ANESTHETIC MANAGEMENT OF A PATIENT WITH BECKWİTH-WİEDEMANN SYNDROME
B.KARSLI
1, E.DOĞANBAKI
2, C.ÜNAL
2, N.KAVRUTÖZTÜRK
2
AKDENİZ ÜNİVERSİTESİ TIP FAKULTESİ 1
ANTALYA EĞİTİM ARAŞTIRMA HASTANESİ 2
Introduction
Beckwith-Widemann Syndrome (BWS) occurs in the neonatal period at a frequency of approximately one in 13 700 live births.
We present anesthetic management of a patient with BWS having massive tongue undergoing tonsil-adenoid resection.
Case report
8 years old patient was admitted to otorhinolaringology clinic with a complaint of snoring and apne periods during sleeping. In physical examination ; macroglossia, earlobe fissures, hepatomegaly, asimetry in facial and extremity were seen. Also he is dumb and has mental and motor regression.His mallamapathy score was IV.
The patient was premedicated with Atropine and brought to the operating room. After an assessment was made that ther would be no difficulty in intubation by laringoscopy, anesthesia was induced with 50mg Propofol. When mask ventilation was seen easy 12,5 mg Rocuronium was administered intravenously. Laringoscopy and orotracheal intubation were performed easier then expected. During bleeding control , we started paracetamol infusion for analgesia .At the conclusion of surgery, after he awaked perfectly, we extubated him but we did not take away the orofarengeal airway for probable airway obstruction.
Discussion
BWS presents with variable anomalies which include omphalocele, macroglossia, visceromegaly and severe cardiac defects (1). Upper airway obstruction and difficulties in endotracheal intubation due to macroglossia are critical problems for anesthetic management . Becouse of macroglossia and sleep apne patients with BWS often requires either awake vocal cord inspection or awake tracheal intubation.
In summary patients with BWS may have varied clinical presentation. A comprehensive preoperative examination should include assesment of the airway, cardiovascular status, endocrin status and electrolytes. Preparations for difficult airway management should be made. We recommended iv paracetamol for postoperative analgesia and awake extubation in patients with BWS.
References
1. Sotelo-Avila C, Gonzalez-Crussi F, Fowler JW (1980) Complete and incomplete forms of Beckwith-Wiedemann syndrome: their oncogenic potential. J Pediatr 96:47-50
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