blitt CD, Connis RT, Nickinovich DG, et al. Practice guidelines for management of the difficult airway: an updated report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway. Anesthesiology. 2013;118:251–70.PubMedCrossRef Apfelbaum JL, Hagberg CA, Caplan RA, Blitt CD, Connis RT, Nickinovich DG, et al. Practice guidelines for management of the difficult airway: an updated report by the American Society of Anesthesiologists Task Force on Management of the Difficult Airway. Anesthesiology. 2013;118:251–70.PubMedCrossRef
9.
go back to reference Lin AE, Bernar J, Chin AJ, Sparkes RS, Emanuel BS, Zackai EH. Congenital heart disease in supernumerary der(22), t(11;22) syndrome. Clin Genet. 1986;29:269–75.PubMedCrossRef Lin AE, Bernar J, Chin AJ, Sparkes RS, Emanuel BS, Zackai EH. Congenital heart disease in supernumerary der(22), t(11;22) syndrome. Clin Genet. 1986;29:269–75.PubMedCrossRef
10.
go back to reference Fraccaro M, Lindsten J, Ford CE, Iselius L. The 11q; 22q translocation: a European collaborative analysis of 43 cases. Hum Genet. 1980;56:21–51.PubMedCrossRef Fraccaro M, Lindsten J, Ford CE, Iselius L. The 11q; 22q translocation: a European collaborative analysis of 43 cases. Hum Genet. 1980;56:21–51.PubMedCrossRef
11.
go back to reference Ohtsuka K, Kiyono S, Takimoto M. A potential hazard of spinal cord injury by spinal and epidural anesthesia in a patient with low-placed conus medullaris (author’s transl). Masui. 1977;26:967–79.PubMed Ohtsuka K, Kiyono S, Takimoto M. A potential hazard of spinal cord injury by spinal and epidural anesthesia in a patient with low-placed conus medullaris (author’s transl). Masui. 1977;26:967–79.PubMed
Metadata
Title
Anesthetic management of pediatric patients with Emanuel syndrome
Authors
Reiko Nishinarita
Takahiro Mihara
Nobuhito Nakamura
Yoshihisa Miyamoto
Koui Ka
Publication date
01-06-2015
Publisher
Springer Japan
Published in
Journal of Anesthesia / Issue 3/2015
Print ISSN: 0913-8668
Electronic ISSN: 1438-8359
DOI
https://doi.org/10.1007/s00540-014-1955-y

Other articles of this Issue 3/2015

Journal of Anesthesia 3/2015 Go to the issue