<![CDATA[Sindroma Apert adalah suatu gangguan genetik yang ditandai dengan penggabungan tulang yang terlalu dini (kraniosinostosis). Penggabungan dini tersebut menghambat pertumbuhan normal tulang dan mempengaruhi pertumbuhan bentuk kepala dan wajah. Penggabungan dini tulang kepala dapat mempengaruhi perkembangan otak bahkan menyebabkan peningkatan tekanan intrakranial, dan pada sindroma Apert juga didapatkan penggabungan beberapa jari tangan dan kaki (sindaktili). Seorang anak berusia 2 tahun dengan sindroma Apert. Tanda klinis peningkatan tekanan intrakranial belum didapatkan sehingga operasi dijadwalkan terencana dan pasien dirawat terlebih dahulu di ruangan. Kemungkinan kesulitan untuk intubasi dengan laringoskopi diantisipasi dengan persiapan intubasi menggunakan optik fiber dan trakeostomi. Pasien diberikan premedikasi midazolam 0,5 mg intravena pada saat pasien akan dibawa ke kamar operasi. Dilakukan anestesi umum, induksi menggunakan propofol 30 mg, fentanil 30 μg diberikan 3 menit sebelum intubasi. Fasilitas intubasi dengan vekuronium 2 mg, pemeliharaan anestesi dengan N2O/O2 dan Sevofluran. Vekuronium diberikan 1 mg /jam. Ventilasi kendali menggunakan ETT no. 5,0. Operasi berlangsung selama 6 jam dengan posisi pasien terlentang. Hemodinamik selama operasi relatif stabil, tekanan darah sistolik berkisar 90-110 mmHg, tekanan darah diastolik 50-70 mmHg, laju nadi (HR) 87-110 x/mnt, SaO2 99-100 %. Setelah operasi berakhir pasien bernafas spontan adekuat dan dilakukan ekstubasi di kamar operasi. Pasca operasi pasien di rawat di PICU hingga hari ke-4 pasien dipindahkan ke ruangan. Gangguan penggabungan tulang kepala yang terlalu dini dapat menyebabkan gangguan pertumbuhan bentuk kepala, otak dan gangguan pendengaran dan penglihatan. Selain itu juga dapat menyebabkan terjadinya peningkatan tekanan intrakranial. Koreksi segera dengan melakukan suturektomi dan dekompresi dapat mencegah kemungkinan-kemungkinan tersebut. Anaesthetic management of patient with Apert syndrome which undergo suturectomy Apert syndrome is a genetic disorder characterized by the premature fusion of certain skull bones (craniosynostosis). This early fusion prevents the skull from growing normally and affects the shape of the head and face. Early fusion of the skull bones also affects the development of the brain and even can increased the intracranial pressure. In apert syndrome there was also fusion of fingers and toes (syndactyly). A 2 years old child with Apert syndrome which undergo suturectomy and decompression. The clinical signs of raised intracranial pressure in this patient has not been obtained yet so the surgery was done as scheduled . Difficulties to perform intubation with direct laryngoscopy were anticipated through the use of fiber optic and preparation of tracheostomy. Patient has been given premedication using midazolam 0,5 mg given intravenously before his admission to the operating room. The surgery is performed with general anesthesia using propofol 30 mg then fentanyl 30mcg, 3 minutes before intubation. Vecuronium 2mg was given to facilitates intubation. Maintenance of anesthesia with Nitroons/O2 sevoflurane and Vecuronium 1mg/hour. Ventilation was controlled by using ETT no 5.0. Patient was in supine position, and it last for 6 hours. There was relatively stable hemodynamics, systolic blood pressure range 90-110 mmHg, diastolic blood pressure 50-70 mmHg, pulse rate 87-110x/minutes, SaO2 99-100%. After the operation, there was adequate spontaneous breathing so extubation was performed in the operating room, then he was referred to PICU. On day 4 patient was moved to the room. Premature fusion of skull bones will cause growth disorders of the head, brain, and hearing and vision impairment. It also can cause increased intracranial pressure. Immediate correction by suturectomy and decompression can prevent this possibility.]]>
