Charge Sendromu Ön Tanılı Prematüre Hastanın Acil Anal Atrezi Cerrahisinde Anestezi Yönetimi
Özet
Bu olgu sunumu, CHARGE sendromu ön tanısı alan 29 haftalık prematüre bir bebeğin acil anal atrezi cerrahisindeki anestezi yönetimini ele almaktadır. CHARGE sendromu; kolobom, kalp kusurları, koanal atrezi, gelişme geriliği, genital hipoplazi ve kulak anomalileri ile karakterize, nadir görülen genetik bir bozukluktur. Olguda, fetal distres nedeniyle sezaryenle doğan ve çoklu konjenital anomalileri (renal agenezi, ASD, PDA, pulmoner hipertansiyon vb.) bulunan 1663 gramlık hastanın yönetimi detaylandırılmıştır. Preoperatif dönemde hastaya, kardiyak anomalileri nedeniyle PDA’nın açık kalması için alprostadil infüzyonu başlanmıştır. Anestezi yönetimi; zor havayolu hazırlığı, ASA 5E risk sınıflandırması ve pron pozisyonunda cerrahi müdahaleyi içermiştir. İndüksiyonda midazolam ve fentanil, idamede sevofluran ve remifentanil kullanılmıştır. Operasyon sırasında vital bulgular stabil seyretmiş, hasta cerrahi sonrası entübe şekilde yoğun bakıma teslim edilmiştir. Postoperatif 2. günde ekstübe edilen hasta, 7. günde ileri tedavi için sevk edilmiştir. Sonuç olarak, prematüre ve çoklu anomalili CHARGE sendromlu hastalarda, kapsamlı preoperatif değerlendirme ve her türlü komplikasyona hazırlıklı bir anestezi yaklaşımının önemi vurgulanmaktadır.
This case report discusses the anesthetic management of a 29-week-old premature infant with a presumptive diagnosis of CHARGE syndrome during emergency anal atresia surgery. CHARGE syndrome is a rare genetic disorder characterized by coloboma, heart defects, choanal atresia, growth retardation, genital hypoplasia, and ear anomalies. The case details the management of a 1663-gram infant born via cesarean section due to fetal distress, presenting with multiple congenital anomalies including renal agenesis, ASD, PDA, and pulmonary hypertension. In the preoperative period, alprostadil infusion was initiated to maintain PDA patency due to cardiac anomalies. Anesthesia management included difficult airway preparation, ASA 5E risk classification, and surgical intervention in the prone position. Midazolam and fentanyl were used for induction, while sevoflurane and remifentanil were utilized for maintenance. Vital signs remained stable during the procedure, and the patient was transferred to the intensive care unit intubated. The patient was extubated on the second postoperative day and referred to an advanced center on the seventh day. In conclusion, the report emphasizes the importance of comprehensive preoperative evaluation and an anesthetic approach prepared for all complications in premature infants with CHARGE syndrome and multiple anomalies.
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