Literature DB >> 27973931

Anesthetic Management of a Patient With Emanuel Syndrome.

Masanori Tsukamoto1, Takashi Hitosugi2, Kanako Esaki1, Takeshi Yokoyama2.   

Abstract

Emanuel syndrome is associated with supernumerary chromosome, which consists of the extra genetic material from chromosome 11 and 22. The frequency of this syndrome has been reported as 1 in 110,000. It is a rare anomaly associated with multiple systemic malformations such as micrognathia and congenital heart disease. In addition, patients with Emanuel syndrome may have seizure disorders. We experienced anesthetic management of a patient with Emanuel syndrome who underwent palatoplasty. This patient had received tracheotomy due to micrognathia. In addition, he had atrial septal defect, mild pulmonary artery stenosis, and cleft palate. Palatoplasty was performed without any complication during anesthesia. Close attention was directed to cardiac function, seizure, and airway management.

Entities:  

Keywords:  Congenital heart disease; Emanuel syndrome; General anesthesia

Mesh:

Year:  2016        PMID: 27973931      PMCID: PMC5157147          DOI: 10.2344/16-00028.1

Source DB:  PubMed          Journal:  Anesth Prog        ISSN: 0003-3006


  8 in total

1.  Anesthetic management of Pallister-Killian syndrome using a Bispectral Index monitor in a patient with severe seizures.

Authors:  Shinichiro Kira
Journal:  J Clin Anesth       Date:  2011-12       Impact factor: 9.452

Review 2.  Prevalence of Emanuel syndrome: theoretical frequency and surveillance result.

Authors:  Tamae Ohye; Hidehito Inagaki; Takema Kato; Makiko Tsutsumi; Hiroki Kurahashi
Journal:  Pediatr Int       Date:  2014-08       Impact factor: 1.524

3.  Anesthetic management of pediatric patients with Emanuel syndrome.

Authors:  Reiko Nishinarita; Takahiro Mihara; Nobuhito Nakamura; Yoshihisa Miyamoto; Koui Ka
Journal:  J Anesth       Date:  2015-01-21       Impact factor: 2.078

4.  The effects of sevoflurane and hyperventilation on electrocorticogram spike activity in patients with refractory epilepsy.

Authors:  Naoko Kurita; Masahiko Kawaguchi; Tohru Hoshida; Hiroyuki Nakase; Toshisuke Sakaki; Hitoshi Furuya
Journal:  Anesth Analg       Date:  2005-08       Impact factor: 5.108

5.  Phenotypic delineation of Emanuel syndrome (supernumerary derivative 22 syndrome): Clinical features of 63 individuals.

Authors:  Melissa T Carter; Stephanie A St Pierre; Elaine H Zackai; Beverly S Emanuel; Kym M Boycott
Journal:  Am J Med Genet A       Date:  2009-08       Impact factor: 2.802

6.  Intubation of infants with Pierre Robin syndrome: the use of the paraglossal approach combined with a gum-elastic bougie in six consecutive cases.

Authors:  F Semjen; M Bordes; A-M Cros
Journal:  Anaesthesia       Date:  2008-02       Impact factor: 6.955

7.  Cardiac output measurement in children: comparison of Aesculon cardiac output monitor and thermodilution.

Authors:  M Tomaske; W Knirsch; O Kretschmar; K Woitzek; C Balmer; A Schmitz; U Bauersfeld; M Weiss
Journal:  Br J Anaesth       Date:  2008-02-27       Impact factor: 9.166

8.  Emanuel syndrome: A rare disorder that is often confused with Kabuki syndrome.

Authors:  Shailendra Kapoor
Journal:  J Pediatr Neurosci       Date:  2015 Apr-Jun
  8 in total

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