Dental rehabilitation under general anesthesia in an outpatient setting for a child with a heterozygous BCL11B variant: a case report.

backgroundBCL11B-related disorder is a rare autosomal dominant neurodevelopmental syndrome. Patients often present with intellectual disability, speech delay, behavioral abnormalities, craniofacial dysmorphism, T-cell immunodeficiency, and a broad spectrum of dental anomalies (e.g., severe caries, crowding, hypodontia). The convergence of potential airway anomalies, immune dysfunction, and neurobehavioral challenges result in significant complexities for perioperative management, underscoring a critical knowledge gap in safe outpatient anesthetic protocols for this population. CASE PRESENTATION: A 7-year-old boy diagnosed withBCL11B-related disorder, harboring a de novo heterozygous variant in BCL11B (NM_138576.2:c.2461_2462insGAGCCACACCGGCG, p.E821Gfs*28), presented with intellectual disability, delayed speech, behavioral disturbances, and epilepsy, which was treated with levetiracetam. He also exhibited T-cell deficiency, and distinctive craniofacial features, along with severe generalized caries. Comprehensive dental rehabilitation was performed under outpatient general anesthesia. Induction utilized nitrous oxide–oxygen and sevoflurane, followed by nasotracheal intubation with videolaryngoscopy. Treatment, which included restorations, root canal therapies, and extractions, was completed in 105 min. Deep estuation was uneventful, and recovery proceeded smoothly. No complications occurred, and follow-ups at 24 h and 3 days confirmed favorable outcomes and high parental satisfaction.

conclusionComprehensive dental rehabilitation under outpatient general anesthesia can be safely performed in children with BCL11B-related disorder. The success of this procedure depends on thorough preoperative assessment, careful airway management, and effective multidisciplinary coordination.