Joubert Sendromlu Hastanın Diş Tedavilerinde Anestezi Yönetimi: Olgu Sunumu

Abstract

Joubert sendromu nadir görülen, psikomotor gelişme geriliği, nistagmus, ataksi ve tekrarlayan takipne-apne nöbetleri ile karakterize otozomal resesif geçişli bir hastalıktır. Mikrosefali, polidaktili, retina displazisi, böbrek kistleri ve dil tümörleri sendroma eşlik edebilen anomalilerdir. Joubert sendromlu hastalarda değişen derecelerde serebellar vermis displazisi görülür. Bu çocuklarda anestezi uygulanması perioperatif solunum problemleri ile komplike olabilir. Kranial magnetik rezonans görüntülemede serebellar vermis hipoplazisinin neden olduğu molar diş görüntüsü nöroradyolojik tanı kriteridir. Bu yazıda Joubert sendromlu bir hastada diş tedavisi sırasında anestezi yönetimimiz sunulmaktadır.

Keywords

• Joubert sendromu
• genel anestezi
• anestezik yönetim
• inhalasyon anestezisi
• remifentanil
• endotrakeal entübasyon

Anesthesia Management in Dental Treatments of a Patient with Joubert Syndrome: Case Report

Abstract

Joubert syndrome is a rare, autosomal recessive disease characterized by psychomotor developmental delay, nystagmus, ataxia and recurrent tachypnea-apnea seizures. Microcephaly, polydactyly, retinal dysplasia, kidney cysts and tongue tumors are anomalies that may accompany the syndrome. Varying degrees of cerebellar vermis dysplasia are observed in patients with Joubert syndrome. Anesthesia administration in these children may be complicated by perioperative respiratory problems. The appearance of molar teeth caused by cerebellar vermis hypoplasia on cranial magnetic resonance imaging is a neuroradiological diagnostic criterion. In this article, our anesthesia management during dental treatment in a patient with Joubert syndrome is presented.

Keywords

• Joubert syndrome
• general anesthesia
• anesthetic management
• inhalation anesthesia
• remifentanil
• endotracheal intubation

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