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Objective To report brain magnetic resonance imaging (MRI) and ultrasonography findings in pediatric patients with congenital idiopathic bilateral vocal fold dysfunction and analyze factors associated with its etiology and resolution. Study Design Case series with retrospective review. Setting Tertiary care multi-institutional setting: Nationwide Children’s Hospital, Indiana University, University of North Carolina, and Cleveland Clinic. Methods Pediatric patients with congenital idiopathic bilateral vocal fold dysfunction were included in this review. Results Congenital idiopathic bilateral vocal fold dysfunction was identified in 74 patients from 2000 to 2018. Brain MRI scans were performed in all patients and ultrasonography in 30 (40.5%). Normal imaging results were most commonly found in patients born full-term ( P < .0001) or via vaginal delivery ( P < .01). Abnormal brain MRI and ultrasound results were found in 38 of 74 (51.3%) and 16 of 30 (53.3%), respectively. Type I Chiari malformation was not identified in any patient. No specific brain MRI or ultrasound abnormality was associated with patients’ bilateral vocal fold dysfunction. Complete/incomplete bilateral vocal fold resolution occurred in 45 of 74 (60.8%) patients over the study interval and was not associated with brain MRI or ultrasound findings or birth complications but was associated with vaginal delivery ( P = .02). Resolution rates were highest for patients with bilateral vocal fold paramedian paralysis ( P = .05). Conclusions In this multi-institutional study, no specific brain MRI or ultrasound abnormality was associated with patients’ bilateral vocal fold dysfunction or subsequent resolution rates. While imaging is often performed to detect and treat any reversible causes of bilateral vocal fold dysfunction, in this series, imaging findings were heterogeneous and did not identify any treatable causes, such as type I Chiari malformation.

2b. Laryngoscope, 2503-2507, 2018.

Background Extramedullary hematopoiesis (EMH) occurs in patients with hematologic disorders, but rarely within the paranasal sinuses. We report a case of EMH in a 17-year-old male with sickle cell disease (SCD) who presented with occipital pain and sinusitis. A computed tomography (CT) scan demonstrated heterogeneous opacification of the right maxillary sinus concerning for allergic fungal sinusitis or a fungal ball with bony erosion. He was taken to the operating room for endoscopic biopsy and a limited endoscopic sinus surgery. Grossly, his maxillary sinus was filled with spiculated osseous tissue. Final pathology demonstrated active hematopoietic bone marrow filling the sinus. Methods We present a case report and literature review of sinonasal EMH. Results We identified 14 articles with 15 patients. EMH was typically associated with SCD or beta thalassemia. The average age of presentation was 30. There was a male sex predilection with a ratio of 11:15. The most common presenting symptom was a headache and nasal obstruction (33% for both). The most common finding on CT was a soft tissue expansile mass (73%). The most commonly affected location was the maxillary sinus (60%). Conclusions This case report serves as a reminder to consider EMH as an uncommon cause of sinus opacification, particularly in patients with SCD or beta thalassemia. The expansion of hematopoietic tissue may be identified as a sinus mass on CT. By recognizing the potential manifestations of chronic anemia, an accurate and timely diagnosis can be made.

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