Progressive Velopharyngeal Insufficiency: A Rare presenting Symptom of Neurofibromatosis Type 1

Johnson AB^1*, Phillips JD², Bright KL³, Ocal E⁴ and Hartzell LD¹

¹Department of Otolaryngology, University of Arkansas for Medical Sciences, Little Rock, AR, USA

²Department of Otolaryngology, Vanderbilt University Medical Center, Nashville TN, USA

³Department of Speech Language Pathology, Arkansas Children’s Hospital, Little Rock, AR, USA

⁴Department of Neurosurgery, University of Arkansas Medical Sciences, Little Rock, AR, USA

*Corresponding Author : Johnson Adam, MD, PhD
Department of Otolaryngology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Tel: 501-364-7546
E-mail: ABJohnson@uams.edu

Received: August 16, 2017 Accepted: September 01, 2017 Published: September 07, 2017

Citation: Johnson AB, Phillips JD, Bright KL, Ocal E, Hartzell LD (2017) Progressive Velopharyngeal Insufficiency: A Rare presenting Symptom of Neurofibromatosis Type 1. J Otol Rhinol 6:6. doi: 10.4172/2324-8785.1000328

Velopharyngeal insufficiency (VPI) is most commonly associated with overt or submucous cleft palate. There are few reports of VPI as a symptom of Neurofibromatosis type 1 (NF1). Here we report a novel case of delayed onset and progressive VPI as the presenting symptom leading to the diagnosis of NF1. Examination revealed nasal air emissions, hypernasality, a persistent velopharyngeal gap and multiple cafe au lait spots. Subsequent neurology consultation revealed changes on MRI consistent with NF1. She was then evaluated by neurosurgery and underwent surgery to address hydrocephalus. With time, the patient has seen improvement in speech. Primary surgical intervention for VPI was avoided. This case underscores the importance of obtaining a complete history and performing a thorough physical exam in all patients.