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Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu

Year 2011, Volume: 18 Issue: 4, 278 - 280, 01.08.2011

Abstract

Beckwith-Wiedemann sendromu; eksofalos, makroglossi, hipoglisemi, organomegali, omfalosel ve gigantizim gibi çeşitli anomalileri içerir. Beckwith-Wiedemann sendromlu olgular sıklıkla infant dönemde cerrahiye gereksinim duyarlar. Anestezi yönetimindeki temel problemler sıklıkla; zor hava yolu ve hipoglisemidir. Makroglossinin maske ventilasyonu ve entübasyonu zorlaştırabileceği akılda tutularak indüksiyondan önce zor hava yolu hazırlığı yapılmalıdır. Dikkatli intraoperatif glukoz takibi hipogliseminin neden olacağı nörolojik sekelleri önlemede oldukça önemlidir. Bu sunuda laringeal maskenin potansiyel zor hava yolu anatomisine sahip Beckwith-Wiedemann sendromlu olguda endotrakael tüpe alternatif olarak kullanılabileceğine değindik. Anahtar kelimeler: Beckwith-Wiedemann Sendromu; Anestezi; Zor Hava Yolu; Laringeal Maske.

References

  • Whizar V, Sinler A, Ontiveros P, et al. Anesthesia for partial glossectomy in a toddler with Beckwith-Widemann syndrome. Anestesia en Mexico 2006; 18: 158-64.
  • Bissonnette B, Luginbuehl I, Marciniak B. Dalens B. Beckwith- Wiedemann syndrome. Syndromes; McGraw-Hill Medical Publishing Division 2006: 87-8.
  • Weksberg R, Shuman C, Smith AC. Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet 2005; 137:12- 34.
  • Gurkowski MA, Rasch DK. Anesthetic consideration for Beckwith-Wiedemann syndrome Anesthesiology 1989; 70: 711-2.
  • Kimura Y, Kamada Y, Kimura S. Anesthetic management of two cases of Beckwith-Wiedemann syndrome. J Anesth 2008; 22: 93- 4.
  • Bahk JA, Han SM, Kim SD. Management of a difficult airways with a laryngeal mask arway under propofol anaesthesia. Paed Anaesth 1999; 9: 163-6.
  • Çelikel V, Basgul E, Karagöz AH. Anesthesia in Beckwith- Wiedemann syndrome. Pediatric Anesthesia 2004; 14: 778-80.
  • Kim Y, Shibutani T, Hirota Y, et al. Anesthesic consideration of two sister with Beckwith-Wiedemann syndrome. Anesth Prog 1996; 43: 24-8.
  • Wiess M, Dullenkopf. Cuffed tracheal tubes in children: past, present and future. Expert Rev Med Devices 2007; 4: 73-82.
  • Goldman LJ, Nodal C, J ımenez E. Successful airway control with the laryngeal mask in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma for central line catheterization. Paediatric Anaesthesia 2000; 10: 445-8.

Anesthetic Management of Beckwith-Wiedemann Syndrome Case: Report

Year 2011, Volume: 18 Issue: 4, 278 - 280, 01.08.2011

Abstract

Beckwith-Wiedemann syndrome consists of various abnormalities, such as exomphalos, macroglossia, hypoglcemia, visceromegaly, omphalocele, gigantism. Patients with Beckwith-Wiedemann syndrome frequently require surgical intervention during infant period. Common problems associated with anesthetic management of Beckwith-Wiedemann syndrome are difficult airway and hypoglycemia. Macroglossia might cause difficult ventilation and intubation for that reason preparation for airway difficulty should be made before induction. Intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae. In this presentation, we addressed the use of laryngeal mask may be an alternation to the endotracheal tube patients with Beckwith-Wiedemann which consist potentially difficult airway anatomy. Key words: Beckwith-Wiedemann Syndrome; Anesthesia, Difficult Airway; Laryngeal Mask.

