Anesthetic management in a pediatric patient with Noonan syndrome, hypopituitarism and hypothyroidism: A case report

Abstract

Noonan syndrome is a genetically transmitted autosomal dominant disorder characterized by various anatomic anomalies and pathophysiologic derangements. Associ­ated anomalies include hyperthelorism, ptosis, microgna­thia, downward sloping palpebral fissures, low-set ears, abnormal helix of ear, deeply grooved philtrum, short and/ or webbed neck, low hairline and cervical vertebral anom­alies. Patients with Noonan syndrome are known to pres­ent with challenging airways. Tracheal intubation can be difficult because of airway and cervical vertebral anoma­lies and bag mask ventilation may be difficult because of asymmetrical face. We present a case of anesthetic man­agement for Noonan syndrome. J Clin Exp Invest 2013; 4 (2): 238-241

Keywords

• Anesthesia, general, noonan syndrome, air­way management

Panhipopituitarizm, hipotiroidi ve Noonan sendromlu pediatrik hastada anestezik yaklaşım: Olgu sunumu

Abstract

Noonan sendromu değişik patofizyolojik düzensizlikler ve değişik anatomik anomalilerle görülen, otozomal domi­nant genetik geçişle karakterize bir hastalıktır. Bu send­romla ilişkili anomaliler, hipertelorizm, pitozis, mikrognati, aşağı dönük palpebral fissürler, düşük yerleşimli-anormal heliksli kulaklar, derin oluklu filtrum, kısa ve/veya yele bo­yun, düşük ense saç çizgisi şeklinde dismorfik bulgulardır. Noonan sendromu tanısı almış hastalar zor hava yolu ile karşımıza çıkar. Trakeal entübasyon hava yolu ve servikal vertebral anomalilere ve maske ile ventilasyon asimetrik yüz yapısına bağlı olarak zor olabilir. Biz Noonan send­romlu bir olguya anestezik yaklaşımımızı sunmak istedik.

Keywords

• Anestezi, genel, noonan sendromu, hava yolu yönetimi

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