1041 Recurrent Retropharyngeal Ganglioneuroma in a Pediatric Patient

Abstract

Abstract Introduction Ganglioneuroma is a rare benign subtype of peripheral neuroblastic tumor that arises from sympathetic ganglion cells. Fewer than 10% of these cases occur in the head and neck and are rarely seen in pediatric patients. Clinical symptoms and treatment are dependent on mass location and extension. The authors present a case of recurrent retropharyngeal ganglioneuroma in a pediatric patient. Report of case(s) Patient is a 2-year-old previously healthy female who presented with progressive snoring and found to have retropharyngeal lymphadenopathy during tonsillectomy. Differential diagnosis included retropharyngeal abscess; however, after no improvement with antibiotics, malignancy was suspected. Pathology results from a needle biopsy confirmed the diagnosis of ganglioneuroma. CT imaging showed extension of mass to bilateral retropharyngeal, posterior mediastinal and paraspinal, and retroperitoneal spaces. Given concerns for significant morbidity with complete resection, patient underwent partial resection of mass, followed by chemotherapy to decrease tumor burden. Patient initially did well, then developed nine-month progressive snoring, positional apnea, persistent stertor, and dysphagia. Sleep study showed severe obstructive sleep apnea (OSA) with apnea hypopnea index (AHI) 74, oxygen nadir 66%, and maximum transcutaneous carbon dioxide (CO2) level of 58 mmHg. Repeat CT imaging showed regrowth of the tumor with large right-sided retropharyngeal mass, mass effect on right oropharynx and associated vasculature, and moderate narrowing of airway. Supplemental oxygen via nasal cannula was initiated as inpatient, due to concerns that noninvasive positive pressure support would further compromise the critical airway. Urgent repeat debulking of the tumor with partial pharyngectomy was completed. Post-operatively, patient's oxygen dependence, stertor, and positional apnea resolved within 24 hours. Repeat sleep study showed considerable improvement with mild OSA, AHI 1.4, oxygen nadir 89%, and maximum transcutaneous CO2 level of 47 mmHg. Her dysphagia and residual mild intermittent snoring are improving with no signs of recurrence of the mass. Conclusion Upper airway obstruction secondary to neck mass in pediatric patients is rare and even less commonly found to be recurrent ganglioneuroma. This case offers further understanding of the complexities of diagnosing and treating insidiously growing retropharyngeal tumors and the prompt management of critically obstructed airways. Support (if any)