Velopharyngeal Insufficiency Secondary to Endoscopic Endonasal Odontoidectomy and Craniocervical Fusion in a Pediatric Patient With Down Syndrome: A Case Report

IntroductionPost-operative morbidity is common (36%) in patients with Down Syndrome (DS) requiring surgical spine intervention. Underlying anatomical factors such as midface hypoplasia, shorter palates and hypotonia are also contributory. However, there are no reported cases on velopharyngeal insufficiency (VPI) following trans-nasal endoscopic odontoid resection and reconstruction.Case PresentationA 6-year old girl with Down Syndrome (DS) underwent an occipitocervical fusion for atlantoaxial instability and cervical stenosis. Post-operative complications and hardware failure necessitated a revision occipitocervical fusion with extension to the C6 vertebral level. Subsequently, the child presented with symptoms consistent with velopharyngeal insufficiency (VPI), including hypernasal speech and a decline in conversation skills. Speech examination confirmed weak oral airflow and poor articulation of phrases. A video nasopharyngeal endoscopy (VNE) demonstrated coronal pattern of movement was seen with lateral wall motion, and incomplete approximation of the posterior pharyngeal wall, consistent with the diagnosis. The child was managed consistently with dedicated speech therapy. Despite an initial decline of 50-60% in spoken skills, consistent subjective and objective spontaneous improvement was seen with speech rehabilitation.ConclusionThis case highlights the development of VPI due to extensive spinal intervention. However, dedicated rehabilitation endeavors with speech therapy were instrumental in mitigating the symptoms of this patient.