Sedation-Based Anesthetic Management using Target-Controlled Ketofol in a Child with Cornelia de Lange Syndrome Undergoing Emergency Neck Abscess Drainage: A Case Report

Cornelia de Lange syndrome (CdLS) is a rare congenital disorder characterized by distinctive craniofacial dysmorphism, growth retardation, intellectual disability, ad multisystem anomalies. These features present substantial anesthetic challenges, particularly related to airway management. We report the case of a 6-year-old girl with CdLS who presented for emergency surgical drainage of a rapidly enlarging left-sided neck abscess following failure of medical therapy. Her medical history included severe developmental delay, cleft palate, chronic kidney disease, and oropharyngeal dysphagia. Airway examination revealed micrognathia, limited mouth opening, and midfacial hypoplasia, raising concern for both difficult mask ventilation and intubation. Given the anticipated airway risk and short duration of the procedure, the anesthetic team opted for monitored sedation using a target-controlled infusion (TCI) of ketofol, a combination of ketamine and propofol. Induction was initiated with sevoflurane via a T-piece circuit, followed by intravenous access and titration of TCI ketofol (1.0–2.5 mcg/mL). The patient remained spontaneously ventilating with a nasal airway and T-piece mask support throughout the 30-min procedure. No episodes of airway obstruction, desaturation, or hemodynamic instability were observed. The patient recovered uneventfully in the PACU and was returned to the ward without complication. This case demonstrates the feasibility of using a sedation-based strategy in a high-risk syndromic child undergoing emergency surgery. It highlights the role of TCI ketofol in preserving airway reflexes and respiratory drive. It reinforces the need for careful patient selection, multidisciplinary planning, and individualized anesthetic approaches in children with congenital syndromes.