Cornelia de Lange Sendromlu Pediatrik Olguda Günübirlik Anestezi Yönetimimiz

Abstract

Cornelia de Lange syndrome (CdLS) is a rare congenital syndrome accompanied by significant craniofacial, cardiovascular, gastrointestinal, and musculoskeletal anomalies with dysmorphic facial appearance. As many systems are affected in these patients, anesthesia management can become complex. An 8-year-old, 17 kg, a 31-month-old girl diagnosed with CdLS due to developmental delay applied for cranial and whole spinal magnetic resonance imaging (MRI). The Mallampati score was III in the preoperative examination, the mouth opening was narrow, and the risk of difficult intubation was high. We have prepared all necessary materials following the guidelines. MRI was successfully performed while maintaining the patient's spontaneous breathing under oxygen support. This case report is aimed to raise awareness for this syndrome, where difficult airway and intubation are expected in specialized units such as nonoperating room anesthesia applications, and to review it in the light of the literature.