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Crouzon's syndrome with adenotonsillitis: conventional surgery in altered anatomy

Sudhir M Naik, Mohan Appaji, Ravishankar S, Goutham MK, Annapurna SM, N Pinky Devi, Sarika S Naik

Background/Objectives: Crouzon’s syndrome is characterized by premature closure of the cranial sutures, midface hypoplasia, orbital deformities & other associated abnormalities. Children with Crouzon syndrome frequently have obstructive sleep apnea due to the underdevelopment of the midface.

Case report: A 12 year old boy of Crouzon’s syndrome with chronic adeno-tonsillitis was managed by adeno-tonsillectomy under general anaesthesia by scalpel cautery method. The boy responded well to surgery & the mild sleep disorder disappeared within a week uveventfully.

Conclusion: Sleep disorders in this condition can be treated by improving the airway by selective procedures like midface advancement, mandibular expansion , adeno-tonsillectomy, uvulo-palatopharyngoplasty, anterior tongue reduction & endoscopic tracheal granuloma excision.