Patau sendromu ön tanılı pediyatrik olguda anestezik yönetim: Olgu sunumu ve literatüre kısa bir bakış

Patau sendromu (trizomi 13) multipl organ sistem anomalileri ile seyreden bir kromozomal sendromdur. En sık bulguları kraniyofasyal ve kardiyak anomalilerdir. Patau sendromlu olgular anesteziyologlar açısından, hava yolu kontrolünün güçlüğü, pozisyon vermede zorluk, ağır olgularda sıvı tedavisinin düzenlenmesinde güçlük gibi sorunlar nedeni ile özelliklidir. 2 aylık kız hastaya konjenital katarakt nedeni ile acil cerrahi operasyon uygulandı. Patau sendromu ön tanısı ile izlenen hastada ASD, VSD, dekstrokardi, hafif mikrognati mevcuttu. Anestezi indüksiyonunu takiben damar yolu açılarak atrakuryum ve remifentanil ile idame sağlandı. Operasyon sonunda sorunsuz derlenen hasta postoperatif dönemde de komplikasyonsuz izlendi. Sonraki süreçte muayene için multipl sayıda inhalasyon anestezisi uygulanan hastanın ek sorunu olmadı. Bu makalede; anesteziyologlar açısından özellikli bir hasta grubu olan, patau sendromu ön tanısı ile izlenen bir hastanın anestezik yönetimini ve anesteziyolojik yaklaşımı literatür ışığında tartışmayı amaçladık.

Anesthetic management of a pediatric patient prediagnosed as Patau syndrome: Case report and short overview of the literature

Patau syndrome (trisomi 13) is a chromosomal syndrome with multisystemic features. The major findings are craniofascial and cardiac abnormalities. Patients with Patau syndrome create a unique group for anesthesiologists because of difficult airways, positional difficulties, and fluid replacement challenges in severe cases. A 2 month old female patient was scheduled for an semi-emergent operation for congenital cataract. The patient was prediagnosed with Patau syndrome and had ASD, VSD, dextrocardia and mild micrognathia. After anesthesia induction and insertion of an iv line intubation was performed with atracurium. For anesthesia maintenance o2/air/sevofluran mixture and a low dose remifentanil infusion was used. The operation and recovery was uneventful. The postoperative period was also uncomplicated. In follow-up, the patient was anesthetised multiple times for evaluation without further problems. Patients with Patau syndrome are a unique group for the anesthesiologist. In this paper we discuss the anesthesiologic management and literature in a patient prediagnosed as Patau syndrome with mild symptoms.

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