References

  • Whizar V, Sinler A, Ontiveros P, et al. Anesthesia for partial glossectomy in a toddler with Beckwith-Widemann syndrome. Anestesia en Mexico 2006; 18: 158-64.
  • Bissonnette B, Luginbuehl I, Marciniak B. Dalens B. Beckwith- Wiedemann syndrome. Syndromes; McGraw-Hill Medical Publishing Division 2006: 87-8.
  • Weksberg R, Shuman C, Smith AC. Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet 2005; 137:12- 34.
  • Gurkowski MA, Rasch DK. Anesthetic consideration for Beckwith-Wiedemann syndrome Anesthesiology 1989; 70: 711-2.
  • Kimura Y, Kamada Y, Kimura S. Anesthetic management of two cases of Beckwith-Wiedemann syndrome. J Anesth 2008; 22: 93- 4.
  • Bahk JA, Han SM, Kim SD. Management of a difficult airways with a laryngeal mask arway under propofol anaesthesia. Paed Anaesth 1999; 9: 163-6.
  • Çelikel V, Basgul E, Karagöz AH. Anesthesia in Beckwith- Wiedemann syndrome. Pediatric Anesthesia 2004; 14: 778-80.
  • Kim Y, Shibutani T, Hirota Y, et al. Anesthesic consideration of two sister with Beckwith-Wiedemann syndrome. Anesth Prog 1996; 43: 24-8.
  • Wiess M, Dullenkopf. Cuffed tracheal tubes in children: past, present and future. Expert Rev Med Devices 2007; 4: 73-82.
  • Goldman LJ, Nodal C, J ımenez E. Successful airway control with the laryngeal mask in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma for central line catheterization. Paediatric Anaesthesia 2000; 10: 445-8.
There are 10 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

M. Ali Erdoğan This is me

Aytaç Yücel This is me

Nizamettin Bucak This is me

Mukadder Şanlı This is me

Zekine Begeç This is me

M. Özcan Ersoy This is me

Publication Date August 1, 2011
Published in Issue Year 2011 Volume: 18 Issue: 4

Cite

APA Erdoğan, M. A., Yücel, A., Bucak, N., Şanlı, M., et al. (2011). Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu. Journal of Turgut Ozal Medical Center, 18(4), 278-280.
AMA Erdoğan MA, Yücel A, Bucak N, Şanlı M, Begeç Z, Ersoy MÖ. Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu. J Turgut Ozal Med Cent. August 2011;18(4):278-280.
Chicago Erdoğan, M. Ali, Aytaç Yücel, Nizamettin Bucak, Mukadder Şanlı, Zekine Begeç, and M. Özcan Ersoy. “Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu Sunumu”. Journal of Turgut Ozal Medical Center 18, no. 4 (August 2011): 278-80.
EndNote Erdoğan MA, Yücel A, Bucak N, Şanlı M, Begeç Z, Ersoy MÖ (August 1, 2011) Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu. Journal of Turgut Ozal Medical Center 18 4 278–280.
IEEE M. A. Erdoğan, A. Yücel, N. Bucak, M. Şanlı, Z. Begeç, and M. Ö. Ersoy, “Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu”, J Turgut Ozal Med Cent, vol. 18, no. 4, pp. 278–280, 2011.
ISNAD Erdoğan, M. Ali et al. “Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu Sunumu”. Journal of Turgut Ozal Medical Center 18/4 (August 2011), 278-280.
JAMA Erdoğan MA, Yücel A, Bucak N, Şanlı M, Begeç Z, Ersoy MÖ. Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu. J Turgut Ozal Med Cent. 2011;18:278–280.
MLA Erdoğan, M. Ali et al. “Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu Sunumu”. Journal of Turgut Ozal Medical Center, vol. 18, no. 4, 2011, pp. 278-80.
Vancouver Erdoğan MA, Yücel A, Bucak N, Şanlı M, Begeç Z, Ersoy MÖ. Beckwith-Wiedemann Sendromlu Olguya Anestezik Yaklaşım: Olgu sunumu. J Turgut Ozal Med Cent. 2011;18(4):278-80.