ELİF DOĞAN BAKI, Ceyda ÜNAL, Nilgün ÖZTÜRK KAVRUT, Mehmet Ümit ÇELİK, BİLGE KARSLI

Beckwith-Wiedemann Sendromlu Hastada Anestezi Yönetimi

Beckwith-Widemann sendromu (BWS) neonatal periyotta yaklaşık 13700 canlı doğumda 1 görülür. Bu sendrom makroglossi, omfalosel, viseromegali, gigantism, neonatal hipoglisemi gibi birçok anomaliler içerir. Havayolu yönetimini makroglossinin sebep olabileceği zor ventilasyon ve endotrakeal entubasyonun komplike hale getirmesi beklenir. Bu yüzden anestezi induksiyonu öncesi zor havayolu hazırlığı düşünülmelidir. BWS’da diğer bir problemde postoperatif analjezidir. Çünkü opioid kullanımı solunumu deprese edebilkirken, nonsteroid antiinflamatuar ilaçlarda kanamaya yol açabileceğinden postoperatif analjezi yönetimi dikkatlice planlanmalıdır. Biz büyük bir dile sahip, adenotonsillektomi ameliyatı yapılacak 8 yaşında Beckwith-Widemann sendromlu hastada anestezi yönetimini sunduk

Anahtar Kelimeler:

Beckwith-Wiedemann sendromu; havayolu yönetimi; postoperatif analjezi

Beckwith-Wiedemann Sendromlu Hastada Anestezi Yönetimi

Beckwith-Widemann Syndrome (BWS) occurs in the neonatal period at a frequency of approximately one in 13 700 live births. This syndrome consists of abnormalities, such as; macroglossia, omphalocele, visceromegaly, gigantism and neonatal hypoglycemia. It is expected that airway management would be complicated by macroglossia which might cause difficult ventilation and endotracheal intubation. Therefore preparations for airway difficulty should be considered before anesthesia induction. Another problem in BWS is postoperative analgesia. Because opioids can depress breathing and non steroid antiinflammatory drugs can increase bleeding, postoperative analgesia should be planned carefully. We present anesthetic management of an eight years old patient with BWS having big tongue undergoing tonsiladenoid resection

Keywords:

management; postoperative analgesia,

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Beckwith JB: Extreme cytomegaly of the adrenal cortex, omphalocele, hyperplasia of kidneys and pan- creas and Leydig-cell hyperplasia: another syndrome? Presented at Annual Meeting of the Western Society for Pediatric Research 1963; Los Angeles.
Wiedemann HR. Complexe Malformatif Avec Hernie Ombilicale Et Macroglossie: Un 'Syndrome Nouveau?' J Genet Hum 1964;13:223-32.
Marret E, Flahault A, Samama CM, Bonnet F. Ef- fects of Postoperative, Nonsteroidal, Antiinflammato- ry Drugs on Bleeding Risk After Tonsillectomy: Meta- Analysis of Randomized, Controlled Trials. Anesthesi- ology 2003;98:1497-502.
Lundeberg S, Lönnqvist PA. Update on Systemic Postoperative Analgesia in Children. Paediatr Anaesth 2004;14:394-7.
Viitanen H, Annila P. Analgesic Efficacy of Tramadol 2 Mg Kg(-1) for Pediatric Day-Case Adenoidectomy. Br J Anaesth 2001;86:572-5.
Graham GG, Scott KF. Mechanism of Action of Paracetamol. Am J Ther 2005;12:46-55.
Wurthwein G, Koling S, Reich A, et al. Pharmacoki- netics of Intravenous Paracetamol in Children and Adolescents Under Major Surgery. Eur J Clin Pharmacol 2005;60:883-8.
Sotelo-Avila C, Gonzalez-Crussi F, Fowler JW. Com- plete and Incomplete Forms of Beckwith-Wiedemann Syndrome: Their Oncogenic Potential. J Pediatr 1980;96:47-50.
Gurkowski MA, Rasch DK. Anesthetic Considera- tions for Beckwith-Wiedemann Syndrome. Anesthe- siology 1989;70:711-2.
Filippi G, McKusick VA. The Beckwith-Wiedemann Syndrome. Medicine (Baltiomore) 1970;49:279-98.
Kim Y, Shibutani T, Hirota Y, et al. Anesthetic Con- siderations of Two Sisters with Beckwith-Wiedemann Syndrome. Anesth. Prog 1996;43:24-8.
Kimura Y, Kamada Y, Kimura S. Anesthetic Man- agement of Two Cases of Beckwith-Wiedemann Syn- drome. J Anesth 2008;22:93-5.
Greenwood R, Sommer A, Rosenthal A, et al. Car- diovascular Wiedemann Syndrome. Am J Dis Child 1977;131:293- in the Beckwith
Alhashemi JA, Daghistani MF. Effect of Intraopera- tive Intravenous Acetaminophen Vs. Intramuscular Meperidine on Pain and Discharge Time After Paedi- atric Dental Restoration. Eur J Anaesthesiol 2007;24:128-33.
Yarkan Uysal H, Akin Takmaz S, Yaman F, Baltaci B, Başar H. The Efficacy of Intravenous Paracetamol Versus Tramadol for Postoperative Analgesia After Adenotonsillectomy in Children.Journal of Clinical Anesthesia 2011;23:53-7.