Overview
Cherubism is a rare, autosomal dominant genetic disorder characterized by bilateral, symmetrical enlargement of the mandible and maxilla, often accompanied by gingival fibromatosis. It typically presents in childhood with progressive bone lesions that can complicate airway management 1.Diagnosis
Clinical Features: Bilateral mandibular and maxillary enlargement, characteristic facial appearance resembling cherubs.
Family History: Positive family history is crucial for diagnosis.
Radiographic Imaging: Multilocular, expansile radiolucencies in the mandible are indicative 1.
Genetic Testing: Not routinely required but can confirm mutations in SH3BP2 gene in some cases 1.Management
Surgical Intervention: Removal of mandibular lesions when symptomatic or causing functional impairment 1.
Anaesthetic Considerations: Careful airway assessment due to potential for difficult intubation; nasotracheal intubation may be necessary 1.
Pharmacological Support: Use of inhalational agents (e.g., sevoflurane) and opioids (e.g., sufentanil) for anesthesia 1.
Local Anesthesia: Peripheral venous access facilitated by topical anesthetics (e.g., lidocaine, prilocaine) 1.Special Populations
Pediatrics: Increased vigilance for airway management due to mandibular enlargement; successful nasotracheal intubation reported 1.
Comorbidities: No specific comorbidities highlighted in the provided abstracts; focus on managing anesthesia risks associated with airway challenges 1.Key Recommendations
Conduct thorough preoperative airway assessment, including radiographic evaluation, in patients with cherubism to anticipate potential difficulties 1 (Evidence: Moderate).
Consider nasotracheal intubation in pediatric patients with cherubism undergoing surgical procedures involving the mandible 1 (Evidence: Weak).
Utilize multimodal analgesia and appropriate inhalational agents for anesthesia management to minimize respiratory complications 1 (Evidence: Expert opinion).References
1 E Gentili M, Levreil E, Bedhet N. Cherubism and anaesthesia. Anaesthesiology intensive therapy 2021. link