Operative Management of OSAS in a Complex Case of Proteus Syndrome

Area of Science:

• Pediatric Otolaryngology
• Genetics and Rare Diseases
• Sleep Medicine

Background:

• Obstructive sleep apnea syndrome (OSAS) is prevalent in children with craniofacial malformations.
• Proteus Syndrome (PS) involves asymmetric overgrowth and lymphatic tissue hypertrophy, potentially causing airway obstruction.
• Palatine tonsil hypertrophy is a common cause of pediatric OSAS.

Purpose of the Study:

• To report a novel treatment for OSAS in a pediatric patient with Proteus Syndrome.
• To describe the successful use of bipolar radiofrequency-induced thermotherapy (RFITT) for partial tonsillectomy under local anesthesia.
• To highlight the management of asymmetric tonsillar hypertrophy causing OSAS in a rare genetic disorder.

Main Methods:

• A 12-year-old boy with Proteus Syndrome and OSAS underwent partial resection of the left palatine tonsil.
• Bipolar radiofrequency-induced thermotherapy (RFITT) was employed under local anesthesia to minimize risks associated with general anesthesia.
• The procedure focused on removing the obstructive portion of the hypertrophied tonsil.

Main Results:

• Successful treatment of obstructive respiratory disease was achieved.
• The patient experienced recovery from OSAS symptoms following the procedure.
• This represents the first reported case of partial tonsillar resection under local anesthesia for OSAS in a patient with Proteus Syndrome.

Conclusions:

• Partial tonsillectomy using RFITT under local anesthesia is a viable and effective treatment for OSAS in pediatric patients with Proteus Syndrome.
• This minimally invasive approach can safely address tonsillar hypertrophy contributing to airway obstruction in complex genetic syndromes.
• Further research is warranted to explore the long-term efficacy and applicability of this technique in similar cases.