Radiological Case of the Month | Congenital Defects | JAMA Pediatrics | JAMA Network
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November 2000

Radiological Case of the Month

Author Affiliations


Arch Pediatr Adolesc Med. 2000;154(11):1167. doi:

Denouement and Discussion: Velopharyngeal Insufficiency Causing Nasopharyngeal Reflux in the Neonate

Figure 1. Swallowing study using fluoroscopy shows reflux of contrast from the oropharynx into the nasopharynx and out of the nostrils.

Figure 2. Swallowing study using fluoroscopy showing part of the contrast passed down the esophagus to the stomach and a silent aspiration episode into the trachea.

Definitive diagnosis of velopharyngeal insufficiency (VPI) was made by fiberoptic and nasopharyngoscopy. Initially, this infant had poor sucking, swallowing, and breathing coordination, and needed to rest between sucks. He was treated with cisapride to enhance esophageal motility, and after treatment for a week with parenteral fluids, he began oral feeding using a "cleft palate" nipple. Gradual improvement in feeding tolerance and reduction in episodes of nasopharyngeal reflux occurred over the next week. He still had occasional regurgitation episodes and was diagnosed with failure to thrive by age 3 months. Follow-up fiberoptic nasopharyngoscopy revealed persistent VPI.

Velopharyngeal insufficiency represents inability to separate the content of the oropharynx from the nasopharynx during feeding and speech by the muscles of the palate and the posterior and lateral pharyngeal wall. It is a relatively uncommon diagnosis in children and has not been reported in the neonate.1 Velopharyngeal insufficiency may be organic or functional, produced by congenital or acquired causes or due to paresis or local disorders. It most frequently occurs as a result of cleft palate, submucous cleft palate (any combination of bifid uvula, muscular diastasis of soft palate, or bony defect of the hard palate) or following adenoidectomy.1 Other entities associated with VPI are DiGeorge syndrome, velocardiofacial syndrome, neurofibromatosis, hemifacial microsomia (facial-auriculovertebral malformation complex),2 Chiari malformation,3 myasthenia gravis, histiocytosis (eosinophilic granuloma), Pierre Robin syndrome, Dubowitz syndrome,4 Kabuki make-up syndrome,5 encephalopathy, peritonsillar abscess, Möbius syndrome, and myotonic dystrophy.6 Familial cases of VPI with autosomal dominant inheritance have been reported.7 Velopharyngeal insufficiency may be complicated by speech hypernasality, snoring,8 Eustachian tube dysfunction, and chronic otitis media with conductive hearing loss and paranasal sinus infections.9

Velopharyngeal insufficiency may be diagnosed by multiview videofluoroscopy10 but the gold standard for diagnosis is flexible fiberoptic nasal endoscopy.11 Rapid magnetic resonance imaging is a costly but sensitive method to evaluate VPI.12 Treatment modalities include speech therapy for minimal VPI and dental prosthesis for cases in which surgery is contraindicated. The mainstay of treatment for posterior pharyngeal wall augmentation includes using materials such as paraffin, fascia, autologous cartilage, and Teflon (DuPont, Wilmington, Del) paste.13 If these measures fail, a push-back palatoplasty can be attempted. Other surgical procedures used are sphincter pharyngoplasty and palatopharyngoplasty (velopharyngeal flap).14 Obstructive sleep apnea has been reported as a complication of pharyngeal flap surgery.

Accepted for publication February 18, 1999.

Reprints: Oussama Itani, MD, Director of Neonatology, Borgess Medical Center, 1521 Gull Rd, Kalamazoo, MI 49048.

Hirschberg  J Pediatric otolaryngological relations of velopharyngeal insufficiency.  Int J Pediatr Otorhinolaryngol. 1983;2199- 212Google ScholarCrossref
Shprintzen  RJCroft  CBBerkman  MDRakoff  SJ Velopharyngeal insufficiency in the facio-auriculo-vertebral malformation complex.  Cleft Palate J. 1980;17132- 137Google Scholar
Gerard  CLDugas  MNarcy  PHertz-Pannier  J Chiari malformation type I in a child with velopharyngeal insufficiency.  Dev Med Child Neurol. 1992;34174- 176Google ScholarCrossref
Lerman-Sagie  TMerlob  PShuper  A  et al.  New findings in a patient with Dubowitz syndrome: velopharyngeal insufficiency and hypoparathyroidism.  Am J Med Genet. 1990;37241- 243Google ScholarCrossref
Lan  KMichiwaki  YSunaga  SMichi  K Congenital velopharyngeal incompetence in Kabuki make-up syndrome.  Int J Oral Maxillofac Surg. 1995;24298- 300Google ScholarCrossref
Caldarelli  DD Incidence and type of otopathology associated with congenital palatopharyngeal incompetence.  Laryngoscope. 1978;881970- 1978Google ScholarCrossref
Andres  RBixler  DShanks  JCSmith  WL Dominant inheritance of velopharyngeal incompetence.  Clin Genet. 1981;19443- 447Google ScholarCrossref
Ishikawa  YAmitani  R Nasal and paranasal sinus disease in patient with velopharyngeal insufficiency.  Arch Otolaryngol Head Neck Surg. 1994;120861- 865Google ScholarCrossref
Durr  DGShapiro  RS Otologic manifestations in congenital velopharyngeal insufficiency.  AJDC. 1989;4375- 77Google Scholar
Stringer  DAWitzer  MA Velopharyngeal insufficiency on multiview videoflouroscopy: a comparison of projections.  AJR Am J Roentgenol. 1986;14615- 19Google ScholarCrossref
Conley  SFGosain  AKMarks  SMLarson  DL Identification and assessment of velopharyngeal inadequacy.  Am J Otolaryngol. 1997;1838- 46Google ScholarCrossref
Yamawaki  YNishimura  YSuzuki  YSawada  MYamawaki  S Rapid magnetic resonance imaging for assessment of velopharyngeal muscle movement with phonation.  Am J Otolaryngol. 1997;18210- 213Google ScholarCrossref
Smith  JKMcCabe  BF Teflon injection in the nasopharynx to improve velopharyngeal closure.  Ann Otol Rhinol Laryngol. 1977;86559- 568Google Scholar
Gosain  AKConley  SFMarks  SLarson  DL Submucous cleft palate: diagnostic methods and outcome of surgical treatment.  Plast Reconstr Surg. 1977;71497- 1509Google Scholar